A CONTRIBUTION 
TO 
THE STUDY OF 
FRIEDREICH'S ATAXIA. 
WITH EXHIBITION OF CASES. 
BY 
J. P. CROZER GRIFFITH, M.D., 
INSTRUCTOR IN CLINICAL MEDICINE IN THE UNIVERSITY OF PENNSYLVANIA, AND 
ASSISTANT PHYSICIAN TO THE HOSPITAL; PHYSICIAN TO ST. AGNES' HOSPITAL; 
PATHOLOGIST TO THE PRESBYTERIAN HOSPITAL. 
REPRINT FROM TRANSACTIONS. A CONTRIBUTION 
TO' 
THE STUDY OF 
FRIEDREICH'S ATAXIA. 
WITH EXHIBITION OF CASES. 
BY 
4 
J. P. CROZER GRIFFITH, M.D., 
INSTRUCTOR IN CLINICAL MEDICINE IN THE UNIVERSITY OF PENNSYLVANIA, AND 
ASSISTANT PHYSICIAN TO THE HOSPITAL; PHYSICIAN TO ST. AGNES' HOSPITAL J 
PATHOLOGIST TO THE PRESBYTERIAN HOSPITAL. 
REPRINTED FROM THE 
TRANSACTIONS OF COLLEGE OF PHYSICIANS OF PHILADELPHIA, 
FEBRUARY 1, 1888. 
PHILADELPHIA: 
WM. J. DORNAN, PRINTER. 
1889. A CONTRIBUTION 
TO 
THE STUDY OF FRIEDREICH'S ATAXIA. 
WITH EXHIBITION OF CASES. 
By 
J. P. CROZER GRIFFITH, M.D., 
ASSISTANT PHYSICIAN TO THE HOSPITAL OF THE UNIVERSITY OF PENN- 
SYLVANIA: PATHOLOGIST TO THE PRESBYTERIAN HOSPITAL. 
[Read February 1, 1888.] 
No better commencement of this subject can perhaps 
be made than by repeating the oft-quoted definition for- 
mulated by Friedreich himself, as follows: " There exists 
a chronic inflammatory degeneration of the spinal cord 
leading to atrophy, and which seems especially liable 
to develop at puberty under the influence of an heredi- 
tary predisposition. It is chiefly confined to the pos- 
terior columns, and, beginning in the lumbar part of the 
cord and spreading in both an upward and downward 
direction, it finds its limits in the medulla, after involving 
the centres and trunks of the hypoglossal nerves. The 
affection is characterized clinically by a disturbance of 
the coordination and harmony of the bodily movements, 
which develops very gradually, advances from the lower 
to the upper portion of the body, and at last invariably 
involves the organs of speech; though there is no dis- 
turbance of sensibility, and the special senses and cere- 2 
GRIFFITH, 
bral functions remain intact. There are neither paralyses 
of the sphincters nor trophic affections. Less constantly 
there appear curvature of the vertebral column, vertigo, 
and nystagmus. From a clinical point of view the dis- 
ease may be regarded as a chronic progressive paralysis 
of the power of combination of movements; and from 
the standpoint of pathological anatomy, as a chronic de- 
generation of the posterior columns of the spinal cord." 
This definition still holds good in part, though in the 
light of the cases which have been reported since it was 
given, it must undergo certain modifications. The fol- 
lowing, based upon it, will perhaps better accord with 
our present knowledge of the affection: Friedreich's 
ataxia is a chronic systemic inflammatory degeneration 
of the spinal cord, developing usually in infancy or 
childhood, and occurring in cases in which there has 
probably been an arrest of development of the cord dur- 
ing foetal life, this being the result of some hereditary 
predisposition. It is situated chiefly in the posterior 
columns, the lateral pyramidal tracts, the direct cerebel- 
lar tracts, and the columns of Clarke, though other parts 
of the white and gray matter are very commonly some- 
what affected, and the sclerosis extends slightly into the 
medulla. The affection is characterized clinically by a 
disturbance of the coordination of the bodily move- 
ments, developing gradually, advancing from below 
upward, and finally involving the organs of speech. 
Curvature of the spine, talipes, vertigo, and nystagmus 
are frequent. The patellar reflex is nearly always abol- 
ished. Paralysis and sensory disturbances are not un- 
common in advanced cases. Trophic, vaso-motor, and 
visceral affections are unusual, and any involvement of 
the intellect is probably accidental. Friedreich's ataxia. 
3 
In the course of some studies on this disease I found 
so many omissions in and discrepancies between the tab- 
ulated lists of cases made by various authors, and so 
many new cases in the literature, that I have investigated 
for myself all the reports of instances of the disease, so 
far as I have been able to learn of any. To render the 
results as accurate as possible, the original publications 
have been consulted in every instance but three; these 
being the article of Erlenmeyer, of which, however, a 
literal translation was found; that of Jakubowitsch, an 
abstract of which was seen in one of the German jour- 
nals, and that of Palma, whose case is nevertheless de- 
tailed in full by Vizioli. Personal communication with 
Drs. J. J. Putnam, G. B. Shattuck, W. B. Gowers, W. 
Everett Smith, W. C. Warren, and Morton Prince has 
aided me greatly, and I take this opportunity to express 
my thanks to these gentlemen for their kind and valu- 
able assistance. 
I desire to offer to the College a new tabular arrange- 
ment of cases, with their most important symptoms. 
Table I. contains 143 cases,1 comprising all those from 
1 Through an oversight, the case of C. L. Dana, and that of V. P. G-ibney 
(New York Med. Record, Oct. 1, 1887, p. 465) were omitted. The important 
features of these cases are as follows: 
Case 144.-Dana's; male; aged nineteen. Father probably alcoholic; no 
other case in family. Ataxia of gait and speech, and constant thirst, appeared 
at seventeen years. Examination showed marked ataxia of gait; Romberg's 
symptom; some ataxia of arms; slight oscillation of head; slight paralysis; 
slight curvature of spine; slight talipes equinus ; knee-jerk normal; no atrophy; 
hands cold and red; no pain ; slight anaesthesia; slight diminution of muscle 
sense; legs sometimes numb ; speech slow, thick, and scanning ; almost imper- 
ceptible nystagmus ; apparently beginning optic atrophy ; sometimes vertigo ; 
polyuria. 
Case 145.-Gibney's; male; aged seventeen to twenty years. Disease known 
to have existed at six to nine years ; ataxia of gait very marked ; speech ataxic ; 
no pain; no cause of disease known. 4 
GRIFFITH, 
the literature appearing to have any just claim to be con- 
sidered probable instances of Friedreich's ataxia, and 
including those exhibited to-night by Dr. Sinkler, Dr. 
Osler, and myself. Objections might with propriety be 
urged against some of the number, but it is not the pur- 
pose of this paper to assume the right of discrimination 
beyond that which is absolutely necessary, but simply to 
collect and analyze the cases as an aid to future study. 
To Table II. (Additional List) are relegated: A. Reputed 
cases, containing all those reported and related by blood 
to those in Table J., but which have not themselves come 
under professional observation. B. Possible or probable 
cases, merely referred to and without published descrip- 
tions, so far as 1 have been able to ascertain. C. "Too 
doubtful cases," as they might be named, claimed by the 
observer or by critics to be instances of the disease, but 
which differ so widely from its complex of symptoms 
that it seemed almost certain that the general consensus 
of medical opinion would reject them. 
I beg leave this evening to exhibit to the College three 
cases of Friedreich's ataxia, all of them inmates of the 
Home for Incurables and Cancer Hospital, of this city, 
where I have repeatedly examined them during my terms 
of service. For their presence here to-night I am in- 
debted to the courtesy of my colleague. Dr. Horace G. 
Hill. 
Case I.-Sadie T., aged twenty-three; single. Father and 
mother living and well; maternal grandmother died of apo- 
plexy; no history of any nervous disease in family. There 
were 7 children, of whom 1 died of measles, 1 of some febrile 
affection; 1 brother and 2 sisters are well, and 1 brother 
(George) suffers from this same disease. Friedreich's ataxia. 
5 
The history obtained from the mother and the patient 
herself is as follows : 
She was well and active until about ten years of age, at which 
time the mother noticed that the child " was nervous." Her 
hands shook while eating, and her gait was somewhat unsteady 
and staggering, though she did not often fall. The mother, 
who is an intelligent woman, is positive in the statement that 
the disease did not resemble chorea, of which she had seen 
instances. On account of the nervous affection she was en- 
tered, a few months later, at the Children's Hospital of this 
city, and soon after passed through a severe attack of ty- 
phoid fever, followed very soon by measles. After recovery 
the nervous affection was found to be much worse, and the 
patient was unable to walk without crutches; and, even after 
strength returned to other parts, the legs remained weak. The 
affection of the upper extremities had not meantime grown 
worse, and was but slight. The patient complained at this time 
of a sense of burning in the feet and aching in the legs, but 
there is no history of true lightning pains. Sensibility in the legs 
to touch and to pain appears to have been undisturbed, for the 
mother used frequently to test it herself with the point of a pin, 
and could perceive no alteration. The patient walked with 
crutches until the age of sixteen, was able to go upstairs on 
the hands and knees, and to rise from the floor by grasping the 
furniture; yet the disease had been constantly progressing in 
the lower extremities. 
In November, 1880, at the age of sixteen, she was examined 
at the Infirmary for Nervous Diseases, of Philadelphia, and the 
following note entered in the case-book: " She can feel per- 
fectly. No tendon reflex. Adduction of thighs very weak, but 
the patient can make all movements of feet and legs, but is 
utterly unable to stand. Patient stout and of a good color. 
When on back, on attempting to lift legs, they fall outward. 
There is some choreic movement of the hands." She entered 
the Home for Incurables December 29,1880, being still able to 6 
GRIFFITH, 
walk with crutches, though with difficulty. The incoordination 
of the hands now grew worse. She rapidly lost the power to 
walk at all, and her speech became affected. It was impos- 
sible for her to sit upright, and a brace was made for her. 
Her mother says that her intellect has remained unaffected. 
Present condition, August 16, 1887 : The patient is of average 
size, and of a bright and intelligent expression. She sits dur- 
ing the day in a wheel-chair, which she is able to propel. She 
cannot sit erect, but slouches down in the chair, and holds her 
head far forward. There is some scoliosis to the right in the 
dorsal region. She is unable to walk or stand, or, indeed, 
barely to move the legs at all; nor can she raise herself from 
a recumbent to a sitting position while on the bed. Both feet 
are in a position of slight equino-valgus, but there are no other 
contractures. The muscles, both of the thighs and legs, are 
firm, and there is no more atrophy than would naturally follow 
lack of use. The patellar reflex is abolished. The grip of the 
hands is good; that of the left being somewhat better than 
that of the right hand. In grasping an object the fingers and 
hands are extended over it like a claw. The fingers are held 
rather flexed, and the patient is unable to extend those of the 
right hand very well, though there is no resistance to passive 
motion. There is no motion or tremor of the hands when in 
repose, but on voluntary effort a slow ataxic movement of the 
fingers develops, somewhat resembling athetosis. She picks up 
a pin with the greatest difficulty, and with marked incoordina- 
tion, using the whole finger and not the tip, much as an infant 
would. She cannot cut her own food, but can feed herself. 
She can scarcely touch the tip of the nose with the finger, but 
this difficulty is not definitely increased when the eyes are shut. 
The muscles of the arms are perfectly well developed, and 
there is apparently no diminution in strength. The handwrit- 
ing is almost illegible. During the effort to write, or under 
other excitement, the head develops irregular lateral oscillatory 
movements: and a slight jerking motion is almost constantly 
present when speaking, though there is no true tremor. The 
tongue is extended steadily. The speech is peculiar, somewhat Friedreich's ataxia. 
7 
jerking, but not scanning; several words being spoken rapidly, 
then a brief pause ensuing, followed by another series of words, 
the pause sometimes occurring between syllables. The pupil- 
lary reflexes are normal. There is very slight and inconstant 
nystagmus on extreme lateral movement. The result of the 
ophthalmoscopic examination of the eye-ground, kindly made 
for me by Dr. Charles S. Turnbull, was entirely negative. The 
functions of the bladder and rectum are normal, though the 
urine is alkaline, albuminous, and contains numerous leuco- 
cytes. There is no affection of the thoracic and abdominal vis- 
cera, and menstruation is regular. There are no marked dis- 
turbances of sensation. The head and point of a pin are 
differentiated perfectly, both on the upper and lower extremi- 
ties. The points of the aesthesiometer are recognized at six 
centimetres on the extensor surface of the legs, at four centi- 
metres on the forearm, and at about one-half centimetre on the 
tip of the third finger. The sensibility to pain and to temper- 
ature is unaffected. There are no pains in the legs, but a girdle 
sensation is sometimes perceived. Owing to the slowness in 
speaking, it is uncertain whether any retardation of the con- 
duction of sensation exists, though it seems probable.- No 
electrical examination was made. 
The history of the next case is most interesting, and, 
I believe, in certain respects, unique. It would seem as 
though some other nervous affection, perhaps neuritis oi 
anterior poliomyelitis, had preceded the onset of Fried- 
reich's ataxia by about eight years, and that the atrophy 
of the left leg is probably to be attributed to it. 
Case II.-George T., aged twenty-one, brother of last 
patient. The mother relates that he was well as a child, but 
had a fall from a high step when two years old and hurt his 
left hip, though he soon recovered, and no traces of injury re- 
mained as far as could be detected. Soon after this he got his 
feet wet while wading, and on the same evening developed a 8 
GRIFFITH, 
high fever, which disappeared the next day, but left him with 
feet and legs very much swollen as far as the hips, and entirely 
unable to stand. He was taken to see Dr. G. W. Norris, who 
pronounced it paralysis; and again, a few days later, to Dr. S. 
D. Gross, who gave the same opinion, but was not sure whether 
it was due to the fall or to taking cold. After one to two 
weeks the swelling began to disappear, and the child com- 
menced to creep, and a few weeks later to walk. The right 
limb now appeared to be perfectly normal, but the left was 
weaker and decidedly wasted, and a brace was fitted to it. Slow 
improvement followed, and after two or three years the brace 
was replaced by a stiffened shoe. At the age of eight years he 
" threw " the left lower limb when he walked, and both leg and 
thigh exhibited considerable wasting; but the mother says that 
sensation to a pin-prick seemed entirely unaffected. She had 
further never noticed any affection of the hands, nor any other 
disturbance of the gait except that described. At nine years 
of age (in 1875) he was severely sick with measles, followed by 
pneumonia. On recovery it was found that he had lost the 
power of his lower limbs, and had difficulty in sitting erect. 
Curvature of the spine soon developed, but the upper extremi- 
ties remained uninvolved; and after four months he was able 
to walk with the aid of some one or with crutches. He then 
learned to walk from one article of furniture to another rapidly, 
much as an infant would; but slow locomotion was accom- 
panied by much swaying and frequent falls, from which he was 
able to rise again by putting his hands on his knees, or by 
grasping objects in the room. Improvement was very slow, if 
indeed there was any; and in November, 1884, at the age of 
fourteen, he was admitted to the Infirmary for Nervous Dis- 
eases, where the Achilles tendon of the left foot and some 
other tendon of the right foot were cut. After three months' 
treatment he was fitted with a brace for the left leg, reaching 
up to the waist, and with a short one for the right leg; walking 
tolerably well with the aid of these and of a crutch. He says 
the doctors at the Infirmary used to remark that it was strange 
he could not use his right leg better, as it was so well devel- Friedreich's ataxia. 
9 
oped. He was admitted to the Home for Incurables in 1884, 
at the age of eighteen, and could still walk with crutches. 
Since admission his hands have become unsteady, and he is no 
longer able to sit erect without support, or to walk. The date 
at which the affection of speech began is uncertain. He has 
never had lightning pains or girdle sensation, though he some- 
times experiences painless spasm of the extensors of the right 
leg at night. 
Present condition, August, 1887: The patient, of average size, 
has a good color and general nutrition. He sits through the 
day in a wheel-chair, in the same unnatural position as his 
sister, though to a less degree. The lower extremities are 
rather cold and red. The muscles of the right leg and thigh 
are quite firm, and there is no marked wasting. He can flex 
the thigh on the trunk with some difficulty, and partially ex- 
tend the leg, but the muscular power is decidedly diminished. 
The left lower extremity is very greatly wasted, the patient 
being barely able to move it at all. There is no knee-jerk on 
either side. The spinal column shows a marked scoliosis to 
the right in the dorsal region, and the patient cannot raise him- 
self upright in his chair without the aid of his hands. The 
muscles of the arms are unusually large and well developed, 
and the grip is powerful. When in repose the hands lie quietly 
in the lap, but voluntary movements are executed with marked 
incoordination, which is not notably increased on closing the 
eyes. The patient buttons his clothes with difficulty, and is 
unable to bring the forefinger tips together or to touch the ear 
or nose with accuracy. He can feed and dress himself, and 
write, but the handwriting is very unsteady. There is occa- 
sionally a slight ataxic movement of the head, though the 
tongue is extended without tremor; the speech is a little jerk- 
ing. There is slight ataxic nystagmus on lateral motion; the 
pupillary reflexes are normal. An ophthalmoscopic examination 
by Dr. Turnbull revealed no alteration of the eye-ground. The 
thoracic and abdominal organs appear to be normal. There is 
no pain; no girdle sensation; no affection of the bladder or 
rectum. There is retardation of the conduction of sensation 10 
GRIFFITH, 
in the right leg, with slight diminution of tactile sensibility on 
the outer surface, but not over the anterior portion of the tibia. 
No positive sensory changes can be detected in the left leg. In 
the hands and arms there is evidently a diminution in the 
power to distinguish the point from the head of the pin, and the 
aesthesiometer points are recognized on the dorsum of the right 
hand at four centimetres and on that of the left hand at six cen- 
timetres. The muscle sense is decidedly involved in the lower 
extremities, as the patient cannot clearly define the position ot 
his feet when his eyes are shut. 
Case III.-Annie C., aged twenty-six years. The parents are 
living and well, and no history of any nervous disease in the 
ancestors can be elicited. Of 10 children, 2 died in infancy, 
6 are in good health, and 1, a son, die'd of " weakness" at the 
age of nine years, having developed, after scarlet fever, the same 
unsteady gait as Annie at one time exhibited. She was well 
until five years of age, when she had scarlet fever, though not 
in a severe form, and without complications. She seemed, 
however, after recovery, to be very weak in the legs; had a 
swaying, somewhat staggering gait, with frequent falls, and 
could not run upstairs. When eight years old she wore, for a 
year, braces on both legs, extending to the hips. She went to 
school till twelve, but walking then grew so much worse that 
she was obliged to lean on her sister for support, and soon to 
leave school altogether, and she began the use of crutches. 
Weakness in the legs progressed, and at sixteen she could no 
longer walk at all. At twenty she was an inmate of the Jef- 
ferson College Hospital, where she received electrical treat- 
ment, but without benefit. When about twenty-two years old 
she first began to notice weakness in the arms, but she does not 
remember when the difficulty in sitting erect or in speaking 
began. She says that at times she cannot talk as fast as she 
wishes, and that she sometimes chokes when swallowing liquids; 
and I have myself once seen her in a severe attack of choking 
from swallowing saliva. She does not suffer from headache or Friedreich's ataxia. 
11 
other pain, but complains often of cold feet. The left leg is 
sometimes drawn up spasmodically at night. 
Present condition, August, 1887 : The patient is of average 
size, for the most part well nourished, and of a slightly apa- 
thetic expression. Her position in sitting is like that of Case 
I. There is some scoliosis in the dorsal region. The feet are 
shorter than natural, with some talipes equino-valgus, and with 
some contraction of the flexors of all except the great toes. 
There is but very slight atrophy of the muscles of the lower ex- 
tremities. She is not able to extend the legs at all, and can flex 
the thighs but very little. The knee-jerk is abolished on both 
sides. The hands are rather claw-shaped, with the proximal 
phalanges partially extended on the dorsum of the hand, and the 
remaining ones flexed; but the patient can straighten them at 
will, and there is no resistance to passive motion. The ataxia 
of the upper extremities is very great, and she is scarcely able 
to pick up a pin, or with the eyes closed to bring the forefingers 
together, or to touch the ear or nose. With the eyes opened 
this incoordination is very little, if any, less, but when in re- 
pose the hands remain quiet. The arms are somewhat wasted; 
the grip fairly good, but rather infantile, small objects being 
grasped with the radial side of the index finger and the thumb. 
The head exhibits slight irregular swaying and nodding move- 
ments, intensified when talking or under excitement. The 
tongue is extended with a slight trembling motion, and speech 
is slow and scanning. There is slight lateral nystagmus on 
extreme lateral movement of the eye; the pupillary reflexes are 
normal; and Dr. Turnbull reports ischaemia of the retina and 
venous pulsation in both eyes, but no other alteration of the 
eye-grounds. The intellect is entirely unaffected; the func- 
tions of the bladder and rectum are normal, and there is no 
disturbance of menstruation. Physical examination of the 
abdominal and thoracic organs reveals no change in them. 
There are no lightning or other pains, and no girdle sensation. 
The tactile sensibility of the legs is evidently diminished, al- 
though the head and point of a pin are differentiated quite 
well. She is unable to feel the two points of the aesthesiometer 12 
GRIFFITH, 
at a distance of ten centimetres on the forearm, and on the 
dorsum of the hand their recognition is very uncertain, but she 
can nearly always distinguish the point and the head of a pin. 
The slowness of speech renders it difficult to determine whether 
the conduction of sensation is retarded. 
The cases of Dr. W. C. Warren, of Waterford, Mis- 
sissippi, referred to by Dr. Hammond in the Journal of 
Nervous and Mental Diseases for 1882, are so briefly 
reported there that I have written the observer for further 
particulars concerning them, which he has kindly sent 
me, with permission to publish the history of the cases 
up to date, including the account of a fourth patient in 
the same family. 
Family history: The paternal grandmother is still living, but 
has been paraplegic for over thirty years. The father is healthy, 
but of a very excitable disposition. With these exceptions 
there is no nervous disease in the family, and no known history 
of tuberculosis, alcoholism, or syphilis. There are 9 children, 
4 of them the patients to be described. The disease affected 
the 1st, 4th, 5th, and 8th children; the others being healthy 
and well developed. 
Case I.-Nannie AV.; aged twenty-nine; was healthy up to 
the age of eight years, when the parents noticed that she stag- 
gered when first rising in the morning, and could only stand 
steadily after balancing for some time with extended arms. 
After a little exercise the unsteadiness would disappear, but as 
time elapsed it grew constant, the gait being oscillating from 
side to side, and the feet striking the floor with considerable 
force. At about the age of twelve curvature of the spine was 
noticed and the arms became involved. At thirteen she had a 
severe attack of bilious remittent fever, to which affection she 
was subject, and has never been able to walk since. The mo- 
tions of the arms also grew very incoordinate and of a chorei- 
form nature, and the patient was unable to button her clothes FRIEDREICtt'S ATAXIA. 
13 
or to touch the tip of her nose with her index finger with the 
eyes shut. Talipes was noted at an early period. There had 
been only slight pain, between the foot and knee. At twenty- 
one the affection of speech began, and has advanced to an ex- 
treme degree. Up to the age of twenty-three the patient was 
able to extend her hand in salutation, though with difficulty, 
while the arm oscillated involuntarily up and down and from 
side to side several times before the hand of a friend could be 
grasped. For the last two years she has been entirely helpless. 
Menstruation began at twelve to thirteen, and has continued 
regular. The patient complains of vertigo, and suffers from 
some incontinence of urine, while at times there are involun- 
tary painless spasms of the right leg, occurring chiefly at night. 
Present condition: She lies curled up in bed with the knees 
nearly touching the chin. There is marked curvature of the spine 
(lordosis). Intelligence at first appears deficient, but careful 
examination shows the diminution to be slight, as far as it is 
possible to judge in the presence of the great affection of 
speech. For a few months she has been almost constantly cry- 
ing without any known cause. She is able to read ordinary 
print for hours, but sometimes vision fails, and the type seems 
blurred, though no ophthalmoscopic examination has ever been 
made. There is no nystagmus. Speech is nearly unintelligi- 
ble, even to the parents, and there is slight trembling of the 
tongue. The arms are almost powerless, and are held flexed, 
as are the hands and fingers; and there is marked atrophy of 
the muscles, especially of the extensors. The legs are also very 
helpless; the muscles wasted, particularly the extensors; talipes 
equinus is present; the knee-jerk absent. There is no girdle 
sensation, and at present no pain. Cutaneous sensibility is 
about normal. The electrical reactions were not tested, but 
three years ago the faradic contractility was exaggerated. The 
surface of the body is cold; the pulse persistently rapid and 
small. 
Case II.-Thomas W.; aged seventeen; fourth child; healthy 
till eight years of age, when the disease began in exactly the 14 
GRIFFITH, 
same way as in the case of the sister,-i. e., with unsteadiness 
when arising from bed. The gait became more and more 
oscillating and uncertain, and the feet struck the floor with 
force. Finally, at fifteen, walking without assistance was no 
longer possible. At twelve the arms became involved, and at 
fifteen very great incoordination with choreiform movements 
had developed. The speech has quite recently become affected. 
There has been at times slight pain between the foot and knee, 
as in the sister's case. More recently there has been pain in 
the region of the dorsal spine. 
Present condition: The intellect is unaffected; there is no 
vertigo; vision is good; no nystagmus; speech slightly slow 
and hoarse; no trembling of the tongue; head held steadily; 
no ataxic movements of the trunk; decided scoliosis in the 
dorsal region. The hands and arms exhibit marked incoordi- 
nation, and the patient cannot button his clothes, and touches 
the tip of the nose with the index finger with difficulty. These 
and all motions become much more ataxic when the eyes are 
closed. Station is swaying, and the patient has a tendency 
to pitch forward. On closing the eyes the oscillation increases 
very greatly, and he falls unless holding to some support. 
The muscles in general are somewhat atrophied, and there is 
some loss of power. Knee-jerk is lost; talipes equinus of both 
feet is present; there is a slight tendency to spasmodic contrac- 
tions of the legs at night. Cutaneous sensibility is normal; 
there is no girdle sensation, and no affection of the bladder or 
rectum. The surface of the body is cold, and the circulation 
feeble. 
Case III,-Robert W.; aged fifteen; fifth child. The disease 
in this case also began when the patient was eight years of age, 
and with the same staggering when first rising in the morning. 
The oscillation in walking progressed; the arms became in- 
volved at twelve years of age; the speech is still unaffected. 
Some pain in the legs below the knees, and a sense of weak- 
ness in the back is complained of. 
Present condition: Intelligence good; there is decided vertigo;. Friedreich's ataxia. 
15 
the vision is good; no nystagmus; no ophthalmoscopic exami- 
nation has been made. The speech is unaffected, and there is 
no trembling of the tongue. The head does not oscillate; the 
movement of the arms is very ataxic and of a somewhat cho- 
reiform nature, and buttoning of the clothes is impossible ; 
while incoordination is increased on closing the eyes. There 
are no ataxic movements of the trunk. Scoliosis in the dorsal 
region is present. The gait is oscillating, staggering, only pos- 
sible when the patient supports himself by the furniture or uses 
a cane. The station is swaying, and becomes impossible when 
the eyes are shut. Talipes equinus is present. There is no 
patellar reflex. Decided atrophy of the muscles is observed in 
the upper and lower extremities, and there is a certain amount 
of paralysis. The cutaneous sensibility is unaffected. There is 
no girdle sensation, and no affection of the bladder or rectum. 
The surface of the body is cold and the circulation poor. 
Case IV.-Nettie W.; aged eight; eighth child; is just be- 
ginning to show symptoms of the disease. She was always 
healthy, but since the age of six years it has been noticed that 
she staggers when rising in the morning, and only after some 
effort, and by repeated placing of the feet on the floor and bal- 
ancing with the arms outstretched, can she succeed in preserv- 
ing her equilibrium. After a little exercise the ataxia of station 
disappears. There is no affection of speech; no nystagmus; no 
curvature of the spine; no paralysis. The knee-jerk is dimin- 
ished in both legs. 
To study the characteristics of Friedreich's ataxia 
more in detail, the subject may conveniently be divided 
under the following headings : 
History. 
The first cases, 6 in number, were reported by Fried- 
reich in 1861 before the Thirty-second Congress of Ger- 16 
GRIFFITH, 
man Naturalists and Physicians, held at Spiel, but they 
were not published in full until 1863. In 18651 Carre 
described a case in his monograph on Tabes dorsalis. 
From 1872 to 1876, inclusive, 6 cases were reported in 
England by Bradbury, Carpenter, and Dreschfeld, and 2 
in America by Kellogg; and in 1876 Friedreich pub- 
lished his last set, thus making 20 instances in the lit- 
erature, including the 2 of Quincke quoted by Friedreich, 
but which, for the sake of convenience, are tabulated 
later, since Riitimeyer has described them more fully. 
By the end of 1880 the number had increased to 31; 
with the close of 1882 to 47; with the beginning of 
1885 to 71; and now reaches 143. There have appeared 
several valuable critical reviews, with or without reports 
of cases, as, for example, those of Fere, Mobius, Cuche, 
Ormerod, Bury, Sepilli, Vizioli, and others. I would 
here especially acknowledge my indebtedness to those 
of Ormerod, Vizioli, and Bury, of which 1 have made 
free use. 
N AME. 
Friedreich first described the disorder as " Degenera- 
tive atrophy of the posterior spinal column," but this 
title quite fails properly to distinguish it. The name 
" Friedreich's disease," favored by Brousse and proba- 
bly by most writers, though at one time perhaps the 
best, has recently become very objectionable, since para- 
myoclonus multiplex is frequently spoken of under the 
same title. " Congenital ataxia," as employed by Mas- 
tin, may lead to misapprehension, since, though it is 
1 This is the date given in the reference in Friedreich's paper, and also that 
of the copy of Carre's work which I consulted, and which there is no reason to 
suppose was other than a first edition, though the usual date assigned by 
authors is 186*2. Friedreich's ataxia. 
17 
probable that the affection is congenital, it has not been 
proved so. " Hereditary ataxia " as it was called by 
Friedreich, is in few cases a proper appellation, for 
reasons which will be explained later. " Family ataxia,'' 
suggested by Fere, is a less objectionable name than 
the preceding, since all the 143 cases have occurred 
in but 77 families of brothers and sisters. 2 sets of 
Musso's cases were related as cousins, as were 4 sets 
of Riitimeyer's; while 2 of Vizioli's patients and 4 of 
Smith's were children of ataxies. The number is thus re- 
duced by 6, making 71 entirely unrelated families. Yet 
it is to be noted that the disease does not always occur 
in several brothers and sisters, since there have been 39 
instances in which but 1 ataxic child was seen by a phy- 
sician in the family, and of 24 of these it is stated that 
the patient was the only child affected by the malady. 
It becomes evident, therefore, that this title is inappli- 
cable, though in 4 of the 24 cases (99, 107, m, 116) 
the patients were the oldest children, and others may 
consequently have developed the symptoms of the dis- 
ease later; while in Cases 56 and 122 the patient was 
the only child, and in 30, 117, 118, and 127 the exist- 
ence of other children is not mentioned. Smith proposes 
the name " Postero-lateral spinal sclerosis (generic 
origin)," or short, "Generic ataxia." The full title 
describes the pathological changes, but does not distin- 
guish it from Ataxia paraplegia of Gowers-the Com- 
bined postero-lateral sclerosis which is so ably discussed 
by Westphal; while the term " generic " is open to the 
same objection which applies to " hereditary " and 
" family " ; viz., that it is not always applicable, and 
describes as characteristic and even indispensable that 
which is by no means such. A still better title is, I 18 
GRIFFITH, 
think, "Friedreich's Ataxia," which designates the most 
prominent characteristic, gives the disease a specific 
name, and does not impose any limitations to be violated. 
Though not preferring the use of a writer's name for 
this purpose, I cannot but think that in the present state 
of our knowledge this is the best title that can be 
chosen. 
Etiology. 
Heredity.-Friedreich's ataxia is essentially one of 
the hereditary diseases, but the adjective must be un- 
derstood here in its broadest sense. 
A. Direct and similar inheritance-i. e., inheritance of 
the disease itself or of some form of ataxia-has occurred 
in only 33 cases in 16 families of brothers and sisters; 
some of which, however, are very doubtful examples of 
it. Some of the cases of Ormerod (66, 70), those 
of Musso, and the Blattner family of Rutimeyer had 
respectively the grandfather, great-uncle, and great- 
great-great-grandfather reputedly ataxic. Descended 
from the last mentioned, who went by the name of 
" Stulzi " (the stum bier), were 8 instances of Friedreich's 
ataxia (2 in Table II. A) in 4 families. The grandfather 
of 2 of Bury's patients (112, 113) had probably an 
ataxic gait. In Bradbury's, Brousse's, and Carre's cases 
the mothers are spoken of as ataxic, and in the last there 
were in addition 6 brothers and sisters of the patient, 8 
of the mother, the grandmother, and a cousin, who were 
said to ha ve the same disease. The father of Fowler's 
cases was reported to have a peculiar gait; 2 uncles of 
Mastin's patients were almost certainly subjects of 
Friedreich's ataxia; and the father as well as the brother 
were without doubt affected by the same symptoms as Friedreich's ataxia. 
19 
Botkin's case exhibited. But in no instance were the 
reported ataxic parents actually under medical observa- 
tion except in the case of Smith, and in 2 of those of 
Vizioli; and here a direct inheritance of Friedreich's 
ataxia appears to have been witnessed. The father (100) 
of Smith's patients (ioi, 104) became ataxic after the 
age of sixty-six, while the 2 cases of Vizioli referred 
to are especially interesting. Vincenzo Vitielli (81), 
namely, was the father of Antonio and Luigi (89, 90), 
and the table also contains a cousin (91) and 7 uncles 
and aunts of these boys who were subjects of Fried- 
reich's ataxia. A sister of 91 is to be found in Table 
II. A. It is interesting to note that there are sometimes 
instances of crossed heredity-e. g., among Musso's pa- 
tients there were 3 females inheriting the disease through 
the male line; and 3 males (2 in Table I. A) through 
the female line; while in Mastin's cases, again, 3 males 
inherited it through the female line. 
B. Polymorphic inheritance-as seen in the develop- 
ment of a nervous disease in the children of parents 
affected by some other neurosis-is of frequent occur- 
rence, and even the simple constitutional degeneration 
of the antecedents from various causes may account for 
the production of Friedreich's ataxia or other disorders 
in the descendants. Under this broad definition of 
heredity in nervous affections as emphasized by Mobius 
there exist as possible additional ancestral predisposing 
causes of Friedreich's ataxia: 1. Other Neuropathies, 
or exceedingly nervous disposition in the parents, grand- 
parents, uncles, or aunts. 2. Alcoholism. 3. Tuber- 
culosis. 4. Syphilis. 5. Consanguinity. 6. Other 
unclassified causes. 
1. Neuropathies of various kinds, including great 20 
GRIFFITH, 
nervousness, have been reported present in the parents or 
the relatives mentioned in 58 cases. In the remainder 
there were either none discovered or no reference is made 
to the matter. In 25 of the 58 cases one or both of the 
parents (marked + in the table) alone suffered from 
nervous symptoms; in 23 (marked 4-), grandparents, 
uncles or aunts were also nervously affected; and in 10 
(marked -) only the latter (?'. e., excluding parents) 
were neuropathic. 
2. Intemperance of the ancestors1 is a feature to which 
considerable importance was attached by Friedreich, 
who found it present in 6 of his'9 cases. In the Siiss 
family, indeed, the mother maintained that her children 
were conceived while the father was intoxicated. I find 
intemperance of the father-and in Mastin's cases, of 
the mother also-in 31 instances (marked + in the table) 
occurring in 10 families ; while in 13 other cases (marked 
-) the parents were sober, but the grandparents or 
other parental relatives had been intemperate. In some 
of the remaining 98 cases it is stated that there was no 
intemperance in the family, but in most of them the 
subject is not referred to, and it is fair to presume that 
the habit was absent in a large number. In only 7 in- 
stances (i, 2, 20, 21, 74, 75, 134) could it be said to be 
the only hereditary predisposing cause detected, and 
intemperance is unfortunately so common that it may 
have been here a pure Coincidence. Though it seems to 
be not without some influence, I think but compara- 
tively little importance can be attached to it. 
3. Tuberculosis of the parents only was noted in 9 
cases (marked +). In Palma's case the mother was 
1 The term " ancestors" in this paper denotes parents, grandparents, uncles, 
and aunts. Friedreich's ataxia. 
21 
consumptive and sprang from a very consumptive family, 
and in one of Bury's (i 14) both father and uncle died 
of the disease. In 10 instances (marked -), including 
the 3 cases of Freyer where tuberculosis had probably 
existed, grandparents, uncles, or aunts had died of the 
affection. In 21 cases, therefore, all told, tuberculosis 
was present in the ancestors, though in but 11 was it 
the only cause known. It is probable that its presence 
in the forefathers, by producing debility of the offspring 
and a liability to degeneration of all sorts, may predis- 
pose to the development of Friedreich's ataxia. 
4. Syphilis is said to have been present in the grand- 
father of Vincenzo Luongo (91), and some of Freyer's 
patients had exhibited an eruption which was possibly 
syphilitic. Except in these instances there was no evi- 
dence of a deterioration of the nervous system through 
inherited syphilis. 
5. Consanguinity of the parents (grandparents in Mas- 
tin's cases) is reported as existing in but 7 cases in 4 
families, viz., those of Seeligmuller, Erlenmeyer, Mastin, 
and Mendel. In the last 3 it would appear to have been 
the principal predisposing influence, but in the first 
family there also existed a very neurotic history. 
6. We read of no other possible ancestral causes, ex- 
cept that the mother of Freyer's cases is said to have 
been feeble, and that some of the uncles and aunts of 
Musso's patients suffered from general debility and other 
signs of pool* constitution. 
As regards conditions discovered in the other children of 
the same family which rendered probable the existence 
of a common inherited taint or tendency to degenerative 
processes, we find that in 20 families of brothers and sis- 
ters there are said to have been 31 individuals presenting 22 
GRIFFITH, 
the symptoms of Friedreich's ataxia, but who did not 
come under professional observation; this does not in- 
clude the relations of the cases reported by Fellows, 
where the number is not stated precisely. Other neu- 
roses, including exceedingly nervous temperament, are 
reported present to a greater or less degree in 14 families 
-32 cases. In 13 families-27 cases-there existed 
other suspicious conditions in the brothers and sisters 
indicating an inherited feebleness of constitution. Thus 
in 5 families (13-15, 19. 56-58, 96-97- there 
had been numerous early deaths, and in Musso's 2 
families 7 stillbirths were observed. In 2 of Ormerod's 
families (69-75) one or more children had feeble re- 
flexes, and in Freyer's there had possibly been syphilis 
in several children beside the patients themselves. 
The brother of Smith's oldest case (100) lacked all 
moral sense, and the younger brother of Botkin's patient 
is described as having moral insanity. The sister of 2 
of Seguin's cases (96, 97) died of phthisis. In Palma's 
report a curious occurrence is described. The phthisical 
mother suckled but 2 of her children, of whom one was 
the patient, Giuseppi; the other being a sister who died 
of phthisis. The others were nursed by another woman 
and remained healthy, and Palma believes that the milk 
from the phthisical mother was a factor in the develop- 
ment of the ataxic disease. 
It must be noted in this connection that there are cer- 
tain cases where no trace of any inherited or of inheritable 
taint whatever has been discovered, or at least reported 
either in the ancestors or other descendants. An in- 
spection of the table will show this to be true in 11 
instances (24, 44, 55, 76, 99, 107, 109, 116, 1 17, 122, 
140), though the patient of Erlicki and Ry balkin was friedkeich's ataxia. 
23 
a foundling, and nothing was known of her relatives; 
while the family history of McAlister's case is not pub- 
lished. 
As regards heredity, then, statistics show that the 
existence of ataxia or of some other nervous disorder 
in the ancestors exerts a strongly predisposing influence 
on the development of Friedreich's ataxia in the descen- 
dants ; that intemperance has a much less powerful and 
even questionable effect, and that the agency of other 
conditions is insignificant, except as they debilitate the 
parents' constitution. 
Age.-The influence of age in determining the onset 
of Friedreich's ataxia has been variously estimated. 
Friedreich made the claim, based upon his own cases, 
that the disease developed at about the time of puberty; 
but this is clearly too narrow a limit. Numerous in- 
stances have been reported in which it appeared much 
earlier than this, and a few are recorded in which it was 
not detected until after the ag*e of 20. Looking* throng'll 
the table it will be seen that the first symptoms were 
observed as follows: 
In 15 cases ..... by 2 years of age. 
and, including these, 
In 39 cases ..... before 6 " " 
Further, 
In 45 cases . . . . .at from 6 to 10 " " 
" 20 " " 11 " 15 " " 
" 18 " ' " 16 " 20 " " 
" 5 " " 20" 25 " 
The a<je of 14 of the 1G cases is not accu- 
rately enough stated to allow of placing it in any of the 
periods given above; but in all it was less than 16 years, 
and in all but 2 (4, 112) less than 12 years, while in 24 
GRIFFITH, 
4 (76, 81, 82, 95) it was probably less than 5 years. 
The 2 remaining cases are those of Seeligmiiller (23) and 
Smith (100). In the former, difficulty in walking was 
not observed by the patient until after the age of 27, 
though it is uncertain just when ataxia actually devel- 
oped ; and in the latter no symptoms were perceived 
until the age of 66. (See remarks on cases in Table I.) 
ddie statistics show, then, that in over one-half the re- 
corded cases the first symptoms were perceived before 
the age of 11 years, and that in only a small number 
did they develop after 16. 
Sex.-The statements as to the sex chiefly affected 
vary somewhat, depending simply on the number of 
cases of the disease which the various tabulators had 
collected. Of the 143 cases reported here 86 were males 
(60.14 per cent.), and 57 females (39.86 per cent.) 
Acute Diseases.-It is a most interesting fact that 
the occurrence of certain acute, and usually infectious, 
diseases has sometimes precipitated the appearance of the 
ataxia. Most probably the necessary conditions for its 
development were already present and were only incited to 
activity. This precipitation is described in 20 instances 
as follows: Typhoid fever (16, 52); pertussis (47); 
variola (64) ; meningitis (56) ; acute spinal affection of 
a rather uncertain nature (116) ; periostitis (119) ; mor- 
billi (142) ; scarlatina (49, 90, 105, 143) ; scarlatina 
and morbilli (114) ; rheumatism (100, 132); probably 
diphtheria (69, 70, 71) ; diseases not clearly defined 
(117, 137). As a rule, the febrile disease precedes the 
development of the ataxic symptoms either immediately 
or with only a short interval. In 3 of Musso's cases, 
however (64 and 2 in Table II. A.), 2 years had elapsed 
since the patients had suffered from variola, which the Friedreich's ataxia. 
25 
author nevertheless considers the exciting cause; while 
in Erlenmcyer's, 1 of Dreschfeld's (16), and 1 of R ti- 
meyer's (47) cases the acute disease had occurred respec- 
tively 5 months, 2 years, and 1 year previously. A 
degree of doubt cannot but attach to the existence of 
any causal relation in some of these cases. Bury 
believes that chorea may precipitate Friedreich's ataxia, 
but it seems more probable that the choreiform move- 
ments witnessed in one of Riitimeyer's cases (in Table 
II. A.) and in certain other instances were but anoma- 
lous early symptoms of the ataxic disease. 
Clinical History. 
The disease usually begins early in life, and nearly 
always attacks the lower extremities first; the initial 
symptoms being unsteadiness and weakness, with an os- 
cillating, staggering gait accompanied by frequent falls. 
Exceptionally other symptoms, perhaps accidental, ap- 
pear before the affection of the legs. Eclampsia occurred 
repeatedly in a patient of Vizioli's (91) ; and in one of 
Riitimeyer's (53) it was seen at 6 weeks of age and left 
the patient feeble, so that he never learned to walk as 
well as the other children. Friedreich describes the early 
occurrence of hysterical attacks in one case, but considers 
them independent of the ataxia. In Botkin's case the 
symptoms were ushered in by a fit of uncontrollable 
laughing coming on in church,-possibly of an hysteri- 
cal nature. Pain in the head or other parts has been 
noted in a few cases before, or with the earliest ataxic 
symptoms, as have been vomiting, vertigo, curvature of 
the spine, dorsal flexion of the toes, palpitation of the heart, 26 
GRIFFITH, 
choreiform movements, etc. They will be discussed in the 
proper place. 
All these early symptoms, however, are of very un- 
usual occurrence, if indeed they are other than purely 
accidental; and disregarding them, it appears that the 
lower extremities were first attacked in 114 of the 143 
cases. In 10 instances the involvement of the legs and 
arms was noted almost simultaneously, though in one of 
these (99), Sinkler's, the mother seemed to think that 
the awkwardness was first in the arms. In 8 instances 
the speech and the four extremities were affected to- 
gether ; in Descroizilles' case, and very probably in one 
of Vizioli's (91) also, the legs and the speech were 
simultaneously involved, no reference being made to the 
time when the arms became ataxic; and finally in 2 
instances (106, 134) the upper extremities were first 
diseased. The part first attacked in the remaining 8 
cases is not definitely stated. 
The speed with which the disease spreads varies very 
greatly. The average lapse of time in 35 cases between 
the first affection of the upper and lower extremities, where 
the date was accurately enough stated for calculation, 
and not including the cases of simultaneous involvement, 
was about 6 years; but the variation is so great that 
this average is of little value, and a better conception 
can be obtained by consulting the table itself. 
In some of the patients the disease bad not reached 
the upper extremities when reported, and in others it is 
not clearly stated whether or not they were affected. 
The longest interval mentioned, before the involvement 
of the arms was noticed, equals 20 years, and occurred 
in one of Dreschfcld's cases (16). Next in order comes 
one of my own (143) with an interval of 17 years. Friedreich's ataxia. 
27 
These are, however, unusual, and the ataxia perhaps 
most frequently reaches the arms after the lapse of 1 to 
3-5 years. 
In Case 6 one leg was first attacked and then the arm 
of the same side, next the remaining leg and finally the 
other arm; or one leg may be involved and then that of 
the opposite side, as in Cases 2, 3, 35, 126. In certain 
instances one side of the body always remained more 
diseased than the other; as in 1, 6, 107, 122, and in 
Teissier's case (59) the upper limb was more inco- 
ordinate than the lower. 
Ataxic movements may develop even in the head and 
trunk; either with those in the upper extremities or later. 
Bulbar symptoms, usually represented by nystagmus and 
affection of speech, are very constantly present. It is 
generally stated that they appear late in the disease, com- 
ing considerably after the affection of the arms; yet in 
31 cases in which the date of the development of both 
classes is definitely given, the appearance of the bulbar 
symptoms (usually the affection of speech) averaged only 
about 1J years later than that of the incoordination of 
the arms; and the range of variation was not at all 
great. In 18 of these cases both developed at the same 
time, and in 6 the interval was but 1 year or less, while 
the longest intermission was but 9 years, viz., in one of 
Warren's cases (39). Yet these statements do not 
strictly express the truth; for doubtless bulbar symp- 
toms would have developed later in many cases in 
which none were reported, and the average lapse of time 
would probably be increased. 
As the disease advances, more or less paralysis with 
some muscular atrophy often makes its appearance, con- 
tractures of the muscles may develop, and talipes and 28 
GRIFFITH, 
curvature of the spine commonly occur. Pain some- 
times is present and exceptionally is severe. The patient 
becomes unable to walk, and loses more and more con- 
trol over the bodily movements, until he is rendered 
almost completely helpless, while speech may be nearly 
or quite unintelligible. Finally death ensues from pro- 
gressing asthenia; or oftener from some intercurrent 
disease. 
Infectious or other febrile diseases have power to ac- 
celerate as well as to precipitate the ataxic disease. This 
aggravation has been either general, or limited to certain 
regions of the body, and is reported 14 times in 13 
patients; one of Sinkler's cases (98) having once been 
made worse by scarlatina, and again by malaria. The 
acute diseases which have accelerated the progress of 
the nervous disorder are: rheumatism (13, 48); scar- 
latina (69, 89, 98, 133); malaria (98); typhoid fever 
(76, 83, 85); typhoid fever followed by measles (141) ; 
probably diphtheria (74) ; bilious fever (40) ; a teta- 
noid attack (128); some affection not clearly defined 
(102). The increase of the ataxia after recovery from 
the acute disease was so great in 5 cases (76, 83, 85, 
98, 141) that the patients were found to have lost the 
power of walking entirely or except when assisted. Not 
included in the 13 cases is the second patient of Fried- 
reich, in whom parturition at the age of 23 so augmented 
the severity of the symptoms that she was never again 
able to walk without assistance or unless holding to ob- 
jects. A patient of Musso's (61) is also to be mentioned, 
in whom epileptiform seizures appeared to precipitate 
the involvement of speech. Friedreich's ataxia. 
29 
Symptomatology. 
Motor Symptoms. 
Motor Ataxia.-Motor ataxia of the lower extremi- 
ties, shown either in the gait or in intended movements, 
or in both, is reported present, or as having been present 
before loss of power supervened, in 128 cases. In the 
remaining 15 ataxia of the legs is not directly mentioned, 
though it is certainly implied in some of them. Freyer 
distinctly states that the gait was good in one of 
his cases (121), but the disease was only beginning. 
Hammond's effort in his article was to prove that the 
cases reported by Friedreich were not at all like loco- 
motor ataxia. While, therefore, he will not attribute the 
motor affection in his own 6 patients to ataxia, he never- 
theless admits that the feet " were moved exactly like 
those of a drunken man when he attempts to walkand 
in one case (36) distinctly speaks of lack of coordina- 
tion, while in another (35) he implies that closing the 
eyes made an ataxia of station worse. 
34 of the 128 cases exhibited increase of incoordina- 
tion on closing the eyes, or in some instances had done 
this before the power of motion in the legs was lost; in 
19 instances there was no such increase; in 1 (6) 
closure of the eyes had at one time produced it, but 
later did not, though the legs could still be moved. In 
the remaining cases no note is to be found in the original 
report. 
The gait may be that characteristic of tabes dorsalis- 
i. e., with the extreme anterior propulsion and elevation of 
the foot, and heavy fall of the heel; but I have found 
this stated in very few cases, as in 18, 25, 60, 80. 
Certain others-e. g., 17, 66, 98-are described as of 30 
GRIFFITH, 
" typically ataxic " gait; which may very probably indi- 
cate a gait like that seen in tabes. On the other hand, 
in over 45 instances 1 have noted it designated as " like 
that of a drunken man," or " oscillating," or as " not 
typically ataxic," etc. Musso described it as exhibit- 
ing a weakness, tremor, and a tendency to a lateral 
projection of the feet; and even as early as 1871 Brad- 
bury said his case was not like tabes on account of the 
way in which the legs were thrown out at random. One 
of Musso's patients (6i) said of herself that when walk- 
ing she " occupied the whole road." In many other 
cases I have made no exact note of the character of 
the gait, or it is not mentioned by the authors; but 
there would seem to be no question that the oscillating, 
drunken, cerebellar gait is by far more characteristic of 
Friedreich's ataxia than is that usually seen in tabes. 
The eyes arc sometimes kept fixed on the floor while 
walking, as in both patients of Massalongo's, and in that 
of Carre's; sometimes the gaze is directed straight out- 
ward, as in Mendel's and some of Vizioli's cases. 
.Ataxic station is expressly mentioned or implied in 73 
cases, and undoubtedly existed in many more. In 7 of 
the 73 it was certainly or probably absent when the eyes 
were open, but developed when they were closed. Of 
the 66 remaining it was increased by closing the eyes in 
42 instances-i. e., Romberg's symptom existed-and in 
10 there was no such increase. In 13 it is not distinctly 
stated whether Romberg's symptom existed or not, and 
in 1 (6) it had been witnessed earlier in the case but 
afterward disappeared. 
on motion of the upper extremities is usually 
present and well marked. The movements are irregular 
and often jerky. Buttoning the clothes or threading a Friedreich's ataxia. 
31 
needle becomes difficult or impossible; and even less 
delicate actions, such as touching the tip of the nose 
with the finger or carrying food to the mouth, are equally 
badly performed. In the attempt to make any volun- 
tary motion the action is often overdone; thus in trying 
to grasp an object the hand may be advanced past it, as 
in 97. Prehension is often very peculiar, the fingers 
being spread over an object like a claw; as was well 
illustrated by one of my own cases (141). Bury also 
speaks of the claw-shaped grasp in Case 115, and 
Seguin describes the over-extension and spread of the 
fingers before grasping in 93 and 95. I have already 
referred to the peculiar infantile grasp in 2 of my own 
cases (141, 143). I find incoordinate movements of 
the hands or arms reported in 111 cases; increased by 
closing the eyes in 21 of these; not increased in 26 ; 
while in the remaining 61 the question of increase is not 
referred to by the authors. Many of the 32 cases in 
which ataxia of the upper extremities is not mentioned 
in the table undoubtedly possessed it, but the reporter 
has failed to state the fact clearly. In only 11 instances 
(17, 18, 23, 24, 28, 43, 58, 63, 75, 100, 108) is it dis- 
tinctly said that there was none. Irregularities in dis- 
tribution are sometimes witnessed; thus one of Orme- 
rod's cases (71) exhibited ataxic movements in only 
one of the hands, viz., the left. 
Muscle-sense.-Though coming somewhat out of 
its natural order, muscle-sense can best be considered 
here. Whether Romberg's symptom is due to a disturb- 
ance of muscle-sense, or to some entirely different cause, 
forms a most interesting question, but one too extended 
to be fully discussed in this paper. Friedreich claimed 
that his cases proved both locomotor and static ataxia 32 
GRIFFITH, 
to be independent of any disturbance of sensibility. The 
case of Galassi's rendered that writer sceptical as to any 
relation between Romberg's phenomenon and muscle- 
sense. Musso, too, found Romberg's symptom in 4 of 
his cases, while in none of them was the muscle-sense 
otherwise affected, though carefully tested in various 
ways. 
The muscle-sense is described in the table as normal 
in 32 instances. In some of these the authors merely 
state that it was unaffected and probably include the 
absence of Romberg's symptom, while in others the 
manner in which it was tested is detailed. It happens, 
now, that 15 of these 32 cases exhibited increased inco- 
ordination of the motion of the legs or of station on clos- 
ing the eyes, yet the muscle-sense, strictly named, was 
undisturbed, as shown by the testing with weights, grasp- 
ing with eyes closed after an object whose position was 
known, and in the knowledge of the position of the 
members in space. This would seem to confirm the view 
that Romberg's phenomenon depends on something more 
or other than the loss of this sense. An additional proof 
is found in the fact that in 11 cases the muscle-sense 
was more or less diminished, in 4 of which there was no 
Romberg's symptom, and in 3 others no mention is 
made of it, if it existed. 
Static Ataxia.-By this title Friedreich designates 
the ataxia of quiet action-i. e., of the muscular force 
required to keep any part of the body still while unsup- 
ported, as when holding the arm extended. In contra- 
distinction to this, the term " locomotor ataxia," or, 
better, " motor ataxia," would indicate the incoordina- 
tion which appears only when a voluntary movement is 
attempted. The ataxia of station is, of course, a variety Friedreich's ataxia. 
33 
of static ataxia, and has already been discussed, but 
nothing has yet been said of its other forms. Friedreich 
states that static ataxia is almost the last to appear, and 
Charcot and Ormerod express the same opinion, while 
the former also claims that it is characteristic of the 
disease and does not appear in tabes. Though Pitt says 
that the history of the reported cases indicates that it is 
an earlier symptom than the locomotor ataxia, my own 
search through the literature would confirm quite de- 
cidedly the views of the writers first mentioned. I find 
it described in the extremities in 2, 6, 1(3, 15, 22, 46, 49, 
68, 113, 114, 115, 117, 124, 127, 137, 138, 139, 141, and 
many others, usually of the severe forms. The exact 
number I have failed to record, though it is, I think, 
oftener absent or not described than present. 
In the hands while lying passive in the lap, it may be 
seen as peculiar slow movements of the fingers, which 
sometimes resemble athetosis, as in Charcot's second case 
(127), one of Sinkler's (139), and one of my own patients 
(141). In the arms it appears as slow wavy oscillations 
when the member is held outstretched, or even as 
marked choreiform movements, as Erlenmeyer describes 
in his patient, which he, however, classifies as evidences 
of static ataxia, as does Charcot the constant non- 
rhythmic oscillations of the legs, seen in his first case 
(67). Botkin speaks of a similar condition in his own 
patient, and in one of Seeligmuller's cases also (22) there 
were observed the same constant trembling movements 
when the legs were in certain positions. Numerous 
other instances of static ataxia of the limbs are reported, 
but it is seen to best advantage in the oscillating move- 
ments of the head, which are described in 51 instances; 
and undoubtedly most of the cases exhibiting " tremor " 34 
GRIFFITH, 
or " choreiform movements " of the head should be added 
to these. The oscillations may be slight or extensive, 
constant or seen only under excitement. Friedreich 
described in Case 6 an irregular nodding motion of the 
head, "like one going to sleep,'' and this has since been 
often witnessed, as in Cases 45, 127, and others. 
Ataxia of the trunk is reported in 22 instances. In 
others its occurrence is implied, though with but one 
exception no reference is made to it. 
Tremor.-Tremor and choreic movements may nat- 
urally be next described, since they are so closely allied 
to static ataxia. Tremor of some part of the body is 
referred to in 8 cases. In most of these it should pro- 
bably be attributed to static ataxia-e. 7., 2 of Smith's 
cases (102, 103) had tremor of the head on voluntary 
motion, and one of Teissier's (59) exhibited little oscil- 
lations of the whole body when at rest. There is noth- 
ing found which at all recalls the intention-tremor of 
multiple sclerosis, except in Glynn's case, where there 
existed oscillations of the head and trunk, a forward 
and backward oscillation of the tongue, with a shaking 
of the arms which became violent when reaching for a 
cup. In Cases 22 and 133 the muscles of the face ex- 
hibited slight tremor. 
Choreiform Movements.-Choreiform movements 
are referred to in 17 instances, and are, like tremor, pro- 
bably in most cases evidences of static ataxia; being, 
in fact, recognized as such by some of the authors re- 
porting them. They are usually seen in the extremities, 
but in 6 cases grimaces and twitchings of the face are 
alluded to (23, 56, 69, 71, 97, 127). Chorea has ap- 
peared as one of the earliest or even the initial symp- 
tom in certain cases, as, for example, in 2 cases of FKIEDREICll's ATAXIA. 
35 
Ormerod (69, 71) and in that of Gower's and Pitt's 
(27). Pitt states that 3 other cases in the family began 
with choreiform movements, which were, however, not re- 
ferred to when Gowers reported them ; and I do not think 
that statistics corroborate Pitt's view "that the first 
symptoms noticed are disorderly choreiform movements." 
Erlenmeyer's case is especially interesting in this con- 
nection, since Erb pronounced it to be an instance of 
chorea, while Friedreich deemed it some spinal affection, 
though not ready to admit it into the same class with 
his own cases. Erlenmeyer, however, insisted that it 
was an example of Friedreich's ataxia, and it has since 
been unquestionably accepted by Rutimeyer. There 
seems no reason to consider it other than a genuine 
case. 
Spasm.-Involuntary spasmodic contraction of the 
muscles has been reported in 21 instances. These are 
sometimes painful, perhaps oftener not, and occur chiefly 
in the lower limbs when the patient is lying in bed or 
sitting. 4 of Friedreich's cases exhibited this symptom, 
apparently with cramp-like pains. It was seen also in 
a case each of Hammond's (33), Riitimeyer's (47), 
and Smith's (102) ; the legs of the last patient being at 
times drawn up to such an extent that she begged to 
have weights put on them to pull them down. In 2 of 
my own cases (142, 143) these spasms occurred at night, 
and the patients were unable unassisted to straighten 
the limbs immediately. 
Fagge's patient suffered from a spasmodic action of 
the muscles of the face when an effort was made to talk, 
drawing the mouth into a meaningless smile; and in 
Botkin's patient there occurred spasm of the muscles 36 
GRIFFITH, 
of the neck, hands, and feet, with constant grinding of 
the teeth, the case being unique in this last particular. 
Paralysis.-More or less paralysis is a very com- 
mon feature in advanced cases, and it may, indeed, 
appear early in the disease; but as Ormerod justly re- 
marks, the initial weakness in the legs, and even the 
loss of the power of walking called by the friends 
" paralysis " are probably due simply to the incoordina- 
tion in most instances. Many of the cases which have 
been subjected to careful medical examination in the 
early stages support this view. (See " Inability to 
Walk.") Muscular paralysis is reported present in 56 
cases, absent in 53, and is not directly mentioned in 
34, though in a number of these it almost certainly 
existed. Many of the cases in which paralysis is 
reported absent were not far advanced in the dis- 
ease, and the percentage of cases affected would cer- 
tainly be increased could the clinical histories be fol- 
lowed further. It may become extreme in advanced 
cases, as in one of Vizioli's (87), who had been affected 
in early infancy and who died at the age of 46, having 
reached a state of complete immobility apparently of all 
the muscles, excepting those of mastication and respi- 
ration. In Therese R. (123), diseased 28 years, the legs 
were totally paralyzed, and the arms and trunk largely 
involved; and Emma W., one of Smith's cases, was in a 
similarly helpless condition. The rapidity of the devel- 
opment of paralysis does not, however, always depend on 
the duration of the disease; since in Glynn's case, for 
example, which had lasted but 4 years, the muscular power 
of the limbs was decidedly diminished, and in Kahler's 
and Pick's patient, diseased 8 years, it was nearly gone; 
while in one of Freidreich's cases (6), on the other hand, Friedreich's ataxia. 
37 
the patient died of typhoid fever with normal muscular 
power after suffering from ataxia for 24 years, and in 
one of Smith's (103) there was no paralysis after 18 
years of the disease. 
Inability to Walk.-In a number of cases stand- 
ing or walking had become impossible as the disease 
advanced. This was due in some of them to the paral- 
ysis, but in others it clearly depended on the existence 
of excessive incoordination, since the actual strength 
did not seem to be at all diminished. Friedreich calls 
especial attention to this condition in Case 6. A review 
of the table shows that in 54 cases the patients were no 
longer able to walk, except with the aid of crutches or 
when assisted by some person. In 4 instances the au- 
thors do not make any statement concerning it, and in 
86 the power of walking was still preserved, though 
often only when the patient steadied himself by the fur- 
niture or walls. The relation between the inability to 
walk and the paralysis may be seen by comparing these 
two columns in the table. In 10 of the 54 cases paral- 
ysis is stated to be absent; in 9 it is not mentioned, 
and in the remaining 35 there was more or less of it, 
though it is very certain that in many of them the term 
was wrongly applied, especially where the table shows 
that no atrophy of the muscles was to be found. In 
some of them paralysis is expressly stated to be very 
slight or even questionable, and was clearly not suffi- 
cient to account for the loss of the power of locomotion. 
The age at which this loss occurred is noted in the table 
whenever given in the original article, but the lapse of 
time from the onset of the disease to this event is so 
variable that to average it would be useless. In Cases 
37, 38, 39, 87, and 88, the patients never walked; while 38 
GRIFFITH, 
in i, 32, 34, 44, 55, 139, and 141, walking unassisted was 
impossible within a year after the disease began, and 
in the last case within a month after the first onset. In 
Case 5 the patient was able to walk through 27 years 
of the ataxia, up to the time of death from an intercur- 
rent disease, and the table shows other cases in which 
the ability persisted for years. 
Contractures.-Contractures of various parts are 
referred to in a number of instances, and are usually 
among the later symptoms; and may be divided into 
Talipes, Curvature of the spine, and Other contractures. 
Talipes is reported in 27 cases, in 14 of which it was 
of the variety equino-varus. The remaining 13 are 
named as follows: equinus (40, 41, 42, 58, 114, 115, 
122); equino-valgus (141, 143); valgus (112); varus 
(134); cavus (126, 136). The last case, though not 
named directly " cavus " by the author, is best summed 
up by this word. Talipes is a late symptom, and, indeed, 
has only been seen in 7 cases which still preserved the 
power of walking. It is associated often (12 cases), 
though not always (9 cases), with some degree of mus- 
cular atrophy. In 2 of Smith's patients (101 and 102) 
the talipes was immovable; in one of Bury's (114) 
there was very little motion at the ankle-joint, and in 
one of Sinkler's (139) the tendo Achillis was very tense. 
Curvature of the spine, considered by Rutimeyer a 
form of contracture, appears to be largely independent 
of the degree of paralysis. It is usually one of the later 
symptoms, though in Cases 4 and 10 there was curva- 
ture of the spine-perhaps accidental-2 years before 
other symptoms appeared, and in Cases 9, 71, and per- 
haps others it was among the early symptoms. It is 
reported present in 57 cases; being sometimes slight, Friedreich's ataxia. 
39 
sometimes very marked; and is described in 28 of these 
as scoliosis, in 20 as cypho-scoliosis, in 8 as cyphosis; 
while in 1 the variety is not designated. 
Contractures of other parts of the body are not infre- 
quent as late symptoms, though in one of Bury's cases 
(i 13) they were present from the first. They are men- 
tioned in 24 cases; all except 100, 101, 102, and 115 
exhibiting dorsal flexion of the toes. There were also 
contractures of some of the fingers or of the hands 
in Cases 49, 50, 51, 101, 113, 115. In one of Smith's 
cases (101) the elbows were flexed; in another (102) 
the muscles of the thighs and calves were much con- 
tracted; in another (100) the calf-muscles were tense, 
and in one of Sinkler's (98) the plantar fascia was con- 
tracted. 
Electrical Contractility.-The electrical con- 
tractility has been tested in only a few instances, and 
appears to reveal nothing characteristic. In 31 cases it 
is reported normal, sometimes the faradic current being 
specified, often the nature of the current not being named. 
In 2 instances (66 and 100) it was normal or increased. 
In 9 cases (2, 17, 19, 81, 89, 91, 103, 124, 136) it was 
slightly diminished; and in 2 (59, 60) the faradic con- 
tractility was increased. In 3 instances (113, 114, 115) 
the reaction of degeneration was detected; the faradic 
contractility being diminished in the first and normal 
in the second. One of Smith's cases (101) exhibited 
decreased faradic contractility but increased response 
to the galvanic current; while in Descroizilles' patient 
the faradic reactions were normal and the galvanic pos- 
sibly diminished. 
Examination of the electrical sensibility has been re- 40 
GRIFFITH, 
ported in a few instances, but has taught nothing of 
importance. 
Reflexes.-One of the most important studies con- 
nected with Friedreich's ataxia is that of the patellar 
reflex. In the 143 cases reported the knee-jerk was 
abolished in 91, while in 30 the condition is not stated. 
The absence would seem, then, to be a very constant 
symptom, as it is a very early one. In 2 of Ormerod's 
cases (72, 73), for example, it was observed before the 
diagnosis could be made. In Leubuscher's case the 
reflex was lost during the first half year; in Jakubo- 
witsch's, which had only been diseased a month or so, it 
was very greatly diminished; and in 15, 21, 28, 56, 
74, and others it disappeared within 1-3 years after 
the first appearance of the symptoms. The patellar 
reflex is nevertheless not always absent, though this 
claim has frequently been made. It is reported dimin- 
ished in Fellows' patient and one of Warren's (43), and 
much diminished in 7 instances. But in 6 cases (63, 65, 
85, 100, 137, 139) it is said to be normal; in Descroi- 
zilles' patient, normal or exaggerated; and in 6 cases 
(22, 23, 66, 68, 107, 138) exaggerated. Though all 
of these have a fair claim to be considered instances 
of Friedreich's ataxia, objection might be raised to some 
of them on the ground that in still other respects they 
were anomalous. (See remarks on Table I.) The patient 
of Prince's, however, though having exaggerated reflexes, 
appears to be an unquestionable instance of it, and Cases 
63 and 85, with normal reflexes, are typical in other 
respects and belong respectively to the families reported 
by Musso and Vizioli, which contain some of the most 
striking cases known. Massalongo's 2 patients are 
beautiful examples of the disease; yet in one the knee- Friedreich's ataxia. 
41 
jerk was normal, and in the other exaggerated; and I 
have personally examined the later patients of Sinkler- 
the MacDonald children-and cannot but agree with 
others who have seen them, that they are undoubted, 
well-advanced cases of Friedreich's ataxia, yet without 
the absence of knee-jerk. 
As Westphal states, the knee-jerk will not be abol- 
ished unless the lumbar enlargement be involved; and 
it is easily conceivable that this involvement might fail 
to occur in undoubted cases of Friedreich's ataxia. 
We cannot, therefore, but conclude that though abo- 
lition of the patellar reflex is a very important diagnostic 
sign of the disease, yet genuine cases may occur in 
which the reflex is slight, normal, or even exaggerated. 
Very exceptionally ankle clonus has been observed. 
I do not recollect reading of it in any cases but those of 
Descroizilles' and Fellows'. 
As regards the cutaneous reflexes the plantar reflex 
was normal in 40 instances, absent in 11, diminished in 
4, and increased in 7. The other cutaneous reflexes 
were normal in 26 cases in which they were tested to 
some extent, diminished, at least in part, in 7, absent in 
1, and increased in 1. 
Trophic and Vaso-motor Symptoms. 
These two sets of disturbances are so nearly allied 
that I have classified them together. 
Atrophy of the Muscles is comparatively unusual 
even where paralysis is well marked. In 60 cases there 
was no muscular atrophy, and undoubtedly it was absent 
in many, if not most, of the instances in which no refer- 
ence is made to it. The lower extremities are the parts 
usually attacked, though the arms are sometimes in- 42 
GRIFFITH, 
volved. In 11 cases atrophy is said to be slight, and in 
24 marked. This does not include my own case (142 ), 
in which the great atrophy of the left leg and thigh was 
evidently independent of the ataxic disease. Some of 
the patients exhibited extreme atrophy ; thus Case 50 
had both legs and arms much wasted, as had Cases 19, 
40, and 64. 
Other Trophic Changes-which are tabulated in 
the same column with vaso-motor affections-are very 
unusual. Bedsores are said never to develop in Fried- 
reich's ataxia. One, however, was observed in Case 2, 
but was superficial and soon healed. The skin of the 
legs was in parts firmer, thickened, and adherent to the 
subjacent tissues in one of Sinkler's earlier cases (99); 
Vizioli reports an extreme development of adipose in 
the atrophied legs of his patient Vincenzo (81), and 
Smith reports enlargement of the feet of one of his cases 
(101), while in a patient of my own (143) they seemed 
smaller than normal. Two other patients, of Smith's and 
Vizioli's, respectively (102 and 85), and one of Seguin's 
(93), suffered from chilblains, which easily suppurated in 
the last case. These are so equally a vaso-motor affec- 
tion that other disorders of this class may be expected 
to occur. 
Vaso-motor Affections are represented in 19 cases 
by coldness of the surface of the body or especially of the 
feet, with more or less blueness and sometimes oedema 
(19, 40, 41, 42, 46, 47, 48, 51, 54, 57, 69, 74, 75, 93, 
95, 101, 102, 142, 143). In 2 instances flushings are 
reported; of the head and face (63), and flashes of 
heat (2). [When both vaso-motor and trophic affec- 
tions, not including muscular atrophy, are present, the 
sign in the table is T.j Friedreich's ataxia. 
43 
Sensory Symptoms. 
Pain.-The absence of the severe initial lancinating 
pain of tabes is one of the most characteristic symptoms 
of Friedreich's ataxia; and their occurrence in Dresch- 
feld's and a few other cases contributed to throw doubt 
upon the diagnosis. Pain of some sort, before the other 
symptoms appeared, or early in the course of the disease, 
was present to some degree in only 22 cases, absent in 
79, and probably wanting in most of the remaining. 
Botkin's and one of Musso's cases (61), as well as 2 of 
Friedreich's (13, 14), suffered from headache before other 
symptoms appeared, and Bradbury's case began with 
pain and weakness in the back. The patient of Carre's 
suffered from severe pain in the. legs and feet; and that 
of Jakubowitsch's from pain in the knees, shoulders, and 
head at the very beginning of the ataxic affection, while 
Friedreich's Cases 3, 4, and 5 complained of considerable 
early pain of the lower extremities. With these excep- 
tions pain at the beginning of the disease has been in- 
significant, and in many cases was probably accidental. 
Pain after the disease has become well established is 
comparatively more common. I find it recorded in 47 
cases, in many of which it was certainly unconnected 
with the disease itself. In about half of this number it 
was well marked, and in the others slight; while in 63 
cases it is stated to be absent and in the balance was 
presumably so. Among the cases in which it was severe 
may be noted 2 patients of Musso's (61 and 62), who 
suffered from intense headache at certain periods, accom- 
panied or followed by a temporary increase of the ataxic 
symptoms. Severe lightning pains are rare, and are 
perhaps best illustrated by one of Fowler's patients (77). 44 
GRIFFITH, 
Lissette Suss (5) suffered from gnawing and neuralgic 
pains and painful spasms in the limbs ; and several other 
of Friedreich's cases exhibited similar sensory disturb- 
ance. Some of Seguin's (96, 97) and of Smith's (101, 
102, 103) patients had marked pain in the lower extremi- 
ties, though in 102 these seemed rather to be due to the 
intercurrent and fatal acute myelitis. Maria Vitielli 
(85) suffered from headache and pain in the articulations. 
Tenderness on pressure over the spine has been reported 
in Cases 63, 64, 85, and a few others. 
Cutaneous Sensibility.-The cutaneous sensibility 
is generally not much involved. In 74 instances it was 
normal, in 16 no reference is made to the subject, and in 
47 there was more or less diminution of tactile sensi- 
bility. In 2 cases (7 and 25) it was normal or increased, 
and in 3 cases (40, 60, 136) increased. In Descroizilles' 
patient it was at one time slightly diminished, but later 
normal. 
The diminution of sensibility-usually tactile-has 
generally been slight and often questionable. Some- 
times it could be detected by the aesthesiometer; some- 
times the touch of the finger could not well be felt. 
Often the sensibility to pain was blunted also, and in 
certain cases the conduction of sensation was retarded. 
Among the most marked instances of anaesthesia may 
be noted the second case of Friedreich's, the first of 
Musso's, 1 of Dreschfeld's (16), and 2 of Smith's (102, 
(103) ; the last 2 patients being scarcely able to distin- 
guish pinching and pricking of the skin from simple 
touching. In one of Freyer's cases (119) there was 
total anaesthesia in spots ; but the most striking example 
is Therese R. (123), in whom the lower limbs had become 
totally anaesthetic after 7 years' illness from Friedreich's Friedreich's ataxia. 
45 
ataxia. Evidences of a degree of anaesthesia are further 
seen in Cases 55, 103, and 100, in which there was re- 
spectively a sensation of cushions, of velvet, and of a 
sponge under the feet. 
Muscle-sense has been already discussed in connec- 
tion with Romberg's symptom. 
Paresthesia.-Some form of paraesthesia, including 
girdle sensation, is reported in only 18 cases. In 43 
instances (o in table) there was no mention of any form 
of paraesthesia, and it is stated that either girdle sensa- 
tion, or paraesthesia of other kinds, or both classes were 
absent. It is fair to presume that the majority of the 
remaining cases were free from any such affection. In 8 
instances a girdle sensation is reported, namely, in 22, 
103, 132, 141, 100, 102, 114, 119. In the first 4 it was 
very slight, but in the others well marked; Smith's 
patient (102) having it perhaps most strongly devel- 
oped. Seeligmuller's second patient felt as though the 
clothes everywhere were too tight. Paraesthesias of other 
kinds, such as formication, tingling, numbness, etc., 
were observed in 14 instances (6, 7, 9, 17, 22, 45, 46, 
54, 69, 100, 102, 107, 119, 135). In few were they more 
than very slight, and in none severe. 
Bulbar and Cerebral Symptoms. 
Affection of Speech.-Affection of speech is one 
of the most prominent of the bulbar symptoms, as it is 
one of the most frequent characteristics of the disease, 
and one which would probably develop in all cases not 
cut short by some intercurrent affection. It is generally 
a later appearance, occurring as a rule about the same 
time or somewhat before nystagmus. As already shown 46 
GRIFFITH, 
under " Clinical History," it does not always develop 
so lono- after the affection of the arms as has often been 
supposed, and may be affected from the first. It was 
present in 107 cases, absent in 21, and not referred to in 
the remainder. Speech may be scarcely perceptibly in- 
volved, or so greatly so that it is almost or quite unin- 
telligible, as in one of Warren's (40) and one of Smith's 
(101) cases. It may be drawling and slow, as in one of 
Bury's (115) and in Joffroy's patients, or jerking and 
interrupted, or confluent, as in Jakubowitsch's case, or 
typically scanning. In Kudolf Blattner (49) there were 
frequent changes of pitch; in Hollis's case, speech was 
low and indistinct. Glynn's patient slurred his sylla- 
bles, pronouncing " actually " as though written " ax- 
ially." In Power's case it was sometimes slow, some- 
times rapid and slurring, etc. Friedreich spoke of 
'' ataxia of speech ; " and the term applies especially 
well to the rapid pronunciation of words and syllables 
followed by a pause, and this again by the same jerking 
articulation as I have described it in one of my own 
cases (141). He considers it due to an incoordination 
of the articulating movements of the tongue. The 
peculiar rushing, tumbling speech which Seeligmuller 
observed in one of his cases (23) induced him to char- 
acterize the trouble in that instance as an " ataxia of 
thought; " since the patient seemed to begin a new sen- 
tence before he had finished the old one. 
I have attempted to express by signs in the table the 
degree of the disturbance of speech in the different cases, 
but the character of this disturbance is too varied to be 
well shown. The scanning articulation is quite com- 
mon, though it is not, I think, mentioned in the majority Friedreich's ataxia. 
47 
of cases. As instances of it may be noted Cases 26, 59, 
60, 61, 62, 64, 102, 112, 114, 122, 127, 143. 
Face, Mouth, and Throat.-The tongue is not 
infrequently affected; usually by a fibrillar tremor, some- 
times by an irregular jerking or a to-and-fro movement. 
It is reported involved in 24 cases, and not affected in 
30, including Case 2, where a previously existing tremor 
had disappeared. In Quincke's and Rutimeyer's first 
case (46) the tongue could not be held still in the mouth, 
but this very active movement was afterward substi- 
tuted by a simple tremor. In Erlenmeyer's case it 
would not remain quiet; in one of Teissier's (59) it ex- 
hibited little oscillations, and in Glynn's these were 
larger, while the tongue was seen " curling and uncurling 
in a most curious way." In Ferrier's it was unsteady 
when projected; in the first of Friedreich's it trembled 
and twitched, and in that of Mendel moved backward 
and forward but was without fibrillar tremor. Nearly 
all the other cases exhibited fibrillar tremor only. At 
one time in Case 2 the tongue would become motion- 
less for some moments and the patient unable to speak. 
There was also some paresis in Joffroy's patient. 
Mastication and Deglutition are usually spared, but 
may become involved in advanced cases. Thus deglu- 
tition is described as almost impossible in 2 of Smith's 
cases (101, 103), and a similar difficulty was present to 
some degree in Cases 33, 45, 49, 50, 94, 143. Choking 
attacks of a peculiar nature seemingly not dependent on 
deglutition were observed in a case of Ormerod's (133). 
There is sometimes inability to retain the saliva in 
the mouth ; apparently distinct from or in addition to 
ptyalism. This was the case in one of Seeligmuller's 48 
GRIFFITH, 
patients (22), one of Smith's (ioi),one ofSinkler's (139), 
and others. True salivation will be referred to later. 
The face has often a very expressionless appearance; 
as, for example, in 11, 64, 65, and 136; and this condition, 
when combined with great difficulty in articulation, 
sometimes gives an erroneous impression of an affection 
of the intellect. Slight twitching or tremor occasionally 
is witnessed, as stated elsewhere. In one of Dreschfeld's 
cases (17) there is said to have been slight atrophy of 
some of the muscles, and in Joffroy's some paresis; 
while in Ferrier's the imperfect movement of the upper 
lip did not properly disclose the teeth. 
Eye.-Strabismus is mentioned in a few cases-e. g., 
in 8, 23, 56, 71, 80, 109, 115, 131 ; but it is uncertain in 
how many of these it was accidental. Diplopia is re- 
ferred to in a few instances, as 7; 68, 136. Blepharo- 
spasm I remember to have seen described in but 3 cases, 
those of Botkin, Descroizilles, and Erlenmeyer, in the 
first 2 of which the spastic element elsewhere was 
very well marked. Some degree of ptosis was observed 
in Joffroy's patient and in one of Ormerod's (71). 
But the most important feature connected with the 
organ of vision is nystagmus, which Friedreich regarded 
as a very characteristic symptom, though it is one of the 
last to appear. It was present in 56 cases, absent in 46, 
and not mentioned in the remainder, though it certainly 
would be found to have appeared in many of them 
could the subsequent histories of the cases be followed 
up. It is a much less common symptom than is affec- 
tion of speech, as is exemplified by Friedreich's cases; 
the latter symptom being observed in all of them, and 
the former in but 5. It very frequently develops at Friedreich's ataxia. 
49 
the same time with the affection of speech or a little 
afterward, but it may appear much later; not being 
detected in one of Friedreich's cases (2), for example, 
until 10 years, and in another (6) until 19 years after 
the disturbance of articulation was first noticed. So in 
one of Warren's patients (40), speech had been affected 
for 5 years and had become almost unintelligible, yet 
no nystagmus could be discovered. On the other hand, 
in Erlenmeyer's patient nystagmus appeared at the out- 
set of the disease, lasted 3 weeks and then disappeared 
permanently. A survey of the table shows that there 
are but 4 cases (22, 48, 124, 125) stated to have had 
nystagmus without difficulty of speech, while in 2 others 
(92, 103) the latter is not mentioned. The form is 
nearly always what Friedreich called " ataxic nystag- 
mus ; " namely, a more or less extended, transverse, 
bilateral oscillation of the bulbs, appearing only when 
the eyes are fixed upon an object, especially if held 
at the side. The nearer the object is approached, 
the more marked does the symptom become, but 
when the eyes are at rest it disappears completely. 
It is of less rapid motion than the ordinary or static 
nystagmus, and, indeed, differs widely from it. In Leo 
von K. (22) it appeared to cease only when the eyes 
were directed at certain angles, which varied at differ- 
ent times. Botkin's case is said to have had involun- 
tary turning of the eyeball upward, a motion which 
appears to be allied to nystagmus. " Static nystagmus " 
also-i. e., that seen wThen the eyes were at rest-was 
observed in Cases 46, 51, 54. The pupillary movements 
are in some instances said to be sluggish, as 64, 69, 111, 
and others; or to be dilated, as in 45, 65, 66, or con- 50 
GRIFFITH, 
tracted; but in no case were they found not to react to 
light and on accommodation. 
Atrophy of the optic nerve, so common in tabes, occurs 
in Friedreich's ataxia with the greatest rarity, if at all. 
I have found ophthalmoscopic examinations recorded in 
38 cases, and in none of them was there any change of 
importance noted except in Power's case, in which the 
disks were rather white; and in one of Seguin's ( 96), in 
which there was partial atrophy of both optic nerves. 
The condition of the color fields might prove of interest 
in showing the possible relation of the disease to loco- 
motor ataxia. Very little has been done in this direc- 
tion, though Oliver made a careful examination of one 
of Sinkler's cases (99) and found narrowed fields, leading 
him to believe that there existed ocular changes allied to 
those of tabes. 
Vision is reported impaired in a number of instances in 
which no ophthalmoscopic examination had been made, as 
for example in 7, 11, 17, 35, 40, 51, 96, 97, 123. In Bot- 
kin's case there was total blindness of the left eye, but 
probably not dependent on the principal disease. 
Hearing was deficient in a few cases, as in 50, 51, 
and 123, but this was probably purely accidental. 
Intellect.-One of the features which distinguish 
Friedreich's ataxia so sharply from disseminated sclero- 
sis is that it rarely has any effect on the mental power. 
In 79 instances the intellect is reported normal, while in 
21 it was possibly more or less affected, though the study 
of these cases shows that the deterioration was generally 
only apparent or trifling. Thus it is stated that the in- 
telligence was low, but not out of proportion to the social 
position (19) ; that the patient often forgot words (35) ; 
that the intellect seemed at first to be decidedly affected, Friedreich's ataxia. 
51 
but was in reality very slightly, if at all so (40); that 
the patient said he thought slowly (98), etc. In only a 
few instances does the intellect seem to have been actu- 
ally impaired. In one of Smith's cases (102), for ex- 
ample, the memory had become very deficient, and the 
simplest mental acts were the result of laborious effort; 
while a sister (101) would frequently scream for hours 
for no discoverable reason, and had also had epileptic 
seizures during fifteen years, though it is not certain 
whether they had any connection with the ataxic dis- 
order. A case of Seguin's (94) became practically in- 
sane during the last two months of life; and Power's 
case had but weak intelligence, and often laughed spas- 
modically. In Brousse's case the intelligence was low, 
and one of Hammond's patients (33) appeared to have 
suffered an arrest of mental development to some extent. 
It seems open to question whether Friedreich's ataxia 
had any direct bearing upon the deficient cerebration 
of any of these cases; and even Seeligmuller's patients, 
sometimes excluded on the ground of cerebral disturb- 
ance, exhibited no greater affection of this nature than 
did their father, who was in other respects a healthy 
man. 
Vertigo.-Vertigo is a not infrequent early or late 
symptom, being present in 29 cases and absent or not 
recorded in the remainder. 4 of Friedreich's cases had 
it to some degree, and the patient of Ferrier's was 
early so annoyed by it that he was unable to play with 
his companions, though the symptom later disappeared 
entirely. In Botkin's patient it preceded other symptoms 
by about two years, but had probably nothing to do 
with the ataxic disease; and in one of Friedreich's (3), 
one of Rutimeyer's (46), and one of Bury's (112) it was 52 
GRIFFITH, 
among the early symptoms. Vertigo later in the disease 
was seen, for example, in Power's patient, who suffered 
from attacks of giddiness, with loss of consciousness and 
fainting; and in Catharine L. (105), where it was well 
marked, and brought on by reading or in other ways, as 
also in one of Smith's patients (100), where it was a con- 
stant symptom, greatly increased by standing. Other 
cases have experienced it even when in the recumbent 
position, as in Friedreich's second case, in which it was 
for a time the chief complaint. In certain other instances 
it was severe, though it usually is a symptom of no great 
importance, and is often probably accidental. 
Visceral and Secretory Symptoms. 
These are usually slight and possibly accidental; are 
reported present in 42 cases in all, and are best consid- 
ered in classes, as follows: [In the table marked + if 
more than one class is present; if only one, the sign is 
-■] 
Affection of the Bladder is rarely met with, being 
referred to in 13 cases; in 8 of which (8, 23, 55, 59, 96, 
in, 114, 133) it is described as incontinence which was 
slight or occasional, or as occurring when laughing (55), 
etc. In Power's case, and in one of Warren's (40), in- 
continence seems to have been more marked, though 
never excessive. Sinkler's first patient (98) had some 
difficulty at the beginning of urination ; one of Musso's 
(61) was unable to urinate while lying in bed; and Fried- 
reich's second patient could only pass water in drops, 
and after long straining. 
Affection of the Rectum is very rare, excepting 
that several cases had troublesome constipation-cer- Friedreich's ataxia. 
53 
tainly no unusual feature in many conditions. In one of 
Seguin's patients (97) this is said to have been extreme. 
In the first case of Seeligmuller (22), on the other hand, 
the feces were sometimes expelled before the clothes could 
be removed; and the brother (23) was obliged to hasten 
as soon as the desire to defecate or urinate was felt. 
Descroizilles' patient suffered from constipation alter- 
nating with involuntary passages of urine and feces. 
Disorders of the Sexual Apparatus have been 
claimed by some writers to be quite common, being 
represented in the males by impotence and in the females 
by affections of menstruation. This claim was probably 
based on the fact that 2 of Friedreich's cases were impo- 
tent, while some of the women had disordered menstrual 
functions. Later experience shows that impotence has 
only been reported in 3 cases, all told (1, 6, 17), though 
the perverted sexual feelings of Seeligmuller's patient 
(22) rendered him virtually impotent, and he might be 
properly classified with the others. On the other hand, 
the males of Vizioli's first family, though severe cases 
of Friedreich's ataxia, exhibited normal or unusually 
great sexual power. Disturbances of menstruation were 
considered by Friedreich to be a complication rather than 
a result of th$ spinal affection. They are reported in 10 
instances (2, 4, 19, 30, 54, 61, 62, 69, 116, 117), consisting 
of such disorders as "scanty menstruation, beginning in 
the sixteenth year" (19); "menstruation at twenty, 
regular but scanty" (62); "has not menstruated at 
nineteen " (69), etc. One of Friedreich's cases (2), who 
had at times decided menstrual irregularity, conceived 
and bore a child while suffering from ataxia. Probably 
the most serious disturbance reported is that of Salome 
Siiss (4), who did not menstruate until the age of twenty- 54 
GRIFFITH, 
two, and then very irregularly and with intervals of 
several years. Among any 57 females suffering from 
any chroAic debilitating disease, it is probable that 10 
instances of menstrual disorders would be found equally 
severe with those reported here. 
Other Visceral and Secretory Affections, some 
of which are allied to vaso-motor disturbances, are re- 
corded as follows: Salivation in 5 cases (2, 50, 56, 59,68) ; 
palpitation of the heart in 13 cases (2, 61, 62, 63, 5, 4, 19, 
22, 23, 101, 102, 103, 104), in the last 8 of these being one 
of the earliest symptoms; persistent acceleration of the 
pulse in 8 cases (2, 19, 40, 59, 60, 119, 123, 124) ; profuse 
sweating in 3 cases (2, 50, 63) ; gastric disturbance in 9 
cases (8, 57, 132, 133, 45, 101, 102, 103, 104), being one 
of the initial symptoms in the last 5 ; dyspnoea in 5 cases 
(2, 4, 5, 19, 102). Besides these there are mentioned 
polyuria (2), cough (4), intermittent albuminuria (13), 
nervous crises (61), and praecordial anxiety (62, 63). 
Pathological Anatomy. 
Among the 12 autopsies which have been made I have 
included that of Erlicki and Rybalkin, whose case was 
an excellent example of Friedreich's ataxia, except that 
nystagmus and the affection of speech had not yet de- 
veloped. The authors admit that these symptoms would 
not appear until late, and make their case a text for the 
discussion of Friedreich's ataxia, yet hesitate to call it 
an example of it on account of the absence of these 
symptoms. Since the symptoms in other respects and 
the post-mortem lesions are those of the affection in 
question, there is every reason to include the case here. Friedreich's ataxia. 
55 
The autopsies, arranged in the order in which they 
were reported, are as follows : 
I. Andreas Lotsch (I); Friedreich; reported 1863. 
Died at 35 of typhoid fever. Ataxic 18 years. 
II. Justinia Siiss (3); Friedreich; reported 1863. Died 
at 31 of typhoid fever. Ataxic 16 .years. Specimens 
re-examined 16 years later by Schultze. 
III. Salome Suss (4); Friedreich; reported 1863. 
Died at 28 of typhoid fever. Ataxic 12-13 years. 
IV. Friedreich Suss (6) ; Friedreich; reported 1877. 
Died at 38 of typhoid fever. Ataxic 23 years. Micro- 
scopical examination made by Schultze. 
V. Josefa Strasik (19); Kahler and Pick; reported 
1878. Died at 23 of phthisis. Ataxic 8 years. 
VI. Charlotte Lotsch (2); Friedreich; reported 1880 
by Schultze. Died at 51 of nephritis. Ataxic 34 years. 
VII. Marie R. (30) ; Brousse; reported 1882. Died 
at 32 of cerebral hemorrhage. Ataxic 8 years. 
VIII. Clara W. (102); Smith; reported 1885. Died 
at 29 of acute, myelitis. Ataxic 19-20 years. Micro- 
scopical examination by Putnam. 
IX. J. W. (117); Erlicki and Rybalkin; reported 
1886. Died at 18 of phthisis and typhoid fever. Ataxic 
2 years. 
X. Robert Shaw (27); Gowers; reported 1886-7 by 
Pitt. Died at 28-29 of cardiac dilatation. Ataxic 9-10 
years. 
XI. Heinrich Kern (46); Quincke and Rutimeyer; 
reported 1887 by Rutimeyer. Died at 20 of fatty heart. 
Ataxic 13-15 years. 
XII. Bertha Kern (48); Rutimeyer; reported 1887. 
Died at 16; cause of death not clear. Ataxic 9-10 
years. 56 
GRIFFITH, 
The following analysis will perhaps best indicate the 
lesions found: 
Brain.-The brain (not including the medulla) was 
certainly examined in 10 cases and nothing of importance 
discovered in 9 of them (I., II., III., IV., V., IX., X., 
XI., XII.). An ex-amination was probably made in VI., 
but it is not positively stated; in VII. there were the 
lesions of cerebral hemorrhage, and the pons was small; 
and in VIII. the skull was not opened. 
Cord and Medulla.-As regards the microscopical 
appearance-i. e., the size and shape of the cord and me- 
dulla-the latter is stated to have been very atrophic in 
IV.; and the cord was "small" in IV., V., VI., X., and 
XI.; while in 10 instances, viz., I., II., III., IV., VI., 
VIII., X., XI., XII., the flatness of the posterior col- 
umns is particularly specified. In 11 instances, there- 
fore, all told, the size of all or part of the spinal cord was 
below normal, no allusion being made to this p »int in 
VII. Meningitis was observed in 10 cases, viz., I., II., 
III., IV., V., VI., VIII., IX., XL, XII.; limited in 6 of 
them (I., II., III., IV., VIII., IX.) to the posterior portion 
of the cord. In XI. and XII. the pia was diffusely thick- 
ened in places ; in V. and VI. the position of the inflam- 
mation is not clearly stated, and in the former could 
scarcely be called a meningitis; III. and IV. exhibited 
thickening of the ependyma of the fourth ventricle, while 
in VII. and X. there was no meningitis at all. Con- 
siderable fluid was found in the sac of the dura in a 
number of cases, and in Friedreich's first 3 autopsies the 
posterior fissure was obliterated. 
Medulla.-The medulla has been examined micro- 
scopically in 8 instances; though whether lesions were 
found is not always clearly stated. In I., II., III., and Friedreich's ataxia. 
57 
IV. there is said to have been slight extension of the 
posterior sclerosis to the medulla, with traces of change 
in the fourth ventricle. In IV. there was also sclerosis 
of the right anterior pyramidal tract extending a little 
above the decussation of the pyramids, and with slight 
increase of connecting tissue with numerous corpora 
amylacea in the restiform bodies. The funiculi graciles 
and funiculi cuneati are described as sclerosed in IX., X., 
and XII., but in the last up to the level of the upper 
decussation only, while in the first it is further stated 
that there was no change above the decussation. In IX. 
the funiculi rotundi were also involved to some extent. 
Case VII. exhibited a patch of sclerosis reaching to the 
level of the hypoglossal nucleus ; in VI. there was noth- 
ing found to note, and in V., VIII., and XI. the medulla 
was not examined. 
Cord.-A microscopical examination has been made 
in all cases, though the improved means of investigation 
now at our command might show more extended changes 
in I. and III. than were reported. 
The Posterior Columns were sclerosed from the lumbar 
to the cervical enlargement in all the cases, and even 
higher and into the medulla in those just described; and 
the sectional area was usually sclerosed very nearly 
throughout. The postero-median columns were greatly 
degenerated in all the cases, but the postero-external 
columns less intensely and uniformly affected. In I., II., 
and III. the sectional area of degeneration was greatest 
in the lumbar region, though everywhere complete; but 
in IV. the degeneration was most marked in the cervical 
and dorsal regions. The whole sectional area of V. was 
very uniformly sclerosed, except a healthy layer next to 
the gray matter. Case VI. is said to have been just like 58 
GRIFFITH, 
IV. Iii VII. only the portion was affected adjacent to 
the central canal and to the cornua, and the sclerosis was 
most pronounced in, and reached the periphery only in 
the lumbar region, though almost doing so in the cer- 
vical cord. In VIII. the posterior columns were diseased 
nearly throughout, though more completely in the dorsal 
than in the lumbar region, and a narrow portion adjoin- 
ing the posterior commissure was not involved. IX. 
exhibited a degeneration in section which was most in- 
tense above, but very general everywhere, except a small 
tip where the posterior commissure joined the posterior 
horns, and even this was diseased in the upper part of the 
cord. In X., also, the sclerosis was general, except a 
small portion contiguous to the neck of the horn in the 
lumbar and cervical regions. Finally, in XI. and XII. 
the degeneration of Burdach's columns increased some- 
what in a downward direction as far as the lower dorsal 
region, below which it again diminished; in these cases, 
too, there remained a narrow strip of healthy tissue at 
the junction of the posterior horn with the posterior 
commissure, or along the posterior horn. 
Gowers states that the cervical root zone never es- 
capes in Friedreich's ataxia, as it sometimes does in 
tabes; but in the cervical cord of XI. the postero- 
external columns were scarcely at all involved. 
The Lateral Pyramidal Tracts are reported sclerosed 
throughout nearly their whole length in 11 cases; and 
possibly the disease was overlooked in the remaining 
one for reasons already given. The degree of degen- 
eration in sectional area in these cases varies considera- 
bly. In II. and III. only the portions adjacent to the 
posterior horns were affected throughout the cord, and in 
the latter the change could only be detected by the mi- Friedreich's ataxia. 
59 
croscope. The degeneration in IV. occupied merely the 
posterior parts of the lateral tracts, but reached quite to 
the periphery, while nearly the whole length and sec- 
tional area were sclerosed in V. Case VI. is described 
as resembling IV. In VII. the degeneration was situ- 
ated at the junction of the anterior and posterior horns, 
and reached the periphery in the lumbar cord only; 
VIII. exhibited more sclerosis in the dorsal than in the 
lumbar region; it was especially well marked in IX. in 
the cervical swelling, and in X. in the dorsal cord; while 
in XI. and XII. it seemed to decrease from above down- 
ward, and at any rate was less marked in the lumbar 
portion. 
A very important feature is still to be remarked, 
namely, that in 7 cases (IV., V., VI., IX., X., XI., XII.) 
the diseased portion of the lateral tracts did not imme- 
diately join the posterior horns; but was separated by 
a band of healthy white tissue. No direct statement is 
made concerning the matter in II., III., and VII., but 
it would seem that no such band existed in these cases, 
and it was clearly absent in VIII. 
The Direct Cerebellar Tracts would appear from de- 
scriptions and drawings to have been more or less in- 
volved in 7 cases (IV., V., VI., VIII, X., XI., XII.) 
throughout all or nearly all of their length, with a greater 
or smaller sectional area of degeneration. 
A Peripheral Degeneration passing forward from the 
sclerosed cerebellar tracts has been described in 5 cases, 
viz., IV., VI., VIII., X., and XI. An excellent ex- 
ample is the case of Gowers' and Pitt's (X.), in which it 
was well marked, and extended even along the anterior 
median fissure from the cervical to the upper dorsal 
region, though below this point it was irregular -and 60 
GRIFFITH, 
slight. In IV. it was to be seen only in the cervical 
cord, and in VI. the position was the same, but the zone 
of degeneration was not so complete, and did not extend 
so far forward. Gowers figures slight peripheral scle- 
rosis in the lumbar cord in a section which he examined 
from Smith's case (VIII.). Case XI. had but a partial 
development of this sclerosed zone, and only in the upper 
cervical region. 
The Anterior Pyramidal Tracts were sclerosed to 
some extent in 6 cases (IV., V., VI., VII., VIII., X.). 
In IV. and V. the degeneration extended downward on 
the right side into the dorsal region, and in VI. both 
sides were irregularly sclerosed from the decussation 
downward. Case VII. showed irregular degeneration 
on both sides, chiefly in the neighborhood of the anterior 
cornua; in VIII. there was a bilateral sclerosis in the 
dorsal region ; and in X. from the upper dorsal cord 
upward. 
Changes in the gray matter are reported as follows : 
The Columns of Clarice were found to be degenerated 
in 8 instances, viz., IL, IV., V., VI., IX., X., XL, XII. 
Friedreich makes no reference to them in I., IL, and III.; 
but Schultze found the degeneration years later in II.; 
arid perhaps could have detected it in I. and II I. Brousse 
(VII.) does not speak of the condition of gray matter. 
Smith (VIII.) says that the nerve cells of the posterior 
cornua were at all levels far less numerous than usual, 
and in this statement probably includes Clarke's col- 
umns, which would increase the number of cases to 9. 
Other changes in the gray matter were sometimes seen. 
Thus in II. there was sclerosis of the middle and posterior 
portions from the cervical enlargement to the mid-dorsal 
region, and even the anterior horns were affected to Friedreich's ataxia. 
61 
some extent. In IV. there was diminution of the 
volume of the gray matter, especially evident in the 
cervical enlargement. In VIII. the cells of the anterior 
horns as well as of the posterior horns (see above) 
were diminished in number. There was degenera- 
tion between the anterior and posterior horns in IX.; 
and in X. portions of the posterior horns were affected 
in some sections. Inflammation in and about the cen- 
tral canal was seen in Cases II., VII., and VIII., where 
the canal was filled with small round cells ; and in XI., 
in which it was found obliterated in most sections. Sup- 
plementary canals have also been noticed in rare in- 
stances. In VIII. a small channel of this nature was 
situated a little posterior to the main tube, and ex- 
tended throughout nearly the whole lumbar region. In 
X. there were 2 canals not more than 1 inch in length, 
lined by columnar epithelium, and situated one behind 
the other in the central portion-of the cord at the decus- 
sation of the pyramids, thus taking the place of the 
usual single central canal. In III. there were 2 sup- 
plementary canals, about 1 line in diameter, beginning 
at the lumbar swelling and extending half way to the 
cervical enlargement. They were situated one on each 
side, chiefly in the gray matter, at the junction of the 
posterior and anterior horns, and were filled with a clear 
fluid. In the upper half of the cord they were represented 
by foci of gelatinous material occupying similar posi- 
tions, and downward in the lumbar enlargement some 
traces of these were found. 
Nerves.-The posterior nerve roots were sclerosed in 
all the cases, and in general extensively, as in VIII., 
where there was scarcely a fibre not degenerated. In a 
few cases the sclerosis was less severe, as in VII., where 62 
GRIFFITH, 
the nerve roots were only affected in the lumbar region; 
and in X., where only some of the fibres were diseased. 
Some strands of the anterior nerve roots were sclerosed 
in VIII.; slight changes were found in the sciatic, crural, 
brachial, and hypoglossal nerves in I.; and the median 
and sciatic were somewhat degenerated in XII. 
Histology.-The histological nature of the changes 
found in the spinal cord is described by Friedreich as 
simple sclerosis; an overgrowth of the neuroglia at the 
expense of the nerve fibres. A finely fibrillated or 
granular tissue develops, traces of the nerve fibres are 
scarcely to be found, corpora amylacea are frequently 
very numerous, and the large cells of the gray matter 
shrink and lose their processes. In Case III. the lateral 
columns underwent a simple softening instead of the 
usual sclerotic change. 
Pathology. 
The nature of Friedreich's ataxia and its position 
among other diseases have been matters of considerable 
discussion. Friedreich, Erb, Mobius, Carre, Ormerod, 
and Grasset classify it as a variety of tabes only. In 
fact, Eulenburg, Topinard, and Carre do not distin- 
guish it at all from this affection, and the last two 
writers include Friedreich's cases in their works on loco- 
motor ataxia. Friedreich, however, would seem to sep- 
arate it from locomotor ataxia rather more sharply than 
do the others. Bourneville considered it a combination 
of tabes and disseminated sclerosis. There are, indeed, 
clinical reasons why the belief in its intimate connection 
with disseminated sclerosis could be entertained, for 
many of the symptoms, especially in some cases, are Friedreich's ataxia. 
63 
strikingly similar, yet none of the autopsies which have 
been made reveal any instance of lesions en plagues. 
Friedreich's ataxia certainly belongs to quite another 
group of diseases, while both anatomical and clinical 
symptoms distinguish it also from tabes. Hammond 
was of the opinion that the ataxia was caused by cere- 
bellar disease, but this is entirely unconfirmed by the 
results of post-mortem examinations; and though Vizioli, 
Galassi, and Musso agree with him regarding its cere- 
bellar origin, they do not consider it due to disease of 
the cerebellum, but to interference with the innervation 
from this centre in its descent through the diseased 
cord. They entertain the view expressed by Seppilli, 
Brousse, and Charcot, and probably most generally held, 
viz., that Friedreich's ataxia is a distinct, independent 
affection. It is placed by some writers intermediate 
clinically between tabes and disseminated sclerosis, but 
more exactly by Gowers between tabes and ataxic para- 
plegia. The early age at which it appears led Fried- 
reich to the conclusion that there existed a congenital 
disposition to it; that there was an hereditary arrest of 
development of the spinal cord in toto ; and that the 
poorly developed organ was readily attacked by a scler- 
osing process. This same view is adopted by Schultze, 
Mobius, Vizioli, Musso, and others. 
The question as to the primary lesion is an exceed- 
ingly interesting and difficult one. Friedreich says that 
the nature and position of the anatomical changes ren- 
der it certain that the spinal disease cannot be sec- 
ondary to an alteration in the medulla, or vice versa; 
and, basing his belief on the first 3 autopsies, he claimed 
that the meningitis was the primary cause and that other 
lesions were secondary to this. After the autopsy on 64 
GRIFFITH, 
Friedreich Suss (IV.), however, he concluded that the 
degeneration of the posterior columns in all his cases wss 
primary, but that the disease of the lateral columns, and 
in IV. of the peripheral zone, was secondary to the men- 
ingitis which the posterior sclerosis had set up. Smith 
entertains the same opinion, though claiming that the 
secondary inflammation is not of a diffuse nature. 
Brousse maintains that the posterior sclerosis is primary 
and systemic, and that extension from it occurs in two 
ways: the one by means of the inflamed central canal, 
the other peripherally by a leptomeningitis ; the disease 
of other parts of the cord being secondary and diffuse. 
Schultze considers the affection in Cases IV. and VI., 
which he examined, to be a degenerative atrophy of the 
entire posterior half of the cord, with certain slight excep- 
tions, and claims that though the distribution of the scle- 
rosis is too regular for disseminated sclerosis, it is too 
irregular for a genuine systemic disease. Kahler and 
Pick think that it is not an arrested growth of the cord 
in toto, but rather an imperfect development of the fibres 
of certain nervous systems which is the primitive cause 
of the disease. This development ceases at about the 
time of the sheath formation, and is induced by heredi- 
tary influences; and later in life, under the action of 
some agencies unknown to us, degeneration takes place 
in these imperfectly formed fibres. These authors were, 
I think, the first to assert that Friedreich's ataxia was a 
hereditary combined systemic disease of the cord-i. e., 
a simultaneous and independent degeneration of two or 
more systems of nerve fibres. They regard the periph- 
eral degeneration as a later and accessory change, per- 
haps due to a subsequently developing chronic men- 
ingitis. Rutimeyer adopts the same view regarding Friedreich's ataxia. 
65 
both the nature of the disease and the origin of the 
peripheral sclerosed zone, and Erlicki and Rybalkin 
claim that the post-mortem lesions in their case can only 
be accounted for by admitting the presence of a com- 
bined systemic spinal disorder. Pitt proposes a very 
rational theory which well explains the arrested de- 
velopment of the cord in toto, and at the same time 
harmonizes with the views of Kahler and Pick. He 
believes that the general or partial smallness of the cord 
is congenital, being a consequence of an inherited ten- 
dency to early vascular degeneration. The posterior 
columns, being naturally the most vascular, of course 
suffer the most, and are the most diminished in size. 
The earliest histological changes consist less in an ex- 
cessive growth of neuroglia than in an imperfect devel- 
opment of the medullary sheaths, followed later by a 
degeneration; the first cause being probably an end- 
arteritis. These views necessitate the belief in a systemic 
degeneration of various parts of the cord, and are sus- 
tained by the fact that the tracts most sclerosed corre- 
spond very nearly to those which are the last to acquire 
medullary sheaths in fcetal life ; and further that puberty 
or an acute illness, with the vascular changes which 
accompany both, often precipitates or accelerates the 
development of the symptoms of the ataxic disorder. 
Again, it is impossible in Cases X. and VII. that the 
affection could have spread from a meningitis, in spite 
of the peripherally sclerosed zone in the former, since 
no meningitis existed, and in IX. too, it did not pass 
beyond the surface of the posterior columns ; and on 
the other hand, the 7 instances in which there was a 
band of healthy tissue in the lateral tract adjacent to 
the posterior horn confute the supposition that in these, 66 
GRIFFITH, 
-the majority of cases,-there had been an extension 
of degeneration by contiguity from tract to tract, or 
through the posterior horn to the lateral tract. There- 
fore, although more autopsies are needed to settle the 
question positively, the evidence is strongly in favor 
of the view that Friedreich's ataxia is a combined sys- 
temic spinal disease, at least for certain parts of the 
cord. Whether there may also occur in some instances 
a diffuse secondary inflammation of other districts does 
not seem so clear, though it is possibly the case. 
The cause of the bulbar symptoms cannot be satisfac- 
torily explained by any of the pathological lesions yet 
discovered. The usual absence of pain and of dimi- 
nution of cutaneous sensibility is likewise not easy of 
explanation. There is reason to believe that these dis- 
turbances are not connected with involvement of the 
exterior portion of Burdach's columns, as has been sup- 
posed, since in certain cases, as IX., the whole transverse 
section of the posterior columns was sclerosed, yet the 
cutaneous sensibility was unaffected. Rutimeyer ad- 
vances the theory that they depend on disease of the 
boundary layer of the posterior horn {Rand Zone of 
Lissauer), since in his 2 autopsies this zone was unaf- 
fected, while in tabes its involvement is one of the most 
characteristic changes in the gray matter. Gowers 
states that Friedreich's ataxia differs anatomically from 
ataxic paraplegia in the greater degree of affection of the 
posterior columns in the lumbar region, and in the in- 
volvement of the posterior nerve roots. It is on this 
account that the exaggerated knee-jerk and ankle clonus 
are absent in the former and present in the latter disease. Friedreich's ataxia. 
67 
Diagnosis. 
The recognition of Friedreich's ataxia is not always 
easy; the principal and usual conditions on which a 
diagnosis may be based being, 1, the evidence of some 
hereditary influence; 2, the occurrence of several cases 
in a family; 3, the early age at which the symptoms 
appear; 4, the presence of motor ataxia spreading from 
below upward; 5, static ataxia in more advanced cases ; 
6, affection of speech; 7, nystagmus late in the disease; 
8, talipes ; 9, curvature of the spine; 10, frequently 
paralysis late in the disease. Negative symptoms are 
the absence, 1, of patellar reflex; 2, of marked sensory 
lesions, at least early in the disease; 3, of decided 
trophic lesions; 4, of affection of intellect; 5, of affection 
of the optic nerve; 6, of visceral or vaso-motor disturb- 
ances. But some of these symptoms may be absent in 
certain cases, and others may appear which suggest the 
presence of some other affection; so that in this, as in all 
diseases, the diagnosis must be based on the aggrega- 
tion of symptoms rather than on individual ones. (For 
anomalous cases see Remarks on Table I.) 
Clinically, Friedreich's ataxia is most closely allied to 
tabes dorsalis, disseminated sclerosis, and ataxia para- 
plegia. 
Uncomplicated tabes dorsalis rarely appears before the 
age of 25-30, and the cases occurring during childhood 
are of the utmost rarity, if, indeed, genuine. It exhibits 
neither incoordination of speech nor nystagmus ; though 
lesions of the optic nerve, affection of the pupillary 
reflex, and other disturbances connected with the eye 
are frequent and early symptoms. Severe pain is one 
of the first and most common signs of the disease; 68 
GRIFFITH, 
other affections of sensation are usual and appear early ; 
and visceral and atrophic affections are generally present. 
The gait of tabes is peculiar; the feet being projected 
too far forward, while in Friedreich's ataxia the projec- 
tion is often more lateral, and the gait is oscillating, as 
has been described. Charcot says that " static ataxia " 
is rarely seen in tabes. 
Disseminated sclerosis, whose symptoms still more 
nearly resemble those of Friedreich's ataxia, usually 
develops after the age of 20, though it may appear in 
infancy. The influence of heredity is exceptional, and 
the progress of the disease is not infrequently marked 
by remissions which seem almost like recovery. Nys- 
tagmus and affection of speech are common to both 
disorders, but in disseminated sclerosis the former is 
usually more extreme than in the other disease, and is 
not simply " ataxic nystagmus; " while the speech is 
typically scanning-a feature which may or may not be 
present in Friedreich's ataxia. Some of the more dis- 
tinguishing symptoms of disseminated sclerosis are the 
exaggerated knee-jerk, ankle clonus, rhythmic oscilla- 
tions, violent jerking movements,- intention tremor, 
rigidity and spastic paraplegia, disturbance of intellect 
often with epileptiform and apoplectiform attacks, and 
the absence of Romberg's phenomenon. These symp- 
toms either do not occur at all in Friedreich's ataxia, 
or are seen only exceptionally. 
Ataxia paraplegia develops, according to Gowers, 
between 30 and 40 years of age, and does not occur in 
several members of a family. The knee-jerk is exag- 
gerated, and there is no nystagmus. Marked affection 
of speech is wanting, though there is sometimes slight 
defect in articulation. fkiedreich's ataxia. 
69 
Of the other diseases with ataxic symptoms, cerebellar 
tumor can hardly be confounded with the disease in 
question. The gait is " drunken," and resembles many 
cases of the spinal disease; but there is no involve- 
ment of the arms, and the tendon reflexes are present; 
while tumor also produces headaches, optic neuritis, 
vomiting, epileptiform attacks, and marked vertigo. 
Certain instances of Friedreich's ataxia exhibiting ex- 
cessive static ataxia or choreiform movements, as in 
Sinkler's last 3 cases (137, 138, 139), might raise the 
question as to the diagnosis of the affection from hered- 
itary chorea. This disorder, however, is distinctly he- 
reditary (direct similar inheritance) for generations, and 
without a break; nearly always appears after the age of 
35-40 years; has no truly ataxic symptoms, and is 
almost invariably sooner or later associated with severe 
disturbances of intellectual power with a tendency to 
suicidal mania. There is, further, no diminution of the 
patellar reflex. 
Duration, Prognosis, Treatment. 
Duration.-The course of Friedreich's ataxia is 
steadily onward, and though there frequently occur 
periods during which no decided progress of the dis- 
ease can be detected, yet improvement in the symptoms 
rarely takes place. This is the opinion generally ac- 
cepted, and I recollect but 3 cases-one of Smith's (100), 
one of Teissier's (60), and that of Leubuscher-in which 
notable improvement was observed. In Teissier's case 
the symptoms largely disappeared during 2 years, and 
then returned; though not to so great a degree up to 
the time when the case was last examined. The dura- 70 
GRIFFITH, 
tion of the disease is very extended, unless it be termi- 
nated by some intercurrent affection. Many patients 
are able to follow their avocations for years, with very 
little increase in the severity of the symptoms. 2 of 
Friedreich's cases (2 and 5) were ataxic 34 and 27 years 
respectively, and died finally of typhoid fever. One of 
Smith's cases (101) has had the disease 31 years; one 
of Stintzing's (123) 28 years; and one of Vizioli's (81) 
41 years. But the longest duration reported is in an- 
other patient of Vizioli's (87) where the ataxia lasted 
from birth till he died at the age of 46. On the other 
hand, some cases have grown rapidly worse, and have 
died with advanced symptoms in but a few years from 
the onset. One of Hammond's cases (33), for example, 
suffered but 8 years; 2 of Coleman's patients (38, 39) 
never walked, and died at 3 years of age; and the 
other (37), as well as a case of Vizioli's (88), died at 6 
years, and like the others had never walked. As far as 
I have been able to discover, 25 of the cases in the table 
are reported by the observers to have died. 5 of these 
(1, 3, 4, 6, 14, 38) are said to have died of typhoid 
fever; 1 (117) of typhoid fever and phthisis; 2 (19, 
107) of phthisis; 1 (38) of some pulmonary affection; 
1 (2) of nephritis ; 1 (30) of apoplexy; 4 (5, 33, 34, 94) 
apparently of advancing weakness; 1 (46) of fatty 
heart; 1 (27) of fibroid heart; 1 (102) of acute mye- 
litis; 1 (86) of "taking cold;" and 1 (88) of diarrhoea. 
In 1 (48) the cause of death is not clear, and in the re- 
maining 4 (37, 39, 87, 128) there is no intimation given 
as to its nature. 
Prognosis.-It will be seen from what precedes, that 
the prognosis is usually favorable as regards the dura- 
tion of life, but most serious as regards recovery from Friedreich's ataxia. 
71 
the disease. The patient will die of the progressive 
weakness in the course of Friedreich's ataxia, if not 
carried off by some intercurrent affection. How soon 
the habits of life will be seriously interfered with, and 
when the lethal exitus will probably take place, must be 
determined for each individual patient, by considering 
the former rapidity of progress in that particular case. 
Treatment.-Treatment has proved unavailing in 
almost all cases. One of Teissier's patients (60) im- 
proved greatly, apparently through better hygienic sur- 
roundings. One of Smith's cases (100) improved tem- 
porarily under the use of nitrate of silver. This drug 
naturally suggests itself, and has, indeed, been used 
in a number of other cases, but without any decided 
benefit. Arsenic, phosphorus, iodine, zinc, may also 
be tried, and cauterization over the vertebral column 
has been proposed. The constant current applied to the 
spine seemed to be of benefit in Leubuscher's case. One 
of the chief indications is to sustain the general nutri- 
tion by tonics, iron, cod-liver oil, change of air, salt and 
other bathing, etc., and to stimulate by electricity and 
massage muscles which are apparently beginning to 
lose power. When the spinal curvature and the lack of 
control of, or lack of power in, the dorsal muscles render 
the patient unable to sit upright, a plaster jacket may 
be applied with great advantage. I have found this 
give great relief to one of my own patients (142). Case 
102 (Smith's) also improved surprisingly under electri- 
cal treatment and the employment of a plaster jacket. 72 
GRIFFITH, 
Remarks ox some of the Cases in Table 1. 
I -have already stated that 1 do not by any means con- 
sider that all the cases in the table are undoubted in- 
stances of Friedreich's ataxia, but that all of them have 
at least some claim to be considered such. The cases 
about which doubt has been or might perhaps be enter- 
tained are 7, 8, 11-12, 16-18, 19, 22-23, 31-36, 37-39, 
55-56, 60, 68, 100, 108, 109, in, 114-115, 117. Most 
writers have no question concerning Case 7 (Carre), but 
a few-e. g., as Seeligmiiller-doubt its genuineness 
chiefly on account of the very marked degree of initial 
and later pain. 8 (Bradbury) is not admitted by Bury, 
but has, I think, a good right to a place with other cases. 
Kellogg's cases (11-12) are not as well reported as 
could be desired, and are on this account excluded by 
many tabulators, but included by the Italian writers 
and by Bury. There seem to be good reasons to con- 
sider them genuine. Dreschfeld's patients (16-18) are 
certainly questiona ble, and some of them resemble tabes 
very greatly. They are tabulated by Ormerod, but re- 
jected by Smith. The fact that there were 5 attacked 
in one family is their greatest claim for membership 
among this class of cases. Most authorities do not 
hesitate to call 19 an instance of Friedreich's ataxia, 
though Riitimeyer questions it, and Gowers assigns it 
to ataxic paraplegia. 22-23 (Seeligmiiller's) are ex- 
cluded by many writers, but considered genuine by 
Smith, Vizioli, Prince, and others, with whom I am 
disposed to agree. Hammond's cases (31-36) are so 
briefly and imperfectly described that Smith rejects them 
altogether, and they are very generally called in ques- TABLE I.-OBSERVED AND REPORTED CASES. ARRANGED CHRONOLOGICALLY. 
Number. 
Name. 
X* 
0) 
X 
Observer. 
! (Brackets indicate 
those of one family 
of brothers and 
sisters.) 
Ataxia in parents, grand 
parents, uncles or aunts. 
Other neuroses, including 
great nervousness in 
parents, etc. 
Alcoholism in parents, etc. 
Tuberculosis of parents, etc. 
Syphilis, etc. 
Consanguinity of parents or 
grandparents. 
Other causes in parents, 
grandparents, etc. 
Number of cases observed 
in children of one family. 
Number of other children 
reputed ataxic. 
Other neuroses in children 
of family. 
Other suspicious conditions 
in children of family. 
Where the ataxic symptoms 
began. 
Age when first attacked. 
Age when upper extremities 
affected. 
Age when bulbar symptoms 
appeared. 
Age when last examined, or 
when died. 
Ataxia precipitated by acute 
febrile disease. 
Ataxia accelerated by acute 
disease. 
Ataxia of lower extremities. 
Ataxic station. 
Ataxia of upper extremities. 
Ataxia of head (neck) 
Ataxia of trunk. 
Tremor. 
| Choreiform movements. 
. Spasmodic contractions of 
1 muscles. 
Paralysis. 
Inability to walk ; and age 
when it developed. 
Curvature of spine; 
cyphosis; scoliosis. 
1 Talipes. 
| Other contractures. 
Electrical contractility. 
Patellar reflex. 
| Plantar reflex. 
Other cutaneous reflexes. 
| Atrophy of muscles. 
Other trophic changes; and 
vaso-motor affections. 
Pain, initial, eras early 
symptom. 
| Pain, later in disease. 
Cutaneous sensibility. 
Muscle sense. 
i Girdle sensation, or other 
I paraesthesias. 
Affection of speech. 
| Tremor of tongue. 
5 
s 
hO 
i 
Affection of optic nerve. 
Affection of intellect. 
Vertigo. 
Visceral and secretory dis- 
1 turbances. 
1 
Andreas Lotsch, 
M. 
Friedreich, 
0 
+ 
... 
2 
0 
0 
0 
1 
17 
25 
26 
35* 
... 
... 
... 
± 
+ 
... 
... 
4- 
18 
0 
... 
N 
... 
0 
0 
0 
N 
N 
... 
± 
- 
0 
... 
0 
0 
- 
2 
Charlotte Lotsch, 
F. 
0 
... 
+ 
2 
0 
o 
0 
I 
17 
25 
25 
51* 
p 
f + 
± X 
+ 
4" 
0 
+ 
-1- 
<31 
c s 
e r 
0 
-F 
0 
N 
+ 
0 
+ 
= 
N 
0 
Of- 
4- 
0 
0 
4- 
4- 
3 
Justinia Siiss, 
F. 
" r 
o 
+ 
+ 
+ 
4 
0 
0 
0 
I 
15 
19 
20 
31* 
... 
... 
+ 
4- 
4- 
... 
... 
4- 
o 
cs 
... 
N 
... 
... 
0 
... 
+ 
4- 
- 
± 
+ 
0 
4- 
... 
4 
Salome Siiss, 
F. 
° 
4- 
+ 
+ 
... 
4 
0 
0 
0 
I 
15-16 
19 
25 
28* 
... 
... 
4- 
... 
... 
... 
- 
+ 
c s 
... 
N 
... 
... i 
0 
+ 
+ 
N 
... 
4- 
... 
... 
0 
5 
Lisette Siiss, 
F. 
° 
+ 
+ 
+ 
... 
4 
0 
0 
0 
I 
14 
<28 
... 
41* 
... 
4- 
+ 
... 
4- 
0 
cs 
... 
... 
4- 
N 
... 
± 
+ 
0 
0 
Of 4- 
4- 
6 
Friedreich Siiss, 
M. 
" I 
0 
4" 
+ 
+ 
4 
0 
0 
0 
I 
14 
16 
17 
38* 
... 
±X fl 
4-XfI 
±x 
+ 
... 
0 
>24 
s 
N 
0 
N 
N 
0 
... 
... 
N 
N 
± 
... 
4- 
0 
4- 
- 
7 
Sophie C., 
F. 
Carre, 
... 
... 
1 
6 
I 
22 
... 
28 
+ X 
+ 
- 
... 
0 
0 
... 
0 
± 
N,(- 
... 
4- 
4- 
0? 
0 
... 
8 
M. C. 
M. 
Bradbury, 
+ 
... 
1 
1 
I 
17 
... 
18 
... 
± 
+ 1 
4- I 
0 
0 
0 
... 
N 
+ 
- 
N 
... 
0 
... 
... 
0 
+ 
4- 
9 
M. 11. 
F. 
Carpenter, 
0 
0 
2 
1 
I ? 
12' 
... 
28 
... 
... 
±1 
±1 
4-1 
... 
0 
= 
0 
s 
0? 
0 
0 
0 
- 
... 
0 
- 
... 
0 
... 
10 
E. H. 
F. 
0 
0 
2 
1 
1 
14 
26 
... 
... 
+ 
4- I 
... 
0 
... 
... 
0 
0 
... 
... 
- 
... 
0 
M. 
Kellogg, j 
0 
o 
Q 
0 
I 
6 
10 
± 
4- 
0 
... 
N 
4- 
4- ? 
=? 
M. 
0 
o 
2 
1 
0 
I 
5 
7 
4- 
... 
0 
0 
0 
0 
0 
13 
Louise Schulz, 
F. 
Friedreich, 
0 
0 
0 
... 
0 
3 
0 
0 
I 
12 
12 
13 
30 
... 
R 
± X 
±X 
± X 
+ 
+ 
... 
= 
... 
0 
0 
... 
N 
0 
N 
N 
0 
... 
... 
- 
= 
-? 
N 
0 
± 
0 
0 
- 
14 
Katharina Schulz, 
F. 
0 
0 
0 
... 
0 
3 
0 
0 
+ 
1 
12 
<13 
13 
19* 
... 
... 
± X, 
0 X 
± X 
... 
... 
... 
0 
0 
... 
0 
... 
- 
... 
N 
0 
+ ■ 
... 
0 
... 
... 
15 
Marie Schulz, 
; f. 
0 
0 
0 
... 
0 
3 
0 
0 
+ 
I a 
12 
12 
<13 
18 
... 
+ X 
+ x 
1-x 
... 
... 
... 
... 
0 
0 
0 
N 
N 
0 
... 
0 
0 
N 
N 
4- 
0 
+ 
... 
0 
... 
16 
James B. 
M. 
Dreschfeld, 
0 
... 
... 
3 
2 
- 
... 
l 
20 
40 
... 
42 
T 
... 
+1 
+1 
- X 
... 
0 
... 
... 
N 
... 
... 
0 
± 
... 
= 
0 
0 
0 
... 
... 
17 
John B. 
M. 
cc 1 
0 
... 
... 
... 
3 
2 
- 
... 
I 
23 
... 
... 
32 
... 
... 
- I 
ox 
... 
0 
... 
= 
4- 
± 
4- 
N 
- 
0 
... 
0 
0 
- 
18 
Mrs.Martha C.(neeB). 
F. 
0 
... 
... 
3 
2 
- 
... 
I 
20 
... 
34 
... 
+1 
... 
0 
... 
... 
... 
0 
0 
... 
0 
+ 
... 
- 
... 
0 
... 
... 
... 
19 
Josefa Strasik, 
F. 
Kahler & Pick, 
0 
... 
+ 
1 
0 
+ 
I 
15 
22 
22 
23* 
... 
... 
f 4- 
... 
4-X 
- 
- 
... 
... 
... 
± 
22 
s 
0 
= 
0 
... 
4- 
- 
0 
0 
N 
N 
0 
4- 
0 
0 
-? 
4- 
20 
. George B. 
M. 
Schmidt, j 
0 
0 
+ 
... 
0 
2 
0 
0 
0 
I 
11 
... 
21 
... 
... 
±x 
f ±x 
± X 
4- 
+ 
0 
... 
0 
0 
>19 
N 
0 
N 
N 
0 
0 
0 
- 
N 
0 
4- 
0 
4~ 
0 
... 
21 
Linus B. 
M. 
« I 
0 
0 
+ 
... 
0 
2 
0 
0 
0 
1 I 
10 
12 
... 
... 
+ 
0 
... 
0 
0 
0 
0 
... 
... 
N 
N 
... 
... 
0 
... 
22 
Leo v. K. 
M. 
Seeligmiiller, | 
± 
... 
... 
+ 
... 
2 
1 
... 
I 
9-12 
<22 
26 
+1 
+1 
= 1 
- 
... 
... 
0 
0 
s 
N 
+ 
0 
- 
... 
4- 
N 
4- 
0 
4- 
* 
- 
- 
4- 
23 
Alexander v. K. 
M. 
± 
... 
... 
+ 
2 
1 
... 
1 
27? 
28 
... 
+ 
0 X 
0 
0 
- 
... 
0 
0 
c 
... 
4- 
0 
... 
0 
... 
... 
- 
N 
= 
= 
... 
- 
4- 
24 
Harry G. 
M. 
Hollis, 
0 
0 
... 
1 
0 
0 
0 
I 
9 
... 
13 
0 
... 
+ 
+1 
0 
... 
0 
0 
N 
0 
N 
... 
- 
N 
... 
0 
4- 
... 
0 
... 
4- 
25 
Shaw, 
M. 
Gowers, [ 
... 
± 
... 
0 
5 
0 
0 
0 
I 
21 
39 
... 
+ 
+ 
... 
... 
... 
- 
0 
0 
N 
N 
0 
0 
N, 4- 
... 
+ 
... 
0 
... 
0 
... 
... 
26 
Shaw, 
F. 
... 
± 
... 
... 
0 
... 
5 
0 
0 
0 
I 
18 
... 
... 
29 
... 
+ 
+ 
0 
- 
0 
0 
... 
0 
0 
N 
... 
4- 
0 
... 
0 
... 
27 
Robert Shaw, 
M. 
... 
± 
... 
... 
0 
... 
5 
0 
0 
0 
I 
19 
... 
28-29* 
... 
... 
4- 
+ 
4- 
4- 
4- 
0 
0 
0 
N 
- 
0 
0 
N 
+ 
... 
0 
0 
... 
28 
Shaw, 
M. 
± 
... 
... 
0 
... 
5 
0 
0 
0 
I 
19-20 
22 
... 
... 
- 
-I 
0 
... 
... 
... 
0 
... 
0 
= 
... 
0 
0 
... 
= 
0 
... 
0 
... 
29 
Shaw, 
M. 
... 
± 
... 
0 
... 
5 
0 
0 
0 
I 
18-19 
... 
19 
... 
- 
01 
= 
- 
0 
0 
... 
0 
- 
0 
0 
- 
+ 
... 
0 
... 
0 
30 
Marie R. 
F. 
Brousse, 
+ 
- 
... 
... 
1 
... 
I 
24 
32* 
... 
4- 
+1 
4-1 
4- 
0 
0 
... 
0 
0 
... 
- 
+ 
0 
+ 
- 
31 
J. T. 
M. 
Hammond, < 
0 
... 
... 
2 
0 
... 
I 
4 
6 
6 
19 
... 
X 
... 
4- 
8 
... 
N 
0 
N 
0 
4- 
0 
32 
H. T. 
M. 
" I 
0 
... 
2 
0 
... 
I 
10 
>11 
>11 
21 
... 
... 
X 
X 
... 
>U 
... 
... 
... 
... 
... 
... 
N 
0 
4- 
... 
33 
M. B. 
M. 
J 
0 
0 
... 
2 
0 
0 
0 
I 
6 
<8 
8 
14* 
... 
X 
... 
+ 
0 
... 
... 
0 
4- 
N 
N 
0 
± 
... 
-T 
4- 
34 
C. B. 
M. 
1 
0 
0 
... 
... 
2 
0 
0 
0 
I 
8 
9 
... 
15* 
... 
... 
... 
... 
... 
... 
... 
9 
... 
0 
- 
N 
0 
4" 
... 
35 
E. M. 
M. 
0 
0 
... 
... 
2 
0 
I 
3 
6-7 
6-7 
30 
... 
... 
I 
... 
4- 
4- 
0 
... 
N 
0 
0 
0 
N 
... 
0 
4- 
+ 
... 
+ 
... 
36 
H M. 
M. 
0 
0 
2 
0 
I 
7 
... 
26 
... 
... 
- 
... 
- 
... 
... 
0 
0 
N 
... 
0 
4- 
... 
... 
... 
37 
Green R. 
M. 
Coleman, ( 
0 
0 
... 
3 
0 
0 
0 
I 
1 
... 
6* 
... 
... 
... 
... 
+ 
4- 
0 
0 
0 
N 
4- 
... 
... 
... 
... 
38 
Frank R. 
M. 
" s 
0 
0 
3 
0 
0 
0 
I 
... 
... 
3* 
... 
... 
... 
4- 
4- 
... 
0 
0 
0 
N 
... 
4- 
... 
0 
39 
Willie R. 
M. 
0 
0 
... 
... 
3 
0 
0 
0 
I 
in 
3* 
... 
... 
... 
... 
... 
4* 
4- 
0 
... 
0 
0 
N 
... 
... 
4- 
... 
... 
... 
40 
Nannie W. 
F. 
Warren, (- 
0 
± 
0 
0 
0 
0 
4 
0 
0 
0 
I 
8 
12 
21 
26 
0 
B 
f + 
f + 
f± 
4- 
+ 
± 
13 
c 
e 
0 
± 
- 
- 
- 
4- 
... 
0 
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0 
0 
- ! 
... 1 
2 
0 
0 
15-16 
21 
21 
22 
0 
0 
+ I 
4-1 
4- 
0 
21 
cs 
vl 
... 
N 
0 
... 
- 
0 
0 
± 
N 
- 
... 
4- 
0 
4- 
... 
0 
... 
113 
William G. 
M. 
1 
- 
0 
- 
0 
0 
... 
2 
0 
4- 
0 
I 
12-13 
14-15 
... 
19 
0 
... 
+ I 
+ I 
+ 
+ 
4- 
- 
0 
cs 
e r 
+ 
r d 
0 
N 
N 
... 
0 
N 
- 
4- 
0 
0 
0 
... 
114 
John W. 
M. 
li 
0 
0 
0 
± 1 
... 
1 
0 
0 
0 
I 
7 
12-13 
16 
M&Sc 
4-1 
4- 
4- 
4- 
0 
0 
c 
e 
4" 
rd 
0 
N 
- 
0 
N 
N 
4- 
4- 
- 
- 
0 
0 
... 
- 
115 
Jane R. 
F. 
u 
0 
0 
... 
1 
0 
0 
I a 
7 
7 
11 
12 
0 
0 
+ 
4- 
4- 
4- 
4- 
8-9 
s 
e 
± 
r d 
0 
N 
N 
4- 
0 
0 
N 
= 
... 
4- 
0 
- 
0 
0 
... 
116 
F. 
0 
0 
0 
0 
o 
0 
0 
1 
0 
o 
o 
8 
8 
8 
16 
0 
4- I 
+ I 
4- X 
4- 
4- 
- 
0 
s 
... 
N 
0 
N 
N 
0 
= 
- 
- 
N 
... 
4- 
0 
4- 
0 
0 
0 
- 
117 
J. N. 
F. 
Erlicki & Rybalkin,; 
- 
1 
16 
18 
... 
18* 
+ 
... 
± 
4- I 
+ 
... 
... 
... 
0 
0 
0 
N 
0 
N 
0 
0 
0 
N 
= 
0 
0 
4- 
0 
0 
0 
0 
- 
118 
Frederick B. 
M. 
Glynn, 
0 
+ 
0 
0 
0 
0 
1 
0 
... 
... 
I 
17 
... 
... 
21 
0 
0 
+1 
4- I 
+ 1 
+ 
4- 
4- 
... 
4- 
0 
... 
0 
... 
- 
N 
... 
4- 
4- 
... 
f 
... 
119 
Otto N. 
M. 
Freyer, 
0 
0 
••• 
,? 
+? 
-L 
3 
0 
- 
- 
I 
10 
... 
... 
19 
Ps 
... 
+ 1 
+ 
... 
... 
0 
0 
... 
N F 
0 
N 
N 
0 
. - 
... 
= 
N 
4- 
4- 
... 
4- 
0 
0 
4- 
- 
120 
Hans N. 
M. 
u S 
0 
0 
... ; 
? 
+? 
+ 
3 
0 
- 
- 
1 
<5 
... 
... 
16 
... 
+ 
+ 1 
4- 
... 
0 
... 
... 
0 
0 
0 
- 
... 
121 
N. 
M. 
0 
0 
-? , 
+? 
+ 
3 
0 
- 
- 
I 
... 
9 
... 
... 
0 
0 I 
... 
... 
- 
0 
0 
... 
... 
... 
0 
0 
N 
0 
0 
... 
0 
... 
122 
Paul P. 
M. 
Blocq, 
0 
0 
... 
1 
0 
0 
0 
I 
10 
12 
>12 
20 
0 
0 
± X 
± X 
+ 
0 
18 
e 
... 
N 
0 
0 
0 
0 
N 
... 
4" 
... 
4- 
... 
... 
... 
123 
Therese R. 
F. 
Stintzing, 
0 
0 
0 
... 
3 
1 
- 
0 
I 
4 
... 
32 
... 
... 
I + 
... 
= f 4- 
... 
... 
± 
20 
... 
0 
0 
0 
- 
4~ 
4- 
... 
... 
- 
124 
Rosalie R. 
F. 
cc S 
0 
0 
0 
3 
1 
- 
0 
I a 
8 
8 
15 
... 
+ 
+ 1 
- X 
... 
0 
0 
- 
0 
1' 
0 
N 
N 
0 
... 
4~ 
... 
0 
- 
125 
Joseph R. 
M. 
0 
0 
0 
3 
1 
- 
0 
8 
13 
... 
4- 
4- 
0 
0 
... 
... 
0 
0 
... 
... 
N 
... 
0 
4- 
0 
... 
126 
A. F. 
M. 
Ferrier, 
0 
... 
+ 
1 
0 
0 
0 
l 
7 
14 
18 
... 
... 
+1 
... 
+ 1 
+ 
0 
0 
0 
14 
s 
c V 
4- 
N 
0 
0 
N 
0 
0 
... 
... 
4- 
4- 
0 
0 
0 
of ± 
... 
127 
Paul X. 
M. 
Charcot, 
... 
... 
... 
1 
10 
20 
... 
... 
+ 
4- I 
+ T 
4- 
+ 
... 
0 
0 
... 
0 
... 
0 
0 
0 
N 
4- 
... 
+ 
... 
... 
128 
Willis F. 
M. 
Mastin, f 
- 
+ 
+ 
3 
0 
0 
0 
I 
10-12 
25 
>25 
32* 
0 
+ 
+ 
+ 
... 
... 
... 
4- 
4- 
... 
... 
0 
0 
... 
0 
0 
N 
0 
°n 
... 
... 
... 
129 
Ervin F. 
M. 
- 
+ 
+ 
3 
0 
0 
0 
I 
10 
... 
28 
0 
0 
+ 
0 I 
+ 
... 
... 
... 
+ 
0 
cs 
... 
- 
... 
0 
- 
0 
0 
N 
... 
0 
... 
0 
0 
0 
130 
Abraham F. 
M. 
- 
+ 
+ 
3 
0 
0 
0 
I 
8 
12 
17 
0 
0 
+ 
0 I 
+ 
... 
0 
c 
0 
... 
== 
0 
0 
0 
N 
... 
0 
0 
0 
0 
0 
131 
Emma F. 
F. 
Mendel, 
0 
0 
... 
0 
+ 
1 
1 
... 
... 
11 
1 
... 
4 
... 
... 
± X 
± X 
- 
= 
o 
... 
0 
0 
... 
0 
0 
N 
4- 
-r 
0 
0 
... 
132 
John Brand, 
M 
Ormerod, 
0 
± 
... 
... 
2 
0 
+ 
+ 
I 
18 
25-26 
R 
+ 
4-1 
f = 
+ 
0 
0 
0 
N F 
0 
N 
0 
4- 
- 
- 
- 
4- 
0 
- 
133 
Linda Brand, 
F. 
0 
± 
2 
0 
_1_ 
+ 
I 
5 
17 
17 
29 
Sc 
f + 
± 
... 
- 
... 
+ 
28-29 
0 
0 
NF 
0 
0 
4" 
N 
N 
... 
4- 
0 
4- 
... 
4- 
134 ■ 
Bridget Sullivan, 
F. 
0 
0 
+ 
... 
... 
1 
0 
- 
0 
a 
16 
16 
21 
... 
... 
± 
± I 
- 
- 
... 
... 
... 
0 
cs 
v r 
NF 
0 
0 
... 
= 
N 
N 
= 
... 
- 
0 
0 
135 
H. O. 
M. 
Shattuck, 
0 
0 
0 
0 
0 
0 
0 
1 
2 
0 
0 
l 
8 
12 
14 
0 
+ 1 
4-1 
4-1 
+ 
4- 
... 
0 
0 
8 
0 
0 
N 
... 
0 
0 
4- 
4- 
- 
0 
0 
... 
136 
M. 
Joffroy, 
0 
0 
+ 
... 
... 
1 
1 
0 
0 
I 
10 
15 
15 
19 
0 
0 
+ T 
4-1 
+ 
0 
... 
- 
0 
S 
C V 
- 
- 
0 
4- 
+ 
N 
4- 
... 
0 
0 
- 
137 
Annie MacDonald, 
F. 
Sinkler, 
0 
0 
0 
0 
0 
0 
0 
3 
0 
0 
0 
I a s 
3 
3 
3 
15 
+ 
... 
+ 
4-X 
+ x 
4- 
4- 
0 
0 
... 
N 
0 
0 
0 
N 
... 
0 
4- 
0 
4- 
... 
0 
0 
138 
Michael MacDonald, 
M. 
0 
0 
0 
0 
0 
0 
0 
3 
0 
0 
0 
I a 8 
2 
2 
2 
12 
... 
... 
+ 
4-X 
+ x 
+ 
+ 
... 
0 
0 
... 
... 
... 
4- 
0 
... 
0 
0 
N 
0 
4- 
0 
- 
... 
0 
0 
... 
139 
Bernard MacDonald, 
M. 
0 
0 
0 
0 
0 
0 
0 
3 
0 
0 
0 
las 
2 
2 
2 
11 
... 
+ 
4- 
+ x 
± 
4- 
0 
10 
s 
e r 
... 
... 
N 
- 
0 
0 
N 
0 
4- 
0 
... 
0 
0 
... 
140 
Blanche Roberts, 
F. 
Osler, 
0 
0 
0 
1 
0 
0 
0 
las 
3 
3 
3 
11 
... 
+ 
4-1 
4- 
+ 
0 
0 
0 
0 
0 
0 
... 
= 
0 
... 
0 
0 
N 
... 
0 
4- 
... 
0 
... 
0 
... 
141 
Sadie T. 
F. 
0 
0 
... 
2 
0 
0 
0 
I a 
10 
10 
>16 
23 
0 
T&M 
f + 
± X 
4- 
0 
0 
± 
10-11 
s 
el 
0 
0 
! ••• 
0 
0 
4- 
N 
- 
4- 
0 
- 
0 
0 
... 
142 
George T. 
M. 
Griffith, | 
0 
0 
... 
... 
2 
0 
0 
0 
I 
9 
>18 
21 
M 
0 
+ 
+ X 
- 
0 
0 
4- 
+ 
>18 
s 
0 
0 
... 
4- 
0 
0 
- 
= 
0 
- 
0 
- 
0 
0 
... 
143 
Annie C. 
F. 
0 
1 
1 
0 
0 
I 
5 
22 
26 
Sc 
0 
f + 
± X 
+ 
0 
0 
+ 
± 
16 
s 
e 1 
4- 
0 
... 
0 
4- 
0 
0 
- 
0 
4- 
- 
0 
0 
... 
... 
EXPLANATION OF SIGNS USED IN TABLE I. 
+ increased, or marked. 
± much increased, or very marked. 
- diminished, or slight. 
= much diminished, or very slight. 
0 none, or absent. 
? not clearly stated, or the author himself is in doubt. 
, or. 
> [as >12] after or more than the age of. 
< [as <12] before or less than the age of. 
* died at the age mentioned. 
a upper extremities. 
B bilious fever. 
C cyphosis [or lordosis], 
cv cavus. 
D diphtheria. 
e equinus. 
el equino-valgus. 
er equino-varus. 
F faradic. 
f that the condition indicated by the fol- 
lowing sign was formerly present. 
G galvanic. 
I increased on closing the eyes. 
in infancy. 
Z lower extremities. 
M measles. 
Ml malaria. 
Mn meningitis. 
My myelitis. 
N normal. 
P parturition. 
Ps pertussis. 
K rheumatism. 
r d reaction of degeneration. 
S scoliosis. 
s speech. 
Sc scarlatina. 
T typhoid fever. 
V variola, 
vl valgus, 
vr varus. 
X not increased on closing the eyes. Friedreich's ataxia. 
73 
tion by European writers, though tabulated by Ormerod 
and Vizioli. Careful reading " between the lines " of 
the author's report renders it probable that they are 
genuine, as he claims. 37-39 are also most incom- 
pletely described, but information received from Dr. 
Coleman through Dr. Smith renders it at least possible 
that they are instances of this disease, and I feel there- 
fore obliged to place them in the table. There certainly 
appears to be no other affection to which they can be so 
well assigned. Jakubowitsch's case (55) is by no 
means typical, and is excluded by Bury; yet it seems 
to accord with the symptom-complex of Friedreich's 
ataxia better than with any other disease. Erlenmeyer's 
case (56) most probably belongs here, and Rutimeyer 
is of the same opinion, though Friedreich and Erb were 
opposed (see " choreiform movements ''). The girl (60) 
described by Teissier might be excluded from the list 
on account of the improvement in the symptoms, had 
not her brother (59) been a well-marked case of the dis- 
ease. 68 is a very anomalous case and might possibly 
be disseminated sclerosis; but the distinct hereditary 
and family history strengthen Botkin's claim that it 
was Friedreich's ataxia. He believes that accidental 
focal lesions may also have existed. The age of the 
patient and some of the symptoms lead Gowers to rele- 
gate 100 to ataxic paraplegia. Considerable corre- 
spondence with Dr. Smith as to the course of the case 
since it was reported, leads me to place it in the table. 
108 is a very doubtful case, but not sufficiently so to 
nullify Fellows' claim that it is an instance of Fried- 
reich's ataxic. The report is most incomplete. 109 
(McAlister's) is a case included in this class by Bury, 
but of which an exceedingly imperfect account is pub- 74 
GRIFFITH, 
lished. Descroizilles' patient (m) had some symptoms 
much like disseminated sclerosis, though it very proba- 
bly belongs to Friedreich's ataxia. Bury hesitates to 
call 2 of his own cases (114-115) instances of this 
affection, on account of the absence of a history of 
heredity. They seem in other respects to be good ex- 
amples of it. Erlicki's and Bybalkin's patient (117) 
has already been referred to under " Pathological Anat- 
omy." Friedreich's ataxia. 
75 
Table II.-Additional List, arranged Chronologically. 
A. Reputed cases, related by blood to those in Table I., but not under professional 
observation. 
Reporter. 
1 No. 
Relationship to cases in Table I. 
Carre, 
17 
Grandmother, mother, 8 uncles and aunts, 6 brothers and sisters and a cousin 
said to have similar symptoms. 
Bradbury, 
2 
Mother and brother similarly affected. 
Carpenter, 
1 
A third member of the family later became ataxic. 
Kellogg, 
3 + 
A brother, aged 5-6, and several cousins ataxic. 
Dreschfeld, 
2 
A brother attacked at 19, and a sister at 21 years of age. 
Seeligmiiller, 
1 
A sister, aged 21, possibly affected. 
Brousse, 
1 
The mother had the same disease. 
Power, 
2 
A brother attacked at 15, and a sister at 17. 
Riitimeyer, 
3 
Great-great-great-grandfather said to have been ataxic; 2 brothers of Case 54 
showed the first symptoms in their 4th year. 
Musso, 
3 
Possibly the great-uncle had the same disease ; 2 brothers of Case b4 were 
certainly affected. 
Botkin, 
Ormerod, 
2 
Father and a brother said to have had similar svmptoms. 
2 
Grandfather of Cases bq-73 reputed ataxic. Brother of Cases 74-75 attacked at 
age of 13 
Fowler, 
1 
Father said to have had a peculiar gait; possibly the same disease. 
Fazio, 
2 
2 brothers with the same disease. 
Vizioli, 
1 
A sister of Case qi died at 5 years of age with all the symptoms of Friedreich's 
ataxia. 
Seguin, 
1 
A sister attacked at 15 years of age. 
Smith, 
1 
A sister attacked at 6-7 years of age. 
Fellows, 
2 + 
Several members of the family are said to be similarly,affected. 
Bury, 
1 
Grandfather of Cases 112-113 probably had an ataxic gait, and may have had 
the same disease. 
Stintzing, 
1 
A brother died at 31 with the same disease from which the others of the family 
suffer. 
Mastin, 
3 
2 uncles attacked at 15 and 12-14; and a cousin at 9 years of age. 
Mendel, 
1 
A brother died at 4% with symptoms of Friedreich's ataxia. 
Shattuck, 
2 
A brother attacked at 7, and a sister at 5-6 years of age. 
Joffrov, 
1 
A sister affected at 12. 
Gri filth, 
1 
57 + 
A brother of Case 143 died at 9 with symptoms of the disease. 
B. Possible and probable cases; under observation and reported, but without 
description. 
Reporter. 
No. 
Eulenburg, 
3 
Mentions a case of "locomotor ataxia" under 10 years of age, and 2 under 20 ; 
both illustrating heredity. Are very possibly instances of Friedreich's 
ataxia, as the author does not distinguish this affection from tabes. 
Duchenne, 
3 
Says he saw with Trousseau 3 brothers attacked by ataxia. 
Vulpian, 
? 
Says he has seen cases analogous to those mentioned by Duchenne. 
Schultze, 
i 
Reported a case briefly before the Wanderversammlung der Stidwestdeutschen 
Neurolog. u. Irrenarzte; but I can find no description of it. 
Putnam, 
2 
Saw 2 separate cases which he believes to be of this type, though no others of 
the family were affected. 
Goodhardt, 
2 
Saw an ataxic child with an ataxic father. No detailed description. They 
may be instances of Friedreich's ataxia. 
Ormerod, 
4 
2 cases of this disease, brothers, were shown him by Dr. Hughlings Jackson. 
Also saw a sporadic case under the care of Dr. Jackson, and another under 
that of Dr. Gee. 
C. 1 oo doubtful cases; claimed as cases by those reporting them, or by others, 
but probably without good reason. 
Reporter. 
No. 
Bouchut, 
2 
A boy of 11 and a girl of 14 are reported by the author as examples of loco- 
motor ataxia. They are occasionally supposed to be Friedreich's ataxia, 
but are very properly rejected by nearly every writer on the disease. 
Hitzig, 
3 
The author rather intimates that these cases belong to progressive muscular 
atrophy. Kahler and Pick suggest that the disease may have been Fried- 
reich's ataxia, but it does not seem clear on what grounds, except that the 
mother was tabetic. 
Fellows, 
1 
A case described as Friedreich's ataxia, but without, I think, any claim to be 
considered an instance of it. 76 
GRIFFITH, 
Bibliography of Reported Cases, and of Table II. A. 
Friedreich, Virchow's Archiv, 1863, xxvi. 391, 443; xxvii. 1; 1876, 
Ixviii. 145; 1877, Ixx. 140. Autopsy, Case 2, reported by 
Schultze, Virchow's Archiv, 1880, Ixxix. 132. 
Carre, De 1'Ataxie Locomotrice Progressive, 1865, 65, 248. 
Bradbury, British Medical Journal, 1871, ii. 499. 
Carpenter, Lancet, 1871, ii. 779. See also Gowers, Trans. Clin. Soc., 
1881, xiv. 4. 
Kellogg, Archives of Electrol. and Neurology, 1875, ii. 182. 
Dreschfeld, Liverpool and Manchester Hospital Reports, 1876, 93. 
Kahler and Pick, Archiv fur Psychiat. und Nervenkrankh., 1878, viii. 
251. 
Schmid, Correspondenzblatt fiir Schweizer Aerzte, 1880, 97. 
Seeligmuller, Archiv fiir Psychiat. und Nervenkrankh., 1880, x. 222. 
Hollis, British Med. Journal, 1880, ii. 167. 
Gowers, Transactions Clin. Society, London, 1881, xiv. 1. Also Diseases 
of the Nervous System, Eng. ed., 1886, i. 353. Autopsy, Case ■?/, by 
Pitt, Guy's Hospital Reports, 1887, xliv, 373. 
Brousse, De 1'Ataxie Hereditaire, 1882, 68. 
Hammond, Journal of Nervous Diseases and Mental Disorders, 1882, 
485. 
Coleman, ibid. Also through personal communication from Dr. W. 
Everett Smith. 
Warren, ibid. Also personal communication. See Case histories, this 
article. 
Leubuscher, Berliner klinische Wochenschr., 1882, 590. 
Power, St. Bartholomew's Hospital Reports, 1882, 305. 
Rutimeyer, Virchow's Archiv, 1883, xci. 106; 1887, ex. 215. 2 cases 
also seen by 
Quincke, Virchow's Archiv, 1876, Ixviii. 164. 
Jakubowitsch, Jeschened klin. Gas., 1883, No. 14. Abstract in Jahr- 
buch fiir Kinderheilkunde, 1883, xx. 510. 
Erlenmeyer, Centralblatt fiir Nervenheilk., 1883, vi. 385. Translated 
by Ohmann-Dumesnil in Alienist and Neurologist, 1884, v. 449. 
Walle, Correspondenzbl. fiir Schweiz. Aerzte, 1884, xiv. 33. 
Teissier, Lyon Medical, 1884, xlvi. 45. The second case reported in 
full by 
Cuche, Maladie de Friedreich. These de Lyon. 1887. 
Musso, Rivista Clinica di Bologna, 1884, iv. 865. 
Massalongo, Rivista Veneta di Scienze Mediche, 1884, 393. 
Charcot, Gazette des Hopitaux, 1884, 393. 2d case [/2/J in Gazette 
des Hopitaux, 1887, 413, 463. Also in Progres Medical, 1887, 453. 
Botkin, Med. Obozr. Mosk., 1885, xxiii. 32. Friedreich's ataxia. 
77 
Ormerod, Medico-Chirurgical Transactions, 1885, Ixviii. 147. Later cases 
reported in Brain, 1888, 461. 
Buzzard, Proceedings of the Royal Medical and Chirurgical Society of 
London, 1885, 347. 
Fowler, ibid., 1885, 348. 
Fazio, Rivista Clinica e Terapeutica, 1885, vii. 73. 
Vizioli, Giurnale di Neuropatologia, 1885, 1, 145. Also Neurolog. Cen- 
tralbl., 1886, 111. 
Palma, Relazione delle Cure termo-ininerali del Gurgitello practicata in 
Napoli .... durante la state del 1884. Reported also in full by Vizioli. 
Seguin, N. Y. Medical Record, June 13, 1885, 645. Also Boston Med. 
and Surg. Journal, Oct. 15, 1885, 379. 
Sinkler, Philadelphia Medical News, July 4, 1885. Later cases in 
Transactions of College of Physicians of Philadelphia, 1888. 
Smith, Boston Med. and Surg. Journal, Oct. 15, 1885, 361. Also personal 
communication. 
Putnam, ibid., 367. Also personal communication. 
Prince, ibid., 371. Also personal communication. 
Fellows, The Clinique [Chicago], 1886, 1. 
McAlister, British Medical Journal, 1886, i. 19. 
Fagge, Practice of Medicine, 1886, i. 480. 
Descroizilles, Progres Medical, 1886, 569. 
Bury, Brain, July, 1886, ix. 145. 
Galassi, Bullettino della Societa Lancisiana degli Ospedali di Roma, 
Aug. 1886, 57. 
Erlicki and Rybalkin, Archiv fur Psychiat. und Nervenkr., 1886, 693. 
Glynn, Liverpool Medico-Chirurgical Journal, Jan. 1887, 191. 
Freyer, Berliner klin. Wochenschrift, 1887, 91. 
Blocq, Archiv de Neurologic, 1887, xiii. 217. 
Stintzing, Miinchener med. Wochenschrift, 1887, 389. 
Ferrier, British Med. Journal, 1887, i. 1214. 
Mastin, Phila. Medical News, Aug. 27, 1887, 225. 
Mendel, Berliner klin. Wochenschrift, 1887, 779. 
Shattuck, Boston Med. and Surg. Journal, Feb. 16, 1888, 168. Also 
personal communication. 
Joffroy, Bulletin Medical, 1888, 247. Also Gazette Hebdomadaire, 
1888, 149. 
Osler, Transactions of the College of Physicians of Philadelphia, 1888. 
Bibliography of Table II. B and C. Arranged Chronologically. 
B. 
Eulenburg, Lehrbuch der Nervenkrankheiten, 1878, ii. 459. 
Vulpian, Maladies du Systeme Nerveux, 1879, 245. 
Duchenne, ibid. 78 
Friedreich's ataxia. 
Schultze, Neurolog. Centralblatt, 1883, ii. 290. Also Archiv fur Psychi- 
atric und Nervenkr., 1884, xv. 263. 
Putnam, Boston Med. and Surg. Journal, Oct. 15, 1885, 367. 
Goodhardt, Diseases of Children, 1885, 455. 
Ormerod, Brain, January, 1888, 461 
c. 
Bouchut, Gazette des Hopitaux, 1874, No. 38, 297. 
Hitzig, Berliner klin. Wochenschrift, 1875, 143. 
Fellows, The Clinique [Chicago], January, 1886. 
Bibliography of Reviews and Articles not containing Cases. 
Arranged Alphabetically. 
Bianchi, Gazzetta degli Ospitali, 1882, No. 99. 
Bourneville, Nouvelle Etude sur Quelques Points de la Sclerose en 
Plaques Disseminees, 1869, 211. 
Bristowe, System of Medicine, 6th ed., 1059. 
Editorial, British Medical Journal, 1883, i. 627. 
Erb, Ziemssen's Handb. der Spec. Patholog. und Therapie, 1878, B. xi. 
H. 2, Abt. 2, 194. 
F. W., Philadelphia Medical Times, 1882-3, xiii. 372. 
Fere, Progres Medical, 1882, x. 890. 
Grasset, Traite Pratique des Maladies du Systeme Nerveux, 1881, 321. 
Longuet, L'Union Medicale, 1884, xxxvii. 877. 
Mobius, Volkmann's Sainmlungen klin. Vortriige, 1879, No. 171, 1507. 
Also Schmidt's Jahrbiicher, 1884, B. cciv. 24. 
Ormerod, Brain, 1884, 105. 
Raymond, Dictionnaire Encyclopedique des Sciences Medicales, 1885, 
xv. 397. 
Seeligmuller, Gerhardt's Handb. der Kinderkrankh., 1880, B. vi. Abt. 
i. 2. Halfte, 187. 
Sepilli, Rivista Sperimentale di Frenatria, 1883, 335. 
Smith, Boston Med. and Surg. Journal, 1888, i. 627. 
Topinard, L'Ataxie Locomotrice, 1864. INDEX. 
ACUTE diseases, accelerating progress, 
28 
precipitating onset, 24 
Age, influence of, 23 
Albuminuria, 54 
Alcohol (see Intemperance). 
Anaesthesia, 44 
Ataxia, of arms and hands, 26, 30, 33 
of head, 27, 33 
of legs, 26, 29, 33 
of speech, 26, 45 
of station, 30 
of trunk, 27, 34 
locomotor (see Tabes), 
motor, 29 
relation to closing of eyes, 29, 30, 
31, 32 
static, 32, 33 
Ataxic paraplegia, distinct from, 66, 68 
Atrophy of muscles, 27, 41 
Authors consulted, 3 
Autopsies reported bv different authors, 
55 
Cord, spinal, peripheral degeneration, 59 
anterior pyramidal tracts, 60 
columns of Clarke, 60 
other parts of gray matter, 60 
central spinal canal, 61 
meningitis, 56 
Cutaneous sensibility, 44 
Cyanosis, 42 
DANA'S case, 3 
Death, causes of, 70 
Definition, author's, 2 
Friedreich's, 1 
Deglutition, 47 
Diagnosis, 67 
from ataxic paraplegia, 68 
from cerebellar tumor, 69 
from disseminated sclerosis, 68 
from hereditary chorea, 69 
from tabes, 67 
Diplopia, 48 
Disease, course of, 25 
Diseases, other, influence of (see Acute 
Diseases), 24, 28 
Disseminated sclerosis, distinction from, 
62, 68 
Duration, 69 
Dyspnoea, 54 
Bed-sores, 42 
Bibliography, 3, 76 
Bladder, 52 
Blepharospasm, 48 
Bowels (see Rectum), 52 
Brain, morbid changes, 56 
ECLAMPSIA (see Convulsions), 25 
Electrical reactions, 39 
Etiology, 18 
Eyes, 30, 48 
Eyesight, 50 
CASES exhibited by author, 4 
fatal (see Death), 70 
note on Dana's and Gibney's, 3 
reported to author by Warren, 12 
said to have existed in other chil- 
dren of family, 21 
system of tabulating, 4 
Cerebellar tumor, distinct from, 63, 69 
Cerebral symptoms, 45 
Chilblains, 42 
Chorea, hereditary, distinct from, 69 
Choreiform movements, 26, 34 
Clinical history, 25 
Clonus, ankle (see Reflexes), 41 
Coldness of body, 42 
Consanguinity, influence on heredity, 
21 
Contractures, 25, 38, 39 
Convulsions, 25, 51 
Cord, spinal, changes in, 56, 57 
posterior columns, 57 
lateral pyramidal tracts, 58 
direct cerebellar tracts, 59 
FACE, involvement of, 47, 48 
Families, number of affected, 17 
Fingers, flexion of (see Contractures), 
39 , 
Flushings, 42 
GAIT, varieties of, 29 
Gibney's case, 3 
Girdle pains, 45 
Grasp, claw-like, 31 
HEAD, ataxia of, 27, 33 
Headache (see Pain). 
Hearing, 50 
Heart, palpitation of, 25, 54 
praecordial anxiety, 54 
Heredity, cases with absence of any 
trace of, 22 
conclusions, 23 80 
INDEX. 
Heredity, direct similar, 18 
polymorphic, 19 
neuropathies, 19 
intemperance, 20 
tuberculosis, 20 
syphilis, 21 
consanguinity, 21 
other causes in ancestors, 21 
suspicious conditions in other 
children, 21 
Histology, 62 
History, clinical, 25 
of disease in the literature, 15 
Hypertrophy of tissues, 42 
Hysterical attacks, 25 
Pulse, acceleration of, 54 
Pupils, movements of, 49 
Rectum, 52 
Reflexes, 40 
Romberg's symptom (see also Ataxia 
and Muscle sense), 30, 32 
SALIVA, inability to retain, 47 
Salivation, 54 
Secretory symptoms, 52, 54 
Sensibility, cutaneous, 44 
Sensory symptoms, 43, 66 
Sex, influence of, 24 
Sexual disorders, 53 
Spasm, 35 
Speech, affection of, 26, 45, 66 
Spine, curvature of, 25, 38 
Stomach, disturbances of, 25, 54 
Strabismus, 48 
Sweating, profuse, 54 
Symptoms, rapidity of progress of, 26 
unusual early ones, 25 
bulbar, 45, 66 
cerebral, 45 
motor, 29 
secretory, 52 
sensory, 43, 66 
trophic, 41, 42 
vaso-motor, 41 
visceral, 52 
Syphilis, influence on heredity, 21 
IMPOTENCE, 53 
1 Intellect, 50 
Intemperance, influence on heredity, 
20 
jj£NEE-JERK (see Reflexes), 40 
Mastication, 47 
Medulla, changes in, 56 
Meningitis, 56 
Menstruation, disorders of, 53 
Mind (see Intellect), 50 
Muscles, atrophy of, 27, 41 
Muscle sense (see also Romberg's Symp- 
tom), 31, 45 
NAME, various names applied, 16 
title preferred, 17 
Nerves, morbid changes in, 61 
Neuropathies, influence on heredity, 
19 
Nystagmus, 48 
ataxic, 49 
static, 49 
TABES, distinct from, 62, 67 
Table I. follows page 71 
remarks on, 72 
Table II., 75 
Tables, explanation of, 4 
Talipes, 38 
Toes, flexion of (see Contractures), 39 
Tongue, involvement of, 47 
Treatment, 71 
Tremor, 34 
of tongue, 47 
Trophic symptoms, 41, 42 
Trunk, ataxia of, 27, 34 
Q7DEMA, 42 
J Optic nerve, atrophy of, 50 
PARESTHESIA, 45 
Palpitation (see Heart). 
Pain, 25, 43 
Paralysis, 27, 36 
Pathological anatomy, 54 
Pathology, 62 
of bulbar symptoms, 66 
of sensory symptoms, 66 
primary lesion. 63 
foetal arrest of development, 64 
combined systemic degeneration, 64 
Perspiration (see Sweating). 
Praecordial anxiety, 54 
Prognosis, 70 
Ptosis, 48 
URINE, polyuria, 54 
albuminuria, 54 
Urination, disturbances of, 52 
VASO-MOTOR symptoms, 42 
Vertigo, 25, 51 
Visceral symptoms, 52, 54 
Vision, 50 
Vomiting (see Stomach). 
WALK, inability to, 36, 37 
relation to paralysis (see Gait), 36 
Warren's cases, 12