PROGRESSIVE LOCOMOTOR ATAXY. 
BY 
S. 0. YANDERPOEL, M. D., 
ALBANY. 
Reprinted from the Transactions of the New York State Medical Society, 1865 
ALBANY: 
YAN BENTHUYSEN’S STEAM PRINTING HOUSE. 
1866. PROGRESSIVE LOCOMOTOR ATAXY. 
In October of last year, Mr. V , a gentleman engaged in business in 
the city of New York, entered my office; his step was jerking and irreg- 
ular, and his countenance indicated prolonged suffering. He came, he 
stated, to seek relief from severe pain which he was suffering at irregular 
intervals, principally confined to the lower extremities; though at times 
encircling the lower part of the abdomen as a constricted band'; also, that 
the lower extremities were partially paralyzed, as evinced by an almost 
total loss of sensation, and irregularity of motion of those parts. 
As my acquaintance with him was limited, inquiry was made as to his 
previous history, and the opinion expressed of his case by his physician 
in New York—a gentleman of some eminence in the profession. His 
replies, as to the early period of his affection, are condensed as follows: 
The first indication of any trouble in the feet was about two years since, 
when he found himself daily taking a seat in an omnibus or car, instead 
of the usual walk to business, but thought little of it at the time; soon he 
could not sit comfortably with the feet resting on the ground, having an 
inclination to raise them; this feeling continued until he always sat with 
the feet on a chair before him, and the knees raised. About this time he 
began to feel a heaviness in the soles of the feet, and a want of sensation, 
which was followed by a prickling, tingling feeling. In walking he invari- 
ably stopped at a street crossing, and hesitated which foot to use in step- 
ping off or on the curb; and if a hydrant or lamp-post were near, always 
put out his hand to assist himself. From that time he always used a heavy 
cane. The heavy numb feeling now extended to the legs, and soon after 
entering the house he found the sofa or bed the most convenient place. He 
experienced trouble in washing, as when leaning over, and happening for 
the moment to close the eyes, found himself staggering backward and 
forward, not being able to keep his balance. He also had trouble in going 
about in the dark, and suffered from pain in the legs and joints, always 
wanting to see his feet when he had occasion to use them. The opinion 
expressed by his physician in New York, he stated, was, that it was 
considered the commencement of paralysis, which must be gradually pro- 
gressive. Recognizing that the tableau of symptoms presented, in no way 
answered to this diagnosis, I instituted a closer scrutiny. The intellect 
showed no impairment, nor were there any indications which induced me 
to suppose this condition imminent, as would be were it progressive para- 
lysis. His sleep was sometimes broken from sudden and sometimes pro- 
longed pains in different parts of the body, chiefly, however, in the lower 
extremities. Vision had not been affected, either from temporary paralysis 
of the muscles of the eye or of the retina itself. The other bodily functions 
were normal or nearly so. 4 
In order to test whether the supposed paralysis were truly such, or an 
anaesthesia of the parts merely, I requested him to be seated, and taking 
hold of the foot directed him to resist flexion and extension of the limb. 
There was no diminution of the muscular force. I then bade him stand 
upright, with the feet placed closely together, and close the eyes; the 
instant he did so the equilibrium was lost, and he would fall unless the 
eyes were promptly re-opened. 
I told him I considered his malady “ Progressive locomotor ataxy,” or 
more definitely a lack of muscular co-ordination. 
While I gave him a change in the signification of his malady, it was not 
accompanied with any more encouraging views as to the result of any 
treatment thus far suggested for its cure. Pathologists placing it in the 
family of neuroses such as chorea and epilepsy, have administered the 
treatment recogmized as proper in those affections. Prominent among the 
remedies for which, has been the prolonged administration of nitrate of 
silver. Little encouragement having thus far followed these efforts, I 
prescribed a scruple of bromide of potassium in two drachms of comp, 
tinct. bark, three times daily. Its prolonged use was favorable in alleviat- 
ing to a great degree the migratory pains, and restoring somewhat the 
co-ordination of the muscles. 
A severe attack of pneumonia which has awakened the softening of 
latent tubercles has suspended all treatment adapted to the ataxy proper. 
Remarks.—Progressive locomotor ataxy, though by no means a new 
malady, has been but recently described as a distinct and recognizable 
affection. True, many of its symptoms have been often noticed, under the 
denomination of tabes dorsalis, but with this were included many other 
conditions and diseases of the spinal cord. Dr. Todd, in the enclyclopedia 
of anatomy and physiology (article “ nervous system,”) refers to the two 
varieties of paralysis of the lower extremities ; the one consisting simply 
in the impairment or loss of voluntary motion ; the other distinguished by 
a diminution or total absence of the power of co-ordinating movements, 
while considerable muscular power remained ; the patient experiencing 
great difficulty in walking, and his gait being so tottering and uncertain, 
that his center of gravity was easily displaced. Though this description 
is faithful as far as it goes, he still fails to recognize the true character of 
the malady, classing it as a paralysis, and ignorant of its history and 
distinctive progress. 
The credit is due Dr. Duchenne of Boulogne, in 1858, of first calling 
the attention of the profession to it as a distinct malady. As Bright 
in acute desquamative nephritis, so Duchenne in this malady, has presented 
so full and accurate a picture, that later descriptions merely portray the 
fuller shadings. And as in the former, the malady is even yet known 
chiefly as “ Bright’s disease,” so enthusiastic admirers have applied to the 
latter the name “ Maladie du Duchenne.” 
Dr. Duchenne defines it a malady essentially chronic, characterized 
specially by progressive abolition of the co-ordination of voluntary move- 
ments, simulating a paralysis with which it contrasts by the integrity of 
the muscular force.” 
From the limited information which must still exist of this interesting malady, it has seemed proper to give as brief a resume as possible of 
some of its most prominent features, at the same time that it may easily 
be recognizable. 
The pains are peculiar, as they are also one of the most constant pre- 
cursors. They are usually intense, coming and going with lightning rapid- 
ity ; again they may be more prolonged, lasting half a minute, and repeat- 
ing themselves ten or twenty times an hour. 
They return by accessions several times a year, without any appreciable 
cause, or again without any save variations in temperature. Authors, 
under the titles of “ General Neuralgias” or “ Neuralgic Rheumatism,” de- 
scribe pains which are but the precursors of Progressive Locomotor Ataxy. 
Among nervous accidents, observed about this period, are nocturnal in- 
continence of urine, and especially spermatorrhea—indeed half the cases 
thus far noted have been subject since puberty to spermatorrhea. Strange 
as it may appear, the opposite condition, which is sometimes observed to 
precede the progressive paralysis of the insane, also occurs, viz : The 
singular faculty of being able to repeat the sexual act, a great number of 
times within a short period. Yet cases occur wheie its repetition eight 
and ten times in the twenty-four hours is noted ; probably this false ap- 
pearance of exaggerated virility is a morbid state which is allied to the 
medullary excitation, the cause of the involuntary seminal emissions, and 
incontinence of urine. 
In this first stage of the malady, and before the want of co-ordination 
in the muscles is apparent, there generally occur paralyses; some transi- 
tory in character, others more .persistent. Among the first is facial para- 
lysis—or again a hemiplegia, which comes on without precursory symp- 
tom, without any trouble of the intelligence, or even momentary loss of 
consciousness, and yields in a few days, which excludes all idea of cerebral 
hemorrhage, or cerebral softening. Also paralysis of the tongue, which 
often disappears as rapidly as it came. 
Paralyses more persistent, are also precursors. The more common are 
those which affect vision. Thus a paralysis of the nerve of the external 
motor oculi, induces convergent strabismus—or again the nerve of the 
common motor oculi, inducing divergent strabismus, and falling of the 
eye-lid. The duration of these forms of paralysis is very variable, with 
some remaining through life, others only a few days, weeks or months. 
Various other forms of local paralysis are noted, but not of sufficient fre- 
quence to call for notice in this paper. 
Another train of precursory symptoms, is a sensation of constriction in 
different parts of the body. The individual complains that his chest is 
constricted as by an India-rubber band ; sometimes their arms and legs, 
seem bound. They think their clothes too tight; or a sensation as a girdle 
bound about the abdomen. 
Soon after the foregoing symptoms, those pathognomic of the affection 
present themselves. This defect of co-ordination in the voluntary move- 
ments, is little marked in the beginning, but augments progressively as 
the malady increases; at first almost exclusively confined to the inferior 
members—the superior are implicated only in the last stage of the ataxy. 
In the lower extremities, the want of co-ordination in the voluntary 6 
movements, is shown at first by light oscillations of the body, of which 
the individual is scarcely conscious. We can establish however that these 
oscillations are due to the fact that certain muscles of the lower extremi- 
ties contract strongly or quickly in rapid succession. As the malady be- 
comes more confirmed, the defect in co-ordination is shown by the difficulty 
shown to preserve an equilibrium when standing, or walking. This diffi- 
culty may extend to an entire impossibility to do either, unless the sense 
of sight comes to aid the disordered movements. 
When those affected rise to walk, especially if they have been for some 
time seated, they do so with difficulty and a sensation of stiffness, accom- 
panied by a pain more or less severe in the limbs, extending to the lumbar 
region. In order to rise they are obliged to lean upon a cane, or upon the 
arm of a chair. Once standing, they make great efforts to maintain them- 
selves; they totter, and perceiving the muscles do not obey the will, they 
fear falling, and if they have nothing to sustain themselves, their arms fly 
instinctively from the body, a little forward, oscillating much as a tightrope 
walker without his balance pole. This want of equilibrium, more marked 
in the first steps, is just as apparent when the individual desires to change 
the direction of his course, or especially to return on his footsteps. It is 
very sensibly diminished if he uses a cane, or leans upon the arm of 
another. 
Once started upon a course, their step, at first slow, becomes involun- 
tarily accelerated ; it is uncertain, hesitating, and as one intoxicated. 
Instead of the ordinary measured flexion of the knee, it is jerked, with a 
forced extension. The feet thrown out right and left, are pushed forward 
as if sent by a spring, and fall back, the heel first striking. In noticing 
this difficult and uncertain walk, it would seem the individual would not 
progress far without exhaustion—yet upon level ground they may walk 
miles, and fatigue walkers with whom there is no nervous affection. In 
later stages, exercise soon exhausts the forces, and after a short distance 
the individual is out of breath, and bathed in perspiration. 
The ataxic difficulty rarely extends to the upper extremities; when it 
docs it is usually at a late period of the malady, and develops gradually 
as in the lower. The aid of vision is even more necessary to guide the 
irregular action of the muscles than in the lower extremities. 
Spasmodic contraction of the muscles, at first scarcely noticeable, 
increases in severity with the progress of the disease. They appear not 
only when the will commands a regular movement, but also in a state of 
repose. These muscular spasms in the latter case consist in energetic 
shocks of the affected limbs, and have a great symptomatic value. The 
sick persons say that in walking, and even when standing quietly, the 
ground seems suddenly to sink under them. When lying we see their 
limbs tremble, sometimes violently. If the thigh is seized the muscles 
tremble under the restraint, while the foot moves with remarkable celerity, 
even against the will of the individual. 
As the disease arrives at its extreme period, the anaesthesia becomes 
more and more pronounced, and more generalized in different parts of the 
body. The feet scarcely recognize the ground on which they travel, and 
when to the cutaneous insensibility is added the muscular osseous and 7 
articular anaesthesia, the individual has no consciousness of standing, or 
of the resistance of the ground upon which he may stand with con- 
tracted muscles—closing the eyes, he feels as if suspended in mid-air. 
This anaesthesia may even extend to the mucous membranes, losing taste 
and smell, or the sensation of heat and cold. 
And yet this anaesthesia, marked as it is, and present in the majority of 
cases, can only be regarded as an epi-phenomenon of the malady. It can 
not be regarded as essential to the disease, for many cases have been 
noticed where it was wholly absent. 
In the latter stages of the malady the temporary paralysis affecting the 
different muscles of the eye usually returns and becomes permanent. 
Despite the grave symptoms thus portrayed, individuals affected with 
progressive locomotor ataxy preserve the integrity of their mental facul- 
ties. Their digestive functions are also regularly accomplished; the 
assimilative functions suffer little. Finally, however, they become some- 
what emaciated, and, as with paraplegics, bed-sores form where parts 
are submitted to continual pressure, on account of this pressure, and the 
modification which the troubles of innervation cause to the parts. 
Diagnosis.—Locomotor ataxy, as will be seen by the previous narrative, 
is not difficult to diagnose when fully established. Still there are certain 
symptoms which have a peculiar significance in the early stages. Thus 
the transitory paralysis of the apparatus of vision, and the singular 
character of the pains, are phenomena of the highest signification. These 
phenomena, taken separately, have a certain nature; taken together they 
are almost pathognomic. Thus, when an individual complains of expe- 
riencing at intervals lightning flashes of pain, erratic, manifesting them- 
selves sometimes in one part and sometimes in another; when also with 
this individual exists a paralysis of the third or sixth pair of cranial 
nerves, inducing a convergent or divergent strabismus, complicated with 
amblyopias or complete amaurosis ; when especially these accidents ap- 
pearing without appreciable . cause, have yielded of themselves, after a 
longer or shorter time, resisting in the mean time any and all treatment, 
we have a right to suspect that progressive locomotor ataxy is com- 
mencing. 
Disease of the cerebellum, arising from some congenital defect, is ac- 
companied by disorder of the muscular movement; but in this disease 
there is a peculiar gait; they rest and roll in walking as if giddy or drunk; 
there is nothing peculiar in the way in which the feet are planted. In 
ataxy, on the other hand, the gait is staggering and precipitate; the legs 
thrown about vaguely and spasmodically, and the heels brought down 
with force at each step. 
In general paralysis of the insane, the hesitation in speech, the tremu- 
lousness of the lips and tongue, the general tremulousness, the true para- 
lytu!r*weakness of the muscles as to voluntary movement, and the mental 
condition of the patient, must readily serve to prevent the unsteadiness 
of gait and other evidences of disordered co-ordinate movement from being 
confounded with those which occur in ataxy. 
In general spinal paralysis and in common paraplegia, there is true 
paralysis, more or less complete of the muscles as to voluntary power; 8 
and the muscles too are damaged as to their nutrition and contractility, 
and generally as to their sensibility. In the majority of cases formication 
is associated with numbness, and what has yet never been noticed with 
ataxy, the sensibility to difference of temperature is much impaired or 
altogether lost in the paralysed parts. Very generally there is tenderness 
in some part of the spine, and not unfrequently pain in the same region or 
in the paralysed parts; and if there be pain, it is as a rule, more like dull, 
rheumatic aching, than like the peculiar excruciating, stabbing, boring, 
nipping pangs of pain, flitting from one spot to another in a very erratic 
manner, and occurring in paroxysms of varying duration, which are rarely 
absent in ataxy. And in those cases where progression is possible, the 
gait is almost always sufficiently characteristic, not as has been shown to 
occur in ataxy but hampered and slow ; partly from want of voluntary 
power over the legs, and partly from the muscles becoming stiff in moving, 
each leg being brought forward with evident difficulty, even with the help 
of an upward hitch of the whole side of the body belonging to it, and the 
part of the foot first brought in contact with the ground, being as a rule 
not the heel but the toes. 
Chorea, considered in relation to the troubles in movements, which 
accompany it, is of all convulsive affections, that which approaches nearest 
to progressive locomotor ataxy. For when one affected with chorea 
wishes to execute determined movements, the perturbation of the locomotor 
functions are most energetically manifested ; the will, still sufficiently 
powerful to command muscular action, cannot control it, once that the 
impulse is given. The difference however is readily apparent. While the 
individual affected with progressive locomotor ataxy, no matter at what 
stage of the malady, can guard as long as he wishes absolute repose ; this 
repose is never as complete in chorea. The troubles in muscular action 
are shown by convulsive movement, more or less limited to certain parts 
of the body, more or less violent, and which really never cease save during 
sleep. It is these spasmodic contractions of the muscles of the lower 
extremities, which renders standing upright difficult—phenomena which 
take place independent of the will, and even in despite of it. 
The duration of locomotor ataxy is usually long ; the mean being from 
four to five years—it may be extended to ten, fifteen or twenty years. 
However long may be its duration the prognosis is none the less unfavor- 
able ; the fatal termination is most frequently hurried by inter-current 
affections, when the perturbation of the nervous system, in taking from the 
system the power of resistance to new morbid impressions, give to them 
peculiar gravity. 
Romberg, a German pathologist, and Luys, of Paris, who ranks as the 
leading microscopist of the nervous system, have carefully investigated 
the pathological anatomy. The dorso-lumbar region of the spinal cord, is 
the seat of the material lesions in muscular ataxy. These lesions occur in 
the posterior columns of the cord, and the roots which emerge from them. 
On opening the canal of the spinal cord, the dura mater presents a reddish 
tint, more or less injected, depending upon an augmentation of its normal 
vascularization, and this vascular injection extends to the most profound 9 
ramifications of the vessels. The dura mater is also somewhat thickened 
and ardematous. 
The pia mater participates in this anomalous vascularization, which 
becomes more pronounced as it approaches more the inferior part of the 
cord, and the posterior roots of the nerves, to which it adheres, so that in 
removing it, portions of the nervous substance are dragged away. 
The lesions of the cord itself consist sometimes in a sort of grey degenera- 
tion, sometimes to a translucid and gelatinous state ; in a diminution 
of consistence, or on the other hand an induration, called schlerosis. 
Ordinarily there is a sensible reduction in the volume of the posterior 
columns. In the posterior roots the same alterations march parallel to 
those in the cord. Topineul suggests, “ that this alteration of the roots 
is if not a consequence, at least a consecutive condition ; in no case 
anterior to disease of the cord, and that the alteration in the cord is the 
only constant lesion. 
And here arises a question of exceeding interest. If it is incontestable 
that the cerebellum is the seat of the faculty of co-ordinating movements; 
if as corollary, certain affections of this organ have for result a lack of 
this co-ordination ; on the other hand we have the clinical fact, that 
between the ataxy arising from disease of the cerebellum, and progressive 
locomotor ataxy, thei'e are remarkable points of dissimilarity. 
It is then in the cord, and a single portion of it that the lesion exists, 
and we seek therefore the reasons for the characteristic symptoms of the 
malady. But between these symptoms and the lesions there are at first 
sight singular contradictions. Locomotor ataxy being essentially charac- 
terized by troubles of motion, and loss of sensibility, entering into it as a 
secondary element, we should expect to find, after what we know of the 
distinct functions attributed to the different columns of the cord, the 
material lesions, not in the posterior columns, which according to the 
doctrine of Sir Charles Bell are the source of sensibility, but rather in the 
anterior columns, which are considered the place of origin of the motor- 
nerves. It is the inverse which we really see. 
This contradiction, however, is not as absolute as it appears, at least as 
to absence of lesion of the anterior columns. If these columns are intact, 
there is no paralysis, a phenomenon which is the consequence of their 
greater or less alteration. The troubles of motion are altogether a differ- 
ent order, and however exaggerated they may be, the muscular force in 
progressive locomotor ataxy (and this is the capital fact) is preserved. 
Treatment.—The multiplicity of remedies by turns employed, witnesses 
the infidelity and powerlessness of medicine in the malady. Nitrate of 
silver has probably been more extensively employed than any other remedy. 
The records of its use give no encouragement of success. In the case we 
have narrated, the salts of bromine have acted favorably in controlling the 
pains, and to a certain extent regulating the co-ordination of movement. 
[P. L. A.]