THE 
PERONEAL TYPE 
OF 
PROGRESSIVE MUSCULAR ATROPHA 
A 
THESIS FOR THE DEGREE OF M.D. IN THE UNIVERSITY OF CAMBRIDGE 
BY 
HOWARD H. TOOTH, M.A., M.D., M.R.C.P. 
of st. John’s college. 
ASSISTANT DEMONSTRATOR OF PHYSIOLOGY IN THE MEDICAL SCHOOL OF ST. 
Bartholomew’s hospital; assistant physician to the 
METROPOLITAN FREE HOSPITAL; LATH CASUALTY AND 
HOUSE PHYSICIAN TO ST. BARTHOLOMEW’S 
HOSPITAL. 
LONDON 
H. K. LEWIS, 13G GOWER STREET, W C. 
1836 PERONEAL TYPE 
OF 
PROGRESSIVE MUSCULAR ATROPHY. THE 
PEBONEAL TYPE 
OF 
PROGRESSIVE MUSCULAR ATROPHY 
A 
THESIS FOR THE DEGREE OF M.D. IN THE UNIVERSITY OF CAMBRIDGE. 
BY 
HOWARD H. M.A., M.D., M.R.C.P. 
of st. John’s college. 
ASSISTANT DBMONSTRATOR OF PHYSIOLOGY IN THE MEDICAL SCHOOL OF ST 
Bartholomew’s hospital; assistant physician to the 
METROPOLITAN FREE HOSPITAL; LATE CASUALTY AND 
HOUSE PHYSICIAN TO ST. BARTHOLOMEW’S 
HOSPITAL. 
LONDON 
II. K. LEWIS, 136 GOWER STREET, W.C. 
1886 London 
PRINTED BY H. K. LEWIS, 
136 Gower Street. THE PERONEAL TYPE 
OP 
PROGRESSIVE MUSCULAR ATROPHY. 
Few subjects in neurology have undergone more 
searching revision of late years than the great 
clinical division known as Progressive Muscular 
Atrophy. Since Cruveilhier, Aran, and Duchenne 
first recognised it, it has been divided into many 
groups, clinically resembling, but pathologically 
distinct from, one another. 
Such being the case it is necessary, before con- 
sidering the immediate subject of this essay, to pass 
in review the several types into which the disease 
has hitherto been resolved. 
The name progressive muscular atrophy was given 
to a classic group of cases now so well known as to 
render a description superfluous; but for the pur- 
poses of comparison, the following features must be 
noted. The commencement of the atrophy in the 
small hand muscles, thenar and hypothenar particu- 
larly, generally in the hand most used. The pro- 
gression of the wasting up the arm to the shoulders, 
down the back, and the comparatively late affection 
of the lower extremities. The age of the patient is 2 
Progressive Muscular Atrophy. 
generally advanced, but the disease may apparently 
begin at any time of life after twenty-one, almost 
never in childhood. Fibrillar tremors of the muscles 
are nearly always present. Contractures of certain 
muscles occur as late phenomena, giving rise to the 
well-known claw-shaped hand. The contractility to 
faradism is diminished in proportion to the wasting, 
but there is a tendency to the late development of 
polar alterations in the galvanic irritability, the so- 
called u reaction of degeneration.” This disease is 
very rarely, if ever, hereditary. The pathological 
changes at the seat of this disease are now generally 
admitted to consist of degeneration of the ganglion 
cells in the anterior horns of the spinal gray matter. 
It was for a long time considered by the early 
authorities to be an affection of the muscles, and not 
of the spinal cord. Cruveilhier it is true expected to 
find grave lesions of the central nervous system, 
and relates in telling language his astonishment at 
finding, in 1848, no disease of the cord as he had 
expected. However, in a subsequent case in 1853,* 
he found great thinning of the anterior roots, which 
we now understand to be secondary to disease of the 
anterior horns. 
So again Aran and Duchenne at first considered 
the disease to be a myopathy, till in 1870 Duchenne 
with Joffroyf found and described disease of the 
anterior horns. 
* Cruveilhier, Arch. Gen. de Med., 1853. 
f Duchenne and JofFroy, Arch, de Physiol., 1870, p. 449. Progressive Muscular Atrophy. 
3 
On the other hand Friedreich* as late as 1873 in 
his valuable monograph, takes up the opposite view; 
but then it must be noted that the cases he quotes 
in illustration of his views, in which he was able to 
make the autopsy, were none of them of the group 
now under consideration, but belong to types since 
shown to be myopathies, or else to the group of 
cases which form the subject of the thesis. 
Now the above rapid sketch of what we may call 
the myelopathic thenar type of progressive muscular 
atrophy, is one of the most common, and as atrophy 
is its most prominent symptom, a very large number 
of cases have been, as it were, labelled with this 
name, and assigned to this group. Hence it is that 
we find ip quite late writers, progressive muscular 
atrophy described as commencing at any age from 
three years upwards, often having no fibrillar 
tremors, and often distinctly hereditary. 
As regards heredity, it must be noted that 
Bamberger,f FriedreichJ and Leyden§ all hint at 
the tendency of the hereditary form to appear in the 
lower extremities. The subject is further compli- 
cated by the fact that apparently true myelopathies 
may commence in other than the thenar muscles. 
* Friedreich, Ueber Prog. Muskelatrophie, 1873, Berlin, 
f Bamberger, Oesterr. Z titling fur Prakt. Heilfc, 1860. Camtatt'i 
Jahresbericht, 1860, p 88. 
J Op. cit. 
§ Leyden, Kiin. dcr. Jiuckenmar/cs Krankheileu, 1875. 4 
Progressive Muscular Atrophy. 
For instance, the shoulder muscles,* the serratus 
magnus,t the armj and neck museles§ have been 
described as being the point of attack of an affection 
apparently myelopathic in origin. 
By the light of late obseryations aided by post- 
mortem examinations, the number of irregular or 
atypical cases is, however, becoming more and more 
narrowed. 
Leaving the myelopathic form of muscular atrophy, 
we come now to the great group of myopathies, or 
cases in which the nervous system, central and 
peripheral, has remained perfectly intact, the affec- 
tion being confined to the muscles only. 
At the head of this group we have the now classi- 
cal type of myosclerosis, familarly known as Pseudo- 
hypertrophic muscular paralysis. Duchenne considered 
this a pure myopathy, and nearly all subsequent 
authors agree with him, though a few have described 
changes in the spinal cord, which however cannot 
be considered primary.|| 
The most striking points to be noted in myo- 
sclerosis are the following. Apparent hypertrophy 
of muscles sometimes associated with considerable 
atrophy. The leg muscles are generally first 
* Schneevogt, Niederlandsch. Lancet, 1854. Canstatt's Jahresbericht. 
Duchenne, L'Electrisation localisee, 3e edit., p. 494 et. seq. 
f Duchenne, Ibid, p. 496. 
X Oppenheimer, Habililationschrift, Heidelberg, 1855. 
§ Erb and Schultze, Archiv fur Pstjchiatr., 1879, p. 369. 
|| Lockhart-Clarke, and Gowers, Med. Chir. Trans., 1874. Progressive Muscular Atrophy. 
5 
affected, but not necessarily always. Contractures 
of groups of muscles make their appearance sooner 
or later, particularly those of the calf leading to 
talipes equinus. Absence of fibrillar contractions. 
Absence of polar electrical changes, but of course 
diminution of faradic irritability in proportion to 
the destruction of muscular fibres. The disease is 
essentially one of childhood, though exceptions to 
this have been recorded lately by Drs. Percy Kidd* 
and Fowler.f Lastly there is a very distinct ten- 
dency to heredity. Here then is a type of myo- 
pathy, combining the conditions hypertrophy and 
atrophy. Had it not been that hypertrophy was so 
marked and constant a symptom, the disease would 
in all probability have been classed from the begin- 
ning as a peculiar form of progressive muscular 
atrophy. In fact this has actually been the case as 
will be seen in considering the following type. 
Closely allied to pseudo-hypertrophic paralysis 
is another form of myopathy, or rather probable 
myopathy, for as yet there are no accounts of 
post-mortem examinations forthcoming, except a 
doubtful case reported by of atrophy 
commencing in the neck and shoulder muscles, in 
which post-mortem there was found no disease of 
* Kidd, “ Pseudo-hypertrophic paral. in an adult,” St. Bartholomew’s 
Hospital Reports, 1881, p. 267. 
f Fowler, Clin. Soc. Trans., 1885. 
} Lichtheim,“ Progr. Muskelatroph. ohne Erkrankung der Vorderhorner 
des Riickenmarks,” Arch, fur Psychiatric, 1878. 6 
Progressive Muscular Atrophy. 
the anterior horns at all. This class of cases was 
differentiated from the general mass of atrophies by 
Erb* in 1*884, in a paper based on the clinical obser- 
vation of twenty cases. This type, which he calls 
the u juvenile form” of progressive muscular atrophy, 
has distinct clinical features, by which it is easy to 
distinguish many cases among those of the older 
authors The salient features of this type may be 
summarized as follows. The age at onset is often 
in childhood, but usually about the time of puberty. 
The muscles usually affected first are the shoulder 
girdle and upper back muscles, very late if ever the 
forearm and hand muscles. Lower extremities 
often affected, but generally late. Hypertrophy of 
single muscles is very common, usually the deltoids, 
supra and infra-spinati, triceps, teretes, tensor fascia? 
femoris, sartorius and calf muscles. There are never 
any fibrillar tremors, no reaction of degeneration, 
but a marked tendency to heredity. Though the 
diagnosis requires further confirmation by post- 
mortem examination, the disease is almost certainly 
closely allied to myosclerosis, and the very fre- 
quent occurrence of hypertrophy of single muscles 
lends colour to this view. 
Here then we have the picture of a myopathy in 
which atrophy of muscles is the predominant fea- 
ture, and hypertrophy an occasional one only, as 
* Erb, “Ueber die juvenile Form der Progr. Muskelatropb.,” Deutsche 
Archiv fur Klin. Med., Bd. xxxiv. Progressive Muscular Atrophy. 
7 
opposed to myosclerosis where these conditions are 
reversed. Cases which answer to the above descrip- 
tion occur frequently among the tables of collected 
cases by Waclismuth,* Robertsf and other writers, 
among which may be mentioned Aran, J Eulenburg§ 
and Zimmerlin.|j 
Another important group of myopathies remains 
to be noticed, and here our information concerning 
the pathology is more satisfactory. 
in 1855 fully recognised the fact that 
progressive muscular atrophy, when it occurs in 
childhood, is possessed of quite peculiar features. 
He calls this the “ infantile form” of progressive 
muscular atrophy. Though the disease is possessed 
of very marked distinctive features, it does not 
appear to have attracted any attention till 1874, 
when Landouzy** recorded the cases of two brothers 
suffering from this affection. MM. Landouzy and 
in 1885, give the further history of these 
cases, and some others, with the account of a post- 
mortem examination. It may be thought strange 
that a disease, the symptomatology of which was 
* Wachsmuth, Zeitschr. Jur Rationelle Medicin, 1855. 
f Roberts, “ On Wasting Palsy,’’ 1858. 
X Aran, Arch. Gen. de Med. 1850, (case 7). 
§ Eulenburg, Deutsche Klinik, 1856. 
|| Zimmerlin, Zeitschr.jur Klin. Med., Bd. 7. 
Duchenne, L'electrisation localisee, 3e edit., p. 518. 
*• Landouzy, Societe de Biologie, Juin 27, 1874. 
ft Landouzs and Dejerine, Rev. de Medecine, Feb. 1885. 8 
Progressive Muscular Atrophy. 
known in 1855, should not have its pathogeny 
investigated till thirty years after. 
This disease, as its name implies, is one of child- 
hood or early adolescence. It has a distinct point 
of attack, namely the muscles of expression. One of 
the earliest phenomena is a fixed expression of 
countenance, owing to the immobility of the mus- 
cles of the upper half of the face. Then the fore- 
head becomes smooth, and devoid of wrinkles, the 
eyes are never quite closed in sleep, and when the 
patient laughs his mouth extends laterally only, the 
rest of the face being unmoved (u rire en travers,y). 
The lips are everted and pouting, and the whole 
appearance is so striking as to deserve the name 
u facies myopathica ” given it by the French ob- 
servers. The muscles of mastication and deglutition 
are quite free. Next attacked are the shoulder and 
upper arm muscles, except the spinati and subscapu- 
laris. The extensors of the forearm, the thenar and 
hypothenar, and interossei, and also the lower limbs, 
are affected, but late in the course of the disease. 
No hypertrophy of any muscles has as yet been 
noticed. The faradic irritability is diminished in 
proportion to the wasting, and there is never any 
reaction of degeneration. There are no fibrillar 
muscular movements, but there is a tendency to 
contractures of certain muscles. There is in this 
disease a very decided tendency to heredity, as- 
oendant and collateral. Lastly, in the single case Progressive Muscular Atrophy. 
9 
in which MM. Landouzy and Dejerine were able to 
make an autopsy, the affection was found to be 
confined entirely to the muscles, the nervous sys- 
tem, peripheral as well as central, being absolutely 
free from disease. It may be here mentioned that 
Cruveilhier (loc. cit.) in 1853 describes two cases of 
general muscular atrophy, with affection of the face 
muscles, in which no disease of the spinal cord was 
found. Several well marked cases of this disease 
have been put on record, among which may be 
mentioned those of Remak,* Westphal,f Mossdorf,J 
Marie and Guinon,§ and and Douillet.|| 
I now proceed to describe the cases illustrating 
the form of progressive muscular atrophy, which it 
is the object of this dissertation to establish as a 
distinct type 
• Remak, Neurolog. Centralbl., No. 15, 1884. 
f Westphal, Berlin. Klin. Wochemchr., 1885, p. 617. 
J Mossdorf, Neurolog. Centralbl., No. 1, 1885. 
§ Marie and Guinon, Rev. de Med., Oct. 1885. 
|| Cenas and Douillet, La Loire Medicale, 1885, p. 170. 
U Since this thesis has been commenced, and some months after the 
lines, on which it was intended to work, had been laid down, there has 
appeared in the Rev. de Medecine for Feb. 1886, a paper by MM. Charcot 
and Marie on the same subject, illustrated by five cases. I take this 
opportunity at the same time, of acknowledging my indebtedness to M. 
Charcot for his able Revue Nosographique in the Progr. Medical, March 
1885, which first directed my attention to the subject of amyotrophy. 10 
Progressive Muscular Atrophy. 
Case I. Progressive atrophy of the peroneal and tibial muscles, 
with resulting talipes varus. No fibrillar muscular tremors. No 
great loss of faradic irritability, but reaction of degeneration in 
some of the affected muscles. 
Harold B,* aged 7 years and 8 months. At the age of 6 
years it was noticed that he dragged the left foot slightly, and 
that he began to walk on the outer part of the foot, in fact that 
a talipes varus was established, a condition which has been in- 
creasing ever since. It was also seen that this leg became 
smaller and weaker than the right. All this time he has been 
complaining of pains, which he cannot describe, in the legs, most 
in the morning when he gets up. He feels the cold in that leg 
very much. No tremors have been noticed nor do the legs 
jump at all at night. During the last 2 months the right leg 
has been getting thinner, and the same varus has been slowly 
making its appearance. 
Family history.—He is one of twins, his twin sister is per- 
fectly healthy and “ twice as big as he is.” He has two 
brothers, aged 17 and 15, quite strong and healthy. No history 
of a similar affection can be made out in any other member of 
the family on either side. Mother died of “cancer.” Father 
is alive and well. 
Present condition.—Small, pale, fragile looking child with an 
intelligent expression. His speech has been stuttering lately 
but he can whistle perfectly, has no deglutition trouble, the lips 
are not everted at all, and the tongue is protruded quite straight. 
Upper extremities.—Thin and feeble, but apparently only shar- 
ing in the general low state of nutrition. 
Trunk and spine natural. 
* This case is one of Dr. Ormerod's to whose kindness I am indebted 
for the use of it. Progressive Muscular Atrophy. 
11 
Lower extremities.—The gluteal region and upper part of the 
thighs seem to be of natural volume and consistency considering 
the general feeble development. 
Fig. 1.—From a Photograph.—The wasting being confined principally to the 
peronei and extensors, is more obvious on measurement than on a general view 
like this. The talipes varus is fairly well shown. 
The thighs at about the middle, measure each 11 in. in circum- 
ference. But 2 in. above the patella the left is slightly the 
smaller of the two, thus, L.=8f in., in. There is also 
some diminution in the circumference of the knee-joint, over 
the middle of the patella, thus, L.=8| in., R.=8f in. 
There is a lively knee reflex, but no clonus. 
Lower Leg.—Left calf fairly firm but smaller than right. 
Peronei, extensors and tibiales very flabby in the left side, less 
so in the right. Well marked pes varus of left foot and com- 
mencement of the same condition in the right. This varus is 
not due to contraction of opposing muscles but to simple loss of 
power in the peronei. The foot can easily be brought to its 
natural position, but then lies very flat. The circumferential 
measurements of the calves are L.=7f in., R.=8| in. Two 
inches above the ankles L.=4£ in., R.=5£ in. The left leg is 
very cold and rather livid, but sensation is perfect all over. 
There are no muscular tremors. All the muscles act well to 
faradism, but in the extensor longus digitorum, ACC=KCC. 12 
Progressive Muscular Atrophy. 
This is a fairly typical example of the class of 
cases now under consideration. The features worthy 
of emphasis are the age of the patient, the point of 
attack, and the electrical reactions. No fibrillar 
tremors were noticed, but they seem to be as often 
absent as present in this disease. 
Heredity in this and the succeeding case was not 
to be made out, but I shall hope to show from the 
accounts of recorded cases, that this is not by any 
means always the case. 
Case II.—Progressive atrophy commencing in the peronei presum- 
ably, but noiv most obvious in the right calf jnuscles, occasional 
involuntary jerking of the muscles of the thigh, some degenerative 
electrical changes, but no true reaction of degeneration. 
Kate E., aged 23, came to me at the Metropolitan Free Hos- 
pital with the history that she has been walking badly since she 
was fifteen years old. She has sprained her right ankle twice 
owing to the weakness of the right leg. During the last year 
this weakness has become more pronounced and the leg has 
wasted in the region of the calf very much. She has also felt a 
bruised feeling of the muscles, most, perhaps, in the thigh, and 
in the gluteal region over the site of the sciatic nerve, but there 
have been no actual pains. She says the muscles twitch occa- 
sionally, but no fibrillar tremors have been noticed. 
There is no history of any similar affection in any of her 
family. She is married and has had two children, one still-born 
at seven months, the other also premature but lived three 
hours. 
Present condition.—She is a healthy-looking young woman 
with a high colour and generally well-nourished appearance. Progressive Muscular Atrophy. 
13 
There is no wasting of face muscles or indeed of any muscles of 
the upper part of the body. The chest and heart yield no phy- 
sical signs of disease. 
Lower extremities.—Left.—Quite natural, of good firm consis- 
tence all over. Knee-jerk natural. 
Fig. 2. 
Fig. 3. 
Right.—Thigh muscles very flabby particularly the anterior, 
but she has good power of extension and still better of flexion, 
circumference of right thigh rather less than that of left. Knee- 
jerk very feeble on right side. 
Right lower leg.—Calf muscles extremely wasted, (Fig. 2) 
almost disappeared. Peronei, tibialis anticus, and extensor 
longus digitorum are all very flabby, but not so far gone as the 
calf muscles (Fig. 3). The small foot muscles do not seem to be 
much affected. She cannot stand on tip-toe on that leg at all, and 
the foot is very flat. Sensation is perfect, but the leg is always 
very cold, the contrast in this respect between this leg and the 
left is very striking. No fibrillar movements have been noticed. 14 
Progressive Muscular Atrophy. 
Measurements.—Middle of right calf =11 in. Left = 13| in. 
Eight ankle 2 in. above malleolus = 7f in. Left = 8| in. 
Electrical reactions.—To faradism the gastrocnemius will not 
react to the strongest currents. The peronei react fairly 
strongly, and so do the tibialis anticus and extensor longus 
digitorum. With the galvanic current the following results 
were obtained :—Gastrocnemius reacts to strong currents only, 
such as 40 milliamperes, and then only very feebly, hut 
the KCOACC. Tibialis anticus, minimal contraction = 9 ma. 
and here KCC nearly equals the ACC. Extensor longus digi- 
torum, KCC= 5 ma., ACC = 8 ma. Peroneus longus, KCC = 
4 ma., ACC = 6 ma. It was not considered safe to use very 
strong currents, as the patient was three months advanced in 
pregnancy. 
In this case the calf muscles are at present the seat of the 
most advanced changes, but the tendency to sprain the ankle as 
far back as the age of fifteen, suggests the possibility that the 
peroneal muscles may have been the earliest affected. 
In addition to these cases I have two others, for 
which I am indebted to Dr. Gee and Sir Dyce 
Duckworth, in whose wards they were. Unfortu- 
nately, though I have spared no pains, I have been 
quite unable to trace them after leaving the hospital. 
Though the notes are scanty, the similarity of these 
cases to the type now under consideration must be 
my excuse for reporting them at second hand. 
Case III.—Simon W., aged 49, a cigar-maker, was admitted 
into Luke Ward, St. Bartholomew’s Hospital, in April, 
1880. The notes are briefly these. At the age of 15 years his 
legs became weak and began to waste. This went on for 9 
years and then the hand muscles, began to waste (? which hand). 
For the last 18 years he has had a slowly increasing dorsal Progressive Muscular Atrophy. 
15 
angular curvature of the spine with some lateral deviation, which 
he attributes to carrying heavy weights, and is evidently not due 
to caries. There is no history of syphilis or lead poisoning. 
His mother had a similar affection of the legs. In walking, the 
toes drag. There is great atrophy of the tibialis anticus. The 
muscles of the calf are smaller than natural. There is no 
atrophy of any other muscles of the leg. Knee-jerk abolished. 
Upper extremities.—Great atrophy of thenar and hypothenar 
muscles and probably interossei. All the other arm muscles 
seem natural. Marked fibrillar contractions in arms and legs. 
The general resemblance of this to the preceding 
cases is apparent, but there are these important dif- 
ferences, the muscle of the leg first attacked is the 
tibialis anticus; the hand muscles are affected, which 
we shall see is a common incident in the later pro- 
gress of the disease; there are marked musculo- 
motor phenomena, and there is heredity. 
Case IV.—Edward M., aged 46, a bricklayer, was admitted 
into John Ward, St. Bartholomew’s Hospital, in June, 1884. 
He has had gradual wasting of the right leg 9 years. The leg 
is very weak and much colder than the left. There has been 
very little change for the last 8 years, but the difficulty in walk- 
ing on it increases slowly. He has occasional cramps in the 
leg. Three weeks before admission he had been suffering from 
rheumatism, which he said has settled in the affected limb. No 
heredity can be made out. Has always been temperate. 
Condition on admission.—Healthy looking man. Chest and 
upper extremities natural. 
Lower extremities.—Left natural. Right thigh natural. Right 
leg and foot much wasted. Muscles on outer and anterior aspect 
of leg, more wasted than calf muscles. Tibialis anticus much 
wasted. Fibrillar contractions occasionally seen. Sensation 16 
Progressive Muscular Atrophy. 
perfect. Eight leg much colder than left. Tendon reflexes 
normal. 
No reaction to faradism or galvanism in tibialis anticus and 
extensor longus digitorum, but peroneus longus, gastrocnemius 
and soleus act fairly well. Electro-sensibility lost over wasted 
muscles. 
I think that these cases afford some proof of the 
existence of a distinct type of “ progressive mus- 
cular atrophy,” commencing in the lower extremities, 
and from the tendency of the affection to attack 
first the peronei and extensors, it is proposed to call 
the disease the “peroneal type” of progressive mus- 
cular atrophy, a provisional term which does not 
involve any theory as to causation. 
It will not be out of place now to consider more 
in detail the several points on which the disease 
may be established as a separate type. In order to 
facilitate reference, there is appended to this essay 
a table of all the cases I have been able to collect, 
after rejecting many which were doubtful, owing to 
scanty reporting. 
In the first place must be noticed the age at which 
the disease appears. Including the four cases above 
recorded, there are available in all, thirty-nine cases, 
many of the same family, as in those reported by 
Eichorst (Table, 10), wdiere we have ten cases closely 
related to one another; as the age in some is only 
to be inferred, I have thought it better in this in- 
stance to give an average age for the whole family. Progressive Muscular Atrophy. 
17 
Arranging the ages then in quinquennial periods, the 
following results are obtained. 
A3 tat. 1-5, -10, -15, -20, -25, 37, 46. 
Cases. 8, 10, 8, 10, 1, 1, 1. 
By this will be seen that in thirty-six of the cases, 
the disease appears first at ages under twenty, and 
of these in twenty-six, or about sixty-six per cent., 
the onset of the disease occurs between the ages of 
two and fifteen, and though it is unsafe to draw 
definite conclusions from so small a number of 
cases, it is probable that these figures do not under 
state the case. The disease then is one of child- 
\ 
hood. 
Next as to sex, here we find twenty-eight males 
as opposed to sixteen females, the proportion of 
females being large compared with that in the 
myelopathic form, in which Roberts, for one, finds 
only fifteen female cases, out of a total of ninety- 
nine cases. 
In estimating the influence of heredity, the ar- 
rangement of the figures must be somewhat altered, 
for of the total number it will be seen that many 
belong to the same family, and a family from the 
point of view of heredity must be regarded as an 
individual. It would obviously lead to an over 
estimation of the hereditary influence if each member 
of a family were considered as a separate proof of its 
existence. Regarded then from this stand-point we 18 
Progressive Muscular Atrophy. 
have for consideration seventeen cases only, but of 
these a marked heredity exists in twelve. 
Even therefore after taking these precautionh 
against over-rating heredity, its influence is suffici- 
ently obvious. The term heredity is used here in its 
widest sense and perhaps somewhat loosely. For 
the present purpose the appearance of the same 
disease in two or more children of the same parents 
is regarded as heredity. Some authors, Eichorst 
for instance, do not consider this collateral form as 
true heredity, but it seems that for the purpose of 
clinical distinction this form of heredity is as valu- 
able as any other. Pure collateral heredity without 
any ascendent or descendent features, occurs in four 
or one-third of the above hereditary cases. (Table, 
5, 7, 12, 17).* 
The mode of onset has next to be considered. In 
all the cases the legs are first affected, and where 
any more accurate description of the order of af- 
fection is given, we find the peronei most off 
bear the first brunt of the attack. This is shown 
particularly well in Schultze’s cases, (Table 12), 
which may be studied as the most typical in- 
stances of this form of disease. Here was 
noticed the appearance from day to day of a pes 
varus, not due to contracture of opposing muscles, 
* For an interesting resume of the subject of heredity consult Mobius, 
“ Ueber der hereditaren Nerven-Krankheiten,” Volkmann's Samml ng> 
Bd. ii., Abth. i., No. 171. Progressive Muscular Atrophy. 
19 
but simply to loss of substance and power in the 
peronei. Closely associated with these, and prob- 
ably next in order of affection are the extensors, 
the tibialis anticus, and the extensor longus digi- 
torum. Lastly come the flexor muscles, the gas- 
trocnemius, soleus and tibialis posticus. It must 
be borne in mind that though the peronei are most 
often affected, the disease may choose any of the 
above groups for its invasion. It is noteworthy 
that the small foot muscles do not seem to be ever 
the first attacked. 
In the progress of the disease the upper ex- 
tremities and small hand muscles are often affected, 
and comparatively early; in one of Schultze’s cases 
about four years after the first symptom. This 
point is also emphasized by Charcot and Marie. It 
must be remembered that never is any hypertrophy 
of muscles present. There is considerable diversity 
of opinion as to the diagnostic significance of fibril- 
lar or fascicular tremors of the muscles. Aran laid 
great stress on their importance in progressive mus- 
cular atrophy, he speaks of them as being “ true 
nervous discharges.” Duchenne on the other hand, 
perhaps rather under-rates their significance, when 
he states that he has noted their absence in one- 
fifth of the cases he has observed. This is no 
doubt true, but it is certain that many cases which 
Duchenne included under the term, progressive 
muscular atrophy, as for instance the “ infantile 20 
Progressive Muscular Atrophy. 
form,” were not of nervous origin, but pure af- 
fections of the muscles, and whatever the true 
relations of the tremors may be to the nervous 
forms, it is quite certain that they are never present 
in the myopathic forms. It is very probable that 
in the true myelopathic form these tremors are 
rarely, if ever, absent. 
In the peroneal type, however, they are absent in 
some typical cases and present in others. Thus, 
they are absent in nineteen cases, not mentioned in 
eight, and present in twelve cases. When present 
they are often described as being more than fibrillar 
tremors, namely, contraction of more or less large 
bundles of muscle; the resulting movement may 
then be called fascicular. Here may be related a 
case, which again I owe to the kindness of Sir Dyce 
Duckworth. 
Case V.—Marked fascicular twitchings of muscles of calves, often 
accompanied with severe pains. Some wasting of both calves. 
Same twitchings in peronei, but less marked. No other muscles 
affected. No electrical changes. Elder brother had same 
affection. 
Alfred L., aged 35, has been a butler, is now a coffee- 
tavern keeper; was admitted to John Ward, St. Bartholomew’s 
Hospital, in October, 1885. 
For nine months he has suffered from constant twitchings 
and “ flickerings ” in the gastrocnemei muscles. During the 
same time the calves have been slowly diminishing in size, 
equally. When first attacked the tremors were accompanied by 
excruciating stabbing and pricking pains worse generally at Progressive Muscular Atrophy. 
21 
niglit. It was for these that he sought admission. He says he 
used to have a large calf, but has lost at least one inch in girth 
since the trouble began. No history of syphilis or intemperance. 
An elder brother had exactly the same condition extending over 
a period of four years, but has since died of some malarial fever 
in Canada. Another brother died of pneumonia in Canada. 
He has a sister living who has seven children healthy, but has 
lost two with “ convulsions.” 
Present condition.—He is an intelligent, well made, healthy 
looking man. There is nothing noteworthy to be made out in 
the chest, or the muscles of the upper part of the body. 
Lower extremities.—Thighs quite natural. 
Lower leg.—Incessant contractions of bundles of muscle in 
the gastrocnemei. These contractions involve an unusually 
large area of the surface, reminding one rather of the effect of 
faradism with a small electrode. These movements can be oc- 
casionally seen in the peronei muscles, but much less frequently 
and of much less amplitude. Since he has been under treat- 
ment the pains have been much less, but they occasionally 
come on severely at night. When first seen there was complete 
loss of knee-jerk, but now (four months after admission) there 
is a feeble reflex on both sides, but decidedly less on the right. 
There is no diminution of sensibility on either side. The calf 
muscles . are flabby, but there is not much loss of power. He 
can stand on tip-toe easily. Measurement in thickest part of 
calf, 13| in. 
Very slight if any diminution of faradic irritability, no 
galvanic changes. 
The symptoms in this case point strongly to some 
lesion of the nerve trunks, and the heredity would 
indicate something more than an accidental neuritic 
origin. It is very probable that this is an early 22 
Progressive Muscular Atrophy. 
case of the type under consideration, but at present 
the atrophy is such a subordinate feature, that the 
case has not been included among the above sta- 
tistics. 
The last factor in the symptomatology to be 
noticed is the electrical condition of the muscles. As 
in the myelopathic type, so in this, the wasting of 
the muscles is accompanied by a corresponding 
diminution of the irritability to the faradic current. 
But in the peroneal type there is a comparatively 
early tendency to the reversal of the polar reactions 
to the continuous current. This is not the case in 
the thenar form, in which, reaction of degenera- 
tion, if it occur at all, is always a late phenomenon. 
The pathogeny of the disease is at present doubt- 
ful. Of the cases at present collected, there is 
recorded & post-mortem, examination in four. These 
are so important as to deserve special notice here. 
The first is given by Virchow (Table, 2) in 1885. 
In this no atrophy of the anterior horns of the gray 
matter was made out, but the posterior columns 
were gray in colour, the degeneration reaching on 
each side to the posterior horns. The roots and 
nerves were not diminished to the naked eye, but 
in section the fibres were seen to be separated from 
one another by a growth of connective tissue. The 
fine nerve branches do not seem to have been 
examined. 
In the second, by Oppenheimer, (Table, 3) the Progressive Muscular Atrophy. 
23 
evidence is principally negative. The existence of 
fibrillar tremors, and absence of hypertrophy of any 
muscles, precludes the diagnosis of pseudo-hyper- 
trophy. The spinal cord was quite free from disease, 
but the state of the nerves was not noticed. 
Friedreich supplies the third (Table, 6). In this 
absolutely no change was found in the central 
nervous system, but marked lesions were made 
out in the nerves. In the muscles of the right 
hand and legs, the nerve branches were found to be 
full of connective tissue growth, surrounding and 
pressing on the single nerve fibres. The neurilemma 
nuclei were greatly proliferated, and the nerve fibres 
much thinned, their medullary sheaths being atro- 
phied and often interrupted for a considerable ex- 
tent. This condition was noted in the intra-muscu- 
lar nerve twigs, and even in the larger mixed 
trunks, such as the posterior tibial, peroneal, and 
median, but in a less degree. Even in the largest 
nerves, the sciatic and the anterior and posterior 
roots, were found some degenerated nerve fibres. 
On the other hand the median nerve of the left side, 
which showed no muscular change, was quite 
healthy. Friedreich calls this a chronic interstitial 
neuritis. 
In the fourth case, also by Friedreich, (Table, 7) 
there was some degenerative change in the spinal 
cord, having its seat in the posterior columns,, 
most extensive in the lumbar region, and thinning 24 
Progressive Muscular Atrophy. 
off towards the upper part of the cord, till it was a 
small gray wedge in the cervical region. All 
the rest of the cord was quite natural. The 
anterior roots of the lower cord were not 
diminished in size, but were wanting in lustre and 
translucency; under the microscope the fibres were 
found to have a single contour, and a serpentine 
course. At the same time there was a general infil- 
tration of connective tissue with many nuclei. 
The same change was found in the lumbo-sacral 
posterior roots, and could be traced down the large 
nerve trunks, to the smaller branches in the lower 
extremities. 
This case somewhat agrees with Virchow’s. The 
lesions of the cord in both cases are obviously 
secondary. 
It would appear then, that such post-mortem ex- 
aminations as we can command, point strongly to 
the conclusion that the disease is a neuropathy, using 
the term in its restricted sense, and that it probably 
belongs to that class known by the vague generic 
term, neuritis. 
Is this conclusion borne out by the clinical aspects 
of the disease ? Of all the diseases of the nervous 
system, neuritis is the most Protean in its sympto- 
matology, and it would be out of place here, even if 
space allowed, to enter, ever so cursorily, upon such 
a subject. But it must be allowed that while there 
are no serious objections to the neuropathic hypo- 
thesis, there are one or two points in its favour. Progressive Muscular Atrophy. 
25 
In the true myelopathic type, the order of the 
muscles affected, indicates the progress of the lesion 
in the gray matter, spreading upwards and down- 
wards from a focus somewhere in the region of the 
first or second dorsal roots, which centre, as 
Ferrier and Yeo* have shown experimentally, and 
Beevorj* clinically, controls the muscles of the hand. 
Here is a distinct continuity between a central cause 
and a peripheral effect. Such a sequence is con- 
stantly wanting in the peroneal type, where the legs 
are affected first, and then, at a comparatively early 
period, the hand or arm muscles, the intervening 
trunk muscles, being apparently unaffected. This 
condition is difficult to account for by a central 
hypothesis, except on the supposition of two distinct 
lesions; while, on the other hand, it is very common 
in so-called multiple neuritis, which has received so 
much attention lately, especially from Leyden, J 
Grainger Stewart,§ Webber,|| and Buzzard.^f 
Pain, especially along the course of nerves, points 
to the probability of direct nerve affection. This 
symptom, however, does not so often occur as it does 
in the ordinary forms of neuritis, in explanation of 
which it must be remembered that the disease 
• Ferrier and Yeo, Proc. Roy. Soe., 1881. 
+ Beevor, Med. Chir. Trans., 1885. 
J Leyden, Zeitsehr. fur Klin. Med , I., 1880, p. 387. 
§ Grainger Stewart, Edin. Med. Journ., 1881, p. 865. 
|| Webber, Arch, of Med., N.Y., 1884. 
Buzzard, “ Harveian Lectures,” Lancet, Nov., 1885. 26 
Progressive Muscular Atrophy. 
probably attacks first the small muscular branches, 
the main trunks, and with them the sensory nerves, 
being attacked at a later stage. 
The importance of an early appearance of reaction 
of degeneration, without motor-paralysis, has already 
been insisted upon. 
The true diagnostic significance of fibrillar or 
fascicular tremors appears to be as follows. These 
tremors are conspicuous by their absence in true 
myopathies; they are very often present in the 
peroneal type, and nearly always in atrophies due 
to central origin. They were present in two of the 
cases (Table, 3,7) in which neuritis was demon- 
strated post-mortem. Moreover, distinct fibrillar 
tremors are recorded in a case of true idiopathic 
neuritis by Joffroy,* which was verified by post- 
mortem examination. Fibrillar tremors then serve 
only to distinguish myopathies on the one hand, 
from atrophies due to nervous lesions, whether central 
or peripheral, on the other. 
From the above related facts may be deduced 
these conclusions. 
1. That there is a form of Progressive Muscular 
Atrophy, which commences in the lower extremities, 
most often in the peroneal muscles, but sometimes 
also in the tibialis anticus, extensor longus digi- 
'torum, or gastrocnemius. 
2, That the hand and forearm muscles are 
attacked at an early period. 
* Joffroy, Arch, de Physiologic., 1879, p. 172. Progressive Muscular Atrophy. 
27 
3. That the disease is one of childhood. 
4. That heredity is a marked feature. 
5. That the disease shows a slight preference 
for the male sex. 
6. That fibrillar or fascicular tremors are fre- 
quently, but not always, present. 
7. That degenerative electrical changes are often 
an early phenomenon. 
8. That from the records of autopsies, as well as 
from the symptomatology, it may be inferred that 
the disease is one of the peripheral nerves. TABLE OF RECORDED CASES OF THE PERONEAL TYPE OF PROGRESSIVE MUSCULAR ATROPHY. 
No. 
Reference. 
Sex and 
Age at 
Onset. 
Heredity. 
Order of Affection of 
Muscles. 
Musculo- 
Motor and 
Sensory 
Phenomena. 
Electrical 
Reactions. 
Remarks. 
I. 
Aran. 
Gaz. des hop. 
1855, No. 74. 
M. 
11. 
Two aunts died of muscular 
atrophy. 
Began in R. leg then L. 8 years 
after. Then R. arm and shoul- 
der muscles, but not hand mus- 
cles. 
No fibrillar 
tremors. 
2. 
Virchow. 
Virch. Archiv. 
1855- 
M. 
21. 
Father died of same disease, 
at. 40. 
Legs first. Upper extremities 
later 
No mention 
made. 
History very scanty. 
P.M. No affection of ant. horns. 
Some deje eration of post, 
columns. Interstitial changes 
of some peripheral nerves. 
3- 
Oppenheimer. 
Ueber progres- 
sive fettige Mus- 
kelentartung. 
Heidelberg 1855 
Canstatt’s 
Jahresbericht, 
1855- 
2 M. 
11. 
10. 
One other brother, two male 
cousins, two uncles, suffered 
with same affection. 
Legs much wasted first. Flexors 
of foot and extensors of thighs 
gone. Then back muscles. 
Hands very weak. 
Marked fibrillar 
tremors. 
Occasional 
cramps in 
calves. 
Loss of farad, 
irritability in 
calf muscles. 
Some contracture of calf mus- 
cles. 
1st. case. P M. No disease of 
cord or roots found, nerves 
not examined. 
2nd. case. Scantily reported but 
seems to resemble first. No 
P.M. 
4- 
Bamberger. 
Oesterr. Ztg. fur 
prakt. Heilk., 
i860. 
Canstatt, p. 88, 
i860. 
2 M. 
7- 
11. 
Two brothers. Paternal aunt 
died of wasting of extremities. 
Maternal aunt and uncle had 
some affection of legs. Mother, 
contraction of legs. Sister, 
wasted forearms. 
Extreme wasting of muscles of 
legs. Also of arms. 
No mention 
made. 
Dim of farad, 
irritability in 
proportion to 
wasting. 
5- 
A. Eulenburg. 
Virch. Archiv. 
1871. 
3 F 
8 in 
each 
case. 
Three sisters at. 16, 12, and 9, 
when seen, no other heredity. 
Legs first, great wasting of thigh 
muscles. Hand muscles af- 
fected early. Also shoulder 
and upper arm muscles. 
No mention 
made. 
Dim, of farad, ir 
ritability in all 
affected muscles 
with R. D. in 
many. 
Contractures of knee and hip 
muscles, also talipes equino- 
varus. No. 
Reference. 
Sex and 
Age at 
Onset 
Heredity. 
Order of Affection 
of Muscles. 
Musculo- 
Motor and 
Sensory 
Phenomena. 
Electrical 
Reactions. 
Remarks. 
6. 
Friedreich. 
Ueber progr. 
Muskelatroph. 
1873. 
(Case I.) 
F. 
5- 
Right leg, then left. Great 
wasting of right hand muscles. 
No mention 
made. 
Extreme pes varus. Contrac- 
tures of fingers of right hand. 
P.M. Interstitial change in 
peripheral nerves of right arm 
and both legs. Spinal cord 
quite natural. 
7« 
Do. 
(Case II.) 
F. 
10. 
One brother affected with same 
disease (next case). 
Legs first, then in arms and 
hands. Later in shoulder 
muscles. 
Marked fibrillar 
tremors. Fre- 
quent painful 
muscular con- 
tractions. 
Talipes (varus ?). Contractures 
of fingers. 
P.M. Interstitial changes of 
intra-muscular nerves and large 
nerve-trunks of affected limbs, 
ant. and post, roots. Secondary 
degeneration of post, columns 
of cord, but no other disease of 
cord. 
8. 
Do. 
(Case III.) 
M* 
12. 
Brother of last case. 
Legs, especially small foot 
muscles. 
Fibrillar tre- 
mors. 
Shooting pains 
along limbs. 
No reaction to 
farad, or galv. 
in affected 
muscles. 
9- 
Do. 
(Case IX.) 
M. 
8. 
• 
Legs first then gluteal region. 
Upper extremities affected 
later. 
Fibrillar 
tremors. 
Faradic irrit. 
dimin. No R. D. 
Talipes of left foot, toes clawed. 
Kyphosis of spinal column. 
Contracture of hands. 
10. 
Eichorst. 
Berlin, klin. 
Wochenschr., 
No. 43, 1873. 
5 M. 
5 F. 
average 
age, 18. 
History of atrophies in six gene- 
rations. 
Ten cases under observation ex- 
tending over three generations. 
Lower extremities in all the 
cases, mostly peronei. Hand 
muscles attacked later. 
No fibrillar 
tremors, or 
pains. 
Farad, irrit. 
dimin. in atro- 
phied muscles. 
> 
Tendency to talipes varus or 
equino-varus in most of the 
cases. No. 
Reference. 
Sex and 
Age at 
Onset. 
Heredity. 
Order of Affection 
of Muscles. 
Musculo- 
Motor and 
Sensory 
Phenomena. 
Electrical 
Reactions. 
Remarks. 
11. 
Osier. 
Archives of Med. 
N. Y.( 1880. 
M. 
46. 
Very marked collateral and 
ascending. 
Wasting of whole left leg parti- 
cularly thigh, buttock, and loins. 
Marked twitch- 
ings of 
muscles. 
Very interesting genealogical 
tree extending over 3 genera- 
tions. No atrophies as yet 
among the descendants. 
12. 
Schultze. 
Berlin, klin, 
Wochenschr. 
1884. 
1 M. 
2 F. 
2 in each 
case. 
Two sisters and one brother, set. 
3) 5i 9> when seen ; no ascen- 
dent heredity. 
Peronei muscles, tibial region, 
extensors, not calf muscles. In 
the eldest the thigh, thumb 
and interossei affected later. 
No fibrillar 
tremors. 
No pains. 
Complete R. D. 
in two cases. 
Talipes varus from the begin- 
ning. Contracture of hand in 
eldest ; knee-jerk absent in 
one. 
13- 
Ormerod. 
Brain, 1884. 
F. 
6. 
Brother and father have the 
same affection. 
Peronei and calf muscles. 
Five years later, hand and fore- 
arm muscles. 
No fibrillar 
tremors. 
No reaction to 
farad, or galv. 
in affected leg 
muscles. 
In left hand 
ACC = KCC. 
In this and the two following 
cases the atrophy came on after 
measles. 
Knee-jerk present on both sides. 
14. 
Do. 
M. 
5- 
Brother of 13. 
Lower extremities, probably 
peronei first. Hand muscles 
affected 4 years later. 
No fibrillar 
tremors. 
Diminished 
reaction to 
farad. Galv. 
irrit. nil in leg, 
but fair in 
thighs. 
15. 
Do. 
M. 
3- 
Father of 13 and 14. 
R. leg, calf and peronei muscles. 
No fibrillar 
tremors. 
Not made out. 
The condition has not pro- 
gressed ; the patient is now 56. 
l6. 
Charcot and 
Marie, Rev. de 
Med., Feb., 1866 
M. 
5- 
First noticed pes varus ('affection 
of peronei). 
Hands attacked 2 years after. 
Fibrillar 
tremors. 
Subject to cramp 
in muscles. 
R. D. in some 0: 
the muscles. 
Hands claw-shaped. Knee-jerk 
feeble. No. 
Reference. 
Sex and 
Age at 
Onset 
Heredity. 
Order of Affection 
of Muscles. 
Musculo- 
Motor and 
Sensory 
Phenomena. 
Electrical 
Reactions. 
Remarks. 
*7- 
Do. 
M. 
3- 
Brother with same. 
Legs first. Talipes valgus. Calf 
and thigh muscles. Hand 
muscles later. 
Fibrillar 
tremors. 
Not mentioned. 
Knee-jerk abolished. 
18. 
Do. 
M. 
3- 
Brother of 17. 
First in peronei muscles Ta- 
lipes varus. No affection of 
upper extremities. 
Fibrillar 
tremors. 
Not mentioned. 
Knee-jerk abolished. 
19. 
Do. 
F. 
14. 
R. leg first. Calf muscles then 
L. Five years after hand 
muscles. 
Fibrillar tre- 
mors. Cramp in 
thigh very often. 
R. D. in many 
muscles of the 
upper extremity. 
No reaction to 
galv. in most ol 
leg muscles. 
Knee-jerk abolished. 
20. 
Do. 
F. 
15. 
First in calves and peronei. 
Four years after, hand muscles 
began to atrophy. 
Fibrillar tre- 
mors. Shooting 
pain in legs. 
R. D. in hand 
muscles. No 
react, to galv. in 
legs. BIBLIOGRAPHY. 
This list contains many atrophies of myelopathic, 
neuropathic, and myopathic origin. But among 
myopathies simple cases of myo-sclerosis have not 
been included. This subject with bibliography up 
to date, has been so fully treated by Dr. Gowers in 
in his work on Pseudo-hypertropliic paralysis as to 
render a repetition unnecessary, especially as com- 
paratively little advance has been made since that 
time. Where possible the original articles have 
been consulted. Abstracts of most of them may be 
found in Canstatfs Jahresbericht now edited by 
Virchow and Hirsch. 
Abercrombie (John). Diseases of Brain and Spinal Cord, 4th Edit., 
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Adams, On Progressive Muscular Atrophy. Boston Med. and Surg• 
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Alexander and Hamilton. Progressive muscular atrophy. Lancet, Jan. 
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Anderson. Two cases of progressive muscular atrophy. Glasgow Med. 
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Union med., No. 48, 1856. 34 
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35 
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no nervous lesion discovered. 
Degos. Del’atroph. muse, progr. These, Montpellier, 1876. 
Deschamps. Contributions a l’etude des atroph. muse, progr. 1885. 
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two autopsies in which no nervous changes were noted. 
De la valeur de l’electrisation localisee comme traitement de l’atroph. 
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Nouveaux cas de l’atroph. muse, progress, de l’enfance chez une 
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et Joffroy. De l’atroph. aigue et chronique des cellules nerveuses de 
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L'electrisation localisee. 3e Edition, 1872, p. 486, and appendix, 
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Dumenil. Atrophie des nerves hypoglosses, etc. Gaz. Hebdom, 1859, 
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of spinal roots and hypoglossal nerves. 
Nouveaux faits relative a la pathologie, etc. Gaz. Hebdom, 1867, Nos. 
27, 29 and 30. Five cases with post-mortem examinations in all, 
which showed great thinning of anterior roots in four and also of anterior 
horns in one of them. 
Durant. Sur l’atroph. muse, dite progressive. These, Strasbourg, 1857. 
Canstatt's Jahresb. Three cases, the first apparently beginning in the 
right muscles of mastication, the other two traumatic. 
Eichorsf. Ueber Hereditat der progress. Muskelatroph. Berlin. Klin. 
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Erb und Schultze. Ein Fall von progress. Muskelatroph. mit Erkrankung 
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Lichtheim. Bibliography. 
37 
Erb. Ueber die juvenile Form der progress. Muskelatroph. Deutsche Arch, 
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Eulenburg. Ueber progress. Muskelatroph. A llg. centr. Zeitung, No. 60, 
1885. 
Ueber progress. Muskelatroph. Deutsche Klinik, 1856, Nos. n-14. 
Among other cases, those of twin brothers, myopathies, with apparent 
hypertrophy of glutasi. 
Progress. Muskelatroph. mit gleichseitizen Defect einzelner Muskeln. 
Deutsche Klinik, 1861, No. 25. 
Fall von aufsteigender progr. Muskelatroph. Ibid., 1863, No. 3. This 
and the last case probably myopathies. 
(A) Ueber successives Auftreten diffuser Muskelerkrankungen bei 
Geschwistern. Virch. Arch., 1871, Bd. 53, p. 361. (See Table, 5, this 
essay). 
Ein Fall von fortschreitender Muskularer Dystrophie an den Unter- 
extremitaten. Deutsche Med. Wochenschr., 1885, p. 178. 
Eyrand. Considerations sur l’atroph. muse, progress. These, Strasbourg. 
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Friedberg. Pathol, und Therapie der Muskelldhmungen. Weimar, 1858. 
An opponent of the neurotia theory. 
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to support an universal myopathic theory, contains several valuable 
autopsies. (See Table, 6-9, this essay). 
Promman. Fall von atroph. muse. prog. Deutsche Klinik, 1857, No 33, 34, 
Apparently a thenar myelopathy, but post-mo.tem no thinning of the 
ant. roots noticed. 
Greenhow. A case of progr. muse, atroph. Clin. Soc. Trans., 1872, p. 210. 
Atrophy began in hands but legs were soon affected. Post-mortem, 
no important affection of cord or nerves were found. 
Acute muscular atroph. Ibid., 1873, p. 149. Sister of above case. 
Course very rapid. Post-mortem results resembled those of the pre- 
ceding case. 
Grimm. Ein Fall von progr. Muskelatroph. Virch. Arch., i86g, p. 445. 
Gros. De l’atroph. muse, progr. au point de vue du traitement electrique. 
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a blow on the neck. Guy's Hosp. Reports, 1858, p. 195. 
Gunther. Ueber die typische form der progress, muskelatroph. Berlin, 
klin. Wochenschr., 1885, No. 20. 
Guthzeit. De novissimis observationibus ad atroph. muse, progr. naturam 
et therapiam spectantibus. Diss. Berol., 1862, also Canstatt's Jahresb. 
1863. 38 
Bibliography. 
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39 
Zjichtheim. Progress. Muskelatroph. ohne Erkrankung der Vordehorner des 
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Bibliography. 
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41 
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muscles. a ■: y ■ 
\'Y . v