Progressive Muscular Dystro- 
phies : the Relation of the 
Primary Forms to one 
another and to Typical 
Progressive Muscular 
Atrophy, 
B. SACHS, M. I), 
BY 
REPRINTED FROM 
j 3Ttc Nclu York ftlpti'cal Journal | 
for December IS, 1888. Reprinted from the Rew York Medical Journal. 
PEOGEESSIVE MTJSCDLAE DYSTEOPHIES: 
THE RELATION OF THE PRIMARY FORMS TO ONE ANOTHER 
AND TO TYPICAL PROGRESSIVE MUSCULAR ATROPHY* 
B. SACHS, M. D. 
The diseases to be discussed in this article have passed 
under so many different names that it will be necessary, 
first of all, to state what shall and what shall not be under- 
stood by the term “progressive muscular dystrophies.” 
This term is intended to designate those forms of dis- 
ease in which a primary progressive wasting of some or all 
of the muscles of the body is the most characteristic feature, 
and in which this wasting (atrophy) may or may not be 
associated with true or pseudo-hypertrophy of some mus- 
cles. These primary progressive dystrophies are our chief 
concern; we have nothing to do with muscular atrophy 
following cerebral, myelitic, or peripheral nerve disease. 
One form of disease, however, which is undoubtedly due 
to changes in the spinal cord we must draw into the dis- 
cussion. I refer to typical progressive muscular atrophy. 
This must, in fact, be the basis upon which our discussion 
shall proceed, for a very large number of the cases and dif- 
* Opening paper of the discussion on “ Muscular Dystrophies,” read 
before the American Neurological Association at its fourteenth annual 
meeting. With some few omissions and corrections, the paper is 
printed as read. 
1 2 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
ferent forms of disease which we shall have to consider 
were once classed under this term. “ Progressive muscu- 
lar atrophy ” was for many years, and with many authors 
still is, a mere clinical designation, just as locomotor ataxia 
was a mere clinical term until the pathological anatomy of 
the disease was established, and the term was finally re- 
stricted to cases of tabes dorsalis. 
Neurologists are at present engaged in sifting the clin- 
ical material, and many cases which only a decade ago 
would have been labeled as “ progressive muscular atro- 
phy ” are now recognized to be cases of amyotrophic lateral 
sclerosis, of syringomyelia, of tumor of the cord, of tabes 
dorsalis, of poliomyelitis anterior infantilis, or of multiple 
neuritis. I insist again that we have nothing to do with 
these issues, and that we are concerned with typical pro- 
gressive muscular atrophy (type Aran-Duchenne) only in 
its relation to the primary myopathies or muscular dys- 
trophies. 
For the purpose of establishing this relation, I propose, 
with the above limitations in mind, to give a short histor- 
ical review of our subject. The discussion which is still 
waging, and which has already succeeded in establishing at 
least six different forms of progressive muscular wasting, 
began along two different lines with the creation of two 
distinct diseases—viz., typical progressive muscular atro- 
phy, and pseudo-hypertrophic muscular paralysis. Let us 
first take up the history of “ progressive muscular atrophy.” 
Duchenne, in 1849, was the first to describe this dis- 
ease, and embodied his observations in a paper presented 
to the French Institute (1). In the next year Aran (2) 
gave a full description of the same disease and reported a 
number of cases; the same was done by Cruveilhier (3) 
three years later, but the full dignity of the disease was not 
recognized until Duchenne’s classic account of it appeared PROGRESSIVE MUSCULAR DYSTROPHIES. 
3 
in liis famous work on “ L’Electrisation localisee ” (4). Du- 
chcnne distinguished two forms—progressive muscular 
atrophy of the adult, and progressive muscular atrophy of 
infancy. The latter will come up for consideration, to- 
gether with the new type of muscular atrophy which 
Landouzy and Dejerine have described and advocated. 
The former type remains almost in all particulars as Du- 
chenne described it. Modern authors, including Charcot, 
Leyden, Strumpell, Hammond, Glowers, and others, have 
been able to add but very little to Huchenne’s original de- 
scription. The chief characteristics of this form are as 
follows: 
Progressive Muscular Atrophy (type Aran-Ducbenne). 
—This form begins in a large majority of the cases with an 
atrophy and corresponding weakness in the small muscles 
of the hand (thenar and hypothenar). The atrophy 
spreads from muscle to muscle (“atrophic individuelle ”). 
Beginning, as a rule, with the adductor pollicis longus, 
it involves, next in order, the opponens pollicis and deep 
muscles of the thenar; from these it extends to the hypo- 
thenar, the interossei, the flexors and extensors in the fore- 
arm. At this stage the disease may remain stationary, or 
it may spread to the flexors in the upper arm, to the del- 
toid, possibly the triceps, and finally to the muscles of the 
trunk, the shoulders, and the back. Duchenne recognized 
the fact that the atrophy may begin, in exceptional cases, in 
the trunk, in the shoulders, or in the legs.* Certain it is 
that in those cases in which the atrophy begins in the 
* Of these exceptional cases mentioned by Duchenne a considerable 
number will now be relegated to other forms of muscular atrophy. 
Duchenne states that only two cases in one hundred and fifty-nine be- 
gan in the legs. Hammond said in the first edition of his treatise on 
“Nervous Diseases,” p. 66, dght cases in twenty-nine; in his last edi- 
tion, p. 585, he says only six in fifty-two cases. 4 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
hands, the legs are not affected until very late in the course 
of the disease. One marked exception to this rule has oc- 
curred in my own practice in the case of a woman aged 
forty, in whom the atrophy attacked almost simultaneously 
the small muscles of the thenar and the anterior muscles of 
the thigh. This case had all the other symptoms of typ- 
ical progressive muscular atrophy. 
The atrophied muscles in progressive muscular atrophy 
exhibit fibrillar contractions and as a ride retain their 
faradaic contractility. There may be a diminution of fara- 
daic or galvanic excitability proportionate to the wasting 
of some muscles, and a complete or partial reaction of de- 
generation may be present in other muscles. The march 
of the disease is steadily progressive. Heredity is a strong 
factor in the disease, as is shown by the remarkable series 
of cases published by Naunyn and Eichhorst in the “Ber- 
liner klinische Wochenschrift,” and by the account of the 
Weathersbee family given in the later editions of Ham- 
mond’s treatise, although the latter cases probably belong 
to the peroneal type, to be discussed later on. Osier’s 
cases (5) also give strong proof of heredity. 
With the exception of the factor of heredity, all the 
clinical features, as given above, were known to Duchenne. 
For many years, too, the clinical features of progressive 
muscular atrophy were beyond question. All discussions 
that followed related to the question whether this disease 
was of spinal or peripheral origin. Duchenne first regarded 
the disease as of peripheral origin, but in his third edition 
retracted this view, convinced, as he says, by the pathologic- 
al and anatomical facts gathered by Charcot and Joffroy 
(6), Lockhart Clarke (7), Hayern (8), and others (9). To 
Clarke, and above all to Charcot and his school, we owe the 
advances made (in the years 1860 to 1870) in our knowl- 
edge of the pathology of progressive muscular atrophy. PROGRESSIVE MUSCULAR DYSTROPHIES. 
5 
The main changes found are these : A sclerotic and pig- 
mentary atrophy of the ganglion cells of the anterior horns, 
inflammatory changes in the neuroglia, increased size of 
the hlood-vessels, and proliferation of the cellular elements. 
In fresh preparations granular corpuscles are found, and, ac- 
cording to the degree and stage of the disease, the anterior 
gray cornua are reduced in all diameters, and the ganglion 
cells either atrophied or entirely lost. The anterior nerve- 
roots are affected secondarily to the lesion of the gray sub- 
stance, The nerve-fibers are not all destroyed, a number of 
them remaining intact. Those that are destroyed exhibit 
the appearances of simple atrophy—a point to which Char- 
cot * alludes as distinguishing these cases from infantile 
spinal paralysis. 
The theory of the disease was and is, that the inflamma- 
tion spreads slowly from the ganglion cells of the anterior 
horns along the anterior nerve-roots without destroying as 
many of these fibers as is the case in infantile poliomyelitis. 
The atrophic changes in the muscles are, on this hypothesis, 
the direct result of the irritation which begins in the cells 
of the anterior horns and is propagated thence through nor- 
mal or only half-wasted nerve-roots to the peripheral mus- 
cular fiber. 
The earlier pathological investigations erred in various 
respects; first of all, all changes in the spinal cord were 
not noted, the white columns of the cord were not carefully 
examined. In consequence of this inadvertence in the ex- 
amination of pathological specimens and on account of in- 
sufficient clinical description, many cases of amyotrophic 
lateral sclerosis were recorded as cases of progressive mus- 
cular atrophy. It is Charcot’s great merit to have done 
pioneer work in this, as in so many other neurological prob- 
lems. In France, Charcot (10) succeeded in making his 
* “ Maladies du syst&me nerveux,” vol. ii, p. 209. 6 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
tephro- (polio-) my elite chronique parenchymateuse the ana- 
tomical substratum of Duchenne’s progressive muscular 
atrophy. 
From this time onward German investigators play a 
very important role in the solution of the problem under 
discussion, attacking the problem both from the pathological 
and from the clinical standpoint. Bamberger and Reckling- 
hausen (11) published two cases of Duchenne’s atrophy in 
which no changes could be found in the spinal cord post 
mortem, but it was not until the appearance of Friedreich’s 
great monograph (12) that the possible peripheral origin of 
progressive muscular atrophy was again pushed into the 
foreground. 
Friedreich maintained that the changes found in the ante 
rior nerve-roots and in the anterior cornua, in cases of pro- 
gressive muscular atrophy, were secondary changes, and to 
this he allowed no exception. According to Friedreich’s 
views, progressive muscular atrophy is a primary chronic 
myositis which is followed in due course of time by second- 
ary changes in the nervous system. The intermuscular 
nerve-filaments are the first to be affected, and from these 
nerve-filaments an ascending neuritis travels along the pe- 
ripheral nerve-trunk to the anterior roots of the spinal-cord 
segment; the neuritis of these anterior nerve-roots may 
spread to the cord and here set up chronic myelitic changes 
which will vary greatly in degree and distribution; the 
extent and character of the changes will, according to 
Friedreich, depend upon the extent of the muscular affec- 
tion. The changes in the peripheral nerve-fibers and in the 
ganglion cells of the anterior horns are the result of the 
impaired motor functions of the affected muscles (op. cit., p# 
118 and 124). 
On this theory alone, Friedreich insisted, can we explain 
why in certain cases a wide-spread muscular atrophy is as- PROGRESSIVE MUSCULAR DYSTROPHIES. 
7 
sociated with changes in the cervical segment only, as in the 
cases of Dumesnil (I?), of Lockhart Clarke and Gairdner 
(14), in the cases of Clarke and Cooper (15), Clarke and 
Johnson (16), and others, in which changes were found in 
the spinal cord and none in the nerve-roots. Friedreich 
claims that the nerve-roots were not properly investigated • 
on the other hand, the cases of Recklinghausen, of Fried- 
herg and Cruveilhier, of Trousseau, and his own cases (Nos. 
4 and 21), proved to him that changes may occur in the 
muscles themselves, or in the nerve-trunks and anterior 
nerve roots, and not in the spinal cord ; but Charcot (op. 
cit., p. 209) very correctly protests that all these cases upon 
which Friedreich’s proof rested were examined before the 
present successful histological methods for staining the 
spinal cord had come into vogue, and that they therefore 
prove nothing. 
While Friedreich’s judgment unquestionably erred in 
regard to many of these cases, the error can be explained, 
since many of the cases upon which he based his views are 
now known to belong to other forms of muscular atrophy 
in which there is no accompanying change in the spinal 
cord. As regards typical progressive muscular atrophy, 
the investigations of later years have put the spinal origin 
beyond question, although, as Schultze (17) has shown in 
his excellent monograph, there are but two cases of Du- 
chenne’s atrophy [cases of Pierret-Troissier (18) and of 
Striimpell (19)] in which the anterior gray matter was the 
only part affected, and alone responsible for the wide-spread 
muscular atrophy. To this list we might add the case of 
Wood and Dercum (20) if the clinical history were not un- 
satisfactory. Schultze arrives at his conclusions by exclud- 
ing even those cases in which the nuclei of the medulla had 
become involved by extension of the process. Without 
wishing to depart from the subject before us, I may inti- 8 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
mate that these pathological researches prove that, although 
progressive muscular atrophy is of spinal origin and is a 
distinct clinical entity, it is not necessarily a morbid entity, 
and in most cases represents an early stage of one of several 
spinal-cord diseases. 
It is now time for us to retrace our steps and note the 
development of our knowledge regarding pseudo-hypertro- 
phic muscular paralysis. 
The history of this form can be related in few words. 
The clinical features as laid down by Duchenne, G-riesingcr, 
Seidel, and others have been universally accepted. These 
authors, and all who followed them, fastened upon the in- 
crease in the size of some muscles as the characteristic 
symptom of the disease, and have largely disregarded the 
wide-spread muscular atrophy which is present in many 
cases of pseudo-hypertrophy. 
The earliest cases of pseudo-hypertrophy of muscles were 
described by Mery on (21) in 1852. Similar cases had been 
described by Charles Bell in 1830, hut were not valued at 
their true worth, and Meryon even maintained that his eases 
were intimately related to Cruveilhier’s (Aran-Duchenne’s) 
atrophy. Oppenheim in 1855 published a thesis at Heidel- 
berg on progressive muscular atrophy in which ho reported 
a number of cases of pseudo-hypertrophy, without, however, 
making a distinction between these cases and Duchenne’s 
type. It was Duchenne again who, in a paper (22) pub- 
lished in 1861, first called attention to the increase in the 
volume of certain muscles as the important feature in the 
disease, and in his “ Electrisation localisee ” established this 
type of disease for all time. Since that time innumerable 
cases have been published, enabling Cowers (23) in 1879 to 
base his studies upon a series of 220 cases, some of these, 
however, evidently belonging to other categories. The clin- 
ical features have been verified so many times over that we PROGRESSIVE MUSCULAR DYSTROPHIES. 
9 
need not in tins paper analyze all the cases, but can, without 
hesitation, present the general features of the disease. 
Pseudo-muscular hypertrophy or pseudo-muscular scle- 
rosis (Jaccoud), atrophia musculorum lipomatosa (Seidel) 
(24), is a disease of early youth, the vast majority of cases 
beginning before the age of six. Boys are affected some- 
what more frequently than girls, and there is good proof of 
heredity, the disease, although largely affecting boys, be- 
ing most frequently inherited through the mother. Mer- 
yon’s cases appeared to form an exception (vide Gowers, 
op. cit., p. 24). The first symptoms are a weakness in the 
muscles of the leg, a waddling gait, and an apparent in- 
crease in the size of some of the muscles of the leg. In 
many cases the calves only are hypertrophied ; in others the 
calves and thighs, and in rarer cases, like one now under my 
observation, the disease is limited to, or at least begins in, 
the thigh muscles. 
Author's Case I.—A. K., aged ten. Mother has had six 
children; one died of “brain fever” and one of croup. Four 
living, one older than patient, all healthy. No history of he- 
redity. Patient, a stout child, a newsboy, had first teeth at 
four months; when one year old began to walk. At one year 
and a half showed weakness and could not walk alone; was pro- 
vided with some sort of machine with which he learned to 
walk. Was treated for rickets. Youngest sister has distinct 
rickets at present. No change until last December, when par- 
ents noticed that he was getting lamer. Mother states that 
thighs were always large; had difficulty in finding trousers that 
would fit the hoy in the thighs. Boy could never walk as other 
children did, and could never run after others. He now com- 
plains of great fatigue, and when walking throws himself down 
on the grass from mere fatigue. Examination shows increase 
of volume of anterior thigh muscles of both sides, most marked 
in the middle portion of the vasti. Calves not hypertrophied; 
no other atrophy anywhere except in the serratus anticus of the 10 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
right side. Grasp of both hands normal; knee-jerks present; 
all electrical reactions normal. With the assistance of Dr. 
Peterson I excised two pieces of muscle from the left vastus 
externus which will be referred to in a later section of this paper. 
The wound healed readily; boy complains of greater weakness 
in the leg from which pieces of muscle were removed. 
Duchenne made out three stages of the disease. In the 
first, difficulty in standing and walking, and weakness of 
muscles of lower extremities and of sacro-lumbar region. 
In the second stage the hypertrophy becomes the prominent 
feature, spreading to various muscles of the body, and in 
the third stage there is increased feebleness of the muscles of 
upper and lower extremity of the trunk. Other authors 
recognize a weakening of the sacro-lumbar region and in a 
general way a weakness of the upper extremities, but, in 
view of Erb’s recent studies, it is due to Gowers to state that 
he called attention to the fact that in many cases of pseudo- 
hypertrophy the “ infraspinati and deltoids are often in- 
creased in size. . . . The latissimus dorsi is commonly 
much wasted, and so also is the lower (sterno-costal) por- 
tion of the pectoralis major. . . . The forearm muscles are 
rarely affected.” 
To complete the clinical picture we must in addition 
refer to the lumbar lordosis (probably due to the weakness 
of the extensors of the hip), to the occasional presence of 
contractures, and to the peculiar difficulties in rising from 
the ground (the patient climbing up upon himself) which 
are present in some cases, but not necessarily in all, and to 
which Gowers attaches too much importance in making it 
the cardinal symptom of the disease. My patient has dis- 
tinct pseudo-hypertrophy, hut rises from the floor with the 
greatest ease. In a general way it is to be noted that there 
are no fibrillar contractions in the affected muscles, no 
changes-in the electrical reaction, except diminished excita- PROGRESSIVE MUSCULAR DYSTROPHIES. 
bility to both currents, no sensory disturbances, and the 
patellar reflex may or may not be present. As a typical 
example of pseudo-hypertrophy with unusual atrophy of the 
upper extremities I will cite the following case now under 
ray observation : 
Author's Case lI.—M. K., girl, aged twelve years and a half; 
mother has one other child living and healthy. One sun died at 
age of twenty-four of meningitis. Patient first seen by me two 
years ago. History showed that child had severe fright at age of 
ten months. Child has always been very nervous; learned to 
stand and walk at usual age, but had diphtheria at age of four, 
since when the disease has become much worse. Legs first grew 
thin. The calves increased in size about four years ago. Child 
has always had characteristic difficulty in walking and rising 
from the floor. Examination showed decided weakness in pos- 
terior group of leg and thigh muscles; calf and thigh muscles 
distinctly hypertrophied. Nerves and muscles of lower legs re- 
act well to faradaic current, much more readily on indirect than 
direct excitation. No atrophies anywhere in the body, none 
around shoulder girdle; hands normal. Child has difficulty in 
getting upon a chair, and in descending comes down with a 
bound. 
Thigh—left, inches; right, 13-^-inches. 
Calves—left, inches; right, 10T56 inches. 
Examined the child again after two years; found condition 
very much the same. Thighs—left side, 16 inches; right, 15|- 
inches. Calf—left side, 11 inches; right, 10|- inches, showing 
that the growth of the calf muscles has not kept step with the 
growth of thigh muscles. Muscles of calf and anterior thigh 
muscles still appear large. Resistance to passive movements very 
much diminished, particularly in extensors of thighs. Atrophy of 
sternal portion of the sterno-cleido-mastoid; left shoulder stands 
out more prominently than right, but shows no hypertrophy. 
All arm and forearm muscles thin ; distinct atrophy in the mus- 
cles of the interosseous spaces; grasp very weak; right 18, 
left 18. In walking, both feet assume valgus position. Arms 
are in marked contrast to legs, Length of arms, 25 inches; 12 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
length of legs, 28 inches. Electrical examination : All muscles 
respond promptly to faradaic current, except interossei and 
vasti of both sides, which require very strong currents. Gal- 
vanic response diminished in interossei and in muscles of thenar, 
but formula not altered. 
Having agreed to accept the foregoing description and 
histories as typical of what is ordinarily called pseudo-mus- 
cular hypertrophy, we must now devote a little more atten- 
tion to the pathological anatomy of the disease. Cases of 
pseudo-hypertrophy with autopsies are relatively few, and 
for that reason the evidence must be sifted carefully. 
Middleton, in his very excellent paper (25), collected 
seventeen cases of pseudo-hypertrophies with autopsies; 
one of these (26) must be excluded from the list as being 
a clear case of amyotrophic lateral sclerosis, Schultze {op. 
cit., p, 36) has added to this list the two cases of Middleton, 
one by Berger (27), two cases described by Gunther (28), 
one by Pick (29), and one by Friedreich {op. cit., p, 347), 
making twenty-three cases in all. 
Of these twenty-three cases, those of Friedreich, Meryon 
(Case II), Kesteven (30), Baeg (31), Brigidi (32), Ross (33) 
(Case I), and of Gunther must be excluded, either because 
the spinal cord was not examined microscopically or be- 
cause the examination was not properly made. Of the fif- 
teen remaining cases, the spinal cord and anterior nerve-roots 
were found absolutely normal in ten, and in five others the 
changes that were found could not be held responsible for 
the changes in the muscles. These ten cases (34) are un- 
objectionable in every point; their clinical histories are very 
similar in every respect and are sufficient proof of the fact 
that pseudo-hypertrophy of the muscles is not dependent 
upon changes in the spinal cord. 
In the endeavor to increase this list I have carefully 
searched for earlier cases with autopsies, in our own litera- PROGRESSIVE MUSCULAR DYSTROPHIES. 
13 
ture in particular, which might have escaped Schultze’s 
notice, and have furthermore endeavored to collect cases 
which have appeared since the publication of Schultze's 
monograph, but the total increase is not great. 
First of all, attention should be directed to Gibney’s (35) 
case, which was presented to the American Neurological 
Association two years ago. The history of the boy, aged 
sixteen at death, who had been under observation for ten 
years, is a typical one of the disease. There was first dis- 
tinct enlargement of the calves, followed later on by atrophy. 
A brother is affected in the same way. Dr. Amidon, who 
examined the cord, reports : “ The only lesion appeared to 
be in the ganglion cells of the anterior horns. . . . About 
one half of the cells seemed to have disappeared, leaving 
no trace. The remaining ones are poorly defined, small, 
and in many instances processless. . . . Lesion more marked 
in the dorsal than in lumbar region.” 
Through the kindness of Dr. Amidon, I have been per- 
mitted to re-examine the specimens, and I must confess that 
the case would appear to show that there are no serious 
cord changes in pseudo-hypertrophic paralysis. Processless 
ganglion cells mean as little in the spinal cord as processless 
pyramids mean in the cortex, and a diminution in the rela- 
tive number of cells in any one section is a point exceed- 
ingly difficult to determine, and, if present, is more apt to 
be a secondary than primary affair. I hope that both Dr. 
Gibney and Dr. Amidon will concur in this view of their 
case. 
The only other cases of pseudo-hypertrophic paralysis 
with autopsies which I have been able to find were these: 
Westphal (36) reported the cases of two sisters, both af- 
fected with typical pseudo-hypertrophy, in the one case 
characterized by unusual increase in the volume of many 
muscles and by slight involvement of the facial muscles. PROGRESSIVE MUSCULAR DYSTROPHIES. 
Westphal found no changes whatever either in the cord or in 
the peripheral nerves. Coming from so distinguished an 
author, these facts deserve the greatest consideration. 
Middleton (37) has described another interesting case 
with enormous pseudo-hypertrophy and a wide-spread 
atrophy, including even the raasseters, but the cord did not 
harden well and a microscopical examination could not be 
made. The case is, therefore, useless for our present pur- 
poses. 
Further autopsies on typical cases of pseudo-hypertrophy 
are extremely desirable, but Westphal’s cases, together with 
the others analyzed above, place the non-spinal origin of 
pseudo-hypertrophy beyond question. 
These facts do not appear to be properly appreciated 
as yet, for we find Dr, Inglis (38) very recently reporting 
several interesting cases of pseudo-hypertrophy and assum- 
ing that all pathologico-anatomical facts point to the spinal 
cord as the seat of disease. Dr. Inglis gets over the dis- 
comforting negative facts by stating that “ the cases in which 
the post-mortem examination shows the cord visibly intact 
do not invalidate this idea (the spinal origin of pseudo-hy- 
pertrophy), and that the defect in the distal ends of the 
motor fibers, while not in every case accompanied by 
atrophy of the central cells, is yet the indication of an im 
paired activity of those cells.” It is more surprising still to 
find Hammond (39) disregarding the evidence of the last 
ten years and adhering to the spinal theory of pseudo-hy- 
pertrophic paralysis, and even going so far as to entitle the 
disease “ pseudo-hypertrophic spinal paralysis.” Ham- 
mond’s conclusions are based on cases of Barth (40), Mul- 
ler (41), and Lockhart Clarke (42). Barth’s case is one of 
amyotrophic lateral sclerosis ; Muller’s case was complicated 
by cerebral disease and therefore useless for the determina- 
tion of the anatomical lesion ; while Lockhart Clarke’s case PROGRESSIVE MUSCULAR DYSTROPHIES. 
15 
showed changes which are not primary and which Gowers, 
whose case this was, acknowledged (in the “Lancet” for 
1879) to have been possibly due to the paralysis of long 
standing and to the frequent pulmonary troubles. 
At this stage of our studies let us note that careful clin- 
ical investigation and post-mortem examinations have shown 
among other facts that a wide-spread atrophy is common to 
progressive muscular atrophy (type Aran-Duchenne) and 
pseudo-hypertrophy, but that the absence of all changes in 
the central nervous system, the absence of fibrillar contrac- 
tions, and the absence of reaction of degeneration in cases 
of pseudo-hypertrophy, separate it widely from the former 
disease. Later on we shall see that a very intimate relation 
exists, however, between pseudo-hypertrophy and certain 
other forms of muscular dystrophy which were formerly in- 
cluded under the general heading of progressive muscular 
atrophy. 
The process of distinguishing these forms from pro- 
gressive muscular atrophy was of slow development, and 
with the steps of this process we shall become best ac- 
quainted by alluding to a few excellent articles published 
between the years 1870 and 1880. 
Lichtheim (43) was one of the first to take up the cud- 
gels for Friedreich’s theory of progressive muscular atropliy. 
In 1878 he published a paper on a case of “ progressive 
muscular atrophy without disease of the ganglion cells of 
the anterior horns.” A condensed history of his case is as 
follows : 
Louise Groth, aged forty-two. With exception of an acute 
fever (typhoid), was well until the age of twenty-seven. Severe 
pains in right arm lasting one day and followed next day by 
weakness in the arm. Continued her work as laundress for 
nearly a year in spite of increasing weakness. Was improved 
by treatment and resumed her work. After another year, a 16 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
severe confinement (forceps); formation of abscess around right 
ankle ; right leg grew weaker ; weakness and atrophy of right 
upper arm were superaddtd. Worked with interruptions for 
seven years longer and gave up work two years and a half pre- 
vious to Lichtheim’s examination. No hereditary influences. 
From the description of loss of function,! infer that the follow- 
ing muscles must have been affected : Muscles, wasted—serrati, 
right > left; latissirni, right > left; pectorals, right > left; 
deltoids, right > left; lower portion of trapeze!, right > left; 
and various facial muscles (can not pull up nose, frowning dif- 
ficult, can close eyes, can not whistle, no difficulty in mastica- 
tion or deglutition). Wasted also—intercostals, supraspinati, 
biceps brachialis, and supinator longus of both sides; no atro- 
phy of forearms or triceps. Thenar and hypothenar wasted, 
left > right. Opponens pollicis in fair condition ; tremors 
when fingers are moved. Gluteal muscles wasted; left calf 
slightly larger than the right; no hypertrophy. Abdominal 
muscles normal. No sensory disturbances; no reaction of de- 
generation ; patellar reflex weak but present. Death from phthi- 
sis. Autopsy (Oohnheim): Wo changes in the cord. Flexors 
of thighs show fatty degeneration ; also fatty degeneration and 
atrophy of right rectus abdominis. Muscular fibers: atrophy 
of contractile elements in diseased muscles; increase of con- 
nective tissue, and remaining fibers exhibited a highly nucleated 
perimysium internum. No changes in peripheral nerves. 
This case of Lichtheim was followed up by one of Erb 
and Schultze (44) and one of Kahler (45). The former 
authors endeavored to disprove Lichtheim’s case by a case 
of typical progressive muscular atrophy with changes in the 
cord. Erb’s criticisms were quite severe, but they have lost 
all their force since Schultze showed in later years that 
the changes which he and Erb found were not sufficient to 
account for the muscular changes, the cells that were atro- 
phied being now known to be in no physiological connec- 
tion with the muscles that wjere atrophied ; and, further- 
more, Erb has since decided that Lichtheim’s case, though PROGRESSIVE MUSCULAR DYSTROPHIES. 
a very important one, belongs to the type which Erb (34) 
first described a few years later. And to this most impor- 
tant class of cases we must now devote our attention. 
ErVs Juvenile Form.—Erb described this new form of 
disease in his “ Elcktrofherapie,” but sufficient attention 
was not paid to this juvenile form until Erb again described 
the disease in an article entitled “ Ueber die juvenile Form 
der progr. Muskclatrophie,” in the “ Dtsch. Arch. f. Idin. 
Med.,” 1884. 
The following is a typical case of Erb’s juvenile form, 
the history of which will bring out clearly enough the dif- 
ferences of this form and typical progressive muscular atro- 
pky- 
Ekb’s Case I.—Male, aged forty-six. No hereditary history, 
no syphilis ; several acute diseases in childhood. At the age of 
fifteen noticed that the right arm was weaker and thinner than 
the left. No pains or paraesthesiae. Trouble did not grow worse 
until about the age of forty; at that time the legs and left arm 
became involved ; no sensory, vesical, or sexual disturbances. 
Examination revealed changes in the following muscles : 
Wasted: Both pectorales major and minor, both trapezii, la- 
tissimus dorsi, serrati ant. maj., rhomboids with exception of 
upper portion of right rhomboid superior, both sacrolumbales 
and longissirai dorsi, deep neck muscles, levator anguli scapulae 
right > left, brachialis anticus right > left, supinator longus 
(both sides), triceps right > left, gluteal muscles right > left, 
ilio-psoas right > left, quadriceps, tensor fasciae ; anterior leg 
muscles weak with exception of tibialis anticus; abdominal 
muscles, diaphragm paretic. 
Normal: Sterno-cleido-mnstoid,levator anguli scapulae dex- 
ter, coraco-brachialis, flexors and extensors of forearm, thenar 
and hypothenar, adductors, flexors of leg, calf muscles, small 
muscles of foot. 
Hypertrophied: Deltoid left > right, infraspinati muscles, 
both teretes. 
No ataxia; patellar reflex present; no fibrillar contractions; 18 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
diminished electrical excitability of muscles, but no trace of re- 
action of degeneration. 
The other cases of Erb resembled this one in every re- 
spect, except that in at least one of his patients a later ex- 
amination revealed an incipient hypertrophy of the calves. 
Erb has taken the trouble to hunt through medical lit- 
erature, and proves very conclusively that similar cases have 
been described by Aran, Duchenne, Friedreich, Ross, and 
others, either as cases of progressive muscular atrophy or 
of pseudo-hypertrophy. Erb thus summarizes the chief 
features of this juvenile form : It is a progressive wasting 
with weakness of certain groups of muscles, beginning 
either in childhood or early youth, involving, as a rule, the 
muscles of the shoulder girdle, the upper arm, the pelvic 
girdle, the thigh, and the back, the forearm and leg mus- 
cles remaining intact for a very long time. The atrophy 
may be associated with true or pseudo-hypertrophy of some 
muscles. Fibrillar contractions and reaction of degenera- 
tion are never present. No sensory or visceral disturbances. 
He adds that the wasting is distributed in a typical manner. 
The pectorals, trapezii, latissimi dorsi, the serrati, the rhom- 
boids, as well as most of the upper arm muscles and supi- 
nators, are apt to be wasted, while the deltoids, supraspinati 
and infraspinati are either normal for a long time or hyper- 
trophied. The preservation, furthermore, of the hand and 
forearm muscles gives a very striking clinical picture. 
This disease Erb has chosen to call the juvenile form of 
progressive muscular atrophy—a very unfortunate term, 
since many of the cases exhibited no symptoms until the 
patient was well advanced in years, and others again began 
in early infancy. Erb’s description has been accepted by 
Nothnagel, Schultze, Charcot, Eulenburg, Remak, Gowers, 
and many others. 
Upon the exact distribution of the atrophy and hyper- PROGRESSIVE MUSCULAR DYSTROPHIES. 
19 
trophy, as demonstrated by bis cases, Erb lays the very 
greatest stress. According to bis view, well-preserved fore- 
arms, atrophied upper arms, hypertrophied deltoids, and 
wasted scapular muscles would be almost sufficient for a 
diagnosis of his special form. In the lower legs an almost 
analogous change occurs; the thighs and glutaei are well 
wasted, while the leg muscles and calves are well preserved. 
The question arises whether Erb did not attach too 
much importance to this exact topographical distribution 
of muscular atrophy and hypertrophy. He alleges perfect 
identity between his juvenile form and pseudo-hypertrophy; 
On page 518 he says; “If this disease occurs in earliest 
childhood and is not associated with any considerable lipo- 
matosis, the disease is what has been termed hereditary 
muscular atrophy. If it happened to be associated with 
early-developed and excessive lipomatosis, particularly in 
the lower extremities, it is synonymous with so-called pseu- 
do-hypertrophy. . . . But all of these forms are identical 
with one another and merely represent different manifesta- 
tions, a different march of the disease ( Verlaufsweisen), and 
varying degrees of intensity of the same disease.” 
The relation to hereditary muscular atrophy I will dis- 
cuss later on, but, as for its relationship to pseudo-hyper- 
trophy, is it not curious that Erb’s form is so far less fre- 
quent than the ordinary pseudo-hypertrophy ? To be sure, 
this might be explained in a number of different ways. 
First, the accuracy of description has been at fault in many 
cases. Most authors have had the hypertrophy, and that 
only, in mind, and have not, with the exception of Fried- 
reich and Gowers, paid much attention to the atrophy in 
the upper extremities; and, if detected, most authors have 
described the atrophy so poorly that a clinical picture such 
as Erb discovered can not be made out from their descrip- 
tions, This is true not only of older writers, but also of PROGRESSIVE MUSCULAR DYSTROPHIES. 
those that have written since the appearance of Erb’s paper. 
I have analyzed all recent cases of pseudo-hypertrophy for 
the purposes of clinical differentiation, but in tbe fewest 
cases have even the functional motor disturbances been 
stated with sufficient clearness to permit an inference as to 
the wasting of certain muscles, and definite statements with 
regard to the atrophy of this or that muscle are entirely want- 
ing in the majority of cases. I wish incidentally to remark 
that every case of pseudo-hypertrophy should be examined 
with the greatest care regarding the condition of the upper 
extremities, and the smallest amount of atrophy or hyper- 
trophy of any muscle should be distinctly noted. I have 
found a slight change in the faradaic response of symmet- 
rical muscles a valuable hint in getting at an incipient wast- 
ing with corresponding paresis. Such a condition would, 
in at least one case, have escaped my notice if I had not 
examined both pectorals and had found that the one gave a 
much more lively response to the faradaic current than the 
other did. 
And yet, allowing for all these possible errors, an ex- 
amination of American cases, for instance, has convinced 
me that Erb’s juvenile form is very much rarer in this coun- 
try than typical pseudo-hypertrophy is. In England, Or- 
merod (46), Ross (47), and Dreschfeld (48) are the only 
ones who have described cases resembling Erb’s form, and 
Ormerod’s case contains several atypical features.* In this 
country none have to my knowledge been published as 
cases of Erb’s juvenile form, though, as Seguin has pointed 
* In one of Ormerod’s cases there was partial reaction of degenera- 
tion in the infraspinatus, the very muscle which Erb found remained 
healthy longest; but Ormerod’s electrical examinations are not very 
satisfactory, for he alludes to the polar formula being altered with re- 
gard to the anode—a condition which is found in perfectly healthy 
muscles, PROGRESSIVE MUSCULAR DYSTROPHIES. 
out, Mastin’s cases of hereditary ataxia may be cases cf 
Erb’s form. I have not been able to get at the original 
paper of Mastin. (See Seguin’s abstract in “ Ann. of the 
Univers. Med. Sci.,” i, 1888.) 
Daring the past two years I have waited patiently for 
an example of Erb’s form to turn up, without, however, 
meeting with a single one. This disease may be as much 
less frequent in America as the Landouzy and Dejerine type 
is less frequent in Germany than it is in France. Further- 
more, the thought naturally occurs to one that Erb’s special 
form may represent in many instances a late stage of 
pseudo-hypertrophy, and that the majority of cases of this 
disease dying at an early age never reach this stage. And 
yet we must not forget that Erb has described several cases 
of his typical form beginning at a very early age; and, on 
the other hand again, we well know that cases of typical 
pseudo-hypertrophy may be associated with atrophy in the 
upper extremity without this atrophy assuming Erb’s char- 
acteristic distribution, as proved by my own case (No. II), 
cited above. 
In view' of such cases as this one and the reasoning fol- 
lowed above, it seems to me that the topographical distribu- 
tion of the atrophy or hypertrophy can not be depended 
upon to prove the absolute identity between pseudo-hyper- 
trophy and the juvenile form, and that for the present 
pseudo-hypertrophy deserves the rank of a special form. 
Their relationship seems to me, however, to rest upon sev- 
eral cardinal symptoms which they have in common : 
First. Upon a progressive wasting beginning in early 
life, associated with hypertrophy at some time during the 
course of the disease. 
Third. Upon the absence of the reaction of degenera- 
tion. 
Second. Upon the entire absence of fibrillar contractions. 22 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
Fourth. Upon the absence of changes in the spinal cord, 
the autopsy in Lichtheim’s case going to prove this last 
statement. My friend Dr. Gray (“ New York Med. Journal,” 
May 18, 1888) is therefore wrong in stating that no autopsy 
has ever been obtained in a case of Erb’s type. 
These cardinal symptoms several other forms of mus- 
cular atrophy have in common with tbe two forms just dis- 
cussed. 
We have now to turn our attention to another type, to 
the so-called hereditary form of progressive muscular atro- 
phy. This type was created by Leyden (49) and warmly 
advocated by Mobius (50). According to Leyden, this 
form is characterized as follows : 
The hereditary form of progressive muscular atrophy 
attacks several members of the same family. It appears at 
an early age, as a rule between the eighth and tenth years, 
in one case not before thirty. Males are more apt to be 
attacked than females (the elder Eulenburg, however, de- 
scribed the affection in three sisters of one family). The 
disease begins invariably with weakness in the back and 
lower extremities, and in these regions a wasting of the 
muscles is first observed. After a lapse of years the mus- 
cles of the upper extremities may be involved. Occasion- 
ally the patient may attain to an old age. Atrophy may 
become so extreme that the patients are absolutely helpless. 
The march of the disease is steadily progressive. Elec- 
trical reactions normal; no fibrillar contractions. The atro- 
phy is associated with hypertrophy, particularly of calf 
muscles. No sensory disturbances, no disturbances of 
speech, of deglutition, or of ocular movements. 
Leyden records the case of a man thirty-seven years of 
age who had trouble in walking from early childhood on, 
and who had decided atrophy of back and thigh muscles, 
with vast increase of calf muscles, without any involvement PROGRESSIVE MUSCULAR DYSTROPHIES. 
23 
of shoulder and arm muscles. The geueral symptoms were 
of the kind stated above. Leyden counted among this 
class of cases a well-known one of Meryon (51), the cases 
of Oppenheimer (52), of Heraptenmacher (53), of Bern- 
hardt (54), and of Eichhorst (55); but all of these cases 
have been considered by most other and later authors to 
belong to the type of pseudo-hypertrophy. Leyden has 
been followed by Mobius, by Zimmerlin (56), by Landouzy 
and Dejerine (57), by Schultze (58), and by others in the 
description of this type ; but of these Mobius and the French 
authors alone can be said to be advocates of this special 
form. 
In my opinion, there is not sufficient reason to create a 
separate type of disease on the points laid down by Ley- 
den. . First, all forms of muscular atrophy may be and often 
are hereditary. This is particularly true of pseudo-hyper- 
trophy. Second, cases with distinct heredity often start in 
the upper extremities; and third, all cases beginning with 
weakness and atrophy in the back and leg muscles are not 
necessarily hereditary, as we shall see when we come to the 
consideration of my case of the peroneal type of progressive 
muscular atrophy. 
As regards the first point, in the cases of Oppenheim, 
Friedreich, and Heraptenmacher the disease began in the 
muscles of the back, but spread to the upper extremities 
instead of the lower. Barsikow (59) has described a num- 
ber of cases occurring in two families. In the members of 
the one family the disease attacked back and leg muscles; 
in the other family the spreading of the atrophy was not 
uniform, attacking the leg muscles in one member and in 
another the shoulder first and then the leg muscles. Zim- 
merlin (loc. cit.) published seven cases—four in one family 
and three in another. In the one family the four cases are 
distinctly of the juvenile type, while in the second family 24 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
the two cases began in the upper extremities, leaving the 
legs intact, while in the third case there was involvement 
of upper and lower extremities and even involvement of 
face muscles—an approach to the type Landouzy-Dejerine. 
Schultze (loc. cit.) describes the cases of two brothers, 
one affected with typical pseudo-hypertrophy, and the other 
with a general wasting of the upper and lower extremities. 
In this country Harrington (59a) has reported seven cases 
in which the onset was in the legs in some, in others in the 
legs and arms simultaneously, and in still others the legs 
were affected first, and only a year later the arms. Or- 
merod’s cases of muscular atrophy in three children after 
measles might be used to show the same differences in the 
mode of onset (cf. No. 70). 
We have, therefore, good reason for insisting that. Ley- 
den’s hereditary form is not entitled to rank as a special 
type of progressive muscular atrophy ; that pseudo-hyper- 
trophy and Erb’s juvenile form are distinctly hereditary, 
and, furthermore, that cases with a distinct heredity are by 
no means necessarily characterized by an atrophy first at- 
tacking the muscles of the back and thighs. All of Ley- 
den’s cases would properly come under the head of pseudo- 
hypertrophy of Erb’s juvenile form, or of the “ peroneal 
type.” 
The next type of progressive muscular atrophy—the type 
facio-scapulo-hurneral, type Landouzy-Dejerine, the infan- 
tile progressive muscular atrophy of Duchenne—can not be 
disposed of so easily. 
Cases of progressive muscular wasting with involvement 
of face muscles have always been considered rare. Du- 
chenne described several; Remak (60), Mossdorf (61), Bern- 
hardt (62), Kreske (63), and Westphal (64) have each de- 
scribed one or two cases, but Landouzy and Dejerine (loc. 
cit.) have succeeded in calling renewed attention to this PROGRESSIVE MUSCULAR DYSTROPHIES. 
25 
form, have made the most careful examinations, and have 
obtained a post-mortem examination in one case. For this 
reason it is just to refer to the features of this type as laid 
down by Landouzy and Dejerine, who have seen more cases 
than all other recent authors taken together. 
The Type Landouzy-Dejerine.—This form of progressive 
muscular wasting begins, as a rule, in early life, and in the 
majority of cases in the muscles of the face, giving rise to 
what the authors term the “ facies myopathique.” The lips 
are considerably thickened, and constitute the “ louche de 
tapir.” Great stress is laid upon this tapir-mouth appear- 
ance. Later on in the course of the disease the atrophy 
spreads to the shoulder and arm muscles; the supraspinati 
and infraspinati, the subscapularis, the flexors of the hand 
and fingers remain normal. The muscles of deglutition, 
mastication, and respiratory and laryngeal muscles, as well 
as the ocular muscles, remain normal. In exceptional cases 
the disease may begin in the shoulder or arm muscles, el- 
even .in the lower extremities. The disease is distinctly 
hereditary. Fibrillar contractions and reaction of degenera- 
tion are never present. 
In their first paper Landouzy and Dejerine published cases 
occurring in two different families; in the first the disease 
could be followed up through five generations. Cases that 
are described relate to a father and four sons, five other chil- 
dren not having been affected. The history of one son is 
characteristic. 
The trouble began at the age of three with atrophy of face 
muscles; no other symptoms observed up to the age of seven- 
teen. From that time on atrophy was noticed in the muscles 
of the shoulder and arm, spreading to the trunk. At the age of 
twenty-one atrophy had become extreme—“ nothing but skin 
and bone”—-facies myopathique and louche de tapir. Sensation 
normal, sphincters also; patellar reflex lost, electrical excitabil- 26 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
ity diminished in proportion to the wasting, but no reaction of 
degeneration. At the age of twenty-four, death from phthisis. 
Autopsy.—Atrophy determined as follows: Frontalis, orbi- 
cularis palpebrarum, zygomatici, orbicularis oris, and buccinator 
of both sides (levator anguli oris, normal), trapezius, deltoid 
(infraspinati and supraspinati, subscapularis, teres major and 
minor, normal), biceps, brachialis anticus, and coraco-brachia- 
lis, triceps, supinator longus and extensor radialis (supinator 
brevis, flexor digitorum sublimis et profundus, normal), exten- 
sor pollicis longus and extensor indicis (extensor digitorum 
communis, extensor digiti minimi, extensor ulnaris, normal), 
abductor longus and extensor pollicis brevis slightly wasted, ab- 
ductor brevis pollicis wasted, other thenar and hypothenar mus- 
cles normal. Lurnbricalis distinctly wasted, and interossei 
slightly wasted; pectorals wasted; serrati and sacro-lumbar 
normal. Lower extremities not so carefully examined; glutsei 
were atrophic; no marked lipomatosis anywhere; no changes 
in the nervous system. Diseased muscles revealed simple atro- 
phy of primitive muscular fibers; slight traces of increase of 
interstitial connective tissue and of fat. No increase in muscu- 
lar nuclei. 
The histories of the cases of Reraak and of the other 
authors quoted are very similar. In some the atrophy set 
in in the extremities first, and in the face later on. In Re- 
mak’s case both sides of the face were involved ; in Kreske’s, 
the one side only. 
The similarity between this form and Erb’s will be ap- 
parent to every one at a glance; it is practically Erb’s form 
plus involvement of face muscles. Erb never observed this 
complication in his own cases, and Landouzy and Dejerine 
argue that their cases are different on account of the ab- 
sence of lipomatosis and the presence of facial symptoms. 
As for Erb’s never having observed the facial atrophy in 
any of his cases, it is worth noting that in a later paper 
(65) Landouzy and Dejerine publish a case (No. VI) of 
their form in which the face muscles appeared normal dur- PROGRESSIVE MUSCULAR DYSTROPHIES. 
ing life, but, on post-mortem examination, revealed decided 
morbid changes. It is possible, therefore, that the same 
changes were present in some of Erb’s cases without so ex- 
cellent an observer as lie being able to detect them. If this 
is allowed (and the French authors themselves urge the 
possibility of this), there is no just reason for making a 
separate type for such cases as they describe. They deny 
the resemblance between the two forms in consequence 
also of the invariable absence of lipomatosis; but Westphal 
(66) again seems to have found a decisive case which shows 
that the face muscles may be associated with typical pseudo- 
hypertrophy, and it must be remembered that Landouzy 
and Dejerine grant that they have found hypertrophied 
fibers in some of the muscles. We can not, therefore, see 
the propriety of creating a separate type such as Landouzy 
and Dejerine have described. There is a slight difference 
between their cases and those of Erb in the topographical 
distribution of the atrophy, and even this is doubtful, while 
their cases resemble Erb’s form in the involvement of the 
upper arm and shoulder muscles chiefly, in the presence of 
hereditary influences, in the absence of fibrillar contractions, 
and absence of reaction of degeneration. 
I wish, however, to enter a special plea for the recogni- 
tion of still another type—the peroneal type of progressive 
muscular atrophy. This form was first described by Char- 
cot and Marie (67), and, independently of them, by Dr. 
Tooth (68), of England, in a Cambridge thesis. Charcot and 
his associate reported five such cases, Tooth four cases, and 
Herringhara (69) has recently reported one case in a family 
in which various members in successive generations have 
been similarly affected. To this list lam able-to add one 
case of considerable interest; and similar cases, although 
not designated by this title, have been described by Ham- 
mond (Weathersbee ail), by Orrnerod (70), by Schultze (71), 28 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
and, no doubt, some other of the cases of hereditary muscu- 
lar atrophy would more properly belong to this class. 
The Peroneal Form of Progressive Muscular Atrophy 
begins in early youth, or may, as in one of Charcot’s cases, 
attack a person beyond the age of puberty. There may be 
distinct family inheritance. According to Herringbam, as 
a rule, boys inherit the disease through the mother, as has 
been shown to be the rule in cases of pseudo-hypertrophy. 
The atrophy begins in the lower extremities, first attacking 
the extensor hallucis longus, then the common extensors of 
the toes, and then the peronei; the small muscles of the 
foot may be affected as well. The calf muscles atrophy a 
little later, while the muscles of the thighs offer greater re- 
sistance, and do not undergo atrophy until the disease has 
well run its course. Several years after the onset of the 
disease in the legs the hands become involved; the inter- 
ossei, the muscles of the thenar and hypothenar, as well as 
the muscles of the forearm, become wasted ; the supinator 
longus, the muscles of the shoulder, of the neck, trunk, and 
face, remain normal. The atrophy need not be entirely 
symmetrical. Fibrillar contractions occur occasionally; the 
reaction of degeneration is present in some muscles ; the 
skin reflexes remain normal. 
My own case is as follows: 
Author's Case III.—R. J., a Russian girl, aged twelve, was 
referred to my department at the Polyclinic by Dr. Gibney. She 
is the third child of healthy parents; two born later died—one of 
diphtheria, and one of cerebral trouble after a fall. No disease 
similar to the one from which this patient suffers has been known 
in any branch of the family. While carrying this child, the 
mother was considerably troubled with swollen feet and legs, 
possibly of nephritic origin, but is now a healthy, stout woman. 
The child was asphyxiated when born; no doctor in attendance. 
Patient to walk at nine months; had a slight fall at the 
age of ten months vyithout doing any injury to herself. At the PROGRESSIVE MUSCULAR DYSTROPHIES. 
29 
age of three, mother noticed that there was something wrong 
with the right knee, and in the hospital in St. Petersburg a plas- 
ter-of-Paris splint was put on. This the child wore for seven 
weeks. She could walk perfectly well after that, and played 
as well and ran as fast as any child. Has had a number of dis- 
eases—measles at the age of one year, small-pox at the age of 
four, scarlet fever at the age of six, and typhoid fever at six and 
a. half. In spite of all, recovered and walked perfectly well. 
Came to this country one year ago; nine months ago fell on left 
hip, and for some weeks had pain in left hip. While recovering 
from this fall she noticed that she had difficulty in moving the 
toes of the right leg. This is now five months ago. The im- 
pairment of motion gradually grew worse until the child was 
not able to move the toes at all. Never had pain on her right 
side. Her present manner of walking developed very slowly. 
At first sight her gait seemed to be characteristic of poliomye- 
litis. Child complains of fatigue, particularly in mounting 
stairs; no other special symptoms. Patient was a bright girl; 
no hysterical tendencies. The history shows that the present 
condition of paresis developed slowly, and was not preceded 
either by convulsions or fever. Furthermore, that there was 
no pain accompanying the paresis at any time. Has distinct 
feeling of movement under the skin. 
Examination.—Girl of medium size. Upper extremities, 
good grasp with both hands. Forearm muscles and hand mus- 
cles well developed; supinators, also biceps and deltoids, well 
marked, the latter not hypertrophied. Trapezii and rhom- 
boids of normal strength ; right pectoral a little thinner than 
left. Eight shoulder blade shows slight winged appearance. 
Right serratus slightly weakened. Distinct wasting of the 
right leg, thigh, and gluteal region. The leg muscles of the 
right side more distinctly atrophied than the thigh muscles. 
The child can not lift toes of right foot while resting the heel 
on the ground. The same movement can be performed fairly 
well on the left side. Can not raise herself on tip-toes on the 
risht side, hut can do so with tiie left foot. Posterior sur- 
faces of thighs proportionately less developed than anterior 
surface, Right extensor quadriceps very weak; left weaker 30 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
than normal, hut stronger than on the right side. Evident 
atrophy, therefore, of anterior tibial and posterior tibial group 
of right leg, of posterior thigh muscles, and the glutaei muscles 
of right side. In the lying position, ten centimetres below 
lower edge of the patella, right leg, 23£ ctm.; left leg, ctm. 
Eighteen centimetres below iliac crest, right thigh, 87 ctm.; left 
thigh, 89 ctm. 
Knee-Jerk absent on right side; on left side it was impossi- 
ble to obtain the knee-jerk for several weeks; it is now present, 
however, and very lively. Occasional fibrillar contractions. 
No sensory disturbances anywhere. No rectal or visceral 
symptoms. The triceps tendon reflex present on both sides but 
weak. Occasional fibrillar contractions have been noticed. 
Electrical Examination.—Faradaic examination of all nerves 
and muscles gives satisfactory responses except in the case of 
the right peroneal nerve, which exhibits diminished faradaic 
excitability. On faradaic excitation of peroneal nerve, tibialis 
anticus muscle contracts very feebly. Serratus also responds 
more powerfully on the left side than on the right to current of 
moderate strength. Left pectoralis major does not respond as 
well as right to faradaic current. Galvanic examination satis- 
factory. The following alone need be mentioned : Examination 
with the 10 ctm square electrode: Right peroneal nerve, KCO, 
2J ma.; AOC, ma.; ACC, ma. Left peroneal nerve, 
KCO, 2£ ma.; AGO, ma.; AOC, 6J ma. Right tibialis 
anticus muscle, direct examination, KCO, 7 ma.; AOC, 8 ma. 
Left tibialis anticus, KCO, ma. ; ACC, 6 ma. Electrical ex- 
amination thus shows a decided diminution of response to the 
faradaic current, and to the galvanic current as well in the 
tibialis anticus of the right side, the KCO being almost equal to 
the AGO. Ormerod would have said that the right peroneal 
nerve showed reaction of degeneration with regard to the 
anode, but this, I insist, is nothing morbid. We have, there- 
fore, slight electrical changes in a single muscle; the other 
muscles of the peroneal group respond normally. 
The diagnosis in this case could have rested only be- 
tween acute anterior poliomyelitis, a peripheral neuritis, or PROGRESSIVE MUSCULAR DYSTROPHIES. 
31 
this form of progressive muscular atrophy. The mode of 
onset, gradually and without pain, without fever or con- 
vulsions, argues against a poliomyelitis anterior acuta, as 
well as against peripheral neuritis. The atrophy, too, is 
not as great as we would expect in a case of spinal infantile 
palsy. All of the symptoms—the paralysis proportionate 
to the wasting of the muscles, the absence of the knee- 
jerk, and the slight changes in electrical reaction—can be 
best explained by the diagnosis we have made. Further- 
more, the disease is not retrogressive as poliomyelitis acuta 
would be, but gradually progressive, and the slight indica- 
tions of this progression in the muscles of the trunk lend 
further support to the view of a progressive muscular atro- 
phy, which is strengthened still more by the occasional 
presence of fibrillar contractions. 
The diagnosis in such cases as these must be made with 
the greatest care, but I have no doubt that some of the 
cases which have hastily been put down as cases of periph- 
eral neuritis will prove to be cases of this type. From 
poliomyelitis anterior acuta it will not be difficult to differen- 
tiate this disease, nor from neuritis. It will be more difficult 
to distinguish between these cases and those of a wide- 
spread atrophy following traumatic joint lesions, in which, 
as I have seen a number of times, the atrophy may spread 
with surprising rapidity, and may affect the entire ex- 
tremity. We must, therefore, either rely upon the history 
in these cases, upon the presence or absence of fibrillar 
contractions, or must exclude a purely traumatic atrophy in 
case the atrophy jumps from the affected part to some 
other portion of the body. 
In many cases of progressive muscular atrophy of the 
typical form the histories show that the disease was first 
noticed after some accident. The question therefore arises 
whether it may not be possible for typical progressive mus- 32 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
cular atrophy to develop after a joint lesion in a subject 
predisposed to this disease. 
We have now to consider the relations of this peroneal 
form of progressive muscular atrophy to the other primary 
dystrophies which we have discussed. 
It will be seen at once that the anatomical distribution 
is entirely different from the four forms of primary myopa- 
thies discussed above. If the atrophy spreads to the upper 
extremities, it involves the muscles more after the fashion of 
a Duchenne’s atrophy than after the fashion of a pseudo- 
hypertrophy or an Erb’s form of atrophy. The analogy to 
Duchenne’s form becomes still closer when we consider that 
this peroneal form is distinguished from the other myopa- 
thies by the occasional presence of fibrillar contractions and 
by alterations in the reaction of degeneration. The spread- 
ing of the atrophy from the muscles of the big toe and the 
small muscles of the foot to the muscles of the legs and 
thighs reminds one of the manner in which the atrophy 
spreads in the upper extremities in cases of typical progres- 
sive muscular atrophy. There seems, therefore, to be good 
reason to separate this form from the simple muscular my- 
opathies and to make it a subdivision of typical progressive 
muscular atrophy. This form might be properly entitled 
the leg type, in contradistinction to the hand type which 
would represent the ordinary form of Duchenne’s progres- 
sive muscular atrophy. 
If the ordinary progressive muscular atrophy is a polio- 
myelitis anterior chronica cervicalis, the leg type might rep- 
resent a poliomyelitis anterior chronica lumbalis. But this 
is speculative pathology and needs corroboration, as indeed 
all the clinical and anatomical features of this form do. 
In the preceding pages I have given an account of the 
commonly received forms of progressive muscular wasting. 
Some cases will surely be found that can not properly be PROGRESSIVE MUSCULAR DYSTROPHIES. 
33 
classed under any one of these heads. Schultze’s case, for 
instance, had some of the features of pseudo-hypertrophy, 
some of those of Erb’s form, and in the presence of the 
fibrillar contractions and reaction of degeneration in some 
muscles approached to the type of typical progressive mus- 
cular atrophy. I have had occasion to observe one case in 
a child about seven years of age in which there was a gen- 
eral wasting of all the muscles of the body excepting those 
of the head. The power of the legs and arms was weak, 
without there being any actual paralysis. There was a 
winged appearance of the scapulae, but there were no other 
disproportionate atrophies or hypertrophies anywhere in the 
body. The wasting was an entirely uniform one. Such a 
case as this one is mentioned by Charcot in his recent vol- 
ume (72) and by Gowers in his text-book. Baeg {loc. cit.) 
and Oppenheim (73) have reported cases with involvement 
of the face, tongue, laryngeal and ocular muscles, which it 
is impossible at present to classify under any of the ordi- 
nary forms of progressive muscular atrophy. 
There is good reason, therefore, for allowing that there 
are mixed cases of progressive muscular wasting, and that 
the exact rank of these cases can not be determined at pres- 
ent, except that, according to their cardinal symptoms, they 
should be classed either with the spinal or primary my- 
opathies. 
To complete this study of the various forms of progres- 
sive muscular atrophy, it will be necessary to add a few 
words regarding the histological changes in the muscular 
tissue. Let me say byway of preface to these remarks that 
no proper inferences can be drawn from mere harpoon ex- 
aminations. All such cases I have therefore excluded from 
the present consideration, and have used with considerable 
caution even those cases in which pieces of muscle were re- 
moved by the knife. As in my own case of pseudo-hyper- PROGRESSIVE MUSCULAR DYSTROPHIES. 
trophy, I believe that very limited inferences only can he 
drawn from the examination of such small pieces, and that, 
after all, post-mortem examinations alone are entirely satis- 
factory in giving evidence regarding all the changes that 
may occur in the atrophied muscles. 
If the histological examination of muscles is to he of 
value in making a differential diagnosis between the various 
forms of muscular atrophy, it must help us, first of all, to 
differentiate between the primary muscular dystrophies and 
the spinal forms of progressive muscular atrophy. 
The changes in muscles atrophied from spinal lesions 
are: A change in the striation of the fibers and a narrowing 
of the fibers, an increase in the number of muscle nuclei, 
and possibly segmentation of the nuclei; granular or fatty 
degeneration of the fibers, and occasional globules of fat be- 
tween the muscle-fibrils, I need not add that some or all 
of these changes will be present according to the length of 
time the atrophy has existed, and all of these changes have 
been found both in spinal forms and in primary dystro- 
phies, but the increase in the muscle nuclei is never as great 
in the latter as in the former form. Furthermore, in the 
spinal forms hypertrophied fibers are never found, while 
they are common in the purely muscular types. Muller is 
the only author who records an exception to this rule, in a 
case of poliomyelitis of old standing. In spite of this fact, 
we may say that the presence of any considerable number 
of hypertrophied fibers, side by side with atrophied and de- 
generated fibers, is sufficient proof to exclude such a case 
from the spinal form. Roth (74) professes to be able to 
differentiate between atrophy from spinal lesion and atrophy 
following nerve lesion. He states, for instance, that the 
atrophy of the muscular fibers in progressive muscular atro- 
phy affects the length of a fiber only, causing a shortening, 
while the breadth of the fiber remains normal. In cases of PROGRESSIVE MUSCULAR DYSTROPHIES. 
35 
muscular atrophy after nerve lesion there is, according to 
this author, simple atrophy and interstitial sclerosis. 
And now the question arises. Is it possible to differenti- 
ate by histological examination between the various primary 
myopathies? The histological changes of ordinary pseudo- 
hypertrophy are too well known to need a detailed descrip- 
tion in this place. We have, as a rule, narrowing of fibers 
with changes in their contour, granular or fatty degenera- 
tion of the fibrils and accumulation of fat globules between 
the fibrils, and increase without marked proliferation of the 
connective tissue. In pieces of muscles excised from the 
living subject, enlarged fibers will be seen in the immediate 
vicinity of fibers of normal or less than normal dimensions. 
In post-mortem examination of muscles in pseudo-hypertro- 
phy we need not expect to find fibers of unusual breadth, 
for the examination generally is made at a time when atro- 
phy has supervened upon a preceding stage of hypertrophy. 
A slight increase in the muscle nuclei is often found in typ- 
ical cases of pseudo-hypertrophy, but unusual increase is sus- 
picious of muscular disease of spinal origin. It was on the 
strength of this increase of these nuclei that Friedreich in- 
sisted that typical progressive muscular atrophy began as a 
myositis. Schultze (Joe. cit., p. 24) mentions peculiar giant- 
cell formation as occurring in cases of pseudo-hypertrophy 
which he regards as the residue of muscular protoplasm with 
its proliferated nuclei. 
Jacoby (75) thinks that the disease consists in the main 
of a chronic inflammation invading both the perimysium 
and the muscle tissue, and would call the process a myositis 
progressiva hyperplastica. The careful drawings Jacoby has 
made are very different from the pathological findings in 
other cases and need further corroboration ; besides, his 
case is a pseudo-hypertrophy of rather unusual type, exhibit- 
ing, as the author says, “hypertrophy to an enormous ex- 36 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
tent of the deltoids, infraspinati, biceps, triceps, and costal 
portion of the pectoralis major.” He likens his case to 
Case X of Friedreich, and this is now taken to be a case of 
Erb’s juvenile type. Westphal, in his typical case of pseu- 
do-hypertrophy, found, on post-piortem examination, enor- 
mous increase of adipose tissue in which the muscular fibrils 
were nearly of normal size ; increase of the interstitial con- 
nective tissue ; no hypertrophic fibers ; strands of connect- 
ive tissue occasionally pass through the fatty tissue, few of 
the muscular-fiber groups appearing to be strangulated by 
strands of connective tissue. 
The sections from the vastus externus of my case of 
pseudo-hypertrophy have been cut from tissue hardened in 
Muller’s fluid and alcohol, and stained with alum-carmin, 
hsematoxylin, and eosin - hsematoxylin. The specimens 
show slight loss of striation, slight increase of connective 
tissue and its cells, and a number of enlarged fibers crowd- 
ing in upon other and narrower fibers. The changes are 
slight in degree, and 1 have no doubt that if a piece had 
been excised from the midst of the muscle, instead of from 
its surface, the specimens would have exhibited more de- 
cided changes; but, slight as these changes are, they con- 
firm me in the belief that hypertrophy is a very marked 
and early change, and is associated with early increase in 
the connective tissue. 
In Schultze’s case, which stands midway between pseudo- 
hypertrophy and Erb’s juvenile form, the muscles were 
examined more exhaustively than ever before. Schultze 
found, in addition to the giant-cell formation alluded to 
above, large numbers of fat cells in the muscular tissue, an 
increase of connective tissue and remnants of hypertro- 
phied, normal, and atrophic fibers, and an enormous increase 
of nuclei which the author thinks greater than in ordinary 
cases of pseudo-hypertrophy, though he quotes one case of PROGRESSIVE MUSCULAR DYSTROPHIES. 
37 
Erb’s, of pare pseudo-hypertrophy, in which the finding was 
entirely similar. The vacuoles which were present in 
Schultze’s case represent a very late stage in the pathologic- 
al process, and, if developed to the extent they were in 
this case, can not be due to hardening. 
In Erb’s juvenile form most of the conditions are ex- 
actly the same, but it is important to note a few points of 
difference. In his first paper, in the “ Archiv f. klinische 
Medicin ” for 1884, Erb insisted that the hyperplasia and 
proliferation of the interstitial connective tissue, and next in 
order the considerable deposit of fat in this connective tis- 
sue, were the most characteristic histological features of 
the disease (p. 492); the atrophy of the fibers and the pres- 
ence of a few hypertrophic fibers were thought to be feat- 
ures of secondary importance. In his later articles Erb 
changes his original opinion and corroborates the hypoth- 
esis first put forth by Barsikow—viz., that the hypertrophy 
of the muscular fibers is the primary and most characteristic 
change; this hypertrophy may finally pass into an atrophy. 
Other and secondary features are a rounding off of the con- 
tours of the fibers, an increase in the muscle nuclei, an in- 
crease of connective tissue and its nuclei, and fissuration 
and vacuolization of the fibers. Lipomatosis may occur. 
Hitzig (78), the latest author on this subject, has pub- 
lished very careful researches which tend to corroborate 
Erb’s views. Hitzig has examined four cases; three are 
typical, one decidedly atypical and suggestive, I think, of 
amyotrophic lateral sclerosis. He concludes that “the pri- 
mary and most important change in juvenile atrophy does 
not consist of any interstitial process, but is decidedly par- 
enchymatous, and, according to the intensity of the dis- 
ease, is represented by slight or excessive hypertrophy of 
the fibers. . . . The anatomical changes in pseudo-hyper- 
trophy, on the other hand, are characterized by active 38 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
changes in the connective tissue.” If this proves to be 
correct—and there is a strong probability in its favor—it 
will corroborate the conclusion I arrived at by an analysis 
of the clinical facts—that pseudo-hypertrophy and Erlds 
juvenile form can not be considered entirely identical. 
Hitzig expresses the opinion also that the atrophy of most 
fibers is due to mechanical compression by hypertrophied 
fibers, the hypertrophy being due to an irritative process. 
The atrophy of the fibers is followed by the deposit of fat, 
the latter being a mere substitute for the diminished mus- 
cular tissue. 
In cases that developed most rapidly the atrophy is 
therefore most extreme. This compression theory of Ilit- 
zig will need further corroboration. Singer (*79) is in 
agreement with Hitzig, except that he regards the increase 
of connective tissue as the first stage in the production of 
fat. 
As to the histological changes in Landouzy and Deje- 
rine’s type, we must rely chiefly upon the changes reported 
in the one case on which Landouzy and Dejerine held an 
autopsy. They found simple atrophy or entire disappear- 
ance of the primitive muscular bundles, a few hypertrophic 
fibers, but note that the case had taken an exceedingly 
rapid course. No indication of degenerative atrophy ; very 
slight increase of interstitial connective tissue and fat, which 
would seem to prove that the atrophy must be due to some 
cause within the muscle fibers; no unusual increase of the 
nuclei. All this, therefore, is in agreement with Erb’s 
form, and goes to prove the close relationship between these 
two forms. 
The changes in the peroneal form of progressive mus- 
cular atrophy have not been reported upon by Charcot and 
Marie, Tooth, Herringham, or Hoffman (80). In ray own 
case I have not yet obtained permission to excise a piece of PROGRESSIVE MUSCULAR DYSTROPHIES. 
39 
muscle, but hope to do so before long, and it will be inter- 
esting to note whether the changes resemble those of pri- 
mary dystrophies or are similar to those found in muscular 
atrophy from nerve and spinal lesions. Leyden states that 
in the hereditary form the muscular changes are the same 
as in typical progressive muscular atrophy, but his heredi- 
tary cases presumably include the peroneal type, and thus 
his statements might be taken as an indirect histological 
O o 
proof of tlie close relationship between the peroneal form 
and typical progressive muscular atrophy. 
From this survey of the histological researches in various 
forms of muscular atrophy we conclude that an examination 
of muscular changes may help us to differentiate between 
typical progressive muscular atrophy and the primary my- 
opathies ; and again, if Ilitzig is correct, between pseudo- 
hypertrophy and Erb’s juvenile form. There docs not, 
however, appear to be a marked distinction between Erb’s 
juvenile form and the remaining primary dystrophies, the 
histological changes in the peroneal form being still unde- 
termined. 
The argument which has been held throughout these 
pages leads to the following conclusions : 
1. Progressive muscular atrophy, type Aran-Duchenne, 
is due to spinal-cord disease. The peroneal type of pro- 
gressive muscular atrophy bears close resemblance to this 
form and may possibly have a similar pathology. 
2. Duchenne’s type of progressive muscular atrophy 
might be termed the hand type, while the peroneal form 
would represent the leg type. 
3. Pseudo-hypertrophy is not of spinal origin. Lipo- 
matosis is a mere incident in the course of the disease and 
is associated with wide-spread atrophy in various parts of 
the body. 
4, There is a close relationship between pseudo-hyper- PROGRESSIVE MUSCULAR DYSTROPHIES. 
trophy and Erb’s juvenile form of progressive muscular atro- 
phy, but not an absolute identity. This close relationship 
is marked by the onset of the disease at an early age, by the 
entire absence of fibrillar contractions in both forms, by 
the absence of reaction of degeneration, and by the occur- 
rence of lipomatosis some time during the course of the 
disease. They differ from each other in the distribution of 
the muscular atrophy, and possibly in the histological 
changes in the affected muscles. 
5, Hereditary muscular atrophy does not deserve the 
rank of a separate clinical entity, all forms of primary my- 
opathies being occasionally hereditary. 
6. The type Landouzy and Dejerine is closely related 
to Erb’s form, the additional involvement of the face mus- 
cles not being a sufficient basis for a wide clinical differen- 
tiation. 
7. Pseudo-hypertrophy and Erb’s form should be re- 
garded as the two representative forms of primary progres- 
sive dystrophies. 
8. Primary progressive dystrophies are distinguished 
from spinal progressive dystrophies by their cardinal symp- 
toms—the onset at an early age, the occurrence of true or 
false hypertrophy, the absence of the reaction of degenera- 
tion, and the absence of fibrillar contractions. 
This paper can not be properly closed without reference 
to the subject of classification. The term “ progressive 
muscular atrophy ” has been variously used both to desig- 
nate the fact of a general and progressive muscular wasting, 
and also as the proper name for Duchenne’s type of atro- 
phy. This has led to great confusion, and it would be 
well if the term “ progressive muscular atrophy ” were to 
be used in a generic sense merely, and if some other name 
were found for Duchenne’s type. Erb’s suggestion seems 
to me to be a good one, and I therefore propose to desig- PROGRESSIVE MUSCULAR DYSTROPHIES. 
nate the type Aran-Duchenne as spinal progressive amy- 
otrophy. 
If ray argument against the validity of anatomical dis- 
tribution of atrophies or hypertrophies as a basis for classi- 
fication be accepted, the classification of muscular atrophies 
could be reduced to the following simple form : 
1. Amyotrophia spinalis progressiva: 
a Hand type ; 
/3 Leg type = peroneal form. 
2. Primary progressive dystrophies : * 
a Pseudo-hypertrophy ; 
/3 Erb’s form. 
Notes and References. 
1. Duchenne, “ Mbmoires de I’acacl. des sciences,” 1849. 
2. Aran, “ Archives g6n. de med.,” 1850. 
3. Oruveilhier, “Arch. gen. de med.,” 1853. 
4. Duchenne, “L’Rlectrisation localistie,” 3d ed., p. 486, 
1872. 
5. Osier, “ Arch, of Med.,” vol. iv, December, 1880. 
6. Charcot et Joffroy, “Arch, de physiol.,” 1869. 
7. L. Clarke, various articles in “British and Foreign Med.- 
chirurg. Rev.,” 1862; Beale’s “Arch.,” 1867; “ Med.-chirurg. 
Transactions,” vol. li, etc. 
8. Hayera, “Arch, de physiol.,” 1869. 
9. Charcot, “ Maladies du systerne nerveux,” vol. ii, p. 209. 
10. Idem, p. 206. 
11. Bamberger and Recklinghausen, “Wiener med. Presse,” 
1869. 
12. Friedreich, “Ueber progressive Muskelatrophie,” 1873. 
13. Dumesnil, “ Gaz. hebdom ,” 1867. 
14. Clarke and Gairdner, Beale’s “Arch.,” 1861. 
15. Clarke and Cooper, “ Med.-chirurg, Transactions,” 1866. 
16. Clarke and Johnson, “Med.-chirurg. Transact.,” 1868. 
* The description of the exact anatomical distribution of atrophy or 
hypertrophy would be left to the individual author. PROGRESSIVE MUSCULAR DYSTROPHIES. 
17. Schultze, “Ueber den mit Hypertropbie verbundenen 
Muskelschwnnd,” etc., 1886. 
19. Striimpell. History given by Schultze {op. cit., p. 75); 
and again by Striimpell, “Dtseh. Arch. f. kl. Med.,” Bd. 42, p. 
230, 1887. 
18. Pierret-Troissier, “Arch, de physiol,” 1875. 
20. Wood and Dercum, ‘‘Therap. Gazette,” March 16,1885. 
21. Meryon, “Med.-cbirurg. Transactions,” 1852. 
22. Duchenne, “Arch. gdner. de med.,” 1861. 
23. Gowers, “ Psendo-bypertropb. Paralysis,” London, 
1879; also “Lancet,” July, 1879. 
24. Seidel, “De atrophia musculorum lipomatosa,” Jena, 
1867. 
25. Middleton, “ Glasgow Med. Journal,” August, 1884. 
26. Barth, “Arch. d. Heilkunde,” xii, 1871. 
27. Berger, “Arch. f. Psych,,” Bd. xiv, p. 625. 
28. Gunther, “ Berlin Dissertation,” 1884. 
29. Pick, “ Arch. f. Psych.,” vol. vi, 1876, p. 682. 
30. Kesteven, “Journal of Ment. Science,” 1871, pp. 41 and 
563. 
31. Baeg, cf. “ Oanst. Jahresber.,” 1877; also Schultze, loc. 
cit., p. 39. 
32. Brigidi, “ Irapareiale,” 1878. 
33. Ross, “Diseases of the Nervous System,” vol. i, p. 991, 
etc., Case I. 
34. These cases are the following; 
Meryon, op. cit., Case I. 
Eulenburg and Oohnheim, “ Verb, der Berl. Ges.,” 1869; 
post-mortem examination seventeen days after death, but result 
accepted by all. 
Duchenne, Charcot, “Arch, de phys.,” 1872. 
Brieger, “ Deutsch. Arch. f. kl. Med.,” 1878. 
Schultze, Virchow’s “Arch.,” 1879, Bd. 75, p. 475. 
Ross, op. cit., Case 11. 
Pekelharing, Virchow’s “Arch.,” vol. Ixxxix, p. 228. 
Middleton, op. cit., two cases. 
Berger, loc. cit. 
The cases of Drummond (“Lancet,” 1881) and of Bramwell PROGRESSIVE MUSCULAR DYSTROPHIES. 
(“ Dis. of the Spinal Cord,” 1882, p. 201), which exhibited nor- 
mal ganglion cells, although there were other changes in the 
spinal cord, would swell the number of these cases to twelve. 
For full literature, see the very complete article by Mary Put- 
nam Jacobi in Pepper’s “System,” vol. iv. 
35. Gibney, “Journal of Nerv. and Mental Dis.,” vol. xiii, 
p. 672, 1886. 
86. Westphal, “ Oharite-Annalen,” 1887, p. 47. 
37. Middleton, “ Glasgow Med. Journal,” June, 1888. 
38. Inglis, “Journal of Nerv. and Mental Dis.,” August, 
1888. 
39. Hammond, “Diseases of the Nerv. Syst.,” Bth ed., 1886, 
p. 504. 
40. Log. cit. 
42. Clarke and Gowers, “Med.-chirurg. Trans.,” 1874, vol. 
Ivii, p. 247. 
41. Muller, “ Beitr. z. Path. d. Eueckenm.,’’ Leipsic, 1871. 
48. Lichtheim, “Arch. f. Psych.,” 1878, Bd. viii, p. 521. 
44. Erb u. Schultze, “ Arch. f. Psych.,” ix, 1879. 
45. Kahler, “Zeitschr. f. Heilkunde,” 1884, p. 209. 
46. Ormerod, “ St. Bartholomew’s Hosp. Reports,” vol. 
xxiii, 1887, Oases YII and VIII. 
47. Ross, op. cit. 
48. Dreschfeld, “ Brain,” July, 1886 
49. Leyden, “Klinik d. Euckenmarkskrankheiten,” vol. ii, 
p. 527. 
50. Mobius, Yolkmann’s “ Yortrage,” No. 171. 
51. Op. cit. (cf. No. 21). 
52. Oppenheimer, Heidelberg, 1856. 
53. Hernptenmacher, “ Dissertation,” Berlin, 1862. 
54. Bernhardt, “ Sitzungsber. d. Berl. med. Ges.,” Jan. 20, 
1875, and “Berl. kl. Wochenschr.,” 1875. 
56. “Berl. kl. Wochenschr.,” 1873, Nos. 42 and 43. 
56. Zimmerlin, “Zeitschr. f. kl. Med.,” vii, 1883. 
57. Landouzy et D6jerine, “ Revue de m6d.,” 1885. 
58. Schultze, “Neurolog. Oentralblatt,” 1885, p. 529. 
59. Barsikow, “ Zwei Familien rait Lipomatosis muscul. 
progr.,” Dissertation, Halle, 1872. 44 
PROGRESSIVE MUSCULAR DYSTROPHIES. 
59a. Harrington, “American Jour, of Insanity,” 1877-’7B, 
pp. 75, 76. 
60. Reraak, “Neurol. OentralbL,” 1884, p. 837. 
61. Mossdorf, “Neurol. OentralbL,” 1885, p. 1. 
62. Bernhardt, “ Berl. kl. Wochenscbr.,” 1887, pp. 763-765. 
63. Kreske, “ Mtinchener med. Wochenscbr.,” 1886, No. 15. 
64. Westphal, “Oharite-Annalen,” 1886. 
65. Landouzy et Dejerine, “Eev. de m6d.,” 1886, p. 979. 
66. Westphal, “Oharite-Annalen,” 1884 (6), pp. 377-408. 
67. Charcot et Marie, “Rev. de m6d.,” 1886. 
68. Tooth, “Dissert.,” 1886; cf. also “Critical Digest,” 
“ Brain,” x, 1888. 
69. Ilerringharn, “ Brain,” July, 1888. 
70. Ormerod, “Brain,” vol. vii. 
71. Schultze, “Berl. kl. Wochenscbr.,” 1884, No, 41. 
72. Charcot, “Neue Yorlesungen,” etc., Freud’s transl., and 
“Revision nosographique,” “ Progres med.,” 1885. 
73. Oppenheim, “ Oharite-Annalen,” 1888. 
74. Roth, “ Oomptes rendus de la soc. de bi01.,” Dec. 17,1886. 
76. Jacoby, G. W., “Journal of Nerv. and Ment. Dis.,” 1887. 
76. Schultze (cf. No. 17). 
77. Erb, “ Neurol. Centralbl.,” 1884, and July 1, 1888. 
78. Hitzig, “Berl. kl. Wochenscbr.,” Nos. 25, 34, 35, 1888. 
79. Singer, “Zeitschr. f. Heilkunde,” vol. viii. 
80. Hoffman, “Neurol. OentralbL,” July 15, 1888. 
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