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Progressive Atrophic Myopathy

erb, muscular, disease, affection, atrophy and dejerine

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For the sake of clearness of presentation I have thought it best to describe separately the two forms of primary myopathy, viz., pseudo hypertrophic and atrophic paralysis, notwithstanding that these two clinical types are of the same nature. We will now take up the con sideration of the simple atrophic form.

In speaking in a general way of the history of primary myo pathies, I have sufficiently indicated in what way the nosological type which we are now considering came to be established. We have seen Leyden, Erb, Landouzy, and Dejerine bring important contributions to this subject, the final analysis of which was made by Erb in 1891.

As in the case of pseudo-hypertrophic paralysis, we have here only the vaguest etiological data. All that we know is that this affection is rare, that it attacks in equal proportion the two sexes, and that it appears usually in later childhood, although it may be developed at any age. Joffroy and Achard have indeed reported a case of this disease occurring in a woman fifty-five years of age. Fatigue and physical overwork have been alleged as factors in the production of the affection, and the possible influence of infectious diseases has also been mentioned. Thus measles and scarlatina have been accused as predisposing to the paralysis, but the only point of real interest in the etiological study of the affection is heredity. It is not rare to see several members of the same family affected with muscular atrophy. Eichhorst, Landouzy, and Dejerine have insisted particu larly upon morbid heredity, and they report several instances in which the number of cases of this affection have gone on increasing from generation to generation, the early mortality of the other chil dren also increasing so that in a short time the entire family of myo pathics would become extinct. We find numerous well-attested facts that show that one or more of the children of a myopatbic are liable to be subjects of the same disease in one or other of its forms.

Pathological anatomy sustains the view that there is a primary form of myopathy, for the nervous system presents no changes what ever, and the only lesions found are those of the muscular tissue. This is the seat of a more or less marked atrophy localized in one or another region according to the variety of the disease with which we have to do. The color of the affected muscles is reduced in intensity. Histological examination confirms what is learned by simple inspec tion, the fact, namely, of a progressive destruction of the muscular fibre.

As I have before remarked, progressive muscular atrophy pre sents several different types which it is possible to describe sepa rately. Thus we have the scapulo-humeral (Erb), the facio-scapalo humeral (Landouzy and Dejerine) , the Leyden-Mobins, the Zimmerlin, the femoro-tibial (Eichhorst), and the femoral with claw-like retrac tion of the toes (Brossard).

The seQprilo-Irtonerrl type described by Erb in 1882 begins during childhood or at puberty, the atrophy showing itself first in the muscles of the shoulder and in those of the arm. The muscles of the back, huu bar and lower extremities may also participate in the paraly sis, and this early in the course of the disease, but usually they are not attacked until much later. The lesions are most commonly sym metrical, although Erb has seen them invade one side first, the other side not participating until after a considerable period. As long as a sufficient quantity of muscular tissue remains the reflexes are pre served. There are no tibrillary contractions, and the electrical con tractility remains normal, showing only a diminished intensity which becomes more and more apparent in proportion as the muscular tis sue disappears. According to Erb, the muscles of the face are never attacked in this form of the disease. Affections of this part are, how ever, occasionally observed and serve as a connecting link between this form and the following which has been particularly studied Landouzy and Dejerine.

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