Progressive Muscular Dystrophy as an Endocrine Disease
نویسنده
چکیده
In the present paper, the author desires to report a somewhat atyp ical form of progressive muscular dystrophy, rather resembling Erb's infantile type, of extremely benign and �low progress. In the cases examined it has occurred as a hereditary affection now in the fourth generation. The number of individuals disabled thus far has been fourteen. Of the seven living members with the disease, all but two have been examined by Roentgen ray, and of these five, four show distinct changes in the pineal gland, producing shadows in the roent genogram. The evidence presented by the cases reported in the pres ent paper, togethet· with that of previous communications in this field, creates a strong probability of the existence of a close relationship between progressive muscular dystrophy and disease of the pineal gland. In order to understand the evolution that has now placed this dis ease among the endocrinopathies, a rapid survey of the development of the syndrome, together with the most important contributions thereto in the last few decades, is almost indispensable and is herewith included. The cases studied have been placed at my disposal by Dr. Pearce Bailey, of the Neurological Institute, and for his valuable sug gestions and actual assistance, I desire to express my appreciation and my thanks. Historical.1-The first observations on muscular atrophies are found in Boerhaeve's Aphorisms (1709), in which Van Swieten mentions the involvement of the deltoid and that of the intrinsic hand muscles. Later, Charles Bell described syndromes including progressive muscu lar atrophy. But it was reserved for Aran and Duchenne in 1850 to give the first good description of progressive muscular atrophy. Fol lowing them, the French physicians called this type of disease "atrophic musculaire progressive type Duchenne-Aran." Shortly thereafter, Cru veilhier, on the basis of a necropsy, stated that the cause of the disease lay in abnormality of the spinal gray; and ever since then the discussion
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