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August 1954

Dystrophia musculorum progressiva: Eine genetishe und klinishe Untersuchung der Muskeldystrophien.

AMA Arch Intern Med. 1954;94(2):328. doi:10.1001/archinte.1954.00250020162012

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This book is based on a study of 86 pedigrees of families in Baden, Germany, suffering from progressive muscular dystrophy. More than 6,000 people are included in the geneologies, and of this number 259 had the disease. Clinical studies were made on 159 sufferers. The author finds in his own study and from a review of the literature that there are two basic types of dystrophy —the shoulder-girdle type and the pelvic-girdle type. The latter has two modes of inheritance, and there are therefore three different genetic forms. The shoulder-girdle type is a Mendelian dominant, affecting the sexes equally. The age of onset is from 7 to 25 years, with a peak at 15 to 16. The course is slow. The face may be affected, but the muscles of the neck and of the hand are rarely involved. Pseudohypertrophy occurs in about 25% of cases. Males are more severely involved

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