A slowly progressing, juvenile form of muscular dystrophy, affecting each sex equally. Onset at any age from first to sixth decade, but usually between 20 and 30 years of age. Occurs in two forms. In Erb’s type the proximal muscles of the arms are first involved, with resultant difficulty in raising the hands above the head. Later there is successive involvement of the deltoid and erector spinae muscles, and eventually the muscles of the trunk, pelvic girdle, and thigh also become affected.
In the rarer Leyden-Möbius type the lumbocostal region is first affected, then involvement of the scapulohumeral, usually occurring within 20 years of the onset. Symptoms generally quite similar to Duchenne’s muscular dystrophy. Involvement of facial muscle as a late event differentiates it from Landouzy-Dejerine syndrome. Inheritance is probably autosomal recessive.
- W. H. Erb:
Über die «juvenile Form» der progressiven Muskelatrophie und ihre Beziehungen zur sogenannten Pseudohypertrophie der Muskeln.
Deutsches Archiv für klinische Medicin, Leipzig, 1884, 34: 467-519.