PRIMARY MYOPATHIES

Abstract

Recent favorable therapeutic reports¹ have revived interest in the muscular dystrophies. A brief historical review, however, revealed that this condition has been recognized at least for over a century. Bell² in 1830 first described cases in which the disease resembled pseudohypertrophic muscular dystrophy; Meryon,³ Duchenne,⁴ Erb⁵ and Landouzy and Dejerine⁶ greatly advanced knowledge of the subject through clinical and postmortem observations. It remained for Erb,⁷ however, to give the first clear conception and differentiation of the myopathies. Shortly thereafter, Batten⁸ suggested the following classification, which I have employed in this study: To this list Spiller⁹ would have added the true hypertrophic type. These forms have the common characteristics of being familial or hereditary and usually