A METABOLIC STUDY OF PROGRESSIVE PSEUDOHYPERTROPHIC MUSCULAR DYSTROPHY AND OTHER MUSCULAR ATROPHIES

Abstract

We have recently had the opportunity to study the metabolism of nine cases of pseudohypertrophic muscular dystrophy in different stages of advancement. These cases will be reported from a clinical standpoint with about twenty more by Dr. R. V. Funsten. We also include some metabolic observations on other types of atrophic muscular involvement. Endocrine disturbance in progressive pseudohypertrophic muscular dystrophy is indicated by (1) the hereditary character of the condition, (2) the metabolic abnormalities, (3) the occasional recovery at puberty when glandular readjustments occur, (4) reported improvement following endocrine therapy in some cases, and (5) the development of the disease in polyglandular dystrophies, notably in association with dystrophia adiposogenitalis. Necropsy findings with special reference to the ductless glands are urgently needed to elucidate further the pathogenesis of the condition. The symptomatology and the pathologic changes have been well reviewed by Timme¹ and by Janney, Goodhart, and Isaacson.² Involvement of the