Electromyographic and Histopathologic Correlations in Arthrogryposis

Abstract

PREVIOUS interpretations of the pathologic processes underlying arthrogryposis multiplex congenita (AMC) have varied markedly. Recently, Smith et al¹ have concluded from electromyographic (EMG) observations in 14 patients and Drachman and Banker^2,3 have reported from necropsy studies that the underlying process was neurogenic. Adams et al⁴ reported both neurogenic and myopathic cases confirmed by necropsy. There were decreased numbers of anterior horn cells in the spinal cord of cases considered to be neurogenic. Skeletal muscles showed small myofibers. In myopathic cases there were no spinal cord changes, but diffuse myopathic changes were present in muscle. Banker et al⁵ reported the autopsy findings in a case of congenital muscular dystrophy, and Pearson and Fowler⁶ described a necropsy of hereditary nonprogressive muscular dystrophy as causes of arthrogryposis. Bargeton et al⁷ found changes indicating a collagen disease in their autopsied patient, and in ther opinon the collagen