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May 1967

Electromyographic and Histopathologic Correlations in Arthrogryposis

Author Affiliations

From St. Jude Children's Research Hospital and the University of Tennessee College of Medicine, Memphis.

Arch Neurol. 1967;16(5):512-523. doi:10.1001/archneur.1967.00470230064009

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 Banker2,3 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