PT - JOURNAL ARTICLE AU - Bharucha, E. P. AU - Pandya, S. S. AU - Dastur, Darab K. TI - Arthrogryposis multiplex congenita AID - 10.1136/jnnp.35.4.425 DP - 1972 Aug 01 TA - Journal of Neurology, Neurosurgery & Psychiatry PG - 425--434 VI - 35 IP - 4 4099 - http://jnnp.bmj.com/content/35/4/425.short 4100 - http://jnnp.bmj.com/content/35/4/425.full SO - J Neurol Neurosurg Psychiatry1972 Aug 01; 35 AB - Sixteen cases with arthrogryposis multiplex congenita were examined clinically and electromyographically; three of them were re-examined later. Joint deformities were present in all extremities in 13 of the cases; in eight there was some degree of mental retardation. In two cases, there was clinical and electromyographic evidence of a myopathic disorder. In the majority, the appearances of the shoulder-neck region suggested a developmental defect. At the same time, selective weakness of muscles innervated by C5-C6 segments suggested a neuropathic disturbance. EMG revealed, in eight of 13 cases, clear evidence of denervation of muscles, but without any regenerative activity. The non-progressive nature of this disorder and capacity for improvement in muscle bulk and power suggest that denervation alone cannot explain the process. Re-examination of three patients after two to three years revealed persistence of the major deformities and muscle weakness noted earlier, with no appreciable deterioration.