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Arthrogryposis multiplex congenita
  1. E. P. Bharucha,
  2. S. S. Pandya,
  3. Darab K. Dastur
  1. Children's Orthopaedic Hospital, Bombay-8, India
  2. The Neuropathology Unit, J.J. Group of Hospitals, Bombay-8, India

    Part 1: Clinical and electromyographic aspects


    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.

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    Part 1: Clinical and electromyographic aspects