Arthrogryposis multiplex congenita. I. 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...

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Bibliographic Details
Published in:Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 1972-08, Vol.35 (4), p.425-434
Main Authors: Bharucha, E P, Pandya, S S, Dastur, D K
Format: Article
Language:English
Subjects:
Adolescent
Ankle Joint
Arthrogryposis - diagnosis
Arthrogryposis - diagnostic imaging
Arthrogryposis - physiopathology
Child
Child, Preschool
Electromyography
Face
Female
Hip Joint
Humans
Infant
Knee Joint
Male
Muscle Denervation
Muscles - physiopathology
Neck
Neural Conduction
Radiography
Reflex
Shoulder Joint
Wrist Joint
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Summary: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.
ISSN:0022-3050
DOI:10.1136/jnnp.35.4.425