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Arthrogryposis multiplex congenita: A clinical investigation

1970; Elsevier BV; Volume: 76; Issue: 2 Linguagem: Inglês

10.1016/s0022-3476(70)80171-8

1097-6833

Robert L. Fisher, William T. Johnstone, William H. Fisher, O Goldkamp,

Neurogenetic and Muscular Disorders Research

Twenty-six patients with arthrogryposis multiplex congenita were studied. All had muscle wasting and congenital joint contractures in at least 2 body areas; none had evidence of progressive neurologic disease. Almost all patients examined had neuropathic electromyographic patterns. A significantly high birth order, elevated paternal age, and suggested high incidence of fetal wastage in other pregnancies of the mothers were also observed. Limited improvement of upper extremity function in these patients was generally possible by nonoperative methods. In the lower extremities, an aggressive approach, using a variety of surgical procedures, was usually successful in producing alignment and stability satisfactory for independent ambulation. Twenty-six patients with arthrogryposis multiplex congenita were studied. All had muscle wasting and congenital joint contractures in at least 2 body areas; none had evidence of progressive neurologic disease. Almost all patients examined had neuropathic electromyographic patterns. A significantly high birth order, elevated paternal age, and suggested high incidence of fetal wastage in other pregnancies of the mothers were also observed. Limited improvement of upper extremity function in these patients was generally possible by nonoperative methods. In the lower extremities, an aggressive approach, using a variety of surgical procedures, was usually successful in producing alignment and stability satisfactory for independent ambulation.