Neurogenic and Myogenic Arthrogryposis Multiplex Congenita

Abstract

Arthrogryposis Multiplex Congenita (A. M. C.) is a syndrome, clinically characterized by persistent joint contractures present at birth. The process may involve one or all extremities, but to varying degrees, so there may be hypotonia even when contractures are present.
The authors have experienced four cases of A. M. C. By muscle biopsy and E. M. G. examinations, three of which, including bizygoid twin, were diagnosed as neurogenic A. M. C. and the other was a peculiar type in which rod bodies were observed in biopsied muscle specimens. One of the neurogenic type was died of pneumonia and the diagnosis was confirmed by autopsy.
The main clinical courses and features were quite similar each other. In the first three cases, M. C. V. revealed subnormal levels and did not seem to increase with age after 3 years of age.
In conclusion; first there might be neuromyogenic A.M.C. caused by certain lesions in neuromuscular system in embryonal stage. Secondly, comparing these cases with Werdnig-Hoffmann disease (W-HD), there might be neurogenic (spinal type) A. M. C. different from W-HD and con-sidering from Lebeuthal's cases, there might be myogenic A. M. C. different from congenital muscular dystrophy.
Thirdly, there were evidences of pathology and electrophysiology which suggest the involvement of motor neurones in neurogenic or myogenic A. M. C.