Abstract
A case of arthrogryposis with full necropsy is presented, in which the central nervoys system and many muscles of the 4 extermi-ties were examined histologically. The cerebrum showed scarcity of large pyramidal (Betz) cells in the pre-central gyri, the spinal cord showed a ''fall-out'' of many anterior horn neurons, whereas in skeletal muscles the histology was compatible with spinal type muscular atrophy with extensive fibrosis. The etiology of the pathological changes in arthrogryposis remains unknown.