Arthrogryposis and multicystic encephalopathy after acute fetal distress in the end stage of gestation

The natural history of the rare association "multicystic encephalopathy-art hrogryposis" was traced in a fetus carefully followed after artificial inse mination. The fetus exhibited normal viability and brain morphology up to t he 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hyd ramnios and signs of fetal distress led to cesarean section. The infant pre sented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicrystic encephalo pathy. Both the clinical and the neuropathological findings established tha t multicystic encephalopathy was neither the cause nor the sequential conse quence of the fetal akinesia, but the result of a recent diffuse, acute mal acic process that also involved the anterior horn cells. Acute fetal distre ss, responsible for major ischemic damage to CNS but compatible with fetal survival, remains an obscure condition which allows for the development of severe arthrogryposis in a few weeks.