The natural history of the rare association “multicystic encephalopathy-arthrogryposis” was traced in a fetus carefully followed after artificial insemination. The fetus exhibited normal viability and brain morphology up to the 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hydramnios and signs of fetal distress led to cesarean section. The infant presented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicystic encephalopathy. Both the clinical and the neuropathological findings established that multicystic encephalopathy was neither the cause nor the sequential consequence of the fetal akinesia, but the result of a recent diffuse, acute malacic process that also involved the anterior horn cells. Acute fetal distress, 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.
Key words
Arthrogryposis - Multicystic encephalopathy - Acute fetal distress
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Prof. Pierre Landrieu
Department of Pediatric Neurology
Laboratory of Neuropathology
CHU, Paris-Sud Bicêtre Assistance Publique Hôpitaux de Paris
94270 Paris
France
Email: pierre.landrieu@bct.ap-hop-paris.fr
