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

Neuropediatrics
A CharollaisP Landrieu

Abstract

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.

Citations

Jan 22, 2008·Journal de gynécologie, obstétrique et biologie de la reproduction·O Dupuis, A Simon
Aug 12, 2009·Seminars in Fetal & Neonatal Medicine·Paul Govaert
Aug 1, 2009·Birth Defects Research. Part A, Clinical and Molecular Teratology·Judith G Hall
Aug 3, 2019·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Kerby C ObergJudith G Hall
Jun 11, 2003·Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine·Rodrigo RuanoMarc Dommergues
Feb 10, 2015·Child Neurology Open·Perrine VenotNathalie Bednarek

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