Expanded mobius syndrome

A woman presented at 33 weeks gestation with reduced fetal movements and a nonreactive nonstress test, Fetal ultrasound examination revealed a peculia r unilateral arm tremor. At emergency cesarean section, performed for fetal indications, a 1,672-gm male infant was delivered requiring intubation for feeble respiratory effort. After delivery the neonate was transiently hype rtonic and later hypotonic. Continuing ventilatory support at minimal setti ngs was necessary. The work-up for aneuploidy, metabolic disorders, and inf ection was negative. The infant died after being removed from ventilatory s upport on day 22. Postmortem examination revealed extensive bilateral brain gliosis and mineralization without evidence of inflammation, partial absen ce of cranial nerve nuclei III-XI, and a total absence of cranial nerve roo ts VI-XI, Together these finding are compatible with a diagnosis of expande d Mobius syndrome. (C) 2001 by Elsevier Science Inc. All rights reserved.