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同卵双胞胎中的发育性福-沙-马综合征

Developmental Foix-Chavany-Marie syndrome in identical twins.

作者信息

Graff-Radford N R, Bosch E P, Stears J C, Tranel D

出版信息

Ann Neurol. 1986 Nov;20(5):632-5. doi: 10.1002/ana.410200513.

Abstract

Foix, Chavany, and Marie described a syndrome of faciopharyngoglossomasticatory diplegia resulting from bilateral anterior opercular infarction. We describe identical twins who have a developmental form of the syndrome. The twins, aged 41 years, were the product of a normal pregnancy and birth, but had subsequent delayed motor milestones, seizures, poor language development, mild mental retardation, drooling, absent gag reflexes, inability to protrude the tongue, brisk jaw jerks, impaired fine finger movements, symmetrical brisk reflexes, flexor plantar responses, and mildly spastic gait. Magnetic resonance imaging showed bilateral perisylvian cortical dysplasia compatible with polymicrogyria and incomplete opercular formation.

摘要

福瓦、沙瓦尼和玛丽描述了一种由双侧额叶岛盖前部梗死导致的面咽舌咀嚼性双侧瘫痪综合征。我们报告了一对患有该综合征发育形式的同卵双胞胎。这对双胞胎41岁,孕期和出生均正常,但随后出现运动发育迟缓、癫痫发作、语言发育不良、轻度智力障碍、流口水、咽反射消失、不能伸舌、下颌反射亢进、精细手指运动受损、双侧反射亢进、跖屈反应、以及轻度痉挛性步态。磁共振成像显示双侧外侧裂周围皮质发育异常,符合多小脑回畸形和不完全岛盖形成。

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