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一例 Fisher-Bickerstaff 谱的儿科病例。

A pediatric case of Fisher-Bickerstaff spectrum.

机构信息

Pediatric Transportation Team SAMU 93, Avicenne University Hospital, Bobigny, France.

出版信息

Pediatr Neurol. 2010 Feb;42(2):147-50. doi: 10.1016/j.pediatrneurol.2009.09.009.

Abstract

Miller Fisher syndrome is classically described as an acute inflammatory polyneuropathy clinical variant, associating external ophthalmoplegia, ataxia and loss of tendon reflexes. Despite recent advances in the comprehension of this syndrome, with the description of anti-GQ1b anti-ganglioside antibodies associated with abnormal neuromuscular transmission in the serum of Miller Fisher syndrome patients, there is ongoing debate on the peripheral or central origin of the symptoms. Some authors argue that there is a brainstem and cerebellar involvement. Indeed, since description of the syndrome, numerous cases have been reported with electrophysiologic and imaging evidences of brainstem involvement in the syndrome. Described and discussed here is the case of a 4-year-old child with Miller Fisher syndrome and cerebral lesions evident on magnetic resonance imaging, suggesting a Fisher-Bickerstaff spectrum.

摘要

米勒费舍尔综合征经典地被描述为一种急性炎症性多神经病临床变异,伴外眼肌麻痹、共济失调和腱反射消失。尽管最近对该综合征的认识有所进展,描述了抗 GQ1b 神经节苷脂抗体与米勒费舍尔综合征患者血清中异常的神经肌肉传递有关,但对症状的外周或中枢起源仍存在争议。一些作者认为存在脑干和小脑受累。事实上,自从该综合征被描述以来,已经有许多病例报告了脑干受累的电生理和影像学证据。这里描述和讨论的是一例 4 岁儿童的米勒费舍尔综合征,磁共振成像显示脑部病变,提示为费舍尔-比克斯特拉夫谱。

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