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[伴有如重症肌无力般日间波动的中脑下垂]

[Midbrain ptosis with diurnal fluctuation like myasthenia].

作者信息

Inoue Y

机构信息

Department of Neurology, Toyama Prefectural Central Hospital, Japan.

出版信息

No To Shinkei. 1998 Apr;50(4):387-92.

PMID:9592832
Abstract

A 51-year-old man was admitted because of bilateral fatigable blepharoptosis with diurnal fluctuation. He had a tendency to take much instant foods before onset. On admission, he showed symmetrical bilateral ptosis, supranuclear upward gaze palsy and vertical diplopia. Ptosis mildly improved on lateral or upward gaze. Bell's phenomenon and pupillary response were intact. Intravenous edrophonium infusion test and serum antiacetylcholine receptor antibody were negative. Cranial MRI and CT demonstrated the lesion in the periaqueductal gray (PAG) region from superior colliculus to upper pontine level, which was remarkably enhanced by Gd-DTPA infusion. He was diagnosed as midbrain ptosis, probably due to atypical Wernicke's encephalopathy. It was conceivable that the PAG lesion might be contributory to fatigable blepharoptosis (pseudomyasthenia) and supranuclear upward gaze palsy in the present case, because the PAG controls levator palpebrae neurons of central caudal nucleus in oculomotor nucleus complex and receives afferents from the limbic system., reticular formation and posterior commissure.

摘要

一名51岁男性因双侧易疲劳性上睑下垂伴日间波动入院。发病前他有食用大量即食食品的倾向。入院时,他表现为双侧对称性上睑下垂、核上性上视麻痹和垂直性复视。向外侧或向上注视时上睑下垂稍有改善。贝尔现象和瞳孔反应正常。静脉注射依酚氯铵试验及血清抗乙酰胆碱受体抗体均为阴性。头颅MRI和CT显示中脑导水管周围灰质(PAG)区域从 superior colliculus到脑桥上段有病变,静脉注射钆喷酸葡胺后病变明显强化。他被诊断为中脑性上睑下垂,可能是由于非典型韦尼克脑病所致。可以推测,本病例中PAG病变可能导致了易疲劳性上睑下垂(假性肌无力)和核上性上视麻痹,因为PAG控制动眼神经核复合体中中央尾侧核的提上睑肌神经元,并接受来自边缘系统、网状结构和后连合的传入纤维。

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