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伴有假性脑瘤的托洛萨-亨特综合征。1例罕见病例报告。

Tolosa-Hunt syndrome with pseudotumor cerebri. Report of an unusual case.

作者信息

Nezu A, Kimura S, Osaka H

机构信息

Department of Pediatrics, Yokohama City University, School of Medicine, Japan.

出版信息

Brain Dev. 1995 May-Jun;17(3):216-8. doi: 10.1016/0387-7604(95)00036-b.

DOI:10.1016/0387-7604(95)00036-b
PMID:7573765
Abstract

An 12-old-year girl with Tolosa-Hunt syndrome (THS) complicated with pseudotumor cerebri is presented. She suffered from alternating, recurrent and painful ophthalmoplegia at the age of 8, and bilateral papilledema which did not affect visual acuity was also recognized. THS subsided readily on steroid therapy, and the remission lasted more than 3 years after discontinuing steroid. However, papilledema did not change despite remission of THS, and resulted in optic atrophy with a mildly enlarged scotoma and 10% decrease in visual acuity 6 months after the onset. MRI disclosed slightly contrasted masses in the bilateral enlarged cavernous sinuses and narrowing of the left carotid siphon. Another small mass with partial gadolinium enhancement was revealed adjacent to the left narrowing of the carotid siphon in the optochiasmatic cistern; however, there was no lesion causing intracranial hypertension. The intracavernous MRI findings were considered characteristic of THS, and papilledema seemed to be due to pseudotumor cerebri by exclusion. Since subsequent MRI confirmed no progression of the above findings, the intracavernous and intracisternal masses were suspected to be non-specific inflammatory granulomas associated with THS.

摘要

本文报告了一名12岁患有托洛萨-亨特综合征(THS)并伴有假性脑瘤的女孩。她8岁时出现交替性、复发性和疼痛性眼肌麻痹,同时还发现双侧视乳头水肿,但未影响视力。THS经类固醇治疗后迅速缓解,停用类固醇后缓解持续超过3年。然而,尽管THS缓解,但视乳头水肿并未改变,发病6个月后导致视神经萎缩,伴有轻度扩大的暗点和视力下降10%。MRI显示双侧海绵窦增大,内有轻度强化肿块,左侧颈内动脉虹吸部变窄。在视交叉池内,左侧颈内动脉虹吸部变窄处附近发现另一个有部分钆增强的小肿块;然而,没有引起颅内高压的病变。海绵窦内MRI表现被认为是THS的特征性表现,通过排除法,视乳头水肿似乎是由假性脑瘤引起的。由于随后的MRI证实上述表现无进展,海绵窦内和脑池内肿块被怀疑是与THS相关的非特异性炎性肉芽肿。

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Brain Dev. 1995 May-Jun;17(3):216-8. doi: 10.1016/0387-7604(95)00036-b.
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