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青年成人急性小脑炎伴梗阻性脑积水。

Acute hemicerebellitis with obstructive hydrocephalus in a young adult.

机构信息

Department of Neurosurgery, Hospital Universitario de Canarias, Tenerife, Spain.

出版信息

World Neurosurg. 2011 May-Jun;75(5-6):726-30; discussion 618-9. doi: 10.1016/j.wneu.2010.10.040.

Abstract

BACKGROUND

Acute cerebellitis is a rare entity, described in the pediatric population, usually of viral or autoimmune origin, bilateral and symmetric, and with good prognosis. Only 13 cases of unilateral cerebellitis have been reported thus far. To the best of our knowledge, this is the first case of hemicerebellitis in the literature reported in a young adult that caused acute hydrocephalus requiring surgical treatment.

CASE DESCRIPTION

We report an unusual case of an 18-year-old man, without previous infectious symptoms, who developed acute obstructive hydrocephalus secondary to hemicerebellitis. A ventricular drainage was placed. The affected cerebellar hemisphere was later explored and biopsied, and edematous tissue, with an inflammatory reaction of the leptomeninges, was obtained. With corticosteroids and antivirals, the neurologic symptoms disappeared and the MRI evolved to normal in 2 week's time.

CONCLUSIONS

Hemicerebellitis must be considered in the differential diagnosis of cerebellar mass lesions, even in young adults. Although rare, clinically significant hydrocephalus may develop. Despite the availability of MRI, a high index of suspicion is still required to accurately diagnose this entity. The histologic substrate seems to be localized meningitis. The edematous reaction of the cerebellar parenchyma supports the usefulness of corticoids in the treatment strategy of this disease.

摘要

背景

急性小脑炎是一种罕见的疾病,主要发生在儿童群体中,通常由病毒或自身免疫引起,表现为双侧和对称性,预后良好。迄今为止,仅有 13 例单侧小脑炎的报道。据我们所知,这是首例文献报道的发生于青年成年人的单侧小脑炎导致急性脑积水需要手术治疗的病例。

病例描述

我们报告了一例不寻常的病例,一名 18 岁的男性,无先前感染症状,因单侧小脑炎并发急性梗阻性脑积水。给予脑室外引流。随后探查和活检受影响的小脑半球,获得水肿组织和脑膜的炎症反应。经皮质类固醇和抗病毒治疗后,神经症状消失,磁共振成像在 2 周内恢复正常。

结论

即使是在年轻成年人中,单侧小脑炎也必须被纳入小脑占位性病变的鉴别诊断中。尽管罕见,但可能会出现临床意义重大的脑积水。尽管有磁共振成像的应用,仍需要高度怀疑以准确诊断这种疾病。小脑实质的水肿反应支持皮质类固醇在这种疾病治疗策略中的有效性。

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