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孤立性颅后窝静脉血栓形成表现为局灶性小脑静脉梗死或出血。

Isolated vein thrombosis of the posterior fossa presenting as localized cerebellar venous infarctions or hemorrhages.

机构信息

Servicio de Neurología y Neurocirugía, Hospital Civil de Guadalajara Fray Antonio Alcalde, Hospital 278, Guadalajara, Jalisco, México.

出版信息

Stroke. 2010 Oct;41(10):2358-61. doi: 10.1161/STROKEAHA.110.588202. Epub 2010 Aug 26.

DOI:10.1161/STROKEAHA.110.588202
PMID:20798372
Abstract

BACKGROUND AND PURPOSE

Cerebellar venous infarction or hemorrhage due to isolated venous thrombosis of the posterior fossa is a rare form of intracranial vein thrombosis that can be unsuspected in clinical practice.

METHODS

We studied 230 patients with intracranial vein thrombosis, identifying 9 (3.9%: 7 women, mean age 34 years) with neuroimaging or histopathologic evidence of localized posterior fossa vein thrombosis causing parenchymal injury limited exclusively to the cerebellum.

RESULTS

All patients had an insidious presentation suggesting other diagnoses. Intracranial hypertension (n=6) and cerebellar (n=4) syndromes were the main clinical presentations. Intracranial vein thrombosis was idiopathic in 3 patients; associated with puerperium in 3; and with contraceptives, protein C deficiency, and dehydration in 1 case each. CT was abnormal but not diagnostic in 5 patients, showing a cerebellar hypodensity with fourth ventricle compression and variable hydrocephalus in 5 patients, and cerebellar hemorrhage in 2. Conventional MRI provided diagnosis in 6 cases, showing the causal thrombosis and cerebellar involvement; angiography was practiced in 2 of them, confirming the findings identified by MRI. In the other 3 patients, diagnosis was reached by histopathology. Thromboses were localized at the straight sinus (n=4), lateral sinuses (n=3), and superior petrosal vein (n=2). The acute case fatality rate was 22.2% (n=2), 1 (11.1%) patient was discharged in a vegetative state, 1 (11.1%) was severely disabled, and 5 (55.6%) were moderately disabled.

CONCLUSIONS

Isolated venous thrombosis of the posterior fossa is infrequent and implies a challenging diagnosis. Risk factors for intracranial vein thrombosis and atypical cerebellar findings on CT should lead to further MRI assessment.

摘要

背景与目的

孤立性颅后窝静脉血栓形成引起的小脑静脉梗死或出血是一种罕见的颅内静脉血栓形成形式,在临床实践中可能未被怀疑。

方法

我们研究了 230 例颅内静脉血栓形成患者,其中 9 例(3.9%:7 名女性,平均年龄 34 岁)存在神经影像学或组织病理学证据,表明局部颅后窝静脉血栓形成导致仅局限于小脑的实质损伤。

结果

所有患者均表现出隐匿性表现,提示其他诊断。颅内压增高(n=6)和小脑(n=4)综合征是主要的临床表现。3 例为特发性颅内静脉血栓形成;3 例与产褥期相关;1 例与避孕药、蛋白 C 缺乏和脱水相关。5 例 CT 异常但无诊断价值,显示 5 例小脑密度降低伴第四脑室受压和可变脑积水,2 例小脑出血。6 例常规 MRI 提供了诊断,显示了因果性血栓形成和小脑受累;其中 2 例行血管造影,证实了 MRI 发现。在另外 3 例患者中,通过组织病理学达到诊断。血栓形成位于直窦(n=4)、外侧窦(n=3)和岩上窦(n=2)。急性病死率为 22.2%(n=2),1 例(11.1%)患者出院时处于植物状态,1 例(11.1%)患者严重残疾,5 例(55.6%)患者中度残疾。

结论

孤立性颅后窝静脉血栓形成很少见,提示诊断具有挑战性。颅内静脉血栓形成的危险因素和 CT 上不典型的小脑表现应导致进一步的 MRI 评估。

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