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首页> 外文期刊>Journal of stroke and cerebrovascular diseases: The official journal of National Stroke Association >Rapidly progressive cognitive impairment, ataxia, and myoclonus: An unusual presentation of a dural arteriovenous fistula
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Rapidly progressive cognitive impairment, ataxia, and myoclonus: An unusual presentation of a dural arteriovenous fistula

机译：快速进行性认知障碍，共济失调和肌阵挛：硬脑膜动静脉瘘的异常表现

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摘要

Dural arteriovenous fistulas (DAVFs) have a wide range of clinical presentations, including dementia associated with white matter changes (WMCs). We report a case of DAVF presenting as a rapid progressive dementia and myoclonus without WMCs. A 64-year-old hypertensive and diabetic man was admitted because of a 3-month history of progressive cognitive decline, extrapyramidal and cerebellar signs, and myoclonus. Magnetic resonance imaging (MRI) scans of the brain showed dilated cerebellar veins and T2WI hypersignal in the basal ganglia without WMCs. After admission, he suffered sequential bilateral deep intracerebral hemorrhages. A repeated angioMRI disclosed thrombosis of the distal sagittal and the proximal lateral sinuses. Angiography revealed a torcullar region DAVF. Embolization of the dural fistula was performed. On follow-up, the patients' cognitive deficits improved and myoclonus disappeared. The clinical picture may be explained by venous hypertension in the deep venous system, producing bilateral basal ganglia/thalamic dysfunction and in the posterior fossa. This case shows that DAVFs can produce subcortical dementia without involvement of the deep white matter.
机译：硬脑膜动静脉瘘（DAVF）具有广泛的临床表现，包括与白质变化（WMC）相关的痴呆。我们报告一例DAVF表现为无WMC的快速进行性痴呆和肌阵挛。一名64岁的高血压糖尿病患者因3个月的进行性认知功能减退史，锥体外系和小脑体征以及肌阵挛而入院。大脑的磁共振成像（MRI）扫描显示无WMC的基底神经节中小脑静脉扩张和T2WI高信号。入院后，他经历了连续的双侧深部脑出血。重复的血管MRI显示远端矢状和近端鼻窦血栓形成。血管造影显示了一个动脉区DAVF。硬膜瘘的栓塞术。随访时，患者的认知缺陷得到改善，肌阵挛消失。临床表现可以通过深静脉系统中的静脉高压来解释，其产生双侧基底神经节/丘脑功能障碍和后颅窝。这种情况表明，DAVFs可以产生皮层下痴呆，而不会涉及深部白质。

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