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自发性颅内低血压伴进行性认知功能减退。

Spontaneous intracranial hypotension presenting with progressive cognitive decline.

机构信息

Department of Neurology, Nordsjaellands Hospital, University of Copenhagen, DK, Hilleroed, Denmark.

Department of Neuroradiology, DK, Glostrup, Denmark.

出版信息

BMJ Case Rep. 2021 Jul 21;14(7):e241285. doi: 10.1136/bcr-2020-241285.

DOI:10.1136/bcr-2020-241285
PMID:34290004
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8296796/
Abstract

A 63-year-old woman presented with headache, progressive somnolence, neurocognitive decline and urinary incontinence through a year. Medical history was unremarkable except for hypertension and hypercholesterolaemia. Neurological examination was normal. Brain MRI showed findings typical for spontaneous intracranial hypotension (subdural fluid collection, pachymeningeal enhancement, brain sagging) and pituitary tumour. The patient's complaints improved dramatically but temporarily after treatment with each of repeated targeted as well as non-targeted blood patches and a trial with continuous intrathecal saline infusion. Extensive work up including repeated MRI-scans, radioisotope cisternographies, CT and T2-weighted MR myelography could not localise the leakage, but showed minor root-cysts at three levels. Finally, lateral decubitus digital subtraction dynamic myelography with subsequent CT myelography identified a tiny dural venous fistula at the fourth thoracic level. After surgical venous ligation, the patient fully recovered. Awareness of spontaneous dural leaks and their heterogeneous clinical picture are important and demands an extensive workup.

摘要

一位 63 岁女性因头痛、进行性嗜睡、认知功能下降和尿失禁而就诊,病程持续一年。除高血压和高胆固醇血症外,既往病史无特殊。神经系统检查正常。脑部 MRI 显示自发性颅内低血压的典型表现(硬脑膜下积液、脑膜强化、脑下垂)和垂体瘤。患者接受了多次靶向和非靶向血补丁治疗以及连续鞘内盐水输注治疗后,症状显著但暂时改善。尽管进行了广泛的检查,包括多次 MRI 扫描、放射性核素脑池造影、CT 和 T2 加权磁共振脊髓造影,但仍无法定位漏口,仅显示三个水平的轻微神经根囊肿。最终,侧卧位数字减影动态脊髓造影和随后的 CT 脊髓造影显示第四胸椎水平有一个微小的硬脑膜静脉瘘。行静脉结扎手术后,患者完全康复。了解自发性硬脑膜漏及其异质性临床表现非常重要,需要进行广泛的检查。

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本文引用的文献

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Spontaneous Intracranial Hypotension With Brain Sagging Attributable to a Cerebrospinal Fluid-Venous Fistula.脑脊液-静脉瘘所致自发性颅内低压伴脑下垂
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Atypical Presentations of Intracranial Hypotension: Comparison with Classic Spontaneous Intracranial Hypotension.颅内低压的非典型表现:与经典自发性颅内低压的比较。
AJNR Am J Neuroradiol. 2016 Jul;37(7):1256-61. doi: 10.3174/ajnr.A4706. Epub 2016 Mar 3.
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Imaging Signs in Spontaneous Intracranial Hypotension: Prevalence and Relationship to CSF Pressure.自发性颅内低压的影像学表现:发生率及其与脑脊液压力的关系
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