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自发性颅内低压——神经症状、诊断及预后

Spontaneous intracranial hypotension - Neurological symptoms, diagnosis, and outcome.

作者信息

Dalby Sebastian Worsaae, Smilkov Emil Andonov, Santos Sofia Gaspar, Olesen Viola, Skjolding Anders Daehli, Vukovic-Cvetkovic Vlasta, Jensen Rigmor Højland, Schytz Henrik Winther

机构信息

Danish Headache Center, Department of Neurology, Copenhagen University Hospital-Rigshospitalet-Glostrup, Copenhagen, Denmark.

Department of Diagnostic Radiology, Copenhagen University Hospital, Rigshospitalet-Glostrup, Copenhagen, Denmark.

出版信息

Eur J Neurol. 2025 Jan;32(1):e16579. doi: 10.1111/ene.16579.

DOI:10.1111/ene.16579
PMID:39655564
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11629099/
Abstract

BACKGROUND

Spontaneous intracranial hypotension (SIH) is a rare secondary headache disorder caused by spinal leakage of cerebrospinal fluid. Specialized treatment of SIH consists of epidural blood patches (EBPs), fibrin patching, endovascular sealing, and surgery. The aim of this paper was to characterize SIH patients identified at a tertiary headache center.

METHODS

Eighty patients with SIH identified at the Danish Headache Center were included in this clinical cohort study. Data on demographics, clinical presentation, imaging findings, effect of EBPs, and invasive procedures were collected in a standardized manner. Prognostic elements of EBP treatment outcome were evaluated statistically with success defined as a minimum 30% intensity reduction sustained for at least 1 month.

RESULTS

The average age was 47 years and 53% were female. Peracute and subacute onset was noted in 35% and 36%, respectively. Orthostatic headache was noted in 85% of cases. The median Bern score was 4, and of identified leaks, 52% were type 1, 22% type 2, 22% type 3, and 4% type 4 (defined as peripheral leakage distally for the root pouch). Successful treatment response was 34% for EBPs and 90% for invasive procedures. Prepontine cistern effacement was more prevalent in patients with successful first EBP than without (p = 0.013).

CONCLUSION

SIH patients presented with a wide variety of symptoms with high prevalence of orthostatic headache. Peracute onset was noted in 35% and calls for more awareness in the acute setting. We found prepontine cistern effacement to be a potential predictor of a successful first epidural blood patch.

摘要

背景

自发性颅内低压(SIH)是一种由脑脊液脊柱漏引起的罕见继发性头痛疾病。SIH的专科治疗包括硬膜外血贴(EBP)、纤维蛋白贴补、血管内封堵和手术。本文旨在描述在一家三级头痛中心确诊的SIH患者的特征。

方法

本临床队列研究纳入了在丹麦头痛中心确诊的80例SIH患者。以标准化方式收集了人口统计学、临床表现、影像学检查结果、EBP效果和侵入性操作的数据。对EBP治疗结果的预后因素进行了统计学评估,成功定义为强度至少降低30%并持续至少1个月。

结果

平均年龄为47岁,53%为女性。分别有35%和36%的患者表现为急性和亚急性起病。85%的病例出现体位性头痛。伯尔尼评分中位数为4分,在已确定的漏口类型中,52%为1型,22%为2型,22%为3型,4%为4型(定义为神经根袖远端的外周漏)。EBP的成功治疗反应率为34%,侵入性操作的成功治疗反应率为90%。首次EBP治疗成功的患者中脑桥前池消失更为常见(p = 0.013)。

结论

SIH患者表现出多种症状,体位性头痛患病率高。35%的患者为急性起病,在急性情况下需要提高认识。我们发现脑桥前池消失是首次硬膜外血贴治疗成功的一个潜在预测因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/bc664b7eb5ca/ENE-32-e16579-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/e32b819de9f2/ENE-32-e16579-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/baa5f247eed2/ENE-32-e16579-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/ad32a80a809e/ENE-32-e16579-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/6ca9984a784f/ENE-32-e16579-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/bc664b7eb5ca/ENE-32-e16579-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/e32b819de9f2/ENE-32-e16579-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/baa5f247eed2/ENE-32-e16579-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/ad32a80a809e/ENE-32-e16579-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/6ca9984a784f/ENE-32-e16579-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea8a/11629099/bc664b7eb5ca/ENE-32-e16579-g001.jpg

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本文引用的文献

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Headache. 2024 Apr;64(4):460-463. doi: 10.1111/head.14697. Epub 2024 Apr 13.
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CT Myelography: How to Do It.CT脊髓造影:操作方法
Radiographics. 2024 Jan;44(1):e230088. doi: 10.1148/rg.230088.
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Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.自发性颅内低血压的诊断和管理多学科共识指南。
J Neurol Neurosurg Psychiatry. 2023 Oct;94(10):835-843. doi: 10.1136/jnnp-2023-331166. Epub 2023 May 5.
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The SIH-EBP Score: A grading scale to predict the response to the first epidural blood patch in spontaneous intracranial hypotension.SIH-EBP 评分:一种预测自发性颅内低血压患者对首次硬膜外血贴治疗反应的分级量表。
Cephalalgia. 2023 Mar;43(3):3331024221147488. doi: 10.1177/03331024221147488.
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Natural history of spontaneous intracranial hypotension: a clinical and imaging study.自发性颅内低压的自然史:一项临床与影像学研究。
J Neurointerv Surg. 2023 Nov;15(11):1124-1128. doi: 10.1136/jnis-2022-019300. Epub 2022 Nov 10.
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Clin Neuroradiol. 2023 Mar;33(1):211-218. doi: 10.1007/s00062-022-01205-7. Epub 2022 Aug 26.
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