目的:确定脊髓脑脊液(CSF)静脉瘘(CVF)引起的自发性颅内低血压(SIH)的不同临床或影像学亚型。
背景:自发性颅内低血压在临床上被认为是体位性头痛和刻板的脑磁共振成像(MRI)发现;然而,大多数先前研究SIH的临床和脑部MRI特征的文献都集中在所有类型的脊髓CSF漏。这项研究旨在评估数据是否支持基于与原发性头痛综合征相似的脑成像特征和临床症状的内部一致亚型的可能性。
方法:这项回顾性横断面单机构研究包括48名连续符合国际头痛疾病分类的患者,由于CVF导致的SIH的第三版标准。临床症状,治疗前脑部MRI,并对症状持续时间进行分析。分析临床和MRI数据以确定症状和影像学发现之间的模式和关联。
结果:共评估了20名男性和28名女性,平均(标准差)年龄为61(10)岁。总之,44/48(92%)患者经历过头痛,尽管18/48(40%)在持平时没有认可救济,包括48人中的6人(13%)在平坦时症状恶化。总之,19/48(40%)患者报告至少有一种偏头痛症状,48例患者中有6例(13%)出现至少一种偏头痛症状,在平坦时没有缓解。临床症状主要集中在“经典”表现中,包括平坦时的缓解,枕骨头部疼痛,共病颈部疼痛,压力/搏动性头痛的质量,和一个“非典型”演示文稿,其特点是有几个不同:平时缓解较少(22个中的9个(41%)与20/23(87.0%),p=0.002;比值比[OR]0.110,95%置信区间[CI]0.016-0.53),更多额头疼痛(14/22(64%)与23个(4%)中的一个,p<0.001;OR35.0,95%CI4.2-1681.0),颈部疼痛较少(21人中有2人(4.5%)与13人中有9人(69.6%),p<0.001;OR0.023,95%CI0.0005-0.196),更多的刺伤/尖锐的头痛质量(22人中有9人(41%)与23人中的两个(9%),p=0.017;OR7.0,95%CI1.18-75.9)。脑部MRI发现分为三组:同时表现出大多数SIH影像学发现的人群,那些大脑下垂但较少的厚膜/静脉充血,和那些有厚膜/静脉充血但大脑下垂较少的人。
结论:本研究强调了因CVF引起的SIH患者的临床和影像学多样性,挑战仅依靠经典的体位性头痛来诊断。研究结果表明,根据临床和影像学表现,存在不同的SIH亚型。强调对疑似CVF患者进行综合评估的必要性。未来的研究应进一步阐明临床症状和影像学表现之间的关系,旨在完善诊断标准,提高对SIH病理生理学的认识。
To identify distinct clinical or imaging subtypes of spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) venous fistula (CVF).
Spontaneous intracranial hypotension is classically understood to present clinically with an orthostatic headache and stereotyped brain magnetic resonance imaging (MRI) findings; however, most prior literature examining clinical and brain MRI features of SIH has focused on all types of spinal CSF leaks concurrently. This study aimed to evaluate whether data support the possibility of internally consistent subtypes based on brain imaging features and clinical symptoms analogous to those seen in primary headache syndromes.
This retrospective cross-sectional single-institution study included 48 consecutive patients meeting the International Classification of Headache Disorders, 3rd edition criteria for SIH due to CVF. Clinical symptoms, pre-treatment brain MRI, and symptom duration were analyzed. Clinical and MRI data were analyzed to identify patterns and associations between symptoms and imaging findings.
A total of 20 males and 28 females were evaluated, with a mean (standard deviation) age of 61 (10) years. In all, 44/48 (92%) patients experienced headaches, though 18/48 (40%) did not endorse relief when flat, including six of the 48 (13%) with worsening symptoms when flat. In all, 19/48 (40%) patients reported at least one migraine symptom, and six of the 48 (13%) presented with at least one migraine symptom and had no relief when flat. Clinical symptoms clustered primarily into a \"classic\" presentation consisting of relief when flat, occipital head pain, comorbid neck pain, a pressure/throbbing headache quality, and an \"atypical\" presentation that was characterized by having several differences: less relief when flat (nine of 22 (41%) vs. 20/23 (87.0%), p = 0.002; odds ratio [OR] 0.110, 95% confidence interval [CI] 0.016-0.53), more frontal head pain (14/22 (64%) vs. one of 23 (4%), p < 0.001; OR 35.0, 95% CI 4.2-1681.0), less neck pain (two of 21 (4.5%) vs. nine of 13 (69.6%), p < 0.001; OR 0.023, 95% CI 0.0005-0.196), and more stabbing/sharp headache quality (nine of 22 (41%) vs. two of 23 (9%), p = 0.017; OR 7.0, 95% CI 1.18-75.9). Brain MRI findings clustered into three groups: those presenting with most imaging findings of SIH concurrently, those with brain sag but less pachymeningeal/venous engorgement, and those with pachymeningeal/venous engorgement but less brain sag.
This study highlights the clinical and imaging diversity among patients with SIH due to CVF, challenging the reliance on classic orthostatic headache alone for diagnosis. The findings suggest the existence of distinct SIH subtypes based on clinical and imaging presentations, underscoring the need for comprehensive evaluation in patients with suspected CVF. Future research should further elucidate the relationship between clinical symptoms and imaging findings, aiming to refine diagnostic criteria and enhance understanding of SIH\'s pathophysiology.