Froin's Syndrome: A Comprehensive Review of the Literature and the Addition of Two New Cases.

IF 3.2 Q2 CLINICAL NEUROLOGY
Lucas Jacobs, Bertil Delsaut, Marta Lamartine S Monteiro, Audrey Cambier, Ibrahim Alcan, Evelyne Maillart, Maxime Taghavi
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Abstract

Background: Froin's syndrome (FS) is a rare entity with uncertain prevalence and prognosis, defined by a pathognomonic triad: cerebrospinal fluid (CSF) xanthochromia, elevated protein levels in the CSF, and hypercoagulated CSF, usually obtained through lumbar puncturing below the level of a partial or complete spinal block.

Methods: We conducted a comprehensive review of the literature on FS from its first description in 1903 to December 2023, utilizing PubMed and Google Scholar, and included two new cases from our clinical practice.

Results: We describe two patients who suffered from Froin's syndrome secondary to spinal abscesses. According to our review, FS is caused by neoplasia in 33% of cases, non-malignant mechanical causes in 27%, infections in 27%, non-infectious inflammatory processes in 6%, and vascular in 6%. The most prevalent symptoms are paraplegia/paraparesis (64%), back pain (38%), altered mental state and/or confusion (23%), sciatica (17%), headaches (17%), leg sensory defects (17%), and urinary retention (14%), and are thought to be linked with the underlying causes rather than the CSF characteristics. FS holds a poor prognosis: only 22% recuperate fully after treatment, 22% die due to the cause leading to FS, and 14% retain sequelae.

Conclusions: Xanthochromia and proteinorachia >500 mg/dL are not specific to any single pathological condition, but indicate defective CSF recirculation and spinal block, causing diffusive and/or inflammatory processes resulting in the hyperproteinosis and coagulation of the CSF. We reviewed the pathophysiology, etiologies, symptoms, outcomes, and workups of Froin's syndrome according to the existing medical literature.

弗罗因综合征:文献综述及两例新病例。
背景:弗罗因综合征(Froin's syndrome,FS)是一种罕见的疾病,发病率和预后均不确定,其病理特征为三联征:脑脊液(CSF)黄变、CSF蛋白水平升高和CSF高凝,通常通过部分或完全脊髓阻滞水平以下的腰椎穿刺获得:我们利用PubMed和Google Scholar对FS从1903年首次描述到2023年12月的文献进行了全面回顾,并纳入了我们临床实践中的两个新病例:结果:我们描述了两名继发于脊柱脓肿的弗罗因综合征患者。根据我们的研究,33%的病例由肿瘤引起,27%由非恶性机械性原因引起,27%由感染引起,6%由非感染性炎症过程引起,6%由血管引起。最常见的症状是截瘫/偏瘫(64%)、背痛(38%)、精神状态改变和/或精神错乱(23%)、坐骨神经痛(17%)、头痛(17%)、腿部感觉缺陷(17%)和尿潴留(14%),这些症状被认为与潜在病因而非 CSF 特征有关。FS的预后较差:只有22%的患者在治疗后完全康复,22%的患者因导致FS的病因而死亡,14%的患者留有后遗症:黄染和蛋白尿 >500 mg/dL 并非任何单一病理条件所特有,而是表明 CSF 再循环缺陷和脊髓阻滞,引起弥散和/或炎症过程,导致 CSF 蛋白质过多和凝固。我们根据现有的医学文献回顾了弗罗因综合征的病理生理学、病因、症状、结果和检查方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Neurology International
Neurology International CLINICAL NEUROLOGY-
CiteScore
3.70
自引率
3.30%
发文量
69
审稿时长
11 weeks
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