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弗洛因综合征:文献综述及两例新病例补充

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

作者信息

Jacobs Lucas, Delsaut Bertil, Lamartine S Monteiro Marta, Cambier Audrey, Alcan Ibrahim, Maillart Evelyne, Taghavi Maxime

机构信息

Department of Internal Medicine, Brugmann University Hospital, Université libre de Bruxelles (ULB), 1020 Brussels, Belgium.

Department of Nephrology and Dialysis, Brugmann University Hospital, Université libre de Bruxelles (ULB), 1020 Brussels, Belgium.

出版信息

Neurol Int. 2024 Sep 29;16(5):1112-1121. doi: 10.3390/neurolint16050083.

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.

摘要

背景

弗洛因综合征(FS)是一种罕见疾病,其患病率和预后尚不确定,由一组具有诊断意义的三联征定义:脑脊液(CSF)黄变、脑脊液蛋白水平升高以及脑脊液高度凝固,通常通过在部分或完全脊髓阻滞水平以下进行腰椎穿刺获取脑脊液。

方法

我们利用PubMed和谷歌学术对1903年首次描述至2023年12月关于FS的文献进行了全面综述,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洛因综合征患者。根据我们的综述,FS在33%的病例中由肿瘤形成引起,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%由血管性原因引起。最常见的症状是截瘫/轻截瘫(64%)、背痛(38%)、精神状态改变和/或意识模糊(23%)、坐骨神经痛(17%)、头痛(17%)、腿部感觉缺陷(17%)和尿潴留(14%),并且认为这些症状与潜在病因有关而非脑脊液特征。FS预后较差:仅22%的患者在治疗后完全康复,22%因导致FS的病因死亡,14%留有后遗症。

结论

黄变和脑脊液蛋白含量>500mg/dL并非特定于任何单一病理状况,但表明脑脊液循环障碍和脊髓阻滞,导致扩散性和/或炎症过程,从而引起脑脊液高蛋白血症和凝固。我们根据现有医学文献综述了弗洛因综合征的病理生理学、病因、症状、结局及检查方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31c4/11510248/c3985c4b24c2/neurolint-16-00083-g001.jpg

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