fox - alajouanine综合征：一项关于表现、管理和结果的系统回顾和荟萃分析。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Neurochirurgie Pub Date : 2025-08-08 DOI:10.1016/j.neuchi.2025.101710

Sean O’Leary , Nathan Fredricks , Peace Odiase , Sonia Pulido , Usama AlDallal , Ariadna Robledo , Christopher Thang , Umaru Barrie , Salah Aoun

{"title":"fox - alajouanine综合征：一项关于表现、管理和结果的系统回顾和荟萃分析。","authors":"Sean O’Leary , Nathan Fredricks , Peace Odiase , Sonia Pulido , Usama AlDallal , Ariadna Robledo , Christopher Thang , Umaru Barrie , Salah Aoun","doi":"10.1016/j.neuchi.2025.101710","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><div>Foix-Alajouanine syndrome (FAS) is a grouping of rare, progressive spinal arteriovenous malformations causing significant neurological morbidity.</div></div><div><h3>Methods</h3><div>A systematic review of PubMed, Google Scholar, Embase, Science Direct, and Web of Science following PRISMA guidelines identified 30 articles addressing FAS presentation, management, and outcomes.</div></div><div><h3>Results</h3><div>In 27 case reports covering 46 patients (mean age 55.1 years, 71.7% male), common symptoms included lower extremity weakness (84.8%), bladder dysfunction (69.6%), sensory deficits (65.2%), and gait disturbance (65.2%). Imaging (MRI in 82.5%, angiography in 70.0%) frequently revealed dural arteriovenous fistulas and spinal cord hyperintensities, primarily in the thoracolumbar region. Among these patients, 58.7% improved, 13.1% showed no change, 21.7% worsened, and 6.5% died. Surgical clipping (OR 10.67, 95% CI [2.12–68.04], p = 0.002) and resection (OR 5.74, 95% CI [1.18–36.47], p = 0.029) were associated with neurological improvement, whereas lesions in the mid-thoracic region (T6–T9) had reduced likelihood of improvement (OR 0.18, 95% CI [0.04–0.76], p = 0.017). Conservative management correlated with higher mortality (OR 33.89, 95% CI [1.39–826.08], p = 0.023). In three retrospective studies covering 66 patients (mean age 61.5 years, 80.3% male), primary interventions were surgical or endovascular; 63.6% improved, 35.0% worsened, and 1.5% died at follow-up.</div></div><div><h3>Conclusions</h3><div>Early detection by clinical and radiologic signs with quick intervention in the subtypes of FAS are crucial. Surgical clipping and resection demonstrated particularly favorable results, whereas conservative management was associated with increased mortality. Clinicians should maintain a high index of suspicion for subacute myelopathy to facilitate timely diagnosis and improve long-term prognosis.</div></div>","PeriodicalId":51141,"journal":{"name":"Neurochirurgie","volume":"71 5","pages":"Article 101710"},"PeriodicalIF":1.4000,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Foix-Alajouanine syndrome: A systematic review and meta-analysis of presentation, management, and outcomes\",\"authors\":\"Sean O’Leary , Nathan Fredricks , Peace Odiase , Sonia Pulido , Usama AlDallal , Ariadna Robledo , Christopher Thang , Umaru Barrie , Salah Aoun\",\"doi\":\"10.1016/j.neuchi.2025.101710\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Objective</h3><div>Foix-Alajouanine syndrome (FAS) is a grouping of rare, progressive spinal arteriovenous malformations causing significant neurological morbidity.</div></div><div><h3>Methods</h3><div>A systematic review of PubMed, Google Scholar, Embase, Science Direct, and Web of Science following PRISMA guidelines identified 30 articles addressing FAS presentation, management, and outcomes.</div></div><div><h3>Results</h3><div>In 27 case reports covering 46 patients (mean age 55.1 years, 71.7% male), common symptoms included lower extremity weakness (84.8%), bladder dysfunction (69.6%), sensory deficits (65.2%), and gait disturbance (65.2%). Imaging (MRI in 82.5%, angiography in 70.0%) frequently revealed dural arteriovenous fistulas and spinal cord hyperintensities, primarily in the thoracolumbar region. Among these patients, 58.7% improved, 13.1% showed no change, 21.7% worsened, and 6.5% died. Surgical clipping (OR 10.67, 95% CI [2.12–68.04], p = 0.002) and resection (OR 5.74, 95% CI [1.18–36.47], p = 0.029) were associated with neurological improvement, whereas lesions in the mid-thoracic region (T6–T9) had reduced likelihood of improvement (OR 0.18, 95% CI [0.04–0.76], p = 0.017). Conservative management correlated with higher mortality (OR 33.89, 95% CI [1.39–826.08], p = 0.023). In three retrospective studies covering 66 patients (mean age 61.5 years, 80.3% male), primary interventions were surgical or endovascular; 63.6% improved, 35.0% worsened, and 1.5% died at follow-up.</div></div><div><h3>Conclusions</h3><div>Early detection by clinical and radiologic signs with quick intervention in the subtypes of FAS are crucial. Surgical clipping and resection demonstrated particularly favorable results, whereas conservative management was associated with increased mortality. Clinicians should maintain a high index of suspicion for subacute myelopathy to facilitate timely diagnosis and improve long-term prognosis.</div></div>\",\"PeriodicalId\":51141,\"journal\":{\"name\":\"Neurochirurgie\",\"volume\":\"71 5\",\"pages\":\"Article 101710\"},\"PeriodicalIF\":1.4000,\"publicationDate\":\"2025-08-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Neurochirurgie\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0028377025000839\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Neurochirurgie","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0028377025000839","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}

引用次数: 0

摘要

目的：fox - alajouanine syndrome （FAS）是一组罕见的进行性脊柱动静脉畸形，引起严重的神经系统疾病。方法：根据PRISMA指南对PubMed、b谷歌Scholar、Embase、Science Direct和Web of Science进行系统综述，确定了30篇关于FAS呈现、管理和结果的文章。结果：27例报告46例患者（平均年龄55.1岁，男性71.7%），常见症状包括下肢无力（84.8%）、膀胱功能障碍（69.6%）、感觉缺陷（65.2%）和步态障碍（65.2%）。影像学（MRI占82.5%，血管造影占70.0%）经常显示硬脑膜动静脉瘘和脊髓高信号，主要发生在胸腰椎区。其中58.7%好转，13.1%无变化，21.7%恶化，6.5%死亡。手术夹持（OR 10.67, 95% CI [2.12-68.04], p = 0.002）和切除（OR 5.74, 95% CI [1.18-36.47], p = 0.029）与神经系统改善相关，而胸中区域（T6-T9）病变改善的可能性较低（OR 0.18, 95% CI [0.04-0.76], p = 0.017）。保守治疗与较高的死亡率相关（OR 33.89, 95% CI [1.39 ~ 826.08], p = 0.023）。在三项回顾性研究中，66例患者（平均年龄61.5岁，80.3%为男性）的主要干预措施是手术或血管内治疗；63.6%好转，35.0%恶化，1.5%死亡。结论：通过临床和放射学征象及早发现，快速干预，可有效预防FAS复发。手术夹持和切除显示出特别有利的结果，而保守治疗与死亡率增加有关。临床医生对亚急性脊髓病应保持高度的怀疑指数，以便及时诊断和改善长期预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Foix-Alajouanine syndrome: A systematic review and meta-analysis of presentation, management, and outcomes

Objective

Foix-Alajouanine syndrome (FAS) is a grouping of rare, progressive spinal arteriovenous malformations causing significant neurological morbidity.

Methods

A systematic review of PubMed, Google Scholar, Embase, Science Direct, and Web of Science following PRISMA guidelines identified 30 articles addressing FAS presentation, management, and outcomes.

Results

In 27 case reports covering 46 patients (mean age 55.1 years, 71.7% male), common symptoms included lower extremity weakness (84.8%), bladder dysfunction (69.6%), sensory deficits (65.2%), and gait disturbance (65.2%). Imaging (MRI in 82.5%, angiography in 70.0%) frequently revealed dural arteriovenous fistulas and spinal cord hyperintensities, primarily in the thoracolumbar region. Among these patients, 58.7% improved, 13.1% showed no change, 21.7% worsened, and 6.5% died. Surgical clipping (OR 10.67, 95% CI [2.12–68.04], p = 0.002) and resection (OR 5.74, 95% CI [1.18–36.47], p = 0.029) were associated with neurological improvement, whereas lesions in the mid-thoracic region (T6–T9) had reduced likelihood of improvement (OR 0.18, 95% CI [0.04–0.76], p = 0.017). Conservative management correlated with higher mortality (OR 33.89, 95% CI [1.39–826.08], p = 0.023). In three retrospective studies covering 66 patients (mean age 61.5 years, 80.3% male), primary interventions were surgical or endovascular; 63.6% improved, 35.0% worsened, and 1.5% died at follow-up.

Conclusions

Early detection by clinical and radiologic signs with quick intervention in the subtypes of FAS are crucial. Surgical clipping and resection demonstrated particularly favorable results, whereas conservative management was associated with increased mortality. Clinicians should maintain a high index of suspicion for subacute myelopathy to facilitate timely diagnosis and improve long-term prognosis.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Neurochirurgie 医学-临床神经学

CiteScore

2.70

自引率

6.20%

发文量

100

审稿时长

29 days

期刊介绍： Neurochirurgie publishes articles on treatment, teaching and research, neurosurgery training and the professional aspects of our discipline, and also the history and progress of neurosurgery. It focuses on pathologies of the head, spine and central and peripheral nervous systems and their vascularization. All aspects of the specialty are dealt with: trauma, tumor, degenerative disease, infection, vascular pathology, and radiosurgery, and pediatrics. Transversal studies are also welcome: neuroanatomy, neurophysiology, neurology, neuropediatrics, psychiatry, neuropsychology, physical medicine and neurologic rehabilitation, neuro-anesthesia, neurologic intensive care, neuroradiology, functional exploration, neuropathology, neuro-ophthalmology, otoneurology, maxillofacial surgery, neuro-endocrinology and spine surgery. Technical and methodological aspects are also taken onboard: diagnostic and therapeutic techniques, methods for assessing results, epidemiology, surgical, interventional and radiological techniques, simulations and pathophysiological hypotheses, and educational tools. The editorial board may refuse submissions that fail to meet the journal''s aims and scope; such studies will not be peer-reviewed, and the editor in chief will promptly inform the corresponding author, so as not to delay submission to a more suitable journal. With a view to attracting an international audience of both readers and writers, Neurochirurgie especially welcomes articles in English, and gives priority to original studies. Other kinds of article - reviews, case reports, technical notes and meta-analyses - are equally published. Every year, a special edition is dedicated to the topic selected by the French Society of Neurosurgery for its annual report.