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

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.

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.

Early detection by clinical and radiologic signs with quick intervention in the subtypes of FAS arecrucial. 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.