A rare case of FOIX Alajouanine Syndrome with lipomyelocele and tethered cord syndrome

Abstract

Background: Foix Alajouanine syndrome (AFS) is a spinal arterio-venous fistula (AVF), which was first described by Foix and Alajouanine in 1926 who called it necrotizing myelopathy. The pathophysiology of AFS is still unclear but it is hypothesized that AFS is a progressive spinal cord venous thrombosis related to a spinal vascular lesions, resulting in necrotic myelopathy. It usually present after the fifth decade of life and is common among men. The most common clinical presentation is that of step-wise progressive myeloradiculopathy. Symptoms may initially fluctuate, but eventually a permanent and progressive paraparesis with sensory disturbances and sphincter dysfunction occur.