Disclosures: Timothy G. Bazil, DO: No financial relationships or conflicts of interest

Case Diagnosis: Progressive Thoracic Myelopathy due to Foix-Alajouanine Syndrome

Case Description or Program Description: A 62-year-old male presented 9/2021 for abnormal MRI with progressive LE weakness for ~1.5 years. MRI L-spine demonstrated increased T2-signal changes in lower thoracic spinal cord with prominent intraspinal vessels from suspected ischemic myelopathy, transverse myelitis, versus Foix-Alajouanine Syndrome. On evaluation he had spastic LE paraparesis, sensory deficits, clonus, and bowel/bladder/sexual dysfunction. MRI T-spine demonstrated vascular tortuosity and prominence around thoracic spinal cord with diffuse edema from T4 through conus. Arteriogram revealed Type 1 spinal dural AV fistula arising from a distal radicular branch off T11 intercostal artery, with cephalad and caudal intradural venous drainage.

Setting: Major academic and referral center with Level I adult trauma and Level II pediatric trauma status

Assessment/Results: Patient was evaluated by Neurosurgery and Interventional Radiology. He underwent endovascular Onyx embolization of the AV fistula. He was transferred to IPR unit and discharged at wheelchair level.

Discussion (relevance): This case demonstrates a patient with progressive myelopathy from spinal AV malformation, otherwise known as subacute ascending necrotizing myelitis, or Foix-Alajouanine Syndrome – first described in 1926 in two men with acute-subacute neurological deterioration from spinal AV malformation/vascular congestion*. The disease is typically in men >50*. It affects extrinsic/intrinsic pial veins of spinal cord vasculature, typically lower thoracic/lumbar spinal cord*. If not diagnosed and treated effectively, it can lead to necrotic myelopathy from thrombosis and infarction*. Of note, intravenous steroids can worsen symptoms in patients with spinal AV malformations*.

Conclusions: This patient had worsening neurologic compromise over 1.5 years; presentation is similar to other neurologic conditions including MS or transverse myelitis, in which intravenous steroids are typical treatment. The overall incidence of Foix-Alajouanine is nebulous but estimated ~5-10 million people/year based on 2001 retrospective study*, although likely underdiagnosed*. Early diagnosis and treatment is essential, and diagnoses of AV malformations shouldn’t be overlooked.

Level of Evidence: Level V

« Back to AAPM&R Annual Assembly 2022

PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/progressive-thoracic-myelopathy-due-to-foix-alajouanine-syndrome-a-case-report/