Foix-Alajouanine syndrome mimicking a spinal cord tumor

Summary Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.

Severe necrotizing myelopathy from toxacariasis

We present a 25 year old female veterinarian technician presenting with rapidly progressive quadriplegia in less then 12 hours. Her symptoms occurred at work with initially bilateral hand weakness followed by arm and leg weakness. Before the end of the day she was on a ventilator in the ICU. MRI showed a hyperintense longitudinal T2 signal extending from the cervical medullary junction to T1. Extensive cervical spinal cord edema with cord expansion was noted. CSF showed normal protein and cell count with no oligoclonal banding. A post-infectious inflammatory process causing transverse myelitis was presumed and she was given IVIG, steroids, and plasmapheresis with no improvement. A serum ELISA test for IgG to Toxocara was reactive at titre of 1:800 at 3 weeks after her initial presentation. Her serum IgE levels was elevated at 169 x 10x3 U/L (Normal <87 x 10x3 U/L). At 4 weeks, she was commenced on albendazole at 800 mg per day for two months. A repeat serum ELISA test at 6 weeks and 2 weeks into her treatment with albendazole showed a declining titre of 1:200 consistent with recent Toxocara infection. At 10 weeks, her ELISA test was non-reactive. Unfortunately she did not respond to albendazole treatment and she shows minimal improvement now 1.5 years later.