Foix-Alajouanine Syndrome: Case Report

ABSTRACT

Acute or subacute myelopathy with spontaneous thrombosis of the veins in a patient with a spinal arteriovenous malformation(AVM) has been referred to as "Foix-Alajouanine syndrome(FAS)". A previously healthy 15-year-old girl developed neurological illness with progressive lower extremity weakness and low back pain. The MRI demonstrated the diffuse swelling of the spinal cord around the 10th thoracic level, suggesting myelopathy. Her neurological symptoms deteriorated to paraplegia, voiding difficulty and areflexia. Spinal arteriography did not demonstrate a vascular lesions such as arteriovenous malformation(AVM) or arteriovenous fistula(AVF). Operation revealed diffuse necrosis of the spinal cord and one abnormal thrombosed drainage vein. Progressive clinical illness, the histopathology of the patchy necrosis of spinal cord tissue and many thickened blood vessels are compatible with Foix-Alajouanine syndrome. We report a case of Foix-Alajouanine syndrome with a review of the literature.