A case of necrotic myelopathy due to a spinal vascular malformation (Foix-Alajouanine's disease)

A woman of 65 years, after a period of painful paraesthesiae in the legs, developed a polyneuritic syndrome and also sphincter disturbance. At first the c.s.f. showed albuminocytological dissociation. The polyneuropathy was initially thought to be of diabetic origin, as the blood sugar was moderately raised. For nearly two years the symptoms were not very disabling, in spite of variations in severity, but then there was a sudden onset of severe paraplegia with a sensory level of D 6--7 and bilateral extensor responses. Myelography was abnormal but inconclusive. Her clinical condition gradually deteriorated and severe pressure sores developed. Death occurred five years after the appearance of the first symptoms. Post-mortem examination revealed diffuse necrosis in most of the thoracic region of the cord which showed at this level a large arterio-venous angioma on its posterior part.