A rare disease of the spinal cord characterised by softening of the grey matter with obliterative sclerosis of the small vessels and thickening of the walls of the large vessel supplying the spinal cord. The lesions are especially located in the veins of the spinal meninges, cord, and nerve roots. This results in spasticity of the lower limbs, sensory changes and sometimes loss of sphincter control. Most common in persons aged 20 to 40. Death takes place within 1 to 2 years.
First described by Foix and Alajouanine in 1926 as «subacute necrotizing myelitis». Acute forms have subsequently been described.
- C. Foix, T. Alajouanine
La myélite nécrotique subaiguë (Myélite centrale angiohypertrophique à évolution progressive). Paraplégie amyotrophique lentement ascendante d'abord spasmodique, puis flasque.
Revue neurologique, Paris, 1926, 2: 1-42.