Dejerine-Sottas disease

Dejerine-Sottas disease is a hereditary neurological disorder in which the peripheral nerves are damaged and lose their myelin coating, a process called demyelination. Over time this produces progressive wasting of muscle and a loss of sensation in the body. The condition arises from mutations in a number of different genes rather than a single one, and no cure is currently known. It is also called Dejerine-Sottas syndrome, hereditary motor and sensory polyneuropathy type III and Charcot-Marie-Tooth disease type 3, which places it within the wider family of inherited peripheral neuropathies. The name honours the French neurologists Joseph Jules Dejerine and Jules Sottas, who first described it.