Déjérine-Sottas disease /dezh″ərin sot″əz, -sotäz″/ [Joseph J. Déjérine, French neurologist, 1849–1917; Jules Sottas, French neurologist, 1866–1943] , a rare congenital spino-cerebellar disorder characterized by the development of palpable thickenings along peripheral nerves, degeneration of the peripheral nervous system, pain, paresthesia, ataxia, and diminished sensation and deep tendon reflexes. Diagnosis is made by a histological examination of a peripheral nerve. There is no specific treatment. Also called progressive interstitial hypertrophic neuropathy.
