A disease of the nervous system characterized by degeneration of the muscles innervated by the affected nerves, particularly amyotrophy of the upper extremities, fibrillation, ataxia, positive Romberg sign, intention tremor, disturbed vision, sluggish pupils, sensory disorders of the hand, and absent or depressed reflexes of the Achilles tendon and foot. Wide distribution of lancinating pains (not constant); nerves are occasionally palpable and tender when touched. Onset in the third decade or later, usually in the fifth.
Its autonomous entity is doubted by some authors. It may be considered also as the adult type of Dejerine-Sottas. The term is no longer commonly used.
- G. Roussy, L. Cornil:
Névrite hypertrophique progressive non familiale de l’adulte.
Annales de médecine, Paris, 1919, 6: 296-305.