Abstract

Two cases are reported of progressive trigeminal neutopathy with nasal ulceration. One patient developed signs of spinal cord involvement 15 years after the onset of trigeminal symptoms and died after a total course of 21 years. Necropsy revealed an unusual trigeminospinal system degeneration with deposition of amyloid-like substances in the affected structures. The other patient is alive eight years after the onset of symptoms, the only indication of a lesion outside the trigeminal nerve being a patch of numbness in one leg.