Perineurial cell hyperplasia in early-onset polyneuropathy with multiple cranial neuropathies

We describe a patient who as a teenager developed a sensory-motor polyneuro pathy with optic atrophy. Over the next three decades, multiple cranial neu ropathies appeared. Striking areas of subperineurial cellular proliferation were observed on sural nerve biopsy. The ultrastructural and immunohistoch emical characteristics of these aggregates were those of perineurial cell h yperplasia. To our knowledge, this is the second full report associating pe rineurial cell hyperplasia with a sensory-motor polyneuropathy and the firs t in the English literature. (C) 2000 Elsevier Science B.V. All rights rese rved.