Peripheral Neuropathyを伴うSpinocerebellar Degenerationの病理像を呈したProgressive External Ophthalmoplegiaの1剖検例

Progressive external ophthalmoplegia: An autopsy case pathologically characterized by spinocerebellar degeneration with polyneuropathy

　進行性眼瞼下垂と外眼筋麻痺からなる症候群progressive external ophthalmoplegia（PEO）の剖検例は少なく，その病理像の多くが不明である。私たちはPEOを呈した症例を剖検する機会を得た。

　A 40 year-old woman with progressive external ophthalmoplegia accompanied by facial and pharyngeal weakness, limb muscle atrophy, deafness, sensory impairment, increased CSF protein, lower mental faculties, abnormal EEG and so on showed the neuropathological features of spinocerebellar degeneration with peripheral neuropathy.

　Electronmicroscopy revealed zebra bodies in Schwann cells of peripheral nerves.