Ophthalmoplegia Plus

CONSIDERABLE interest has been focused on the entity of progressive external ophthalmoplegia (PEO) since the report of Kiloh and Nevin in 1951.1These authors were primarily concerned with the pathogenesis of the ocular muscle weakness, concluding that it was virtually always the result of a myopathic process. They regarded PEO as a distinctive and relatively isolated disorder, although occasionally associated with mild myopathy elsewhere; they considered its association with other neurodegenerative findings to be "extremely rare," citing only the cases of Marburg2and of Walsh.3More recently, however, PEO has been linked with a variety of neurologic abnormalities in increasing numbers of reports. In addition to myopathic involvement of limb musculature, other neurologic symptoms including pharyngeal weakness, peripheral neuropathy, cerebellar ataxia, spasticity, deafness, optic atrophy, and dementia have been observed in conjunction with PEO. Other organ systems also involved have included the heart, endocrine glands, skin,