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June 1968

Ophthalmoplegia Plus: The Neurodegenerative Disorders Associated With Progressive External Ophthalmoplegia

Author Affiliations

From the Department of Neurology and Psychiatry, Northwestern University Medical School, Chicago.

Arch Neurol. 1968;18(6):654-674. doi:10.1001/archneur.1968.00470360076008

CONSIDERABLE interest has been focused on the entity of progressive external ophthalmoplegia (PEO) since the report of Kiloh and Nevin in 1951.1 These 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 Marburg2 and of Walsh.3 More 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,

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