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January 1964

The Syndrome of Progressive Cerebral Poliodystrophy

Author Affiliations

From the Neurology Section, University of Colorado School of Medicine and Denver Veterans Administration Hospital, and Department of Pathology, General Rose Memorial Hospital.

Arch Neurol. 1964;10(1):47-57. doi:10.1001/archneur.1964.00460130051008

The existence of a specific disorder of childhood characterized by a nonlipid neuronal destruction with preservation of myelinated structures has been proposed and has variously been termed Alpers' disease, Christensen-Krabbe disease, and progressive infantile cerebral poliodystrophy.1,2 Clinical descriptions have mentioned mental deterioration, spasticity, convulsions, myoclonic jerks, choreoathetosis, and ataxia. The microscopic lesion has been described as a widespread but unevenly distributed neuronal damage, mainly in the cerebral cortex, accompanied by astrocytic and microglial pro-liferation; the white matter usually is normal, and inflammation is absent. In the latest edition of Standard Nomenclature of Diseases and Operations, poliodystrophia cerebri progressiva is listed as a distinct entity.3

Doubt has been cast upon this concept by several authors who claim that the neuronal loss represents merely a nonspecific response to various types of injury.4-6 The variability of the signs, symptoms, and duration of disease makes it necessary to have histological

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