The Syndrome of Progressive Cerebral Poliodystrophy

Abstract

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