Progressive Cerebral Poliodystrophy--Alpers' Disease. Disorganized Giant Neuronal Mitochondria on Electron Microscopy

Overview

Journal J Neurol Neurosurg Psychiatry

Publisher BMJ Publishing Group

Specialties Neurology
Neurosurgery
Psychiatry

Date 1972 Dec 1

PMID 4647849

Citations 11

Authors

U Sandbank

P LERMAN

Affiliations

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Abstract

Three siblings who suffered from progressive mental retardation, seizures, and rigidity showed degeneration of the cerebral cortex. This was manifested by severe to complete neuronal loss with astrogliosis and microgliosis. In one child a brain biopsy was performed at the age of 3 months. The only lesion found was large disorganized perinuclear mitochondria in the neurones. The possibility that the cerebral poliodystrophy is due to an inherited mitochondrial disorder is discussed.

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