Progressive cerebral poliodystrophy – Alpers' disease

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 l...

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Bibliographic Details
Published in:Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 1972-12, Vol.35 (6), p.749
Main Authors: Sandbank, U., Lerman, P.
Format: Article
Language:English
Online Access:Get full text
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Summary: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.
ISSN:0022-3050
1468-330X
DOI:10.1136/jnnp.35.6.749