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. 1972 Dec;35(6):749–755. doi: 10.1136/jnnp.35.6.749

Progressive cerebral poliodystrophy – Alpers' disease

Disorganized giant neuronal mitochondria on electron microscopy

U Sandbank 1,2,3,4, P Lerman 1,2,3,4
PMCID: PMC494177  PMID: 4647849

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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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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