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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1976 Aug;39(8):803–809. doi: 10.1136/jnnp.39.8.803

Alexander's disease presenting as astrocytoma.

T A French, B D Bower, A H Cameron
PMCID: PMC492454  PMID: 956866

Abstract

A case of progressive fibrinoid degeneration of astrocytes is described. Symptoms began with convulsions at the age of 6 weeks and cerebral tumour was suspected because of enlargement of the head, increased intracranial tension, and marked proliferation of astrocytes in the brain biopsy which was interpreted as an astrocytoma. Fibres and granules staining deeply with PTAH are a constant feature of the few cases so far described. The aetiology is unknown but an inherited metabolic defect of astrocytes may be responsible.

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Selected References

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