American Journal of Neuroradiology, Vol 11, Issue 4 805-810, Copyright © 1990 by American Society of Neuroradiology
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Canavan disease: CT and MR imaging of the brain
J Brismar, G Brismar, G Gascon and P Ozand
Department of Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Canavan disease (spongy degeneration of the brain) is a rare lethal neurodegenerative disorder of infancy; fewer than 100 cases have been reported. We describe a series of nine patients with enzymatic defects and clinical features consistent with Canavan disease; in two patients the disease was proved by biopsy. All patients were examined with CT and seven with MR. In every instance, both CT and MR showed white- matter disease, sometimes sparing the external and internal capsules, the corpus callosum, and the deep cerebellar white matter. Atrophic changes were found in six patients; the changes were marked in one and slight in five. There was no obvious correlation between the severity of the white-matter disease and the clinical presentation--one patient with severe white-matter disease was clinically normal. The CT and MR findings in Canavan disease are nonspecific and somewhat nonuniform: its preautopsy diagnosis relies primarily on biochemical findings.
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