Canavan Disease (Spongy Cerebral Degeneration) - A Case Report -

Abstract

We report a case of Canavan disease in a 6 month-old male Korean baby who presented with aggravating tonic-clonic seizure for 5 days. Pathologic' findings could be summarized as follows; increase in brain volume and weight, spongy degeneration in the deep layers of the cerebral cortex and subcortical white matter, and hyperplasia of Alzheimer type II astrocytes throughout the cerebral cortex. Ultrastructurally, there was an abnormal accumulation of fluid in astrocytes and between splitting myelin lamellae.