Computed tomography in the diagnosis of Canavan's disease.

Computed tomography (CT scan) demonstrated a symmetrical decrease in white matter attenuation of the cerebral hemispheres of two young children with macrocephaly and normal neurological examination. Subsequent developmental delay led to brain biopsy, which documented Canavan's disease (spongy degeneration of the brain, Van Bogaert-Bertrand type). The CT scans obtained from these patients with proved Canavan's disease appeared to be quite characteristic in differentiating this disorder from Alexander's disease and adrenoleukodystrophy (Schilder's disease). The CT scan may decrease the necessity for diagnostic brain biopsy in these white matter disorders.