Presenile‐onset cerebral adrenoleukodystrophy presenting as Balint's syndrome and dementia

Abstract

We describe a patient with presenile-onset cerebral adrenoleukodystrophy presenting as Balint's syndrome and dementia. There were demyelinating MRI changes in the parieto-occipital white matter bilaterally, including the splenium of the corpus callosum. Therapeutic trials using l-deamino-(8-D-arginine)-vasopressin, very long-chain fatty acid-free diet, and μM-globulin were of no benefit.