Presenile-onset cerebral adrenoleukodystrophy presenting as Balint's syndrome and dementia.
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 1-deamino-(8-D-arginine)-vasopressin, very long-chain fatty acid-free diet, and gamma-globulin were of no benefit.
Uyama
E
E
First Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
Iwagoe
H
H
Maeda
J
J
Nakamura
M
M
Terasaki
T
T
Ando
M
M
D000326:Adrenoleukodystrophy / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D003704:Dementia / Q000209:etiology*; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D014960:X Chromosome