[Gadolinium-MRI findings of two adrenoleukodystrophy cases treated with gamma-globulin]

Two cases of adrenoleukodystrophy were reported. We treated them with intravenous gamma-globulin infusion. MRI with gadolinium enhancement was performed before and after the therapy to evaluate the effect of gamma-globulin. Case 1 was a 19-year-old boy, who noticed left hemianopsia when he was 18 years old. Case 2 was a 36-year-old man, who developed ataxic gait and character change when he was 33 years old. Very long chain fatty acids in plasma and RBC membrane were elevated in both cases. On T2-weighted image of MRI, high signal lesions were seen in the white matter of occipital lobe in case 1, cerebellum, internal capsule, and around the corpus callosum in case 2. On T1-weighted image, the rim of every lesion was enhanced by gadolinium infusion. After the gamma-globulin therapy, gadolinium enhancement of the rim remarkably reduced in case 1. No reduction was observed in case 2. MRI findings of case 1 indicate the possibility that gamma-globulin may suppress the inflammation of the adrenoleukodystrophy.