Two unrelated and previously healthy girls, aged 17 and 18, presented with a 
subacute encephalopathy, visual and sensory symptoms and signs, and prominent 
seizures that were difficult to control. Brain MRI showed lesions (high signal 
on T2 weighted images) in the occipital lobes and thalamus; EEG showed slow wave 
activity with superimposed polyspikes. Inexorable downhill progression led to 
death in hepatic failure within eight months of onset. Histopathological 
findings in both patients ((a) chronic hepatitis with prominent bile duct 
proliferation, fatty change, and fibrosis; (b) in the brain a patchy destruction 
of the cerebral cortex, predominantly involving striate cortex) were 
characteristic of progressive neuronal degeneration of childhood with liver 
disease--Alpers-Huttenlocher syndrome--a rare autosomal recessive disorder 
usually seen in infants and young children.