Functional imaging in neuroacanthocytosis

The published functional neuroimaging results indicate that the major finding in neuroacanthocytosis (NA) is severe striatal hypometabolism, either with or without atrophy. There is some evidence that the right side is more affected than the left. Cortical abnormalities are either not present or subtle, but if present appear to be particularly located in the frontal cortex. As with NA, an intriguing variety of devastating movement disorders may arise when specific striatal lesions occur. Due to the low prevalence of NA only a few patients have been investigated to date, and further studies are needed.