Neurocognitive decline in Alexander disease.

Alexander disease is a neurological condition associated with prominent white matter deterioration. Its rarity and relatively rapid disease course have provided limited understanding into the cognitive effects of the illness. We report the serial neuropsychological findings of a 21-year-old with normal development and no medical history until age 9, when he experienced refractory sinusitis, stabbing headaches with vertigo, disorientation, and decline in academic and social settings.