Neurocognitive decline in Alexander disease.

[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 . An MRI scan of the brain found acute demyelinating encephalomyelitis , with a preponderance of white matter degeneration in the bilateral frontal lobes . Interval MRIs showed continued degeneration . Confirmation of Alexander disease was made at age 20 through genetic testing . Four evaluations completed from ages 15 to 21 showed impairment across all cognitive domains . Cognitive deficits were most prominent in new learning and recent memory , executive functions , and fine motor dexterity , and less apparent in information processing and visual scanning speed . These results present evidence for a particular cognitive pattern in individuals with juvenile -onset Alexander disease . Despite extensive white matter degeneration in the frontal lobes , certain tasks associated with frontal lobe integrity were relatively preserved . Further research into the neuropsychological presentation of the subtypes of Alexander disease can enhance diagnostic clarity and treatment planning .