title: Alexander's disease and the story of Louise.
abstract: We describe the rare condition known as Alexander's disease or Alexander's leukodystrophy, which is essentially a childhood dementia. We then present the case of Louise Davies (we are using Louise's real name with the permission and special request of her mother), a woman who was diagnosed with this disease at the age of 5 years and is still alive at the age of 38, making her the longest known survivor of this condition. Although now severely impaired, both physically and mentally, and able to do very little, she has lived far longer than expected. We present some neuropsychological results from her childhood before measuring her decline over the past four years. We conclude by considering whether or not the diagnosis was correct and why she has lived so long.
authors: a Neuropsychology, The Oliver Zangwill Centre , Ely , UK.|b Neuropsychology, The Raphael Medical Centre , London , UK.
authors: Barbara A
authors: BA
authors: Wilson
authors: c Head Office , UCL Institute of Child Health, and Great Ormond Street Hospital for Children NHS Foundation Trust , Tonbridge , UK.
authors: Faraneh
authors: F
authors: Vargha-Khadem
authors: b Neuropsychology, The Raphael Medical Centre , London , UK.
authors: Gerhard
authors: G
authors: Florschutz
mesh_terms: D000328:Adult; D038261:Alexander Disease / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology / Q000523:psychology*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D003704:Dementia / Q000000981:diagnostic imaging / Q000473:pathology / Q000523:psychology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D009483:Neuropsychological Tests
keywords: Alexander's disease; childhood dementia; leukodystrophy; neuropsychological assessment