Alexander's disease and the story of Louise

Neuropsychol Rehabil. 2018 Mar;28(2):199-207. doi: 10.1080/09602011.2016.1222945. Epub 2016 Aug 31.


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.

Keywords: Alexander's disease; childhood dementia; leukodystrophy; neuropsychological assessment.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Alexander Disease / diagnosis
  • Alexander Disease / diagnostic imaging
  • Alexander Disease / pathology
  • Alexander Disease / psychology*
  • Brain / diagnostic imaging
  • Brain / pathology
  • Dementia / diagnostic imaging
  • Dementia / pathology
  • Dementia / psychology*
  • Disease Progression
  • Female
  • Humans
  • Neuropsychological Tests