eprintid: 1513405
rev_number: 41
eprint_status: archive
userid: 608
dir: disk0/01/51/34/05
datestamp: 2016-09-03 20:13:10
lastmod: 2021-12-11 00:03:26
status_changed: 2016-12-16 12:03:30
type: article
metadata_visibility: show
creators_name: Wilson, BA
creators_name: Vargha-Khadem, F
creators_name: Florschutz, G
title: Alexander's disease and the story of Louise.
ispublished: pub
divisions: UCL
divisions: B02
divisions: D13
divisions: G26
keywords: Alexander's disease, childhood dementia, leukodystrophy, neuropsychological assessment
note: This is an Accepted Manuscript of an article published by Taylor & Francis in Neuropsychological Rehabilitation  on 31 Aug 2016, available online: http://dx.doi.org/10.1080/09602011.2016.1222945.
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.
date: 2018
date_type: published
official_url: http://dx.doi.org/10.1080/09602011.2016.1222945
oa_status: green
full_text_type: other
language: eng
primo: open
primo_central: open_green
verified: verified_manual
elements_id: 1156365
doi: 10.1080/09602011.2016.1222945
lyricists_name: Vargha-Khadem, Faraneh
lyricists_id: FVARG91
full_text_status: public
publication: Neuropsychological Rehabilitation
volume: 28
number: 2
pagerange: 199-207
event_location: England
issn: 1464-0694
citation:        Wilson, BA;    Vargha-Khadem, F;    Florschutz, G;      (2018)    Alexander's disease and the story of Louise.                   Neuropsychological Rehabilitation , 28  (2)   pp. 199-207.    10.1080/09602011.2016.1222945 <https://doi.org/10.1080/09602011.2016.1222945>.       Green open access   
 
document_url: https://discovery.ucl.ac.uk/id/eprint/1513405/1/Vargha-Khadem_Final%20Alexander%27s%20Disease%20paper%2019.04.161.pdf