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Natural History and Burden of Huntington's Disease in the UK: A Population-Based Cohort Study

Furby, Hannah; Siadimas, Athanasios; Rutten-Jacobs, Loes; Rodrigues, Filipe B; Wild, Edward J; (2022) Natural History and Burden of Huntington's Disease in the UK: A Population-Based Cohort Study. European Journal of Neurology 10.1111/ene.15385. (In press).

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Abstract

BACKGROUND: Huntington's disease (HD) is a rare neurodegenerative disease that presents with progressive psychological, cognitive and motor impairment. These diverse symptoms place a high burden on the patient, families and the healthcare systems they rely on. This study aimed to describe the epidemiology and clinical burden in individuals with HD compared with controls from the general population. METHODS: This cohort study utilised data from general practitioner (GP) medical records to estimate the prevalence and incidence of HD between Jan 2000 and Dec 2018. A cohort of incident HD cases were matched 1:3 to controls from the general population, in whom common clinical diagnoses, medications and healthcare interventions were compared at the time of first recorded diagnosis and at a time close to death. Incidence rates of common diagnoses and mortality were compared with matched controls in the time following HD diagnosis. RESULTS: Prevalence of HD increased between 2000 and 2018, whilst incidence remained stable. Prevalence of psychiatric diagnoses and symptomatic treatments were higher in HD cases than controls. A higher relative risk of psychotic disorders, depression, insomnia, dementia, weight loss, pneumonia and falls was observed in HD cases. Risk of death was >4 times higher in HD, with a median survival of ~12 years from first recorded diagnosis. CONCLUSIONS: This study demonstrates the significant and progressive clinical burden in individuals up to 18 years after first recorded diagnosis.

Type: Article
Title: Natural History and Burden of Huntington's Disease in the UK: A Population-Based Cohort Study
Location: England
DOI: 10.1111/ene.15385
Publisher version: https://doi.org/10.1111/ene.15385
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Huntington’s disease, comorbidity, incidence, mortality, prevalence
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
URI: https://discovery.ucl.ac.uk/id/eprint/10148423
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