Details

Autor(en) / Beteiligte

• Furby, Hannah
• Siadimas, Athanasios
• Rutten‐Jacobs, Loes
• Rodrigues, Filipe B.
• Wild, Edward J.

Titel

Natural history and burden of Huntington's disease in the UK: A population‐based cohort study

Ist Teil von

• European journal of neurology, 2022-08, Vol.29 (8), p.2249-2257

Ort / Verlag

England: John Wiley & Sons, Inc

Erscheinungsjahr

2022

Quelle

Wiley Online Library Journals

Beschreibungen/Notizen

• 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 medical records to estimate the prevalence and incidence of HD between January 2000 and December 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 with HD up to 18 years after first recorded diagnosis. Prevalence of Huntington's disease (HD) increased throughout the UK between 2000 and 2018, although incidence remained stable. Median survival time was 12.4 years. Incidence rates of psychotic disorders, insomnia, dementia, depression, pneumonia, weight loss and falls were higher in individuals with HD than a matched cohort from the general population. Occurrence of psychiatric comorbidities were notably higher in patients with HD prior to diagnosis and 3 months prior to death. This study provides updated epidemiological estimates on the prevalence and incidence of, and mortality in, HD, which together, increase our understanding of the physical, psychiatric and public health burden of HD.