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Published on: December 10, 2021
Natural history and burden of Huntington's disease in the UK: A population-based cohort study
Hannah Furby1, Athanasios Siadimas2, Loes Rutten-Jacobs2
1Roche Products Ltd, Welwyn Garden City, UK.
Insights
Huntington's disease (HD) prevalence increased from 2000-2018. Individuals with HD face a significantly higher clinical burden, including psychiatric diagnoses and a four-fold increased mortality risk, with a median survival of 12 years.
Area of Science:
- Neuroscience
- Epidemiology
- Clinical Medicine
Background:
- Huntington's disease (HD) is a rare, progressive neurodegenerative disorder.
- HD significantly impacts patients, families, and healthcare systems due to diverse symptoms.
- Understanding the epidemiology and clinical burden of HD is crucial.
Purpose of the Study:
- To describe the epidemiology of Huntington's disease.
- To quantify the clinical burden in individuals with HD compared to the general population.
Main Methods:
- Utilized general practitioner medical records for a cohort study (2000-2018).
- Matched incident HD cases (1:3) with general population controls.
- Compared diagnoses, medications, and healthcare interventions post-diagnosis and near death.
Main Results:
- HD prevalence increased, while incidence remained stable between 2000-2018.
- HD cases showed higher rates of psychiatric diagnoses and treatments.
- Increased risks for psychotic disorders, depression, dementia, falls, and mortality (4x higher) were observed in HD patients, with median survival of ~12 years.
Conclusions:
- This study highlights the substantial and progressive clinical burden associated with Huntington's disease.
- The findings underscore the long-term impact of HD on patient health and healthcare utilization.
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.
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