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[Huntington disease in the Valencian Region]
L Páramo-Rodríguez1, S Moreno-Marro2,1, S Guardiola-Vilarroig1,3
1Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana, Valencia, España.
Revista De Neurologia
|May 26, 2023
Summary
Huntington disease (HD) affects 1.97 per 100,000 people in the Valencia Region, with men experiencing higher mortality and earlier death. This neurodegenerative disorder has a high mortality rate, with an average of 6.5 years between diagnosis and death.
Area of Science:
- Neurology
- Epidemiology
- Genetics
Context:
- Huntington disease (HD) is a rare, fatal neurodegenerative disorder.
- Understanding its epidemiological distribution is crucial for public health planning.
Purpose:
- To describe the geographical, age, and sex distribution of HD in the Valencia Region (VR).
- To determine the prevalence and mortality rates of HD in the VR between 2010 and 2018.
Summary:
- A cross-sectional study identified 225 HD cases in the VR from 2010-2018.
- Prevalence in 2018 was 1.97/100,000, with no significant trend. Median age at diagnosis was 54.1 years.
- Mortality rate in 2018 was 0.32/100,000. Men showed higher mortality and earlier age of death.
Impact:
- Findings align with global HD prevalence estimates.
- Highlights sex-based differences in mortality and age at death for HD.
- Provides essential data for targeted interventions and resource allocation for Huntington disease management.
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