Cause of death in Huntington's Disease

Abstract

Huntington’s disease (HD) is a progressive neurodegenerative autosomal dominant disease characterized by choreatic and hypokinetic movements, disturbed behaviour, and cognitive decline. Death usually results from respiratory complications often caused by dysphagia. Previous studies about the cause of death in HD have shown that the most frequent primary cause of death is pneumonia. The pneumonia is never classified by type, eg community-acquired pneumonia (CAP), hospital-acquired pneumonia (HAP), chemical pneumonia, and aspiration pneumonia. The hypothesis on this study is that the most primary cause of death in HD is aspiration pneumonia. Therefore, date regarding 224 deceased HD patients, collected in the Leiden brainbank, were obtained. A significant difference was found for the patients who definitely/probably died from aspiration pneumonia, and the patients who died from a bacteremic pneumonia (χ2=.001). No significant difference was found for gender and aspiration (χ2=.931).