RT Journal Article
SR Electronic
T1 E04 Causes of death in Huntington's disease
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP A22
OP A22
DO 10.1136/jnnp.2010.22638.4
VO 81
IS Suppl 1
A1 A-W Heemskerk
A1 R A C Roos
YR 2010
UL http://jnnp.bmj.com/content/81/Suppl_1/A22.2.abstract
AB Background Huntington's disease (HD) is a progressive neurodegenerative autosomal dominant disease characterised by choreatic and hypokinetic movements, disturbed behaviour and cognitive decline. Previous studies into the cause of death in HD have shown that the most frequent primary cause of death in HD is pneumonia. These studies did not classify the pneumonia by cause, for example, community acquired pneumonia, hospital acquired pneumonia, chemical pneumonia, aspiration pneumonia, etc. Therefore, it is not known from which type of pneumonia HD patients die. Because most HD patients have dysphagia, especially in the advance stage of the disease, it is most likely that aspiration would be the cause of the fatal pneumonia. It is important to distinguish the different types of pneumonia because when it is known that the most primary cause of death is aspiration pneumonia, interventions studies to dysphagia can be set up to prevent aspiration pneumonia. The aim of the present study is to report on the primary causes of death for HD patients, focusing on pneumonia. Methods We used the documents of deceased HD patients from the Leiden University Medical Centre (LUMC) brainbank in The Netherlands. We collected the following information: CAG repeat determination, gender, year of birth, year of death, age at onset, location of death, naturally death, autopsy of the body, described primary cause of death, described underlying cause of death, reverse pneumonias, other notable aspects (eg dysphagia). Results Results thus far. Data regarding 224 deceased HD patients from 1964 to 2010 were obtained. 55.1% of the patients died from pneumonia. From the patients who deceased from pneumonia, 89.4% died from aspiration pneumonia. Other results needs to be worked out. Conclusion The most primary cause of death in HD is aspiration pneumonia.