Foroud T, Gray J, Ivashina J, Conneally P M
Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis 46202, USA.
J Neurol Neurosurg Psychiatry. 1999 Jan;66(1):52-6. doi: 10.1136/jnnp.66.1.52.
Data from a sample of 2494 patients affected with Huntington's disease (HD), collected as part of the National Research Roster for Huntington Disease Patients and Families, were examined to determine if there was a relation between age at onset and duration of illness.
Sufficient data for inclusion in analysis was available from 2068 patients, of whom 828 were deceased and 1240 were living. The median duration of disease was 21.4 years with a range of 1.2 to 40.8 years. Patients were categorised into one of four groups based on their age at onset.
Significant differences in duration based on the age at onset were found (p<0.025), with juvenile and late onset patients with HD having shorter duration of illness compared with those with an onset between 20-49 years.
Duration of disease is influenced by the age at symptom onset with juvenile and late onset patients having the shortest duration.
作为全国亨廷顿病患者及家庭研究名册的一部分，收集了2494例亨廷顿病（HD）患者的样本数据，以确定发病年龄与病程之间是否存在关联。
2068例患者有足够的数据纳入分析，其中828例已死亡，1240例尚在世。疾病的中位病程为21.4年，范围为1.2至40.8年。根据发病年龄将患者分为四组之一。
发现基于发病年龄的病程存在显著差异（p<0.025），与发病年龄在20-49岁之间的患者相比，青少年型和晚发型HD患者的病程较短。
疾病病程受症状发作年龄的影响，青少年型和晚发型患者的病程最短。
