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J34 Natural History Of Huntington’s Disease In Finnish Patients
  1. J Sipilä1,
  2. K Majamaa2
  1. 1University of Turku, Neurology, Kiinamyllynkatu 10-12, 20521 Turku, Finland
  2. 2University of Oulu, Neurology, Aapistie 5A, PL 5000, 90220 Oulu, Finland

Abstract

Background Huntington’s disease (HD) has been little studied in Finland. We have recently identified a nationwide cohort of 207 Finnish patients with HD.

Aim To study disease progression and survival in Finnish patients with HD.

Methods Patients treated for HD between the years 1987–2010 were identified by using the national hospital discharge registry and outpatient registry, the files of laboratories performing genetic diagnostics of HD in Finland and the files of the Family Federation of Finland. Patient records of the ascertained patients were then reviewed.

Results The mean age at diagnosis was 52.6 ± 12.1 years (range, 14–82 years). At the time of diagnosis, 23% of patients were working full-time, 72% lived independently, 65% were able to drive a car and 95% were able to walk unaided. These abilities deteriorated steadily with time and 10 years after the diagnosis all subjects were retired, unable to drive and lived independently. However, 30% were still able to walk unaided or with minimal aid. The abilities to live unaided and to drive correlated well. At the time of diagnosis, 38% of the subjects had problems in handling money and 26% had problems in dressing, but 10 years later 95% were completely unable to handle money and 48% were completely unable to dress. Mean survival was 67.4 years (95% CI; 64.4, 70.4) among men and 70.2 (67.1, 73.3) among women (p = 0.15 for difference, log-rank statistics). Among the 94 deceased patients the mean age of death was 59.9 years ± 12.8 years (range, 20–86 years).

Conclusions Disability begins to increase soon after the diagnosis of HD. Cognitive deterioration affects the functional status more than ambulatory deterioration during the first 10 years after the diagnosis. Mean survival is shortened compared to the general population with no difference between genders.

KeyWords
  • Huntington’s disease
  • Natural history
  • survival
  • Independent living
  • automobile driving

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