Abstract
The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father.
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Selected References
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- Currier R. D., Jackson J. F., Meydrech E. F. Progression rate and age at onset are related in autosomal dominant neurologic diseases. Neurology. 1982 Aug;32(8):907–909. doi: 10.1212/wnl.32.8.907. [DOI] [PubMed] [Google Scholar]
- Farrer L. A., Conneally P. M., Yu P. L. The natural history of Huntington disease: possible role of "aging genes". Am J Med Genet. 1984 May;18(1):115–123. doi: 10.1002/ajmg.1320180115. [DOI] [PubMed] [Google Scholar]
- Myers R. H., Sax D. S., Koroshetz W. J., Mastromauro C., Cupples L. A., Kiely D. K., Pettengill F. K., Bird E. D. Factors associated with slow progression in Huntington's disease. Arch Neurol. 1991 Aug;48(8):800–804. doi: 10.1001/archneur.1991.00530200036015. [DOI] [PubMed] [Google Scholar]
- Myers R. H., Vonsattel J. P., Stevens T. J., Cupples L. A., Richardson E. P., Martin J. B., Bird E. D. Clinical and neuropathologic assessment of severity in Huntington's disease. Neurology. 1988 Mar;38(3):341–347. doi: 10.1212/wnl.38.3.341. [DOI] [PubMed] [Google Scholar]
- Roos R. A., Vegter-van der Vlis M., Hermans J., Elshove H. M., Moll A. C., van de Kamp J. J., Bruyn G. W. Age at onset in Huntington's disease: effect of line of inheritance and patient's sex. J Med Genet. 1991 Aug;28(8):515–519. doi: 10.1136/jmg.28.8.515. [DOI] [PMC free article] [PubMed] [Google Scholar]
- van Dijk J. G., van der Velde E. A., Roos R. A., Bruyn G. W. Juvenile Huntington disease. Hum Genet. 1986 Jul;73(3):235–239. doi: 10.1007/BF00401235. [DOI] [PubMed] [Google Scholar]