![]() ![]() Huntington’s disease aged late onset Huntington’s disease trinucleotide repeat expansion. Further characterization of this population will aid clinicians in diagnosis. LoHD represents a substantial proportion of new diagnoses of HD and has some unique features. Cognitive impairment rather than chorea may be the major source of disability in this group. Individuals with LoHD may have slower progression of illness. Motor manifestations are the commonest initial presentation, although 29.2% presented with non-motor manifestations as the first clinical feature in one case series. 94.4% of reported cases of LoHD had CAG repeat lengths of ≤44. Proportion of LoHD without a positive family history ranges from 3-68%. Among Caucasian HD cohorts, 4.4-11.5% of individuals have LoHD, and this proportion may be increasing. We included all studies reporting the clinical phenotype of LoHD for more than one participant.Ģ0 studies were identified from a potential list of 1243. Content experts were consulted for any additional studies. Web of Science was then used to search for papers citing identified studies. We systematically searched MEDLINE, EMBASE and Web of Science (inception-November 2016). We can be grateful that Huntington challenged us to consider the role that. To review the epidemiology, genotype and phenotype of LoHD. We assess the degree to which propositions from Samuel Huntingtons The Clash. Diagnosis of Late onset HD (LoHD) can be missed, due to the perceived low likelihood of HD in the over 60-year-olds. Although the typical age of onset for Huntington's disease (HD) is in the fourth decade, between 4.4-11.5% of individuals with HD have a late onset (over 60 years of age). ![]()
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