Parkinsonism Relat Disord 20 7 — J Med Genet 30 12 — Clin Genet 65 4 — J Neurol Sci 1—2 — Myers RH Huntington's disease genetics. NeuroRx 1 2 — Am J Hum Genet 37 3 — J Neurol Neurosurg Psychiatry 48 6 — Int Rev Neurobiol — D, … Singapore E. Parkinsonism Relat Disord — Lancet Neurol 10 1 — Clin Genet 70 4 — Shoulson I, Fahn S Huntington disease: clinical care and evaluation. Neurology 29 1 :1—3. Mech Ageing Dev 2 — Mov Disord 26 5 — Huntington study group. Mov Disord 11 2 — Article Google Scholar.
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If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. Reprints and Permissions. Capiluppi, E. Neurol Sci 41, — Download citation. Received : 29 June Accepted : 25 November Published : 09 December Issue Date : April Anyone you share the following link with will be able to read this content:.
Sorry, a shareable link is not currently available for this article. Follow us on:. Search PracticeUpdate Cancel. This study used data from a European registry database the EHDN Registry , a prospective monitored study of HD, to obtain an overview of this group of patients compared with HD patients with the common age of onset.
The average age of onset in the older group was The average size of the CAG repeat was Late-onset patients were more likely to present with motor symptoms They also tended to have more gait and balance problems. Progression, however, was slower. Nature Neuroscience , ; DOI: ScienceDaily, 5 May Karolinska Institutet. Gene variant determines early or late onset of Huntington's disease. Retrieved November 10, from www. Saliva samples from 5, kids were scanned Published online Jun Sai S.
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Genotype CAG repeat length is an important determinant for the age of onset. Table 1 Characteristics and clinical features at presentation, of individuals with Late Onset Huntington Disease. Cornejo-Olivas et al. Clinical features reported at examination. Davis et al. Nance, Westphal and Nugent [ 52 ] 6 Selected to have no family history Macmillan, Davies and Harper [ 53 ] 4 Warren et al.
Faught et al. People were selected to have reduced penetrance alleles. Ruiz et al. Appollonio et al. Phenotype Table 1 summarizes the clinical phenotype of people with LoHD. Diagnosis and family history As noted, the diagnosis of LoHD may be difficult. Progression While chorea is the predominant manifestation, cognitive decline is often the major determinant of disability.
Prognosis There have been conflicting reports in the literature concerning the survival of individuals with LoHD. Conclusions Age of onset for clinical HD can be difficult to determine, due to its slowly progressive nature, unawareness of manifestations among people with HD, and considerable variation in clinical presentation [ 49 ]. Am J Hum Genet. J Neurol Neurosurg Psychiatry. Severity of cognitive impairment in juvenile and late-onset Huntington disease. Arch Neurol. Age Ageing.
Late onset Huntington Disease: Clinical and genetic characteristics of 34 cases. J Neurol Sci. Parkinsonism Relat Disord. Geriatr Gerontol Int. Clinical and molecular features of late onset Huntington disease in a Peruvian cohort. Mov Disord. Which diagnostic procedures in the elderly?
J Geriatr Psychiatry Neurol. BMJ Open. J Med Genet. Huntington disease in Georgia: Age at onset. High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia.
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