There was no statistical correlation between the age of onset and sex or inheritance. Went et al Ribai et al 6. Huntington’s disease is a genetically inherited degenerative neurological disorder, characterized by motor alterations, including involuntary movements such as chorea, dementia and psychiatric disturbances. The results of brain magnetic resonance performed in some of the patients were also analysed. Shortly afterwards, he moved to Pomeroy in Ohio, where he began work as a family doctor [ 1 ]. A review of the literature found 62 studies that reported the proportion of JHD cases amongst all HD cases.
Young et al Fig 4 summarises these studies from high income countries. Secondly, data are included that are published in more than one paper. By comparison, three studies came from the upper middle income group, two came from Venezuela 10, 44 and the third study was that of Hayden 28 , where the data from the White South African population was included in the high income analysis and data from the mixed population was included in the middle income group. MD thesis, University of London, pp. Almqvist et al
Huntington’s disease: clinical aspects in 81 patients
Creighton et al Forest plot of post studies using MMA from high income countries. His father introduced comments and suggestions that were then included in the final version of the article [ 26 ]. Most studies were conducted in Europe and North America so the effect of considering geography was minimal Figure 4 ; however, the three studies from economically less developed countries Figure 5 were effectively from the South African black population dissertztion Venezuela.
Biographical sketch of George Huntington. Get free access to newly published articles Create a personal account or sign in to: Multiple methods of ascertainment MMA.
The Prevalence of Juvenile Huntington’s Disease: A Review of the Literature and Meta-Analysis
To account for any biases relating to age-based population structure, studies were further divided by economic status as defined by the World Bank Throughout his entire professional life, George Huntington vigorously participated in the activities of several local medical associations, acting for a period as vice-president in and then elected president in of the Dutchess County Medical Society.
Data on childhood and adolescent onset cases was available for 42 studies, representing cases.
Annual Mid-year Population Estimates, Published online Apr Of these, Lima et al Low prevalence rates of less than 1 case perhave been reported in Japanese populations 11, If a faster rate of disease progression can be demonstrated in this group of patients, then any compound which affects the natural history of HD may show an effect more quickly.
Cases in the Hayden South African study 28 were categorised by ethnicity into white, mixed and black populations recognising that the white South African population has a mortality distribution similar to that in countries listed as high income, whereas the mixed population has a mortality distribution similar to countries in the upper middle income group no cases were reported huntingtin the black population.
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Huntington’s chorea in the Netherlands – The problem of genetic heterogeneity. Atac et al The study by van Dijk et al 2 was excluded as this literature survey was specifically searching for articles with JHD cases and did not define a denominator total HD population.
Molecular diagnosis of Huntington disease in Brazilian patients. Ribai et al 6.
His contribution to neuroscience was recognized during his lifetime and, inhe became an honorary member of the Brooklyn Society of Neurology [ 89 ]. Ramos-Aroyo et al Simpson et al The longitudinal study of families from the area around Lake Maricaibo has been important in that it contributed to the original localisation of the gene to chromosome 4 and for an understanding of the natural history of the condition 10 44 80 The mean proportion of JHD cases from these three studies was 9.
Shortly afterwards, he moved to Pomeroy in Ohio, where he began work chlrea a family doctor [ 1 ]. There was no statistical correlation between the age of onset and sex or inheritance.
Prevalence in African and American black populations is also considered to be lower than in white populations chlrea, In addition, the patients with HD living around Lake Maricaibo are a relatively closed community so it is possible that this also has an effect on the proportion of JHD cases. Sign in to save your search Sign in to your personal account.
Akbas F, Erginel-Unaltuna N. Signal intensity abnormalities in T2-weighted images in the putaminal region were observed in 5 patients.
Predictability of Phenotype in Huntington’s Disease
Forest plot of studies using MMA from high income countries. Behavioural problems and cognitive decline are also common in JHD 4, 6with additional features of developmental delay and autism in childhood cases 5. Ten were conducted in high income and three in the upper middle income.