scholarly journals The Prevalence of Huntington's Disease

2016 ◽  
Vol 46 (2) ◽  
pp. 144-153 ◽  
Author(s):  
Michael D. Rawlins ◽  
Nancy S. Wexler ◽  
Alice R. Wexler ◽  
Sarah J. Tabrizi ◽  
Ian Douglas ◽  
...  
Keyword(s):  
North America ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Lower Incidence ◽  
Western Europe ◽  
The Past ◽  
Case Ascertainment ◽  
Asian Populations ◽  
The United Kingdom ◽  
Geographical Regions

Background: Reviews of the epidemiology of Huntington's disease (HD) suggest that its worldwide prevalence varies widely. This review was undertaken to confirm these observations, to assess the extent to which differences in case-ascertainment and/or diagnosis might be responsible, and to investigate whether the prevalence pattern has changed over the past 50 years. Methods: Eighty two relevant studies were identified from Medline and Embase, previous reviews, scrutiny of references from included and excluded studies and enquiry among those interested in the field. Results: The lowest rates were among the Asians and the highest among the Caucasians. The differences are not fully explained by varying approaches to case-ascertainment or diagnosis. There was evidence of an increasing prevalence of between 15 and 20% per decade in studies from Australia, North America and Western Europe. Conclusions: The prevalence of HD varies more than tenfold between different geographical regions. This variation can in part be attributed to differences in case-ascertainment and/or diagnostic criteria, but there is consistent evidence of a lower incidence in Asian populations. There is also evidence that in Australia, North America and in Western Europe (including the United Kingdom), prevalence has increased over the past 50 plus years.

Huntington's disease-like 2 (HDL2) in North America and Japan

2004 ◽  
Vol 56 (5) ◽  
pp. 670-674 ◽  
Author(s):  
Russell L. Margolis ◽  
Susan E. Holmes ◽  
Adam Rosenblatt ◽  
Lisa Gourley ◽  
Elizabeth O'Hearn ◽  
...  
Keyword(s):  
North America ◽  
Huntington's Disease ◽  
Huntington’S Disease

Facts and figures about Huntington’s disease

2020 ◽  
pp. 1-8 ◽  
Author(s):  
Oliver Quarrell
Keyword(s):  
North America ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Original Description ◽  
The World ◽  
History Of ◽  
Different Parts

This chapter gives a brief history of Huntington’s disease (HD) starting with the original description by George Huntington. The prevalence of HD is discussed together with data variations in different parts of the world. For example the incidence of HD in Europe and North America is approximately 1 person in 10,000. Apart from humans, no other animal naturally develops HD. The condition can start at almost any age but it is more frequent in midlife. The duration of the disease can be very variable but 20 years is often given as an approximate average.

I29 Comparison of huntington’s disease in europe and North America

2016 ◽  
Vol 87 (Suppl 1) ◽  
pp. A68.4-A69
Author(s):  
Michael Orth ◽  
Juliana Bronzova ◽  
Christine Tritsch ◽  
Ray Dorsey ◽  
Joaquim J Ferreira ◽  
...  
Keyword(s):  
North America ◽  
Huntington's Disease ◽  
Huntington’S Disease

scholarly journals Genetic and Environmental Contributors to Neurodegeneration: An Exploration of the Effects of Alcohol on Clinical Features of Huntington’s Disease Using the Enroll-HD Global Platform

2021 ◽  
Vol 18 (10) ◽  
pp. 5113
Author(s):  
Alexander L. Symonds ◽  
Antonella Macerollo ◽  
Kevin Foy ◽  
Sundus H. Alusi ◽  
Rhys Davies
Keyword(s):  
Alcohol Use ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Features ◽  
Alcohol Intake ◽  
Alcohol Misuse ◽  
High Alcohol ◽  
The Past ◽  
Post Hoc ◽  
High Alcohol Intake

Huntington’s disease (HD) is a neurodegenerative dementia with a well recognised genetic cause. Alcohol misuse is a major environmental factor relevant to numerous neurological presentations, including HD. We explored the effects of alcohol intake on clinical features of HD by means of data from the Enroll-HD, which is a global registry study. A retrospective observational study making use of the Enroll-HD periodic dataset up to 2020 (in accordance with the Enroll-HD guidelines, encompassing 16,120 subjects with the HD gene (CAG expansion > 36), was carried out. This included 180 sites in 21 countries. The study looked at the association of alcohol use with the clinical presentation of HD, specifically looking into the age of first symptoms and HD severity. We also describe a specific case with manifest HD, a participant in the Enroll-HD study, whereby the patient’s obsessionality was central to her pattern of high alcohol intake and to her successful avoidance of alcohol thereafter. A record of past problems with high alcohol intake was more common in the group with manifest HD (9.0%, n = 1121) when compared with the pre-manifest carriers of the HD genetic abnormality (2.3%, n = 339). Age at onset of symptoms was not significantly influenced by current alcohol misuse, or past misuse. The severity of clinical impairments in HD was influenced by alcohol. Patients who reported high alcohol intake in the past had a statistically significant increase in motor impairments, by the Unified Huntington’s Disease Rating Scale total motor score (Kruskal–Wallis, post hoc Dunn’s, p < 0.001), and a significantly higher burden of psychiatric symptoms by the Problem Behaviours Assessment score (Kruskal–Wallis, post hoc Dunn’s, p < 0.01) compared with those not reporting high alcohol use. However, the past alcohol group did not have a lower Mini Mental State Examination score (Kruskal–Wallis, post hoc Dunn’s, p > 0.05) The first symptom of HD, as determined by the assessing clinician, was more likely to be psychiatric disturbance in patients currently misusing alcohol or those with prior history of alcohol misuse (55% and 31% respectively) when compared with controls (5%). Individual case experience, such as that presented in this study, shows that HD and alcohol, two major genetic and environmental contributors to neurodegeneration, interact in producing clinical problems. However, the complexities of these interactions are difficult to define, and may require larger studies dedicated to exploring the various factors in this interaction.

scholarly journals Comparison of Huntington's Disease in Europe and North America

2016 ◽  
Vol 4 (3) ◽  
pp. 358-367 ◽  
Author(s):  
Michael Orth ◽  
Juliana Bronzova ◽  
Christine Tritsch ◽  
E. Ray Dorsey ◽  
Joaquim J. Ferreira ◽  
...  
Keyword(s):  
North America ◽  
Huntington's Disease ◽  
Huntington’S Disease

scholarly journals Huntington's disease: from gene to potential therapy

2001 ◽  
Vol 3 (1) ◽  
pp. 17-23
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Western Europe ◽  
Molecular Mechanisms ◽  
Late Onset ◽  
Cell Metabolism ◽  
Normal Function ◽  
Model Systems

Huntington's disease (HD) is a progressive, late-onset neurodegenerative illness with autosomal dominant inheritance that affects one in 10 000 individuals in Western Europe. The disease is caused by a polyglutamine repeat expansion located in the N-terminal region of the huntingtin protein. The mutation is likely to act by a gain of function, but the molecular mechanisms by which it leads to neuronal dysfunction and cell death are not yet known. The normal function of huntingtin in cell metabolism is also unclear. There is no therapy for HD. Research on HD should help elucidate the pathogenetic mechanism of this illness in order to develop successful treatments to prevent or slow down symptoms. This article presents new results in HD research focusing on in vivo and in vitro model systems, potential molecular mechanisms of HD, and the development of therapeutic strategies.

Cassette Tape Revival as Creative Anachronism

2017 ◽  
Vol 14 (1) ◽  
pp. 109-117 ◽  
Author(s):  
JOANNA DEMERS
Keyword(s):  
North America ◽  
Popular Music ◽  
Data Storage ◽  
Western Europe ◽  
Cassette Tape ◽  
Hard Drives ◽  
The Past ◽  
Streaming Services

ABSTRACTListeners of a certain age tend to think that the cassette tape fell out of favour sometime during the 1990s, but is experiencing a revival of sorts as curious Millennials discover the pleasures of mixtapes and decaying media. But cassette tapes have been in constant use since their invention in 1963. Outside of North America and Western Europe, the tape is still the predominant phonographic medium, and is unseating hard drives as the preferred medium for data storage. For whom, then, is this a revival? My article argues that the tape revival is less an attempt at neutral recuperation of the past than a purposeful rewriting of history. Cassettes are particularly potent because they signify death and decay more forcefully even than vinyl. Their acoustic imperfections and mechanical frailties are now aestheticized in novels and contemporary popular music. Even curated listening experiences, from podcasts to streaming services, are designed to replicate the mixtape. The second era of the cassette tape represents another example of Simon Reynolds’ concept of retromania, and can be fruitfully understood as a chapter in the evolving story of phonographic waste.

scholarly journals Socioeconomic determinants of teenage pregnancy and early motherhood in the United Kingdom: A perspective

2021 ◽  
Vol 11 (4) ◽  
pp. 426-429
Author(s):  
David Aluga ◽  
Elvis Anyaehiechukwu Okolie
Keyword(s):  
United Kingdom ◽  
Teenage Pregnancy ◽  
Western Europe ◽  
Literature Search ◽  
Population Level ◽  
Environmental Determinants ◽  
Pregnancy And Childbirth ◽  
The Past ◽  
Early Motherhood ◽  
The United Kingdom

The United Kingdom has one of the highest teenage birth rates among countries in western Europe. Government initiatives such as the Teenage Pregnancy Strategy introduced by the labor government in 1999 to reduce the teenage pregnancy rate by half in ten years could be responsible for the steady decline in teenage conception and childbirth for the past two decades. However, to sustain this decrease it is crucial to consider the broader socioeconomic and environmental determinants of teenage pregnancy at the population level. A selected literature search was conducted in this respect to highlight the factors that could be neglected by recent interventions on teenage pregnancy and childbirth in the United Kingdom.

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