↓ Skip to main content

Huntington's disease: a clinical review

Overview of attention for article published in Orphanet Journal of Rare Diseases, December 2010
Altmetric Badge

About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (95th percentile)
  • High Attention Score compared to outputs of the same age and source (81st percentile)

Mentioned by

news
2 news outlets
twitter
4 X users
patent
1 patent
facebook
2 Facebook pages
wikipedia
3 Wikipedia pages
video
5 YouTube creators

Citations

dimensions_citation
698 Dimensions

Readers on

mendeley
1675 Mendeley
citeulike
1 CiteULike
connotea
1 Connotea
You are seeing a free-to-access but limited selection of the activity Altmetric has collected about this research output. Click here to find out more.
Title
Huntington's disease: a clinical review
Published in
Orphanet Journal of Rare Diseases, December 2010
DOI 10.1186/1750-1172-5-40
Pubmed ID
Authors

Raymund AC Roos

Abstract

Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD). The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more) on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation) are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which results in patients requiring full-time care, and finally death. The most common cause of death is pneumonia, followed by suicide.

X Demographics

X Demographics

The data shown below were collected from the profiles of 4 X users who shared this research output. Click here to find out more about how the information was compiled.
Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 1,675 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United Kingdom 7 <1%
United States 6 <1%
Germany 2 <1%
Italy 2 <1%
India 2 <1%
Brazil 1 <1%
Austria 1 <1%
Chile 1 <1%
Canada 1 <1%
Other 3 <1%
Unknown 1649 98%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 365 22%
Student > Master 219 13%
Student > Ph. D. Student 177 11%
Researcher 135 8%
Student > Postgraduate 78 5%
Other 204 12%
Unknown 497 30%
Readers by discipline Count As %
Medicine and Dentistry 259 15%
Biochemistry, Genetics and Molecular Biology 212 13%
Agricultural and Biological Sciences 178 11%
Neuroscience 154 9%
Psychology 88 5%
Other 240 14%
Unknown 544 32%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 24. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 30 December 2021.
All research outputs
#1,591,637
of 25,402,889 outputs
Outputs from Orphanet Journal of Rare Diseases
#171
of 3,114 outputs
Outputs of similar age
#8,147
of 192,417 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#3
of 11 outputs
Altmetric has tracked 25,402,889 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 93rd percentile: it's in the top 10% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 3,114 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.2. This one has done particularly well, scoring higher than 94% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 192,417 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 95% of its contemporaries.
We're also able to compare this research output to 11 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 81% of its contemporaries.