Huntington's disease

@article{Walker2007HuntingtonsD,
  title={Huntington's disease},
  author={Francis O. Walker},
  journal={The Lancet},
  year={2007},
  volume={369},
  pages={218-228}
}
  • F. Walker
  • Published 20 January 2007
  • Psychology, Medicine
  • The Lancet
Huntington's Disease
TLDR
The aim of this article is to give an overview of HD, including its genetics, clinical features, different types of genetic testing and reproductive options as well as to introduce some of the ethical issues that can arise during a consultation.
Huntington's disease: a clinical review
  • R. Roos
  • Medicine, Psychology
    Orphanet journal of rare diseases
  • 2010
TLDR
Huntington disease is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia, which results in patients requiring full-time care, and finally death.
Huntington's disease in children.
Huntington's disease.
  • K. Anderson
  • Psychology, Medicine
    Handbook of clinical neurology
  • 2011
Cognition in Huntington's Disease
TLDR
HD was from the mid-1980s one of the first diseases where it was possible to predict whether an asymptomatic individual had inherited the genetic markers and would therefore become ill in the future.
Treatment of Huntington’s Disease
  • S. Frank
  • Medicine, Psychology
    Neurotherapeutics
  • 2013
TLDR
There are no current treatments to change the course of HD, but education and symptomatic therapies can be effective tools for clinicians to use with patients and families affected by HD.
Juvenile Huntington's disease: a case report and literature review.
TLDR
The characteristics of juvenile-onset Huntington's disease (HD) differ from those of adult-ONSet HD, as chorea does not occur, although bradykinesia, dystonia, and signs of cerebellar disorder, such as rigidity, are present.
Huntington’s Disease
Original Research Paper Review Article HUNTINGTON'S DISEASE: FROM MOLECULAR PATHOGENESIS TO CLINICAL TREATMENT
TLDR
Huntington disease is also emerging as a model for strategies to develop therapeutic interventions, not only to slow progression of manifest disease but also to delay, or ideally prevent, its onset.
Characteristics, pathophysiology and clinical management of weight loss in Huntington’s disease
TLDR
The authors delineate the characteristics of weight loss in both HD patients and genetic models of the disease and highlight the implications for management and care of HD patients with, or at risk of, unintended weight loss.
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References

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Therapeutics in Huntington’s Disease
TLDR
Severe disability results from progressive cognitive deficits and loss of voluntary motor control, which worsen over approximately 15–20 years until the affected individual is left unable to ambulate, communicate, swallow, or perform any of the activities of daily living.
Huntington's disease
Huntington's disease
TLDR
Prenatal exclusion testing offers an alternative method of detecting and terminating at‐risk pregnancies without revealing the genetic status of the at-risk parent.
Huntington’s disease
TLDR
The rate of progression of HD was significantly more rapid with a younger age at onset, and CAG repeat length may be an important determinant of not only the age of onset, but also the rate of disease progression.
Therapeutic interventions for symptomatic treatment in Huntington's disease.
TLDR
No intervention proved to have a consistent symptomatic control in HD, and tetrabenazine is the anti-choreic drug with the best quality data available.
Weight loss in early stage of Huntington’s disease
TLDR
At an early stage of the disease, subjects with Huntington’s disease had lower body mass index than matched controls from the general population and the parallel to observations in transgenic mouse models suggests that it is a significant hallmark of Huntington's disease gene expression.
Clinical and neuropathologic assessment of severity in Huntington's disease
TLDR
The relationship between the age at onset and the extent of neuropathologic involvement suggests that a single mechanism may determine both onset and rate of degenerative disease progression.
Huntington's disease in Venezuela
TLDR
The rate of decline of these untreated patients from Venezuela was similar to that seen in US patients who had received neuroleptic drugs and Juvenile patients declined nearly twice as fast as adult-onset patients.
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