Advances in the Pharmacological Management of Huntington’s Disease

@article{Frank2012AdvancesIT,
  title={Advances in the Pharmacological Management of Huntington’s Disease},
  author={Samuel Frank and Joseph Jankovic},
  journal={Drugs},
  year={2012},
  volume={70},
  pages={561-571}
}
There is inevitable physical, cognitive and behavioural decline in Huntington’s disease (HD), a dominantly inherited progressive neurological disorder. The hallmark of the disease is chorea, an involuntary brief movement that tends to flow between body regions. HD is diagnosed clinically with genetic confirmation. Predictive testing is available; however, it should be undertaken with caution in patients at risk for the disease but without clinical disease expression. Ongoing observational… 
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.
Tetrabenazine: the first approved drug for the treatment of chorea in US patients with Huntington disease
  • S. Frank
  • Medicine, Psychology
    Neuropsychiatric disease and treatment
  • 2010
TLDR
Tetrabenazine (TBZ) is a dopamine-depleting agent that may be one of the more effective agents for reducing chorea, although it has a risk of potentially serious adverse effects.
Huntington’s disease: Pathophysiology and therapeutic intervention
TLDR
The study covers the disease's pathophysiology, as well as plants and phytochemicals that have been shown to be beneficial and medications that can help to regulate chorea, dystonia, mental, and psychiatric disturbances.
Current Pharmacological Approaches to Reduce Chorea in Huntington’s Disease
TLDR
Tetrabenazine, tiapride, olanzapine, and risperidone are the preferred first choice drugs to reduce chorea among HD experts, and in the existing literature, these drugs also show a beneficial effect on motor symptom severity and improvement of psychiatric symptoms.
Managing chorea in Huntington’s disease
TLDR
Although chorea only constitutes part of HD, the movements can be disabling, injurious or bothersome and treating chorea and other motor symptoms may improve the quality of life of sufferers.
African Journal of Neurological Sciences - 2009 Vol. 28 No 1
TLDR
Clinical features, diagnosis, symptomatic approaches, Symptomatic treatment and other therapies involved in the management of Huntington’s disease are concentrated on.
REVIEW / MISE AU POINT HUNTINGTON'S DISEASE: A PERPLEXING NEUROLOGICAL DISEASE
TLDR
Clinical features, diagnosis, symptomatic approaches, Symptomatic treatment and other therapies involved in the management of Huntington’s disease are concentrated on.
Current therapeutic options for Huntington's disease: Good clinical practice versus evidence‐based approaches?
  • A. Killoran, K. Biglan
  • Medicine, Psychology
    Movement disorders : official journal of the Movement Disorder Society
  • 2014
TLDR
The limited evidence available and current expert opinion on medical treatment of the dominant motor, psychiatric, and cognitive features of HD are discussed, and no known effective treatment is available for cognitive dysfunction in HD.
Medical management of motor manifestations of Huntington disease.
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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TLDR
Huntington’s disease is an autosomal dominant, inherited, neuropsychiatric disease which gives rise to progressive motor, cognitive and behavioural symptoms, and the only treatment options available in HD are symptomatic.
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The pathophysiology of Huntington's disease remains unknown, although protein aggregation, excitotoxicity, deficits in energy metabolism, transcriptional dysregulation and apoptosis may all be involved.
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TLDR
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TLDR
A 30-year-old woman with a genetically confirmed sporadic form of HD was referred to the department with a severe untreatable chorea that had lasted for more than a week despite high doses of tetrabenazine, and showed her deficits especially in gait, oral functions, and fine motor tasks together with chorea.
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TLDR
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TLDR
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TLDR
The pharmacological management of the various movement disorders associated with Huntington's disease, the cognitive decline and the commonly encountered behavioral disturbances are reviewed.
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