Pharmacological Management of Huntingtons Disease: An Evidence- Based Review

Bonelli, Raphael M.; Wenning, Gregor K.

doi:10.2174/138161206777698693

Cited by 141 publications

(84 citation statements)

References 228 publications

(230 reference statements)

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“…Chorea Several reviews have summarized the symptomatic treatment of chorea associated with HD [49][50][51][52][53][54][55][56][57][58][59][60][61]. Overall, there is not enough evidence available to guide long-term symptomatic treatment in HD, and double-blind and long-term studies assessing various treatment strategies in HD are needed [55].…”

Section: Pharmacological Treatment Optionsmentioning

confidence: 99%

“…Overall, there is not enough evidence available to guide long-term symptomatic treatment in HD, and double-blind and long-term studies assessing various treatment strategies in HD are needed [55]. Despite the lack of evidence, an American Academy of Neurology Guidelines publication was recently released recommending consideration of tetrabenazine (TBZ), amantadine, or riluzole if chorea requires treatment [46].…”

Section: Pharmacological Treatment Optionsmentioning

confidence: 99%

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Treatment of Huntington's Disease

2014

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Section: Pharmacological Treatment Optionsmentioning

confidence: 99%

Section: Pharmacological Treatment Optionsmentioning

confidence: 99%

Treatment of Huntington's Disease

2014

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“…This is an important finding, as it is often non-motor symptoms that have the greatest impact on a patient's health-related quality of life [46]. Furthermore, suicide is common in HD [1,8,9].…”

Section: Discussionmentioning

confidence: 99%

The Pathophysiology and Pharmacological Treatment of Huntington Disease

Pidgeon

Rickards

2013

Behavioural Neurology

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Abstract.Introduction: Huntington disease (HD) is a progressive neurodegenerative condition characterised by motor, cognitive and behavioural dysfunction, and has an autosomal dominant mode of inheritance. As there is currently no treatment to delay progression of the disease, pharmacological intervention is aimed at symptomatic relief. Methods: We set out to assess the current evidence on the pharmacological treatment of motor and non-motor symptoms in HD by carrying out a systematic literature review across five large scientific databases. Results: The search generated 23 original studies meeting our search criteria. Studies on the following drug classes were obtained: dopamine (DA) depleting agents, neuroleptics, anti-glutamatergic agents, acetylcholinesterase inhibitors, GABA agonists, cannabinoids, antidepressants and potential neuroprotective agents. Tetrabenazine (TBZ), a DA depleting agent, was the only pharmacotherapy shown to have a clinically meaningful, statistically significant effect on chorea. The majority of the reviewed studies focussed on the treatment of motor symptoms of HD. Discussion: Overall, the evidence base for the pharmacological management of HD is poor. There is a clear need for future high quality randomised controlled trials on the symptomatic treatment of HD, particularly on the pharmacotherapy of non-motor symptoms of HD.

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“…33 Higher doses of the typical neuroleptics may not necessarily provide additional benefit in the treatment of chorea, compared with lower doses, but have a potential for a higher incidence of adverse effects, including impairment in the oculomotor, orolingual, motor control, and cognitive functional spheres. 34 …”

Section: Typical Neurolepticsmentioning

confidence: 99%

Symptomatic Treatment of Huntington Disease

2008

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Summary: Huntington disease (HD) is a progressive heredoneurodegenerative disease manifested by chorea and other hyperkinetic (dystonia, myoclonus, tics) and hypokinetic (parkinsonism) movement disorders. In addition, a variety of psychiatric and behavioral symptoms, along with cognitive decline, contribute significantly to the patient's disability. Because there are no effective neuroprotective therapies that delay the progression of the disease, symptomatic treatment remains the cornerstone of medical management. Several classes of medications have been used to ameliorate the various symptoms of HD, including typical and atypical neuroleptics, dopamine depleters, antidepressants, antiglutamatergic drugs, GABA agonists, antiepileptic medications, acetylcholinesterase inhibitors, and botulinum toxin. Recently, surgical approaches including pallidotomy, deep brain stimulation, and fetal cell transplants have been used for the symptomatic treatment of HD. The selected therapy must be customized to the needs of each patient, minimizing the potential adverse effects. The primary aim of this article is to review the role of the different therapies, both available and investigational, for the treatment of the motor, psychiatric, behavioral, and cognitive symptoms of HD, and to examine their impact on the patient's functionality and quality of life.

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Pharmacological Management of Huntingtons Disease: An Evidence- Based Review

Abstract: There is poor evidence in management of HD today. The analysis of the twenty level-I studies fails to result in any treatment recommendation of clinical relevance. High-quality RCT are highly warranted to advance HD treatment in clinical practice.

Cited by 141 publications

References 228 publications

Treatment of Huntington's Disease

Treatment of Huntington's Disease

The Pathophysiology and Pharmacological Treatment of Huntington Disease

Symptomatic Treatment of Huntington Disease

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