Huntington's disease: present treatments and future therapeutic modalities

  title={Huntington's disease: present treatments and future therapeutic modalities},
  author={Raphael Maria Bonelli and Gregor K. Wenning and H P Kapfhammer},
  journal={International Clinical Psychopharmacology},
Huntington's disease (HD) is a devastating neuropsychiatric disorder for which therapeutic interventions have been rather fruitless to date, except in a slight symptomatic relief. Even the discovery of the gene related to HD in 1993 has not effectively advanced treatments. This article is essentially a review of available double-blind, placebo-controlled trials of therapy for this condition which also includes relevant open label trials. Unfortunately, HD research has tended to concentrate on… 
Huntington's disease : a review and therapeutic proposal
There is little evidence for treatment of Huntington’s disease and Olanzapine at 2.5mg twice/day has the most promise for treating HD associated chorea and depression.
Therapeutic interventions for symptomatic treatment in Huntington's disease.
No intervention proved to have a consistent symptomatic control in HD, and tetrabenazine is the anti-choreic drug with the best quality data available.
A systematic review of the treatment studies in Huntington’s disease since 1990
A systematic review of pharmacological therapy in HD using the available papers that were published between 1990 and 2006 found that a surprising amount of current drug therapy of HD in clinical practice is based on studies published before 1990.
Therapeutic interventions for disease progression in Huntington's disease.
Only pharmacological interventions were included and none proved to be effective as a disease-modifying therapy for HD, and the selected interventions were found to be generally safe and well tolerated.
Deep Brain Stimulation in Huntington’s Disease: The Current Status
An overview of the result obtained with DBS in patients with HD suggests that DBS of the globus pallidus internus is safe and well-tolerated, and reduces choreic movements, without substantial effects on psychiatric functions.
Combating depression in Huntington's disease: effective antidepressive treatment with venlafaxine XR
Venlafaxine XR is highly effective in the treatment of depression in HD, although it may produce unpleasant side effects, and further studies are required to establish the most suitable treatment.
A Review on Huntington ’ s Disease
The disease pathology and neurochemistry of HD is described and many of the past and emerging therapeutic strategies are addressed, including cell replacement strategies such as stem cell therapy uncertain.
Perospirone in treatment of Huntington's disease: A first case report
What evidence there is for the drug treatment of Huntington's disease
Evidence supporting the pharmacological management of chorea and the psychiatric manifestations of HD is summarised below and adjuvant psychotherapy, physiotherapy and speech therapy should be applied to provide optimal management.
Effectiveness of Anti-Psychotics and Related Drugs in the Huntington French-Speaking Group Cohort
Although differences in motor or behavioural profiles between patients according to the antipsychotics used were small, there were differences in drug effectiveness on the evolution of functional and cognitive scores.


Huntington’s disease: A randomized, controlled trial using the NMDA-antagonist amantadine
It is suggested that NMDA receptor supersensitivity may contribute to the clinical expression of choreiform dyskinesias in HD and that selective antagonists at that site can safely confer palliative benefit.
Quetiapine in Huntington’s disease: a first case report
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.
Olanzapine for Huntington's disease: An open label study
Riluzole therapy in Huntington's disease (HD)
There was a trend toward lower lactate/creatine ratios during riluzole treatment in the basal ganglia spectra but not in occipital cortex spectra, and additional clinical studies of rILuzole for both symptomatic and neuroprotective benefit in Huntington's disease are warranted.
A controlled trial of fluoxetine in nondepressed patients with Huntington's disease
  • P. Como, A. Rubin, I. Shoulson
  • Psychology, Medicine
    Movement disorders : official journal of the Movement Disorder Society
  • 1997
Although fluoxetine may be a useful antidepressant in depressed HD patients, it failed to exert substantial clinical benefits in nondepressed HD patients.
Huntington disease
The Commission for the Control of Huntington Disease and Its Consequences, established by the Ninety-Fourth United States Congress, recently completed an appraisal of clinical care of the patient and family with Huntington disease.
A controlled clinical trial of baclofen as protective therapy in early huntington's disease
Patients with early Huntington's disease declined at a pace slower than that observed in other prospective studies, a finding likely due to selection criteria, avoidance of neuroleptic therapy, and strong psychosocial support.
Clozapine versus placebo in Huntington’s disease: a double blind randomised comparative study
Clozapine has little beneficial effect in patients with Huntington’s disease, although individual patients may tolerate doses high enough to reduce chorea.
A controlled trial of idebenone in Huntington's disease
Sample size calculations based on progression of the ADL and QNE in this study group revealed that a larger study group is necessary to detect any differences less than an almost complete halting of the disease.
Unified Huntington's disease rating scale: A follow up
The authors suggest performing a total UHDRS evaluation every second year for every HD patient as part of the routine longitudinal evaluation.