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Huntingtons Chorea

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1. Understanding How Chorea Affects Health-Related Quality of Life in Huntington Disease: An Online Survey of Patients and Caregivers in the United States Full Text available with Trip Pro

Understanding How Chorea Affects Health-Related Quality of Life in Huntington Disease: An Online Survey of Patients and Caregivers in the United States Chorea is the hallmark motor feature of Huntington disease (HD) and can negatively impact daily functioning and health-related quality of life (HRQoL).The objective of this study was to evaluate how chorea impacts HRQoL and overall functioning among HD patients participating on the PatientsLikeMe website ( www.PatientsLikeMe.com ).A survey (...) was provided to HD participants and/or their caregivers via PatientsLikeMe (9 February 2017-22 March 2017), comprising multiple-choice and open-ended questions designed to assess how chorea impacts HRQoL and overall functioning, and the importance of treating chorea. The HDQLIFE measurement system was used to evaluate patient-reported outcomes of chorea and compare Anxiety and Stigma scores in participants with high chorea versus those with low chorea [HDQLIFE Chorea scores ≥  60 (n = 45) vs. <  60 (n = 38

2018 The patient

2. Physician perceptions of pharmacologic treatment options for chorea associated with Huntington disease in the United States. (Abstract)

Physician perceptions of pharmacologic treatment options for chorea associated with Huntington disease in the United States. To survey neurologists and obtain clinical perceptions of tetrabenazine for the treatment of chorea in patients with Huntington disease (HD).Board-certified/board-eligible neurologists, in practice for ≥5 years, who had treated treat ≥3 HD patients in the past 2 years, were recruited from an online physician panel to participate in a cross-sectional, web-based survey (...) . Respondents provided information about themselves, their practice, approaches to HD chorea management and perceptions of available treatments.Two hundred neurologists responded to the survey. Based on clinician responses, the most common reasons to treat chorea are impairment in activities of daily living (54%) and quality of life (41%). Although tetrabenazine was the only approved treatment for chorea in HD patients at the time of this analysis, it was only prescribed to ∼50% of patients with HD-related

2018 Current medical research and opinion

3. Efficacy and Safety of SOM3355 in Huntington's Disease Chorea

Efficacy and Safety of SOM3355 in Huntington's Disease Chorea Efficacy and Safety of SOM3355 in Huntington's Disease Chorea - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Efficacy and Safety of SOM3355 (...) in Huntington's Disease Chorea The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT03575676 Recruitment Status : Active, not recruiting First Posted : July 2, 2018 Last Update Posted : March 18, 2019 Sponsor: SOM Biotech SL Information provided by (Responsible Party): SOM Biotech SL Study Details Study Description

2018 Clinical Trials

4. Huntington's disease

Huntington's disease Huntington's disease - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Huntington's disease Last reviewed: February 2019 Last updated: July 2018 Summary Huntington's disease is an autosomal dominant neurodegenerative disorder. Often presents in mid-life but may appear at any age. Clinical manifestations include chorea, cognitive decline, loss of coordination, and personality change. Depression (...) and suicide may be comorbid events. In the absence of available effective treatment, many at-risk individuals forgo predictive genetic testing. Definition Huntington's disease is a slowly progressive, neurodegenerative disorder characterised by chorea, incoordination, cognitive decline, personality changes, and psychiatric symptoms, culminating in immobility, mutism, and inanition. Walker FO. Huntington's disease. Lancet. 2007 Jan 20;369(9557):218-28. http://www.ncbi.nlm.nih.gov/pubmed/17240289?tool

2018 BMJ Best Practice

5. Pharmacologic treatment of chorea in huntington disease

Pharmacologic treatment of chorea in huntington disease Evidence-based guideline: Pharmacologic treatment of chorea in Huntington disease | Neurology Advertisement Search for this keyword Main menu User menu Search Search for this keyword The most widely read and highly cited peer-reviewed neurology journal Share August 07, 2012 ; 79 (6) Special Article Evidence-based guideline: Pharmacologic treatment of chorea in Huntington disease Report of the Guideline Development Subcommittee (...) of the American Academy of Neurology Melissa J. Armstrong , Janis M. Miyasaki First published July 18, 2012, DOI: https://doi.org/10.1212/WNL.0b013e318263c443 Melissa J. Armstrong Janis M. Miyasaki Evidence-based guideline: Pharmacologic treatment of chorea in Huntington disease Melissa J. Armstrong , Janis M. Miyasaki Neurology Aug 2012, 79 (6) 597-603; DOI: 10.1212/WNL.0b013e318263c443 Citation Manager Formats Make Comment See Comments Downloads 13074 Share Abstract Objective: To develop an evidence-based

2012 American Academy of Neurology

6. Anti-ma2 receptor encephalitis mimicking Huntington chorea Full Text available with Trip Pro

Anti-ma2 receptor encephalitis mimicking Huntington chorea 28461817 2018 11 13 1735-1995 22 2017 Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences J Res Med Sci Anti-ma2 receptor encephalitis mimicking Huntington chorea. 31 10.4103/1735-1995.202148 Etemadifar Masoud M Department of Neurology, School of Medicine, Isfahan Medical University, Isfahan, Iran. Salari Mehri M Department of Neurology, School of Medicine, Isfahan Medical University

2017 Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences

7. The effect of interventions for management of chorea in Huntington's Disease

The effect of interventions for management of chorea in Huntington's Disease Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content of this registration record, any associated files or external websites. Email

2019 PROSPERO

8. The effects of deep brain stimulation on Huntington's disease and chorea-acanthocytosis: an IPD meta-analysis

The effects of deep brain stimulation on Huntington's disease and chorea-acanthocytosis: an IPD meta-analysis Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content of this registration record, any associated files

2019 PROSPERO

9. Nocturnal Post‐arousal Chorea and Repetitive Ballistic Movement in Huntington's Disease Full Text available with Trip Pro

Nocturnal Post‐arousal Chorea and Repetitive Ballistic Movement in Huntington's Disease 30363612 2019 02 26 2330-1619 3 2 2016 Mar-Apr Movement disorders clinical practice Mov Disord Clin Pract Nocturnal Post-arousal Chorea and Repetitive Ballistic Movement in Huntington's Disease. 200-202 10.1002/mdc3.12258 Ranjan Surabhi S Department of Neurology University of Virginia Charlottesville Virginia USA. Kohler Scott S Department of Neurology University of Virginia Charlottesville Virginia USA (...) . Harrison Madaline B MB Department of Neurology University of Virginia Charlottesville Virginia USA. Quigg Mark M Department of Neurology University of Virginia Charlottesville Virginia USA. Sleep Medicine Center University of Virginia Charlottesville Virginia USA. eng Case Reports 2015 11 27 United States Mov Disord Clin Pract 101630279 2330-1619 Huntington's disease nocturnal movement disorder parasomnia repetitive ballistic movement sleep apnea 2015 07 15 2015 08 13 2015 08 17 2018 10 27 6 0 2015 11

2015 Movement disorders clinical practice

10. Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial. Full Text available with Trip Pro

Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial. Deutetrabenazine is a novel molecule containing deuterium, which attenuates CYP2D6 metabolism and increases active metabolite half-lives and may therefore lead to stable systemic exposure while preserving key pharmacological activity.To evaluate efficacy and safety of deutetrabenazine treatment to control chorea associated with Huntington disease.Ninety ambulatory adults diagnosed (...) with manifest Huntington disease and a baseline total maximal chorea score of 8 or higher (range, 0-28; lower score indicates less chorea) were enrolled from August 2013 to August 2014 and randomized to receive deutetrabenazine (n = 45) or placebo (n = 45) in a double-blind fashion at 34 Huntington Study Group sites.Deutetrabenazine or placebo was titrated to optimal dose level over 8 weeks and maintained for 4 weeks, followed by a 1-week washout.Primary end point was the total maximal chorea score change

2016 JAMA Controlled trial quality: predicted high

11. Huntington's Chorea

Huntington's Chorea Huntingtons Chorea Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Huntington's Chorea Huntington's Chorea Aka (...) : Huntington's Chorea , Progressive Hereditary Chorea , Huntingtons Chorea , Huntington Chorea From Related Chapters II. Epidemiology inherited disease Onset of symptoms in middle life III. Symptoms Gradual onset and progression of symptoms Failing memory Restlessness Lack of initiative IV. Signs movements Shuffling gait Mental deterioration es Normal or increased Images: Related links to external sites (from Bing) These images are a random sampling from a Bing search on the term "Huntingtons Chorea." Click

2018 FP Notebook

12. The development of a new computer adaptive test to evaluate chorea in Huntington disease: HDQLIFE Chorea Full Text available with Trip Pro

The development of a new computer adaptive test to evaluate chorea in Huntington disease: HDQLIFE Chorea Huntington's disease (HD) is an autosomal dominant neurodegenerative disease associated with motor, behavioral, and cognitive deficits. The hallmark symptom of HD, chorea, is often the focus of HD clinical trials. Unfortunately, there are no self-reported measures of chorea. To address this shortcoming, we developed a new measure of chorea for use in HD, HDQLIFE Chorea.Qualitative data (...) and literature reviews were conducted to develop an initial item pool of 141 chorea items. An iterative process, including cognitive interviews, expert review, translatability review, and literacy review, was used to refine this item pool to 64 items. These 64 items were field tested in 507 individuals with prodromal and/or manifest HD. Exploratory and confirmatory factor analyses (EFA and CFA, respectively) were conducted to identify a unidimensional set of items. Then, an item response theory graded

2016 Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation

13. Wearable Sensors for Quantitative Assessment of Motor Impairment in Huntington's Disease Huntington's Disease

to administer correctly, and only captures impairments in clinic. In this Direct to Phase II SBIR we will develop a wearable sensor system for objective, sensitive, and continuous assessment of Huntington's chorea during activities of daily living. The developed technology could be used clinically to detect changes in motor function in response to medications, or could be used scientifically to expedite and reduce the cost of early stage pharmaceutical clinical trials. Condition or disease Intervention (...) by the National Library of Medicine related topics: related topics: resources: Groups and Cohorts Go to Group/Cohort Intervention/treatment Manifest HD Other: HD Wear Wearable sensor system for monitoring Huntington's chorea during activities of daily living Premanifest HD Other: HD Wear Wearable sensor system for monitoring Huntington's chorea during activities of daily living Control Other: HD Wear Wearable sensor system for monitoring Huntington's chorea during activities of daily living Outcome Measures

2018 Clinical Trials

14. Anaesthetic management of a patient with Huntington's chorea undergoing robot-assisted nephron-sparing surgery Full Text available with Trip Pro

Anaesthetic management of a patient with Huntington's chorea undergoing robot-assisted nephron-sparing surgery 27942067 2018 11 13 0019-5049 60 11 2016 Nov Indian journal of anaesthesia Indian J Anaesth Anaesthetic management of a patient with Huntington's chorea undergoing robot-assisted nephron-sparing surgery. 866-867 Batra Ankita A Department of Anaesthesia and Intensive Care, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Sahni Neeru N Department

2016 Indian journal of anaesthesia

15. The non–Huntington disease choreas: Five new things Full Text available with Trip Pro

The non–Huntington disease choreas: Five new things Chorea can be due to a wide variety of causes. In this review, I provide updates on several recently identified genetic and autoimmune causes of chorea, and review evidence supporting the use of deep brain stimulation in chorea.New genes that may cause chorea include ADCY5 (encoding for adenylate cyclase 5) C9ORF72 (in addition to amyotrophic lateral sclerosis and frontotemporal dementia), and those responsible for the neurodegeneration (...) with brain iron accumulation disorders. Novel autoantibodies are increasingly being identified as associated with a variety of neurologic syndromes, including chorea, in both paraneoplastic and non-paraneoplastic settings. Deep brain stimulation can be a useful intervention in patients with chorea who do not respond to oral medications, whether due to neurodegenerative or nondegenerative causes.New causes of chorea continue to be identified. Correct diagnosis is essential for prognostication

2016 Neurology: Clinical Practice

16. A Randomized, Placebo-Controlled Trial of AFQ056 for the Treatment of Chorea in Huntington's Disease. (Abstract)

A Randomized, Placebo-Controlled Trial of AFQ056 for the Treatment of Chorea in Huntington's Disease. This study investigated the hypothesis that AFQ056 (mavoglurant), a selective metabotropic glutamate receptor 5 antagonist, reduces chorea in Huntington's disease (HD).This 32-day randomized, double-blind, parallel-group, proof-of-concept study investigated AFQ056 (25-150 mg [incremental doses], twice-daily) versus placebo in patients with HD. Primary efficacy assessments were the chorea-sum (...) score and orientation index (nondominant hand) from the quantitative motor (Q-Motor) grasping task at day 28. Key secondary efficacy assessments included finger-tapping in the Unified Huntington's Disease Rating Scale-Total Motor Score and Q-Motor measures. Safety and tolerability were assessed.Overall, 42 patients were randomized. At day 28, no improvement was observed on the primary efficacy assessments (P > 0.10) with AFQ056 versus placebo. The Q-Motor speeded-tapping interonset interval

2015 Movement Disorders Controlled trial quality: predicted high

17. Coexistence of Gait Disturbances and Chorea in Experimental Huntington's Disease Full Text available with Trip Pro

Coexistence of Gait Disturbances and Chorea in Experimental Huntington's Disease Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by an expanded CAG repeat. The clinical features are progressive motor dysfunction, cognitive deterioration, and psychiatric disturbances. Unpredictable choreic movements, among the most characteristic hallmarks, may contribute to gait disturbances and loss of balance in HD individuals. In this study, we aimed to investigate (...) with choreiform movements. Our results showed that the dynamic parameters seem to be more affected than static parameters at this age in tgHD rats. The number of steps and step cycles and swing speed of the paws were increased in tgHD rat in comparison to wild-type controls. Our study demonstrates that gait abnormalities coexist with chorea rather than being caused by it. These symptoms may originate from distinct networks in the basal ganglia and downstream connections.

2015 Behavioural neurology

18. Dexmedetomidine with low-dose ketamine for cataract surgery under peribulbar block in a patient with Huntington's chorea Full Text available with Trip Pro

Dexmedetomidine with low-dose ketamine for cataract surgery under peribulbar block in a patient with Huntington's chorea Huntington's chorea (HC) is a rare hereditary disorder of the nervous system. It is inherited as an autosomal dominant disorder and is characterized by progressive chorea, dementia, and psychiatric disturbances. There are only a few case reports regarding the anesthetic management of a patient with HC and the best anesthetic technique is yet to be established for those

2015 Anesthesia, essays and researches

19. Patient-reported outcomes in Huntington's disease: Quality of life in neurological disorders (Neuro-QoL) and Huntington's disease health-related quality of life (HDQLIFE) physical function measures. Full Text available with Trip Pro

-reported outcome measures in Huntington's disease.A total of 510 individuals with Huntington's disease completed 2 Quality of Life in Neurological Disorders (Lower Extremity Function and Upper Extremity Function) and 3 Huntington's Disease Health-Related Quality of Life (Chorea, Speech Difficulties, and Swallowing Difficulties) measures. Clinician-rated and generic self-report measures were also administered.Reliabilities for the new patient reported physical functioning measures were excellent (all (...) Patient-reported outcomes in Huntington's disease: Quality of life in neurological disorders (Neuro-QoL) and Huntington's disease health-related quality of life (HDQLIFE) physical function measures. There is a need for patient-reported outcome measures that capture the impact that motor impairments have on health-related quality of life in individuals with Huntington's disease.The objectives of this study were to establish the reliability and validity of new physical functioning patient

2017 Movement Disorders

20. Brain structure in juvenile-onset Huntington disease. Full Text available with Trip Pro

Brain structure in juvenile-onset Huntington disease. To assess brain morphometry in a sample of patients with juvenile-onset Huntington disease (JOHD) and several mouse models of Huntington disease (HD) that likely represent the human JOHD phenotype.Despite sharing the mutation in the Huntingtin gene, adult-onset HD characteristically presents as a hyperkinetic motor disorder, while JOHD typically presents as a hypokinetic motor disease. The University of Iowa Kids-JHD program enrolls (...) in the striatal volume. Brain morphology in both mice and human patients with JHD shows proportional cerebellar enlargement. This pattern of brain changes may explain the unique picture of hypokinetic motor symptoms in JHD, which is not seen in the hyperkinetic chorea-like phenotype of adult-onset HD.Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

2019 Neurology

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