Clinical characterization of dystonia in adult patients with Huntington's disease

Zande, Nadine A van de; Massey, Thomas; McLauchlan, Duncan; Roberts, Angela; Zutt, Rodi; Wardle, Mark; Payne, Gareth; Clenaghan, Catherine; Tijssen, Marina A. J.; Rosser, Anne Elizabeth; Peall, Kathryn J.

doi:10.1111/ene.13349

Cited by 30 publications

(11 citation statements)

References 23 publications

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“…Nabilone was beneficial on chorea and behavioral disturbances in a case report study [194] and in a pilot clinical study [195]. Recently, a clinical study investigated the effect of different cannabinoid formulations, Sativex®, nabilone, or dronabinol on dystonia, a frequent symptom in HD [232], and patients experienced motor improvements mainly in dystonia and presented less irritability and apathy [197]. These clinical studies conducted with cannabinoids were focused on symptom relief.…”

Section: Clinical Evidence For the Therapeutic Potential Of Cannabinomentioning

confidence: 99%

Cannabinoid pharmacology/therapeutics in chronic degenerative disorders affecting the central nervous system

Aymerich

Aso

Abellanas

et al. 2018

Biochemical Pharmacology

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The endocannabinoid system (ECS) exerts a modulatory effect of important functions such as neurotransmission, glial activation, oxidative stress, or protein homeostasis. Dysregulation of these cellular processes is a common neuropathological hallmark in aging and in neurodegenerative diseases of the central nervous system (CNS). The broad spectrum of actions of cannabinoids allows targeting different aspects of these multifactorial diseases. In this review, we examine the therapeutic potential of the ECS for the treatment of chronic neurodegenerative diseases of the CNS focusing on Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis. First, we describe the localization of the molecular components of the ECS and how they are altered under neurodegenerative conditions, either contributing to or protecting cells from degeneration. Second, we address recent advances in the modulation of the ECS using experimental models through different strategies including the direct targeting of cannabinoid receptors with agonists or antagonists, increasing the endocannabinoid tone by the inhibition of endocannabinoid hydrolysis, and activation of cannabinoid receptor-independent effects. Preclinical evidence indicates that cannabinoid pharmacology is complex but supports the therapeutic potential of targeting the ECS. Third, we review the clinical evidence and discuss the future perspectives on how to bridge human and animal studies to develop cannabinoid-based therapies for each neurodegenerative disorder. Finally, we summarize the most relevant opportunities of cannabinoid pharmacology related to each disease and the multiple unexplored pathways in cannabinoid pharmacology that could be useful for the treatment of neurodegenerative diseases.

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Section: Clinical Evidence For the Therapeutic Potential Of Cannabinomentioning

confidence: 99%

Cannabinoid pharmacology/therapeutics in chronic degenerative disorders affecting the central nervous system

Aymerich

Aso

Abellanas

et al. 2018

Biochemical Pharmacology

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“…More recently, Hart et al (2013) focused on aspects of predominant choreatic versus hypokinetic-rigid motor phenotypes and described a better capacity for global and cognitive functioning of their choreatic group. So far only a few smaller studies investigated the role of dystonia in HD (Mahant et al, 2003;Zande et al, 2017). Zande et al (2017) described a high prevalence of dystonia as being a late symptom correlating with increasing HD stage and disease duration.…”

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confidence: 99%

“…So far only a few smaller studies investigated the role of dystonia in HD (Mahant et al, 2003;Zande et al, 2017). Zande et al (2017) described a high prevalence of dystonia as being a late symptom correlating with increasing HD stage and disease duration. In addition, a strong relationship with functional status of dystonia and rigidity was described (Carlozzi et al, 2019).…”

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confidence: 99%

Functional and cognitive capacity differ in dystonic motor subtypes when compared to choreatic and hypokinetic‐rigid motor subtypes in Huntington's disease

2020

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Background Motor phenotypes in Huntington's disease vary manifold. Phenotype classification is essential to adapt treatment. The aim of this study was to classify a dystonic subtype closer. Methods A total of 7,512 manifest ENROLL‐HD participants were subdivided into mainly choreatic ( N = 606), dystonic ( N = 402), and hypokinetic‐rigid ( N = 369) subjects. Cognitive (verbal fluency, symbol digit, stroop color, trail making, Mini‐Mental State Examination), functional (total functional capacity, Independence Scale), and psychiatric (problem behaviors assessment, Hospital Anxiety and Depression Scale) performance was evaluated at baseline visit. Results Symptoms onset for dystonic were similar to hypokinetic‐rigid, but earlier compared to choreatic subjects ( p < .001). Cognition was better in both groups compared to hypokinetic rigid (all p < .001). Functionality differed between all groups (all p < .001). Differences remained (all p < .001) after controlling for CAP score, CAG, age, disease duration, and education. Conclusions Motor subtypes differ in functional and cognitive capacities but less in psychiatric. We identified better cognitive and functional capacities and similar onsets in predominant dystonic compared to hypokinetic‐rigid patients.

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“…Dystonia is characterized by abnormal postures that may affect all body segments and is frequently associated with rigidity (12). Dystonia intensity varies from a slight intermittent abnormal posture to severe twitch of muscles with major impact on movements and functions of daily living.…”

Section: Resultsmentioning

confidence: 99%

International Guidelines for the Treatment of Huntington's Disease

et al. 2019

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The European Huntington's Disease Network (EHDN) commissioned an international task force to provide global evidence-based recommendations for everyday clinical practice for treatment of Huntington's disease (HD). The objectives of such guidelines are to standardize pharmacological, surgical and non-pharmacological treatment regimen and improve care and quality of life of patients. A formalized consensus method, adapted from the French Health Authority recommendations was used. First, national committees (French and English Experts) reviewed all studies published between 1965 and 2015 included dealing with HD symptoms classified in motor, cognitive, psychiatric, and somatic categories. Quality grades were attributed to these studies based on levels of scientific evidence. Provisional recommendations were formulated based on the strength and the accumulation of scientific evidence available. When evidence was not available, recommendations were framed based on professional agreement. A European Steering committee supervised the writing of the final recommendations through a consensus process involving two rounds of online questionnaire completion with international multidisciplinary HD health professionals. Patients' associations were invited to review the guidelines including the HD symptoms. Two hundred and nineteen statements were retained in the final guidelines. We suggest to use this adapted method associating evidence base–medicine and expert consensus to other rare diseases.

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Clinical characterization of dystonia in adult patients with Huntington's disease

Cited by 30 publications

References 23 publications

Cannabinoid pharmacology/therapeutics in chronic degenerative disorders affecting the central nervous system

Cannabinoid pharmacology/therapeutics in chronic degenerative disorders affecting the central nervous system

Functional and cognitive capacity differ in dystonic motor subtypes when compared to choreatic and hypokinetic‐rigid motor subtypes in Huntington's disease

International Guidelines for the Treatment of Huntington's Disease

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