Voluntary Inhibitory Control of Chorea: A Case Series

Bonomo, Roberta; Latorre, Anna; Balint, Bettina; Śmiłowska, Katarzyna; Rocchi, Lorenzo; Rothwell, John C.; Zappia, Mario; Bhatia, Kailash P.

doi:10.1002/mdc3.12907

Cited by 6 publications

(2 citation statements)

References 9 publications

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“…16 Voluntary suppression of chorea on demand in some people with chorea. 17,18 Flexible use of proactive and reactive inhibition.…”

Section: Dystoniamentioning

confidence: 99%

Rethinking Movement Disorders

Münchau,

Klein,

Beste

2024

Movement Disorders

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At present, clinical practice and research in movement disorders (MDs) focus on the “normalization” of altered movements. In this review, rather than concentrating on problems and burdens people with MDs undoubtedly have, we highlight their hidden potentials. Starting with current definitions of Parkinson's disease (PD), dystonia, chorea, and tics, we outline that solely conceiving these phenomena as signs of dysfunction falls short of their complex nature comprising both problems and potentials. Such potentials can be traced and understood in light of well‐established cognitive neuroscience frameworks, particularly ideomotor principles, and their influential modern derivatives. Using these frameworks, the wealth of data on altered perception‐action integration in the different MDs can be explained and systematized using the mechanism‐oriented concept of perception‐action binding. According to this concept, MDs can be understood as phenomena requiring and fostering flexible modifications of perception‐action associations. Consequently, although conceived as being caught in a (trough) state of deficits, given their high flexibility, people with MDs also have high potential to switch to (adaptive) peak activity that can be conceptualized as hidden potentials. Currently, clinical practice and research in MDs are concerned with deficits and thus the “deep and wide troughs,” whereas “scattered narrow peaks” reflecting hidden potentials are neglected. To better delineate and utilize the latter to alleviate the burden of affected people, and destigmatize their conditions, we suggest some measures, including computational modeling combined with neurophysiological methods and tailored treatment. © 2024 International Parkinson and Movement Disorder Society.

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“…16 Voluntary suppression of chorea on demand in some people with chorea. 17,18 Flexible use of proactive and reactive inhibition.…”

Section: Dystoniamentioning

confidence: 99%

Rethinking Movement Disorders

Münchau,

Klein,

Beste

2024

Movement Disorders

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“…76 Half of patients with chorea and 20% of patients with dystonia can also voluntarily reduce their movements. 75,77 Tics are the prototypical hyperkinetic movement disorders that may be suppressed voluntarily. 75…”

Section: Volitional Suppression Of Involuntary Movementsmentioning

confidence: 99%

Variability of Movement Disorders: The Influence of Sensation, Action, Cognition, and Emotions

et al. 2020

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A BS TRACT: Patients with movement disorders experience fluctuations unrelated to disease progression or treatment. Extrinsic factors that contribute to the variable expression of movement disorders are environment related. They influence the expression of movement disorders through sensory-motor interactions and include somatosensory, visual, and auditory stimuli. Examples of somatosensory effects are stimulus sensitivity of myoclonus on touch and sensory amelioration in dystonia but also some less-appreciated effects on parkinsonian tremor and gait. Changes in visual input may affect practically all types of movement disorders, either by loss of its compensatory role or by disease-related alterations in the pathways subserving visuomotor integration. The interaction between auditory input and motor function is reflected in simple protective reflexes and in complex behaviors such as singing or dancing. Various expressions range from the effect of music on parkinsonian bradykinesia to tics. Changes in body position affect muscle tone and may result in marked fluctuations of rigidity or may affect dystonic manifestations. Factors intrinsic to the patient are related to their voluntary activity and cognitive, motivational, and emotional states. Depending on the situation or disease, they may improve or worsen movement disorders. We discuss various factors that can influence the phenotypic variability of movement disorders, highlighting the potential mechanisms underlying these manifestations. We also describe how motor fluctuations can be provoked during the clinical assessment to help reach the diagnosis and appreciated to understand complaints that seem discrepant with objective findings. We summarize advice and interventions based on the variability of movement disorders that may improve patients' functioning in everyday life.

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Comment on: Voluntary Inhibitory Control of Chorea

Maciel

Maia

Cardoso

2021

Movement Disord Clin Pract

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We read with interest the case series published by Bonomo 1 presenting patients who were able to voluntarily suppress their chorea. Chorea consists of involuntary movements which are unpredictable but not generally thought to be suppressible or distractible. Interestingly, the reported patients could control their movements for a period of time on command.As pointed out by the authors, the ability to suppress chorea has only rarely been studied. We would like to add to this discussion by presenting a patient with chorea-acanthocytosis (CA) with remarkable improvement of chorea with distraction by motor tasks. This is a 40-year-old man with a five-year history of generalized chorea and auto-mutilation with lip biting. Associated findings include sensory neuropathy diagnosed by EMG, caudate atrophy on imaging, elevated creatine kinase, and more of 10% of acanthocytes on peripheral blood smear. He is the son of consanguineous parents. A diagnosis of chorea-acanthocytosis was made. Over the course of the following years, he developed cognitive decline, obsessivecompulsive symptoms and worsening of motor skills with falls. Despite having severe chorea with frequent episodes of head drop and truncal extension, which have been recognized as hallmarks of the disease, 2 the patient's wife has noted that these movements consistently improve when he focuses on motor tasks, such as using a mobile (Video 1). He is, however, unable to voluntarily suppress these movements on command. Surface EMG ruled out dystonia or myoclonus as the cause of these movements (Fig. 1).Typically, chorea does not improve with distractibility or with performance of motor tasks. Indeed, chorea tends to interfere with voluntary motor tasks, which contributes to the morbidity of chorea. In our patient, however, performing cognitive tasks that involve motor skills (such as unlocking a mobile) dramatically improved chorea. Chorea in CA has previously been reported to improve with voluntary motor activity, mental calculations and sensory tricks, as opposed to chorea in Huntington's disease. 3,4 We theorize that the performance of cognitive tasks or movement patterns requiring a certain degree of attention are capable, in this case, of diverting the basal ganglia cortical loop away from the pattern of increased corticomotor output proposed to be associated with chorea, and this ability could be useful as a form of "cognitive trick" for him to be able to temporarily suppress these abnormal movements.Video 1. Video of patient showing generalized chorea and frequent episodes of head drop and truncal extension, all of which markedly improve when patient uses a mobile.

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Voluntary Inhibitory Control of Chorea: A Case Series

Cited by 6 publications

References 9 publications

Rethinking Movement Disorders

Rethinking Movement Disorders

Variability of Movement Disorders: The Influence of Sensation, Action, Cognition, and Emotions

Comment on: Voluntary Inhibitory Control of Chorea

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