Treatment Of Dystonia Research Articles

A Phase 3 Trial Evaluating the Efficacy, Duration of Effect, and Safety of DaxibotulinumtoxinA for Injection in the Treatment of Cervical Dystonia

A Phase 3 Trial Evaluating the Efficacy, Duration of Effect, and Safety of DaxibotulinumtoxinA for Injection in the Treatment of Cervical Dystonia

Short- and Long-term Central Action of Botulinum Neurotoxin Treatment in Laryngeal Dystonia.

Background and ObjectivesLaryngeal dystonia (LD) is isolated task-specific focal dystonia selectively impairing speech production. The first choice of LD treatment is botulinum neurotoxin (BoNT) injections into the affected laryngeal muscles. However, whether BoNT has a lasting therapeutic effect on disorder pathophysiology is unknown. We investigated short-term and long-term effects of BoNT treatment on brain function in patients with LD.MethodsA total of 161 participants were included in the functional MRI study. Statistical analyses examined central BoNT effects in patients with LD who were stratified based on the effectiveness and duration of treatment.ResultsPatients with LD who were treated and benefited from BoNT injections had reduced activity in the left precuneus compared with BoNT-naive and treatment nonbenefiting patients. In addition, BoNT-treated patients with adductor LD had decreased activity in the right thalamus, whereas BoNT-treated abductor patients with LD had reduced activity in the left inferior frontal cortex. No statistically significant differences in brain activity were found between patients with shorter (1–5 years) and longer (13–28 years) treatment durations. However, patients with intermediate treatment duration of 6–12 years showed reduced activity in the right cerebellum compared with patients with both shorter and longer treatment durations and reduced activity in the right prefrontal cortex compared with patients with shorter treatment duration.DiscussionOur findings suggest that the left precuneus is the site of short-term BoNT central action in patients with LD, whereas the prefrontal-cerebellar axis is engaged in the BoNT response in patients with intermediate treatment duration of 6–12 years. Involvement of these structures points to indirect action of BoNT treatment on the dystonic sensorimotor network through modulation of motor sequence planning and coordination.

The simple and rapid quantification method for L-3,4-dihydroxyphenylalanine (L-DOPA) from plant sprout using liquid chromatography-mass spectrometry.

L-3,4-dihydroxyphenylalanine (L-DOPA) is one of the important secondary metabolites of plants and has been used for various purposes, such as in clinical treatment for Parkinson's disease and dopamine-responsive dystonia. In plants, L-DOPA is a precursor of many alkaloids, catecholamines, and melanin; the L-DOPA synthesis pathway is similar to that in mammals. L-DOPA acts as an allelochemical, has an important role in several biological processes, such as stress response and metabolism, in plants. L-DOPA is widely used in the clinical treatment as well as a dietary supplement or psychotropic drug, understanding of biosynthesis of L-DOPA in plant could lead to a stable supply of L-DOPA. This paper describes an improved method for simple and rapid quantification of L-DOPA content using liquid chromatography-tandem mass spectrometry. The standard quantitative methods for L-DOPA require multiple purification steps or relatively large amounts of plant material. In our improved method, quantification of L-DOPA was possible with extract of one-two pieces of cotyledon without any partitioning or column for purification. The endogenous L-DOPA (approximately 4,000 µg g-1 FW (fresh weight)) could be detected from the one pieces of cotyledon of the faba bean sprout using this method. This method was also effective for samples with low endogenous amounts of L-DOPA such as broccoli, Japanese white radish, pea, and red cabbage sprouts. Therefore, this improved method will allow to measurement of L-DOPA content easily and accurately from a small amount of plant tissue and contribute to understanding biosynthesis, catabolism, and transport of L-DOPA.

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit

IntroductionPatients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered. Materials and methodsDescriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021. ResultsIBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV–V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period “honeymoon”. Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3–25.8). ConclusionsIBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.

Role of Cerebellum in the Treatment of Dystonia and Application of Cerebellum Transcranial Magnetic Stimulation

Dystonia is a specific type of abnormal movement characterized by involuntary movement, abnormal posture, or both due to persistent or intermittent muscle contractions. At present, symptomatic treatment is mainly used including a combination of psychological and rehabilitation treatments, oral medications, botulinum toxin injections and deep brain stimulation surgery. Existing animal studies suggested that the cerebellum affects dystonia through at least three mechanisms: 1) abnormal cerebellar efferent signal patterns; 2) abnormal connections between the cerebellum and basal ganglia nuclei; 3) abnormalities in the shape or structure of cerebellar cells. The specific role of cerebellum in dystonia has been explored from clinical and pathological observations, neuroelectrophysiology and neuroimaging. In the past, the main function of cerebellum was to maintain body balance and coordinate movement, while dystonia was mainly a disorder of basal ganglia. In recent years, with the development of the "cerebellum-thalamus-cerebral cortex" circuit, the cerebellum has been considered as a new node related to dystonia. As the role of cerebellum in dystonia becomes more and more important and non-invasive brain stimulation techniques have been developed, repetitive transcranial magnetic stimulation (rTMS) for cerebellum is gradually being used. Transcranial magnetic stimulation (TMS) is one of the most widely used neurophysiological techniques in human research. A magnetic field of varying intensity is applied to the central nervous system to generate local currents in the cerebral cortex. rTMS is the traditional mode of stimulation for TMS. Low frequency magnetic stimulation (≤1 Hz) inhibits cortical excitability in the stimulating region, while high frequency stimulation (>1 Hz) increases the excitability of cortical area of stimulation. Theta rhythm burst stimulation (θ rhythm burst stimulation, TBS) is a new type of rTMS. Intermittent TBS (iTBS) can increase excitability of thecortical area, while continuous TBS (cTBS) can inhibit activation of the cerebral cortex. Based on the theory of cerebellar-brain inhibition, TMS can improve symptoms by modulating cerebellar-brain inhibition. Stimulation of the cerebellar cortex by iTBS can inhibit the development of subsequent associative plasticity in the motor cortex, whereas inhibition of the cerebellar cortex by cTBS can enhance subsequent plasticity in the motor cortex. Therefore, cerebellum has gradually become a new regulatory target of higher brain functional stimulation.

Oromandibular dystonia seen during pramipexole treatment: A rare case

Dystonia is an abnormal, often repetitive, bending/twisting behavioral disorder characterized by continuous or intermittent muscle contraction. Oromandibular dystonia (OMD) is a type of dystonia involving chewing, mouth circumference, tongue, and platysma muscles. OMD is divided into different clinical types, including jaw opening OMD, jaw closing OMD, and mixed type OMD. OMD may either be primary or secondary to other diseases. The average patient age is between 50 and 60 years, and several studies have shown that it is more common among women. Dystonia may occur either as idiopathic (primary) or resulting from neurodegenerative diseases and other secondary dystonia. OMD can cause difficulty in speaking, chewing, and swallowing and produce pain during these movements. Therefore, OMD can lead to deterioration in an individual’s daily life and social relationships. Although dopaminergic drugs can be used in the treatment of dystonia, the aim of the study was to report that these drugs may also be a factor in further development of dystonia and to attract the attention of clinicians to this anomaly.

EXCESSIVE CRYING IN CHILDREN WITH CEREBRAL PALSY AND COMMUNICATION DEFICITS

Introduction: Pain/discomfort is an under-suspected/underdiagnosed cause of Excessive Crying in Children with Cerebral Palsy and Communication Decits [ECCCPCD] (due to their age or different ability). A vicious cycle of spasm-pain-spasm sets in due to the delay in treatment. To study epidemiology, theObjectives: response of ECCCPCD to drug therapy and a drug taper after 250 days. This was a xed-sequence crossover study ofMethods: 131 consecutive subjects <15 years with>7.5 hours crying duration/day for 30 straight days. Outcome measures: 1. Epidemiological data. 2. Means of total and unexplained cry durations (TECCCPCCD and UECCCPCD) in hours while on the placebo (M1) and four measurements while on treatment (M2-M5). The effect of drug taper was measured (M4). Results: Wilcoxon test between TECCCPCCD of M1-M2 yielded medians of 9.98 (95% CI 9.73 to 10.16), p<0.0001, and 6.27 (95% CI 6.24- 6.28), p<0.0001; between UECCCPCCD yielded medians of M1-M2, 8.22 (95% CI 8.02-8.39), p<0.0001, and 5.14 (95% CI 5.12 to 5.16), p<0.0001, between TECCCPCCD of M1-M5, yielded medians of 9.98 (95% CI 9.73 to 10.16) and 2.67 (95% CI 2.53 to 2.82), p<0.0001, between UECCCPCCD of M1-M5, yielded medians of 8.22 (95% CI 8.02 to 8.39) and 2.16 (95% CI 2.04 to 2.28), ps<0.0001. The dosage could be tapered after 250 days in 67/131 (51%) participants. Secondary outcomes were improvements in swallowing and drooling in 65.12% (56/86). Treatment of spasticity, dystonia, visceral, and neuropathic painConclusions: reduced crying. The drug requirement was less after 250 days of treatment. Parents/caregivers reported simultaneous improvement in dysphagia/drool.

A CAD-CAM-derived needle guide for safe treatment of oromandibular dystonia

A CAD-CAM-derived needle guide for safe treatment of oromandibular dystonia

A Phase 3 Trial Evaluating the Efficacy, Duration of Effect, and Safety of DaxibotulinumtoxinA for Injection in the Treatment of Cervical Dystonia (P1-1.Virtual)

A Phase 3 Trial Evaluating the Efficacy, Duration of Effect, and Safety of DaxibotulinumtoxinA for Injection in the Treatment of Cervical Dystonia (P1-1.Virtual)

Treatment of Cervical Dystonia Using Shorter IncobotulinumtoxinA Injection Intervals Improves Patient-reported Outcomes in Those With Inadequate Benefits from Standard Intervals (P1-1.Virtual)

Treatment of Cervical Dystonia Using Shorter IncobotulinumtoxinA Injection Intervals Improves Patient-reported Outcomes in Those With Inadequate Benefits from Standard Intervals (P1-1.Virtual)

Focal or Segmental Dystonia Resistant to Botulinum Toxin: How Do You Treat Patients in Whom Botulinum Toxin Treatment Has No Effect?

Botulinum toxin treatment is most commonly used for blepharospasm, spastic torticollis, upper limb dystonia, and local dystonia in Japan. Botulinum toxin treatment is the first choice in these conditions. However, it has the disadvantages that the therapeutic effect is transient, that there are cases in whom the treatment is ineffective, and a high cost. In ineffective cases, botulinum toxin treatment involves medication and rehabilitation. Various medications have been used for the treatment of focal dystonia mainly in open trials. As these treatments have low evidence levels, each case should be dealt with individually. Operative treatment should be considered for severe cases.

Botulinum Injection Into the Proximal Intestinal Wall of Diet-Induced Obese Mice Leads to Weight Loss and Improves Glucose and Fat Tolerance.

Botulinum neurotoxin (available commercially as BOTOX) has been used successfully for treatment of several neuromuscular disorders, including blepharospasm, dystonia, spasticity, and cerebral palsy in children. Our data demonstrate that injection of Botox into the proximal intestinal wall of diet-induced obese (DIO) mice induces weight loss and reduces food intake. This was associated with amelioration of hyperglycemia, hyperlipidemia, and significant improvement of glucose tolerance without alteration of energy expenditure. We also observed accelerated gastrointestinal transit and significant reductions in glucose and lipid absorption, which may account, at least in part, for the observed weight loss and robust metabolic benefits, although possible systemic effects occurring as a consequence of central and/or peripheral signaling cannot be ignored. The observed metabolic benefits were found to be largely independent of weight loss, as demonstrated by pair-feeding experiments. Effects lasted ∼8 weeks, for as long as the half-life of Botox as reported in prior rodent studies. These results have valuable clinical implications. If the observed effects are translatable in humans, this approach could lay the foundation for therapeutic approaches geared toward robust and sustained weight loss, mimicking some of the benefits of bariatric operations without its cost and complications.

Behavioral aspect of Wilson Disease: Diagnostic & Management Challenge

Wilson disease (WD) is a multisystem disease of defective copper metabolism. Excess copper is accumulated in different organ of body including liver, brain, kidney, eyes etc. Accumulated copper causes dysfunction of different parts of brain and produce signs and symptoms of neurological disease. Epidemiological data suggested psychiatric symptoms may be the presenting problem in 30% of WD patients. Psychiatric symptoms developed almost 100% cases of WD patients at any time of the disease course. Psychiatrc symptoms are affective mood disorder, psychotic behavioral, personality changes, anxiety & depression as well cognitive deterioration. Common neurologic symptoms are dystonia, hypertonia & rigidity, tremors and dysarthria. Rarely patients may present with polyneuropathy or dysautonomia. So both neurologic and psychiatric evaluation and specific treatment is essential for both the conditions. Diagnostic evaluations of Wilson disease include estimation of serum ceruloplasmin, 24 hours urinary copper, MRI of brain. Magnetic resonance (MR) imaging of the brain or computed tomography (CT) may detect structures involved like basal ganglia. Knowledge of behavioral problem of WD is helpful for early diagnosis of many cases and overall management. Mainstay of treatment of Wilson disease is dietary restriction of copper-rich diet, copper-chelating agents, symptomatic treatment for dystonia & rigidity as well as behavioral psychiatric therapy. For dystonia trihexy phenidyl, tetrabenazine, codopa & clonidine can be used. Neurologic as well as psychiatric symptoms would be reduced where chelation therapy is effective. More over sometimes pharmacologic treatment for psychiatric symptoms is required. J Bangladesh Coll Phys Surg 2022; 40: 121-127

Optimal deep brain stimulation sites and networks for cervical vs. generalized dystonia

Dystonia is a debilitating disease with few treatment options. One effective option is deep brain stimulation (DBS) to the internal pallidum. While cervical and generalized forms of isolated dystonia have been targeted with a common approach to the posterior third of the nucleus, large-scale investigations regarding optimal stimulation sites and potential network effects have not been carried out. Here, we retrospectively studied clinical results following DBS for cervical and generalized dystonia in a multicenter cohort of 80 patients. We model DBS electrode placement based on pre- and postoperative imaging and introduce an approach to map optimal stimulation sites to anatomical space. Second, we investigate which tracts account for optimal clinical improvements, when modulated. Third, we investigate distributed stimulation effects on a whole-brain functional connectome level. Our results show marked differences of optimal stimulation sites that map to the somatotopic structure of the internal pallidum. While modulation of the striatopallidofugal axis of the basal ganglia accounted for optimal treatment of cervical dystonia, modulation of pallidothalamic bundles did so in generalized dystonia. Finally, we show a common multisynaptic network substrate for both phenotypes in the form of connectivity to the cerebellum and somatomotor cortex. Our results suggest a brief divergence of optimal stimulation networks for cervical vs. generalized dystonia within the pallidothalamic loop that merge again on a thalamo-cortical level and share a common whole-brain network.

Supraspinal baclofen for the treatment of secondary generalized dystonia: A case series

The aim of the study was to evaluate the use of a novel technique for baclofen delivery using an intrathecal catheter inserted through a lumbar laminotomy with the tip placed at the cisterna magna (supraspinal baclofen (SSB)) for the treatment of severe generalized secondary dystonia. A cohort study of six individuals (4M/2F, mean±SD = 15±4.86 years) with generalized dystonia unresponsive to oral medications were treated with SSB and followed clinically for 9 years. Intrathecal catheter tips were positioned under fluoroscopic guidance just above the level of the foramen magnum, at the cisterna magna. Five of the 6 patients experienced sustained benefit with SSB; the group mean modified Fahn-Marsden scale scores decreased from 95 to 55 (t = 3.29, p = 0.02). One patient suffered complex pump pocket infection, and therefore underwent pump explantation. Supraspinal baclofen may be an effective method for infusing baclofen into the ventricular system of the brain for treatment of secondary dystonia which is unresponsive to oral therapy.

Blockade of M4 muscarinic receptors on striatal cholinergic interneurons normalizes striatal dopamine release in a mouse model of TOR1A dystonia

Trihexyphenidyl (THP), a non-selective muscarinic receptor (mAChR) antagonist, is commonly used for the treatment of dystonia associated with TOR1A, otherwise known as DYT1 dystonia. A better understanding of the mechanism of action of THP is a critical step in the development of better therapeutics with fewer side effects. We previously found that THP normalizes the deficit in striatal dopamine (DA) release in a mouse model of TOR1A dystonia (Tor1a+/ΔE knockin (KI) mice), revealing a plausible mechanism of action for this compound, considering that abnormal DA neurotransmission is consistently associated with many forms of dystonia. However, the mAChR subtype(s) that mediate the rescue of striatal dopamine release remain unclear. In this study we used a combination of pharmacological challenges and cell-type specific mAChR conditional knockout mice of either sex to determine which mAChR subtypes mediate the DA release-enhancing effects of THP. We determined that THP acts in part at M4 mAChR on striatal cholinergic interneurons to enhance DA release in both Tor1a+/+ and Tor1a+/ΔE KI mice. Further, we found that the subtype selective M4 antagonist VU6021625 recapitulates the effects of THP. These data implicate a principal role for M4 mAChR located on striatal cholinergic interneurons in the mechanism of action of THP and suggest that subtype selective M4 mAChR antagonists may be effective therapeutics with fewer side effects than THP for the treatment of TOR1A dystonia.

Influence of Adverse Childhood Experiences and Perfectionism on Musician's Dystonia: a Case Control Study.

Background:Musician’s dystonia (MD) is a task-specific movement disorder characterized by muscle cramps and impaired voluntary motor-control whilst playing a musical instrument. Recent studies suggest an involvement of adverse childhood experiences (ACEs) in the development of MD.Objectives:By investigating the prevalence of ACEs in MD patients with perfectionism as possible mediating factor this study aims to gain further insights into the etiology of MD.Methods:The Adverse Childhood Experiences Scale (ACE-S), the Childhood Trauma Questionnaire (CTQ) and Frost’s Multidimensional Perfectionism Scale (FMPS) were answered by 128 MD patients and 136 healthy musicians. Regression and mediator analyses were conducted to identify relevant predictors of MD and to investigate the role of perfectionism.Results:The CTQ total score (OR: 1.04; 95% CI [1.01, 1.08]) and the sub-score “emotional neglect” (OR: 1.13; 95% CI [1.02, 1.25]) were identified as two predictors of MD. Patients scored significantly higher on the sub-score emotional neglect, but no significant differences were observed for other forms of ACEs. Perfectionism had no mediating function on the association between ACEs and MD.Discussion:Though only slight differences between both groups were found, there is a trend towards higher rates of emotional neglect among dystonic musicians. A possible explanation for the association between musician’s dystonia and emotional neglect could be a lower stress resilience in musicians with a history of ACEs, which increases vulnerability to acquire dysfunctional movement patterns.These tendencies should be further investigated in future studies in which the MD and HM groups are more evenly matched in sex and age.HighlightsWe investigated the role of Adverse Childhood Experiences in the development of musician’s dystonia, comparing a large sample of healthy musicians and dystonia patients. Our findings suggest that experiencing emotional neglect might increase the probability to acquire musician’s dystonia. The findings offer new implications for etiology and treatment of dystonia.

BOTULINUM

Botulinum neurotoxins (BoNT) are toxins produced by bacteria of the genus Clostridium botulinum and are considered as the most potent toxins nowadays. Firstly, known as a food poison, later explored as a biological weapon and currently considered a great therapeutic resource. Seven different BoNT serotypes (A-G) have already been identified. Among them, serotypes A and B are used in clinics, and serotype A is the most used for the highest efficacy and safety. BoNT is widely known and used in aesthetics, through intramuscular injections to attenuate dynamic wrinkles. Additionally, it has been used as therapy in the treatment of strabismus, blepharospasm, cervical dystonia, hyperhidrosis, sialorrhea, hemifacial spasm, and many other clinical conditions in areas such as urology, neurology, ophthalmology, gastroenterology and more recently evaluated in psychiatry. BoNTs are also being studied for a new therapeutic horizon: the treatment of pain. In some painful syndromes that can be treated with BoNT, the mechanism of action is related to the inhibition of muscle contraction through inhibition of acetylcholine release at the neuromuscular junction. This effect involves a selective toxin action on SNARE family proteins (soluble N-ethylmaleimide sensitive factor attachment protein receptor), which mediate the acetylcholine exocytosis process. Moreover, it shows analgesic effect in some clinical conditions that are not related to excessive muscle contraction, such as in the treatment of migraine. Therefore, it has been suggested that BoNT is capable of producing analgesia independent of muscle contraction inhibition. This evidence has stimulated the study of BoNT in other painful conditions, such as trigeminal neuralgia. One of the advantages of BoNT clinical application is its safety. Major adverse effects are application-related and include pain, erythema, pruritus, edema, bruising, hematoma, paraesthesia, and ptosis. In this review BoNT will be analyzed in the historical, pharmacological, biochemical and clinical aspects, but the focus will be on the investigation of the analgesic effect of BoNT that occurs regardless of its muscle relaxant effect.

LIVECHART Patient-Reported Outcome Tool for Botulinum Toxin Treatment in Cervical Dystonia.

ABSTRACTBackgroundMany tools used for recording the response to botulinum toxin treatment are disease‐specific, observer‐based and cumbersome to implement in service settings, especially where clinics treat a variety of disorders. Physicians, clinics, researchers, and patients themselves could benefit from a practical and generic patient‐reported outcome tool. The Liverpool botulinum toxin effects chart (LIVECHART) is a patient‐administered questionnaire developed and used informally over 25 years in a major UK botulinum toxin treatment clinic. In preparation for more formal validation studies, this cross‐sectional study aimed to understand how well LIVECHART captures the effects of botulinum toxin treatment, using patients with cervical dystonia as exemplars.MethodsLIVECHART questionnaires were completed by 90 patients with cervical dystonia who had each experienced at least three previous botulinum toxin injection cycles with completed LIVECHARTs.ResultsThere were significant positive correlations between Likert scores (major deterioration—major benefit) for botulinum toxin treatment effects, and measures derived from weekly visual analog scale (VAS) scores (0–100), including (1) baseline to peak effect, (2) Area Under the benefit Curve (AUC) of current cycle, (3) peak effect duration, (4) duration of acceptable benefit, (5) time back to baseline. The AUC of the current cycle was positively correlated with (1) VAS change baseline to peak effect, and (2) week worn off completely.ConclusionsWe conclude that LIVECHART has high internal consistency and reliability. It adequately reflects amplitude, duration and overall benefit of botulinum toxin treatment, and is worth further formal evaluation to determine its validity and reliability.

Clinical spectrum and management of dystonia in patients with Japanese encephalitis: A systematic review.

BackgroundJapanese encephalitis (JE) is a potentially fatal viral infection with a wide range of manifestations and can also present with a variety of movement disorders (MD) including dystonia. Dystonic features in JE are uncommon. Here, we have tried to summarize the clinical features and management of dystonia among JE patients with a comprehensive literature search.MethodsVarious databases, including PubMed, Embase, and Google Scholar, were searched against the predefined criteria using suitable keywords combination and boolean operations. Relevant information from observational and case studies was extracted according to the author, dystonic features, radiological changes in the brain scans, treatment options, and outcome wherever provided.ResultWe identified 19 studies with a total of 1547 JE patients, the diagnosis of which was confirmed by IgM detection in serum and/or cerebrospinal fluid in the majority of the patients (88.62%). 234 (15.13%) of JE patients had dystonia with several types of focal dystonia being present in 131 (55.98%) either alone or in combination. Neuroimaging showed predominant involvement of thalami, basal ganglia, and brainstem. Oral medications including anticholinergics, GABA agonists, and benzodiazepines followed by botulinum toxin were the most common treatment modalities.ConclusionDystonia can be a disabling consequence of JE, and various available medical therapies can significantly improve the quality of life. Owing to insufficient studies on the assessment of dystonia associated with JE, longitudinal studies with a larger number of patients are warranted to further clarify the clinical course, treatment, and outcome of dystonia.