Dystonia (Limb Dystonia)

An attitude or posture due to the co-contraction of agonists and antagonist muscles in one region of the body. It most often affects the large axial muscles of the trunk and limb girdles. Conditions which feature persistent or recurrent episodes of dystonia as a primary manifestation of disease are referred to as DYSTONIC DISORDERS. (Adams et al., Principles of Neurology, 6th ed, p77)

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Networked: 5088 relevant articles (521 outcomes, 438 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Parkinson Disease (Parkinson's Disease)
2.	Movement Disorders (Movement Disorder)
3.	Essential Tremor (Essential Tremors)
4.	Tremor (Tremors)
5.	Dyskinesias (Dyskinesia)

Experts

1.	Bhatia, Kailash P: 60 articles (01/2022 - 01/2004)
2.	Krauss, Joachim K: 43 articles (01/2022 - 01/2002)
3.	Richter, Angelika: 40 articles (01/2021 - 05/2002)
4.	Volkmann, Jens: 36 articles (01/2022 - 01/2002)
5.	Lin, Jean-Pierre: 33 articles (04/2021 - 02/2010)
6.	Jinnah, H A: 32 articles (01/2022 - 05/2000)
7.	Klein, Christine: 31 articles (11/2022 - 08/2002)
8.	Okun, Michael S: 30 articles (01/2021 - 02/2005)
9.	Pisani, Antonio: 28 articles (11/2022 - 10/2007)
10.	Cif, Laura: 28 articles (07/2022 - 01/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Dystonia:

1.	Botulinum Toxins (Botulinum Toxin)IBA 05/01/2022 - "Botulinum toxin is effective in most patients with focal/segmental dystonia. " 07/01/2010 - "The best treatment for craniocervical dystonia is botulinum toxin injected into the overactive muscles." 09/01/2023 - "Presently, there is no cure for laryngeal dystonia, but botulinum toxin has shown significant success in treating the symptoms of the disorder." 01/01/2023 - "All three groups demonstrated significant improvement in knowledge of module content between their pre- and post-module test scores (PD: p=.0001, dystonia: p<.0001 and botulinum toxin: p=.008), and those who took the dystonia module maintained significant improvement at either a 3- or 6-month follow up compared to pre-module (p <.0001). " 05/01/2022 - "Stereotactic surgery can be the first-line treatment for some types of dystonia because of its high efficacy or because of the absence of official approval for botulinum toxin treatment in Japan. "
2.	Levodopa (L Dopa)FDA LinkGeneric 06/01/2015 - "Levodopa dramatically decreased dystonia and improved spatio-temporal, kinematic and posture parameters. " 06/01/2004 - "The dystonia improved dramatically with levodopa treatment in the absence of additional parkinsonian feature. " 01/01/2003 - "In most patients, this results not in predominating dystonia, a largely nondegenerative condition, but in a progressive often lethal neurometabolic disorder, which can be improved but not cured by L-dopa. " 05/09/2017 - "Furthermore, our literature study on the effect of a therapeutic trial to gain symptomatic relief revealed that l-dopa has occasionally proven beneficial in several established "non-DRDs" and may therefore be considered in selected cases of dystonia due to other causes. " 01/01/2021 - "According to the long-term outcomes, patients were further graded as good (dystonia improved by more than 50% after levodopa, and no further motor symptoms appeared) and poor (dystonia improved by <50% after levodopa, or new motor symptoms appeared). "
3.	BaclofenFDA LinkGeneric 01/01/2019 - "After that, several studies indicate that intraventricular administration of baclofen, is more effective than ITB in refractory dystonia. " 04/21/2003 - "Continuous intrathecal baclofen was effective in treating severe spasticity and dystonia of cerebral origin with major effect on muscles of the lower extremities, pelvis, and back and in particular opisthotonus was relieved. " 01/01/2015 - "Treatment of severe spasticity and dystonia with intrathecal baclofen (ITB) in children has been shown to be effective and has therefore been employed in the Region of Southern Denmark. " 01/01/1992 - "Baclofen can be an effective treatment for childhood dystonia." 01/01/2018 - "Intrathecal baclofen has been suggested as an effective and safe treatment for intractable spasticity and dystonia. "
4.	Type A Botulinum Toxins (Botox)FDA Link 05/01/1989 - "Jaw dystonia was therefore considered and Botulinum A toxin was injected into the right masseter and temporalis which dramatically improved the patient's condition." 09/01/1998 - "OBJECTIVE: Botulinum A toxin has been reported to provide excellent symptomatic relief for patients with dystonia. " 01/01/2012 - "The aim of this study was to examine whether multichannel electromyogaphic mapping improved outcomes in a cohort of antibody-negative onabotulinumtoxinA non-responders by more precisely identifying which muscles were involved in the dystonia. " 06/01/2018 - "In our experience, transoral administration of botulinum toxin type A to treat laryngeal dystonia has proved to be a simple, quick, effective and safe technique. " 04/22/2014 - "Botulinum toxin-A is considered an effective and safe treatment option for refractory oromandibular dystonia in khat-chewers. "
5.	Trihexyphenidyl (Artane)FDA LinkGeneric 10/01/1992 - "The action dystonia was markedly improved by giving trihexyphenidyl. " 02/01/1986 - "Thirty-seven per cent of the 35 idiopathic dystonia patients had a moderate to marked improvement with an average dosage of 21.5 mg of trihexyphenidyl. " 07/01/2011 - "The author hypothesized that trihexyphenidyl may be more effective in specific subgroups and performed a retrospective analysis of 31 children (8.2 ± 5.8 years) with dystonia following treatment with high-dose trihexyphenidyl (>0.5 mg/kg/day). " 09/01/1996 - "Response to medical treatment (trihexyphenidyl dose as high as 40 mg daily) in 5 patients was disappointing; 4 of the 5 patients who underwent functional stereotaxic operations were improved, but dystonia was still present at the end of the follow-up. " 01/01/2023 - "Administration of trihexyphenidyl hydrochloride and concomitant rehabilitation improved his dystonia until return to work, but some residual gait abnormalities remained. "
6.	Antipsychotic Agents (Antipsychotics)IBA 07/01/2015 - "Patients were found to have significant positive results in amelioration of dystonia (P < 0.001) over a 1-year period, without loss of antipsychotic efficacy. " 11/01/2012 - "The incidence of dystonia is 2-3% among the patients treated with antipsychotics, and 50% among the ones cured with conventional antipsychotics. " 08/01/1988 - "Neuroleptic dosage correlated negatively with improvement in trunk and dystonia ratings. " 01/01/2013 - "To study the tonus of tongue muscles as a measure of orofacial dystonia and the total hepatic oxidative capacity as a potential predictor of excessive vulnerability to neuroleptic-induced dystonia in psychiatric patients. " 11/01/1988 - "In a study of 135 schizophrenic and 46 manic male patients treated with neuroleptics, a significantly higher proportion of the manic patients (26.1%) than of the schizophrenic patients (5.9%) developed acute dystonia. "
7.	Cholinergic Antagonists (Anticholinergics)IBA 10/01/2004 - "We describe a 46-year-old woman who presented with lingual dystonia induced only by speaking, which responded well to anticholinergic treatment." 08/01/1988 - "In addition, the incidence of dystonia and the efficacy of anticholinergic prophylaxis were related inversely to age. " 11/01/2022 - "In generalized dystonia, anticholinergic moderately alleviate symptoms. " 11/01/2022 - "Once acute dystonia has appeared, parenteral treatment is to be preferred, but oral cholinergic antagonists may be used after the acute phase to prevent relapse. " 10/01/2017 - "Anticholinergic medication in high doses can be associated with memory impairment in dystonia. "
8.	incobotulinumtoxinAFDA Link 01/01/2023 - "The purpose of this study was to thoroughly evaluate the efficacy of BoNT-A injection in treating WD complicated by lower extremity dystonia. " 01/01/2018 - "The objective of this study was to investigate the effect of incobotulinum toxin A (Xeomin) in the treatment of this type of dystonia in parkinsonian patients, using a randomized, double blind, placebo-controlled trial. " 01/01/2023 - "Overall, our findings indicated that BoNT-A injection led to marked relief of symptoms in patients with WD with lower extremity dystonia." 11/01/2022 - "IncobotulinimtoxinA (Xeomin) for the treatment of adductor laryngeal dystonia: A prospective, open-label clinical trial." 07/19/2019 - "BoNT/A LD0 exceeded 10-fold the dose administered safely to humans for cosmesis and dystonia."
9.	Dihydroxyphenylalanine (Dopa)IBA 03/01/1995 - "Remission in dihydroxyphenylalanine-responsive dystonia." 01/01/2021 - "NR4A2 and Dystonia with Dopa Responsiveness." 06/15/2011 - "We interrogated the complete genome sequences of a 14-year-old fraternal twin pair diagnosed with dopa (3,4-dihydroxyphenylalanine)-responsive dystonia (DRD; Mendelian Inheritance in Man #128230). " 06/05/2010 - "High frequency of multiexonic deletion of the GCH1 gene in a Taiwanese cohort of dopa-response dystonia." 03/01/2005 - "The combination of symptoms, their sensitivity to DOPA, and their persistence throughout childhood constitute, to our knowledge, a new clinical entity, which we propose to categorize as a DOPA-sensitive dystonia-plus syndrome."
10.	Gabapentin (Neurontin)FDA LinkGeneric 09/01/2010 - "All three patients responded well to gabapentin after failing to respond to other anti dystonia drugs." 01/01/2016 - "A four-year retrospective, observational study analysed the use of gabapentin for severe dystonia in children at the Evelina London Children's Hospital. " 01/01/2016 - "Gabapentin can significantly improve dystonia severity and quality of life in children." 02/01/2005 - "There have been only a few previous reports of gabapentin-induced dystonia. " 02/01/2005 - "To report a case of dystonia as an adverse effect of gabapentin. "

Therapies and Procedures

1.	Deep Brain Stimulation 01/01/2023 - "Deep brain stimulation has dramatically changed the management of patients with dystonia, therapeutic approach of dystonia with marked improvement of dystonia and functional disability. " 01/01/2022 - "Deep brain stimulation (DBS) is currently the most effective treatment for medically refractory dystonia with globus pallidus internus (GPi) usually the preferred target. " 07/01/2017 - "The literature review shows that pallidal deep brain stimulation is most effective in patients with isolated dystonia irrespective of the underlying etiology. " 11/01/2013 - "Deep brain stimulation (DBS), specifically of the globus pallidus interna (GPi), has been shown to be extremely effective in primary generalized dystonia. " 07/01/2008 - "Pallidal deep brain stimulation (DBS) is currently the most effective treatment for advanced, medically refractory dystonia. "
2.	Therapeutics 01/01/2021 - "Best treatment results are obtained when BT therapy is integrated in the multimodal and long-term 'multilayer concept of treatment of dystonia'. " 05/01/2006 - "GPi-DBS is a safe and effective treatment for generalized dystonia in patients who remained impaired, despite optimal medical therapy." 11/01/2016 - "To improve the current medical and allied health care treatment options for patients with acquired dystonia, high-quality trials that examine the efficacy of therapies need to be performed." 01/01/2005 - "This transgenic mouse model demonstrates behavioral and pathologic features similar to patients with early-onset dystonia and may help to better understand the pathophysiology of this disorder and to develop more effective therapies." 05/01/2011 - "The results suggest that ITB therapy is an effective treatment for managing spasticity and dystonia, and for improving motor function in children with CP."
3.	Injections 01/01/2018 - "Btx injections are effective for improving clinical state of parkinsonian patients with plantar flexion of toe dystonia." 09/01/1998 - "We confirm that the botuline toxin transcutaneous injections represent a save and effective treatment for laryngeal adductor dystonia." 12/01/2012 - "A comparison of facial muscle squeezing versus non-facial muscle squeezing on the efficacy of botulinumtoxin-A injections for the treatment of facial dystonia." 07/01/2023 - "Yet another line of research grew out of injections for laryngeal dystonia, whereby clinicians began to explore other sphincters in the gastrointestinal tract and eventually to treatment of pelvic sphincters; many of these sphincters are innervated by autonomic nerves. " 11/01/2022 - "US and E-Stim localization guidance techniques provide equivalent efficacy in onabotA injections for spasticity and dystonia. "
4.	Pallidotomy 10/01/2022 - "Bilateral pallidotomy may result in clinically significant improvement in children with acquired/heredodegenerative generalized dystonia, although the benefits should be closely weighed against the risk of irreversible bulbar dysfunction. " 07/01/1998 - "We conclude that pallidotomy is a safe and effective treatment in medically refractory cases of generalized dystonia." 07/01/1998 - "We systematically evaluated the efficacy of both unilateral and bilateral stereotactic pallidotomy in eight patients with generalized dystonia. " 10/01/2022 - "Limited studies are available on the role of pallidotomy in children with acquired/heredodegenerative generalized dystonia. " 01/01/2022 - "The objectives of this study are to evaluate the effectiveness of bilateral subthalamic nucleus (STN) DBS along with unilateral posteroventral pallidotomy (PVP) in patients with dystonia who experienced unsatisfactory GPi-DBS and to address the reported rescue procedures after suboptimal DBS or lesion surgery in dystonia patients. "
5.	Electrodes (Electrode) 01/01/2002 - "However, there is today enough promising evidence for a striking efficacy of pallidal stimulation in dystonia, supporting the need for further investigations in the field, with collaborative projects (regarding to the limited number of eligible patients); double-blind studies, including a consensus about surgical method; and a precise anatomic analysis of the position of the electrode. " 01/01/2018 - "Although mobile dystonia components were slightly improved in the long-term outcome from GPi-DBS the overall therapeutic response 9 years from implantation was limited when comparing "stimulation off" and "stimulation on" despite of proper electrode localization and sufficient stimulation programming. " 08/01/2009 - "Predictors of a favourable long-term efficacy of DBS in primary generalized dystonia patients are young age at surgery, a shorter duration of the disease, a lower severity of disability, and a posteroventrolateral localization of the DBS electrode within the globus pallidus internus (GPi). " 07/01/2009 - "The described technique using stereotactic MRI for planning of the trajectory and direct visualisation of the target, intra-operative MER for delineating the boundaries of the target and macrostimulation for probing the distance to the internal capsule by identifying the threshold for stimulation induced tetanic contractions is effective in DBS electrode implantation in patients with dystonia operated under general anaesthesia. " 05/01/2013 - "Decreased efficacy of pallidal DBS in secondary and NBIA associated dystonia is unlikely to be related to difficulties in achieving the planned electrode placement."