Tardive dystonia …

Lesly G Aguilar Tabora MD

Movement Disorders

Updated 03.11.2026
Released 10.04.1993
Expires For CME 03.11.2029

Tardive dystonia

Author: Lesly G Aguilar Tabora MD

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Editor: Robert Fekete MD

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Tardive dystonia

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Introduction

Overview

Tardive dystonia is the second most common variant of a tardive syndrome after orofacial dyskinesia and is among the most disabling. It is a distinct tardive syndrome characterized by sustained or intermittent dystonic postures arising after exposure to dopamine receptor-blocking agents. It has historically been described using broader terms such as “tardive dyskinesia” or “extrapyramidal syndromes,” though it is now recognized as a separate clinical entity with unique phenomenology, course, and management considerations. Although relatively uncommon overall, tardive dystonia is frequently severe, medically refractory, and associated with low rates of spontaneous remission. Advances, including long-term outcomes with globus pallidus internus deep brain stimulation, have expanded therapeutic options for patients with refractory disease.

Key points

	• Tardive dystonia develops after exposure to dopaminergic blocking agents, most commonly after long-term exposure, and frequently persists despite discontinuation of the offending agent.
	• Tardive dystonia is often reported under the umbrella of tardive syndrome or extrapyramidal syndrome. Accurate use of the term of tardive dystonia improves earlier recognition of phenomenology, and subsequent initiation of appropriate treatment, and allows better data collection regarding true incidence and prevalence.
	• Dopamine blocking agents associated with tardive dystonia are most frequently antipsychotic medications but also include antiemetics such as metoclopramide.
	• The effects of tardive dystonia are often disabling and compromise quality of life due to abnormal movements and the pain produced by these.
	• Medical treatment of tardive dystonia is often symptomatic, with the goal of decreasing pain and dystonic spasms. Discontinuation of the offending dopaminergic blocking agent may be necessary.
	• Deutetrabenazine and valbenazine (level A) are FDA-approved for tardive dyskinesia; their role in tardive dystonia remains uncertain. Updated AAN guidelines list ginkgo biloba as probably effective (Level B) for tardive syndromes; however, supporting trials largely involve tardive dyskinesia, and efficacy in tardive dystonia remains uncertain. Pallidal deep brain stimulation may be considered for tardive dystonia refractory to medical therapy (level C).

Historical note and terminology

The first cases of tardive dystonia were described in the 1950s, shortly after dopamine receptor antagonists were introduced. Keegan and Rajput described these dystonic movements as dystonia tarda (38). In 1982, Burke and colleagues first distinguished patients with tardive dystonia as persistent and late in onset on phenomenological, epidemiological, prognostic, and pharmacologic grounds, in which the dystonia must have developed either during or within three months of a course of neuroleptic treatment (06).

The 2013 Consensus Committee updated the definition for dystonia as “a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Dystonic movements are typically patterned, twisting, and may be tremulous. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation.” Tardive dystonia may be classified along two axes: clinical characteristics and etiology. Further characterization of dystonia may be made based on age at onset, body distribution (focal, segmental, multifocal, generalized, or presenting as hemidystonia), static versus progressive temporal pattern, and associated features, including other movement disorders or neurologic manifestations. Etiology of tardive dystonia may be acquired and due to a known specific cause (01).

Tardive dyskinesia is an umbrella term used to describe multiple phenomenologies occurring from exposure to dopamine blocking agents and includes tardive dystonia (32). Replacing terms such as tardive dyskinesia and extrapyramidal syndrome with more specific designations (eg, tardive dystonia, akathisia, etc.) to describe the tardive movement disorder improves earlier recognition of phenomenology and subsequent initiation of appropriate therapeutic options in managing tardive syndromes. When dystonia is the main feature of the tardive syndrome, it is considered to be tardive dystonia (22).

Clinical manifestations

Presentation and course

Tardive dystonia consists of involuntary, persistent, and sustained muscle contractions that cause twisting and repetitive movements or abnormal postures, usually occurring after months or years of neuroleptic exposure. Tardive dystonia tends to arise insidiously, progress gradually over months to years, and then stabilize. Onset usually occurs in a single body location, typically the face or neck. The arms, trunk, and legs follow in descending order of frequency (06; 69; 19). Tardive dystonia may progress from focal involvement to encompass two or more contiguous or noncontiguous body parts, or it may generalize. Unlike classical tardive dyskinesia, which may be well tolerated or even unnoticed by patients, tardive dystonia is often distressing and disabling (78). The relationship between the age of onset and the distribution of tardive dystonia parallels that of the idiopathic variety: younger subjects, particularly younger male patients, tend to develop generalized dystonia with a more rapid spread and shorter duration of exposure to dopamine receptor blocking agents at onset of dystonia, whereas it more often remains focal or segmental in older patients (06; 42). Cervical dystonia, retrocollis, anterocollis, and torticollis to the right were found to be more common in tardive dystonia than in primary cervical dystonia (42). Late or cranial-onset symptoms, features rarely observed in DYT1 dystonic patients, may distinguish tardive dystonia from primary dystonia (05).

Tardive dystonic movements are clinically identical to those seen in idiopathic dystonia. Tardive dystonia can persist, even after discontinuation of the antipsychotic drug.

Tardive generalized dystonia and stereotypies

This man displays severe tardive cervical, truncal, and oromandibular dystonia and bruxism. He also exhibits generalized stereotypy and akathisia. (Contributed by Dr. Joseph Jankovic.)
Tardive generalized dystonia and stereotypies helped by tetrabenazine

This woman displays severe tardive generalized stereotypy. She improved markedly on tetrabenazine. (Contributed by Dr. Joseph Jankovic.)
Tardive dystonia related to prolonged treatment with risperidone

This is a 23-year-old male with tardive dystonia related to prolonged treatment with risperidone. There is prominent facial grimacing, retrocollis, torticollis to the left, and restlessness. (Contributed by Dr. José Fidel Baizabal...

Trunk involvement in tardive dystonia is best described as arching backward and differs from idiopathic dystonia, where laterocollis and lateral trunk torsion are present (19). The posturing of the arms in internal rotation with flexion of the elbows and wrists is also characteristic of tardive dystonia. Another feature that differentiates between tardive and idiopathic dystonia is the improvement of the dystonic movements with voluntary action in the former. Most patients report some response to sensory "tricks" or task-specific worsening or improvement.

Dystonia with camptocormia helped by a postural maneuver in primary generalized dystonia

This patient has primary generalized dystonia manifested mostly by dystonic camptocormia. Her dystonia is markedly relieved by stepping with one foot onto a chair ("geste antagonistique" or "geste antagoniste")--analogous to a "se...

Retrocollis is a characteristic component occurring in almost half of all patients (36). Less common manifestations include respiratory muscle involvement, oculogyric crises, bruxism, and lingual protrusion dystonia (54; 68; 69). Other movement disorders may coexist, including the orofacial dyskinesias characteristic of tardive dyskinesia, parkinsonism, akathisia, chorea, tremor, and myoclonus (29).

Bruxism

This patient with tardive bruxism experiences constant teeth grinding. (Contributed by Dr. Joseph Jankovic.)
Tardive cranial dystonia and orofacial dyskinesia helped by tetrabenazine

This woman has severe tardive cranial dystonia and orofacial dyskinesia. She improved markedly with tetrabenazine. (Contributed by Dr. Joseph Jankovic.)

As prevention is the key to minimize or ameliorate tardive dystonia, identifying patients who may be at higher risk of developing a tardive syndrome is of absolute importance. Several risk factors for the development of tardive dyskinesia have been described (32; 65). Nonmodifiable risk factors for increased risk of tardive dyskinesia include older patients, female gender, white and African descent, longer psychiatric illness duration, prior brain injury, dementia, and gene polymorphisms involving antipsychotic medication metabolism and dopamine functioning. These risk factors may be applied to all patients with the potential to develop a tardive syndrome, including tardive dystonia, to improve early recognition. However, and in contrast to tardive dyskinesia, tardive dystonia tends to affect younger men. Clinicians are advised to identify and address modifiable risk factors such as the presence of alcohol and substance abuse, the presence of early parkinsonism, and early use of anticholinergic medications, and clinicians are advised to monitor cumulative antipsychotic dosage when determining the current doses of antipsychotic medications.

Table 1. Tardive Dystonia

Population at Risk	Patients exposed to antipsychotics
Course	Typically insidious and focal with progression to other body parts over months to years
Age and gender predilection	Younger male patients
Age of onset and progression
Younger patients	• Tendency towards generalized dystonia • More rapid spread with shorter duration of exposure to DRB agents • Males tend to present with tardive dystonia at a younger age at first exposure
Older patients	• Tendency to remain focal
Cranio-cervical tardive dystonia	• Late onset • Cervical-axial dystonia (most common) • Tardive oromandibular dystonia patients tend to be restricted to the oromandibular area (in idiopathic OMD, patients frequently have neck involvement) • Cervical dystonia, retrocollis, anterocollis, and torticollis to the right are more common than in primary dystonia
Truncal involvement	Opisthotonic truncal extension
Arm posturing	Tendency towards internal rotation with flexion of the elbows and wrists
Response to sensory “tricks” (geste antagoniste)	Minimal
Presence of dystonic storm (status dystonicus)	Rare
Sensory Phenomenon	Infrequent: paresthesias, urge to move
Associated movement disorders	Stereotypies, akathisia, and other movement disorders

Dystonic storm, or status dystonicus, is characterized by dystonia refractory to standard drug therapy. This entity is infrequent amongst patients with primary dystonia, and even rarer in those with tardive dystonia.

Prognosis and complications

Remissions are much rarer than in classical tardive dyskinesia. Remissions hardly ever occur, even when the drug is discontinued, and may take 1 year to 5 years for remission to become complete. In one study, 12% to 33% of patients experience remission after discontinuation of the offending drug, whereas spontaneous remission without discontinuation of the offending agent was described as 2.5% per year (32). A fourfold chance of remission is possible when the dopamine receptor blocking drug is discontinued, yet tardive dystonia is probably irreversible in patients who have been on neuroleptics for more than 10 years (42). Patients who had a remission after cessation of the causative agent have developed permanent tardive dystonia when antidopaminergic drugs were resumed (06).

Unlike classical tardive dyskinesia, the movements of tardive dystonia are often disabling. The following list includes some described complications: death from myoglobinuric renal failure; fracture of the odontoid process in a patient with tardive retrocollis, rheumatoid arthritis, osteoporosis; bilateral rib fractures in a patient with severe axial and limb dystonia with opisthotonos; camptocormia; and Pisa syndrome (49; 42; 44; 27; 08).

Clinical vignette

A 48-year-old man had a history of a single manic episode 10 years earlier. He was treated with lithium for one year and then took no medication for eight years until he developed a “manic” episode. He received haloperidol for four days and became stiff for a week. Sixteen months later, he was “acting bizarre.” He was initially treated with haloperidol and lithium; the former was then changed to thiothixene because of persistent symptoms. He discontinued thiothixene after 21 days because of visual difficulties. A week later, he started moving his head, neck, and jaw, smacking his lips, and blinking uncontrollably. Movements increased on a daily basis. He became unable to walk due to eye closure sustained for 5 to 10 seconds. He had to give up driving and fell into a pit at work because of impaired vision. He also bit his tongue and cheek frequently because of the involuntary clenching of his teeth. He felt his head turning toward the right.

On examination 17 months after onset, there was intermittent tonic and clonic blepharospasm, grimacing, mouth opening with deviation of the lower jaw to the right, jaw clenching with bruxism, and grunting. There was also intermittent flexion and rotation of the head to the right. All movements diminished markedly when he spoke.

Tardive dystonia

There are frequent dystonic spasms involving various combinations of blepharospasm, grimacing, jaw opening or closure, and anterocollis and right torticollis. There is marked attenuation of all movements with speech. (Contributed ...

There was also intermittent flexion and rotation of the head to the right. All movements diminished markedly when he spoke.

Trials of anticholinergics, baclofen, reserpine, botulinum toxin injections, and valproate yielded no benefit. Recurrent psychosis necessitated prolonged neuroleptic therapy, and the use of clozapine was not associated with a change in his dystonia.

Biological basis

Etiology and pathogenesis

Tardive dystonia is a complication of long-term exposure to antidopaminergic drugs; however, the duration of exposure required may be shorter than that necessary for classical tardive dyskinesia. In one series, 21% of patients had been taking their medication for one year or less (36). Although tardive dystonia tends to appear after long-term treatment, there is no minimum period of neuroleptic treatment that may be considered safe. Cases of chronic dystonia after single exposures to antiemetics (eg, metoclopramide) have been reported (75).

All classes of dopamine receptor blockers are potential etiologic agents. A lower incidence of tardive complications from atypical, also known as second-generation antipsychotics, compared with traditional antipsychotics (first generation), has not yet been established through long-term studies (70). A point-prevalence study of tardive dyskinesia in a cohort of more than 20,000 patients with dementia a year after starting treatment found no difference between those treated with typical versus those treated with atypical antipsychotics (50). In Martino and Karnik’s 2018 review of 316 randomized controlled trials, prevalence estimates for tardive dystonia ranged from 1.4% (quetiapine, a second-generation antipsychotic) to 15.3% (L-sulpiride, first generation) for dystonia (53). Nevertheless, the “atypicality” of most second-generation antipsychotics appears to be a relative property that is lost at higher doses (18). Clozapine and quetiapine appear to have little or no propensity to cause tardive dystonia. Other medications linked to tardive syndromes, including tardive dystonia, include aripiprazole, duloxetine and citalopram (serotonin reuptake or serotonin norepinephrine reuptake inhibitors), chronic lithium, flunarizine and cinnarizine, pimozide, tricyclic antidepressant amoxapine, and ziprasidone (11; 58; 32; 41).

Because only a minority of patients with long-term neuroleptic exposure develop tardive dystonia, genetic predisposition is suspected. A genetic association with dopamine D3 alleles has been advanced as a possible explanation for the development of tardive dystonia with atypical antipsychotics (10). Yet, no polymorphisms of the dopamine D2 or D3 receptors were significantly associated with the disorder in another study (55).

The hypothesis of dopamine receptor hypersensitivity due to chronic dopamine blockade used to explain the development of tardive dyskinesia may be applied to tardive dystonia (43; 41). With D2 blockade, antipsychotics inhibition or antagonism of dopaminergic output from the globus pallidus and substantia nigra is lifted, thus leading to hyperkinetic movements. However, tardive dystonia has a somewhat different pharmacology from classical tardive dyskinesia. One theory proposes oxidative stress with increased dopamine turnover and high levels of free radicals leading to striatal degeneration as an explanation to persistence of tardive dystonia even after discontinuation of antipsychotics (41). Another hypothesis to explain the development of tardive dystonia is that of GABA insufficiency due to decreased glutamic acid decarboxylase in the substantia nigra, medial globus pallidus, and the subthalamic nucleus (09). Anticholinergic drugs, for example, are often beneficial in tardive dystonia but tend to aggravate the movements of tardive dyskinesia, suggesting that the pathophysiology of tardive dystonia differs in some key respects from that of tardive dyskinesia. There are reports of tardive dystonia-like syndromes occurring with selective serotonin reuptake inhibitors and with selective serotonin norepinephrine reuptake inhibitors; however, the mechanism is not known.

Epidemiology

There are few articles specifically reviewing the epidemiology or natural history of tardive dystonia. Larger and more recent series include tardive dystonia in the tardive syndrome. Both psychiatric and nonpsychiatric patients treated with dopamine receptor blocking drugs are at risk. Although tardive dystonia, unlike tardive dyskinesia, shows no age or sex predilection, there is minimal male predominance with men presenting tardive dystonia at a younger age at first exposure (36; 42).

Tardive dystonia is greatly underreported. Many observers use the term “tardive dyskinesia” generically for all tardive movement disorders, just as many use the term “extrapyramidal syndromes” to refer collectively to drug-induced acute dystonia, akathisia, parkinsonism, and tardive syndromes (15). The reported prevalence of tardive dystonia ranges between 0.4% to 5% and can be as high as 21% if more inclusive criteria are utilized. In the psychiatric patient population, the prevalence of tardive dystonia has been estimated to be between 1% and 2% for both outpatients and chronic inpatients (79; 23; 60). However, a systematic search for evidence of dystonia in veterans with long-term exposure to antipsychotic drugs found a prevalence rate of more than 20%, most with focal dystonia (63).

These results correlate with a survey of tardive movement disorders (66) that found tardive dystonia to be the second most common type of dopamine receptor blocking drug-related movement disorder, almost as common as classical orofacial tardive dyskinesia, with 56 prevalence surveys and 34,555 subjects yielding an average prevalence of 20% of tardive dyskinesia in patients treated chronically with dopamine receptor blocking drugs (35). In Ferraz and Andrade’s study of 122 patients with a dystonic syndrome, tardive dystonia was the most common cause of secondary dystonia, accounting for 13% of all dystonic patients encountered (20).

Martino and colleagues found the prevalence of new onset tardive syndromes does not appear to be affected by treatment duration or the use of flexible versus fixed dosing of antipsychotic medications (53). Prevalence of tardive syndrome varies with reported lower rates with exposure to second-generation antipsychotics compared to first-generation antipsychotics (20.7% vs. 30.0%, p=0.002). The incidence of tardive syndrome is estimated to increase by 5% annually during the first 5 years of treatment, with an incidence of 49% after 10 years of treatment, and 68% after 25 years of treatment (24; 14; 62).

Differential diagnosis

On a phenomenological basis, tardive dystonia is difficult to distinguish from any other etiology of dystonia. In comparison to idiopathic cervical dystonia patients, those with tardive cervical dystonia have been said to have a higher frequency of involvement of other body parts and more clonic head movements, to be less responsive to sensory tricks, and not to have a head tremor or family history of dystonia (56). Tardive patients often exhibit classic orofaciolingual, limb, and respiratory dyskinesias of tardive dyskinesia (69). Tan and Jankovic also found that tardive oromandibular dystonia (OMD) patients tended to manifest dystonia restricted to the oromandibular area in comparison to idiopathic OMD, where patients frequently had neck involvement.

Confusing conditions

The diagnosis of tardive dystonia can be established only by the history of exposure to an appropriate drug and exclusion of other causes of dystonia. Patients with idiopathic dystonia who are coincidentally being treated with antidopaminergic drugs may be misdiagnosed as having tardive dystonia. Nevertheless, and despite the lack of a proper epidemiologic study, it is widely believed that the proportion of patients taking these drugs who develop dystonia greatly exceeds the expected frequency of idiopathic dystonia in the same population.

Wilson disease and dopamine-responsive dystonia may be considered in those patients developing dystonia at an early age. The presence of neurologic signs other than dystonia, or a positive family history of dystonia, should prompt additional studies to exclude other causes of dystonia.

Head injury, strokes, intracerebral hemorrhages, tumors, or psychogenic etiology causing dystonia can often be excluded as causes of tardive dystonia through careful history and through neuroimaging as indicated.

The presence of paroxysmal opisthotonus and retrocollis as features of tardive dystonia may be confused with seizures. Dystonia may be differentiated from seizures based on patient history, including antipsychotic medication use, and clinical examination to assess the movement disorder phenomenology and detect other neurologic deficits.

Management

	• Prevention and early recognition are essential, as established tardive dystonia is often persistent and rarely remits despite drug withdrawal.
	• Gradual discontinuation of dopamine receptor-blocking agents is first-line management; clozapine should be considered when antipsychotic therapy must continue.
	• Pharmacologic treatment is largely symptomatic; most high-level evidence derives from tardive dyskinesia cohorts, and the benefit of VMAT2 inhibitors in dystonia-predominant disease remains uncertain.
	• Botulinum toxin is effective for focal or segmental forms, and globus pallius internus deep brain stimulation provides durable benefit in medically refractory cases.

The best treatment for tardive dystonia is prevention. Tardive dystonia may be refractory to treatment, and there is no permanent cure. Early detection is key. The main hope for remission lies in the discontinuation of antidopaminergic drugs, ideally with gradual tapering, as abrupt withdrawal may worsen symptoms. Although discontinuation is recommended as first-line management, evidence supporting reversal of tardive dystonia after drug withdrawal remains limited, and remission is uncommon (64; 33; 71). Development of tardive dystonia should prompt a critical reevaluation of the indication for ongoing dopamine receptor blocker therapy. For a nonpsychotic illness, alternative treatments should be sought. No evidence has been found to support switching from a typical antipsychotic to an atypical antipsychotic to reduce tardive symptomatology (03).

Even if the cause is removed, resolution of dystonia is rare. For those in whom the offending drug cannot be discontinued and for the majority whose dystonia will persist despite cessation of the drug, symptomatic therapy is instituted with the goal of decreasing pain and dystonic spasms. For those patients who must continue to receive antidopaminergic treatment, clozapine appears to be a neuroleptic drug that improves rather than exacerbates tardive dystonia (17; 71).

The FDA has approved two vesicular monoamine transporter 2 inhibitors (VMAT2 inhibitors) for use in the management of tardive dyskinesia: deutetrabenazine and valbenazine (33; 57). The recommended initial dose of deutetrabenazine is 6 mg daily. Deutetrabenazine is contraindicated in patients with depression and suicidality, neuroleptic malignant syndrome, parkinsonism, prolonged QT, hepatic impairment, or on MAO-I. Valbenazine may be started at a daily dose of 40 mg. Common side effects include somnolence, fatigue, and sedation. Valbenazine is contraindicated in patients with arrhythmias associated with QT prolongation or with drugs that can prolong the QT interval (03). Tetrabenazine is also a presynaptic dopamine depleter used in the management of tardive syndrome (34; 30; 30; 39; 31; 32; 33; 57).

Anticholinergic drugs, such as trihexyphenidyl and benztropine, are slightly less effective. Guidelines by the AAN describe clonazepam and ginkgo biloba (level B evidence), and amantadine and tetrabenazine (level C evidence) as probably effective in improving tardive syndromes (02; 03; 71). Occasionally, patients may benefit from clonazepam, baclofen, botulinum toxin, or a variety of other drugs (78; 32; 03). Botulinum toxin appears to be most effective in focal or segmental dystonias involving the cranial, cervical, or truncal regions (04; 13; 37; 03). The degree of improvement in many patients is modest. Olanzapine has been effective for up to 12 weeks in this role but is known to cause tardive dystonia de novo (26; 51; 12). Quetiapine has produced considerable symptomatic improvement in several cases, but long-term effects were not reported (61; 25). Dopamine receptor antagonists will suppress the involuntary movements but probably at the expense of contributing to the eventual worsening of the disorder. Nevertheless, these agents may be useful temporarily if immediate control of the situation is necessary.

Botulinum toxin represents an important symptomatic therapy, particularly for focal or segmental tardive dystonia involving cranial, cervical, or truncal regions. Open-label series demonstrate moderate to marked improvement in many patients, with response patterns comparable to primary dystonia, although higher doses may be required in some cervical forms (04; 13; 37; 71). The degree of improvement varies, and repeated injections are typically required.

Deep brain stimulation of the globus pallidus internus (GPi) has been shown to be a promising treatment option for patients with tardive dystonia refractory to medical therapy (48; 67; 21; 15; 73). A meta-analysis of 35 studies with a total of 117 cases demonstrated substantial improvement, which more often occurred gradually over time (52). The optimal response to deep brain stimulation for tardive dystonia is delayed by several weeks (72). Deep brain stimulation of the GPi has been shown to be effective in controlling tardive dystonia, and it provides improvement in the quality of life with changes in cognition (07; 77; 73; 59; 52). Clinical benefit appears durable. The overall improvement was noted to persist even at the last follow-up, averaging 25 months from surgery (52). In other studies, long-term improvement was described in ranges of 63 to 171 months following surgery (45; 47). Side effects of deep brain stimulation include transient worsening of psychiatric disease; surgical or device-related complications such as pain due to traction of cable connection, infection, and infarction during lead placement; and stimulation-related events (dysarthria, gait and balance impairment, visual scotomas, phosphenes, mania, etc.) (52; 45).

Individual cases have reported benefit to vitamin E, intrathecal baclofen, bilateral pallidotomy, and thalamotomy; however, data remain limited and insufficient to support routine use (16; 28; 03). Additional agents, including acetazolamide, thiamine, other vitamins, melatonin, baclofen, calcium channel blockers, levetiracetam, and buspirone, have been reported to improve tardive syndromes in isolated cases or small series, but evidence remains inadequate to support or refute their efficacy in tardive dystonia (02; 03). Mechanical devices that exploit sensory tricks may provide temporary symptomatic relief in selected patients (46).

Table 2. Treatment of Tardive Syndromes

Recommendations by level of evidence
A	• Deutetrabenazine and valbenazine are established as effective treatments of tardive dyskinesia and recommended for tardive syndromes.
B	• Clonazepam and ginkgo biloba probably improve tardive dyskinesia and should be considered as treatment. Supporting data derive primarily from tardive dyskinesia cohorts.
C	• Tetrabenazine might be considered as a treatment for tardive dyskinesia and has supportive open-label data in tardive dystonia. • Amantadine may be considered for tardive syndromes. • In patients with refractory tardive syndrome, GPi deep brain stimulation may be considered and is supported by prospective and long-term follow-up studies.
U	• Insufficient evidence to support or refute tardive syndrome treatment by withdrawing causative agents or switching from typical to atypical DRBA. • Insufficient evidence to support or refute the use of clozapine in patients with tardive dystonia, though case reports and expert reviews suggest potential benefit in tardive dystonia when ongoing antipsychotic therapy is required. • No evidence to support or refute the use of BoNT in patients with tardive syndrome, though open-label data support its use in focal and segmental tardive dystonia.
Contributed by (02; 03). A: multiple populations evaluated/data derived from multiple randomized controlled trials or meta-analysis. B: limited populations to valuated/data derived from a single randomized trial or nonrandomized trials. C: very limited populations, a valuated/only consensus opinion of experts, case studies, or standard of care. U: insufficient evidence.

Treatment of tardive syndromes

(Contributed by Dr. Lesly Giselle Aguilar Tabora.)

Outcomes

The mainstay of treatment remains removal of the offending agent combined with symptomatic treatment. Once tardive dystonia has developed, the dystonia is usually persistent. Epidemiologic and longitudinal studies reviewing outcomes describe 7.5% to 14% remission rate over periods ranging from 2.6 years to 8.5 years after discontinuation of the dopamine-blocking agent (06; 36; 42). More recent reviews continue to emphasize that spontaneous remission is uncommon and that established tardive dystonia is frequently refractory (03; 62; 71). Remission was more likely in younger patients, those with shorter duration of exposure to neuroleptics, and patients with less continual exposure to dopamine receptor agonists after the onset of tardive dystonia. Prolonged neuroleptic use for more than 10 years is more likely to have an increased risk of permanent dystonia.

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References

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Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Author

Lesly G Aguilar Tabora MD

Dr. Aguilar Tabora of Scripps Memorial Hospital La Jolla has no relevant financial relationships to disclose.
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Editor

Robert Fekete MD

Dr. Fekete of New York Medical College received consultation fees from Acadia Pharmaceutical, Acorda, Adamas/Supernus Pharmaceuticals, Amneal/Impax, Kyowa Kirin, Lundbeck Inc., Neurocrine Inc., and Teva Pharmaceutical, Inc.
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Former Authors

David E Riley MD
Joseph Jankovic MD

Patient Profile

Age range of presentation: 0 month to 65+ years
Sex preponderance: male>female
Heredity: none
Population groups selectively affected: Tardive dystonia selectively affects patients exposed to dopamine receptor blocking agents such as neuroleptics, metoclopramide, and certain antidepressants (eg, amoxapine).
Occupation groups selectively affected: none selectively affected

ICD & OMIM codes

ICD-10: Tardive dystonia: G24.09

Medication-induced acute dystonia: G24.02

Other and unspecified abnormal involuntary movements: R25.8
ICD-11: Drug-induced dystonia: 8A02.10

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Tardive dystonia

Associated Disorders

Idiopathic dystonia
Tardive akathisia
Tardive dyskinesia
Tardive myoclonus

Differential Diagnosis

Familial dystonia
Idiopathic cervical dystonia
Idiopathic oromandibular dystonia
Wilson disease
Huntington disease
- Psychogenic dystonia

Also Relevant

Acute drug-induced movement disorders
Blepharospasm
Botulinum toxin treatment of neurologic disorders
Cervical dystonia
Deep Brain Stimulation in Movement Disorders
- Dopa-responsive dystonia
- Idiopathic torsion dystonia
- Limb dystonia
- Oromandibular dystonia
- Peripheral dystonia
- Rating scales of movement disorders
- Tardive Dyskinesia

Tardive dystonia

Cite this article

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Table 1. Tardive Dystonia

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Diagnostic workup

Management

Table 2. Treatment of Tardive Syndromes

Outcomes

Special considerations

Pregnancy

Anesthesia

COVID-19

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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Questions or Comment?

Also in This Category

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Acute drug-induced movement disorders

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Oromandibular dystonia

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