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  • Updated 03.08.2024
  • Released 10.04.1993
  • Expires For CME 03.08.2027

Tardive dystonia



Tardive dystonia has been encompassed under the general term of tardive dyskinesia. Tardive dyskinesia, as well as the term extrapyramidal syndrome, has traditionally been used to describe a variety of delayed-onset, persistent motor and nonmotor syndromes associated with dopamine receptor blocker agent (DRBA) exposure. However, it is recommended to use the more specific designation of tardive dystonia when describing a tardive syndrome characterized primarily by dystonia.

Of the tardive syndromes that occur after exposure to dopamine receptor blocking agents, tardive dystonia occurs more rarely and tends to be refractory to medical treatment with infrequent occurrence of spontaneous remissions. In this article, the author provides updated definition and classification and reviews the basic principles of diagnosis and management. Although long-term use is the most common setting, tardive dystonia has been known to develop after brief exposure and can last only a matter of weeks. Deep brain pallidal stimulation has been used successfully and offers an alternative to those patients who have not shown response to medical therapy.

Key points

• Tardive dystonia occurs after exposure to dopaminergic blocking agents, usually after long-term exposure, and may not improve despite discontinuation of the offending agent.

• Tardive dystonia is often reported under the umbrella of tardive syndrome or extra pyramidal syndrome. Use of appropriate 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.

• Updates in the guidelines for pharmacological treatment of tardive syndromes include: deutetrabenazine and valbenazine (level A), clonazepam, and ginkgo biloba (level B). Use of deutetrabenazine and valbenazine has been granted FDA approval in management of tardive dyskinesia. The use of these medications in tardive dystonia specifically has not been clarified. Pallidal deep brain stimulation may be considered as treatment in 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 designation (eg, tardive dystonia, akathisia, etc.) to describe the tardive movement disorder improves earlier recognition of phenomenology and subsequent initiation of appropriate therapeutic options in management of tardive syndromes. When dystonia is the main feature of the tardive syndrome, it is considered to by tardive dystonia (22).

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