Tardive dystonia

Lesly G Aguilar Tabora MD (Dr. Aguilar Tabora of Sharp Grossmont Hospital has no relevant financial relationships to disclose.)
Joseph Jankovic MD, editor. (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released October 4, 1993; last updated February 21, 2017; expires February 21, 2020

Overview

Of the tardive syndromes that occur after exposure to dopamine receptor blocking agents, tardive dystonia occurs more rarely and is 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.

 

• 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, including discontinuation of the dopaminergic-blocking agent, is often symptomatic with the goal of decreasing pain and dystonic spasms.

 

• Deep brain stimulation has emerged as a therapeutic modality, with reports of rapid response, consistent amelioration of tardive dystonia, and improvement in quality of life.

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 (Keegan and Rajput 1973). 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 3 months of a course of neuroleptic treatment (Burke et al 1982).

Tardive dyskinesia is a term used to describe multiple phenomenologies occurring from exposure to dopamine blocking agents and includes tardive dystonia (Waln and Jankovic 2013). 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 2 axes: clinical characteristics and etiology (Albanese at al 2013). The latter being acquired and due to a known specific cause.

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