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Chapter 37 - Secondary Dystonia

from Section 3: - Hyperkinetic Movement Disorders

Published online by Cambridge University Press:  07 January 2025

By
Vassilena Iankova  and
Susanne A. Schneider
Edited by
Erik Ch. Wolters  and
Christian R. Baumann
Erik Ch. Wolters
Affiliation:
Universität Zürich
Christian R. Baumann
Affiliation:
Universität Zürich
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Summary

Secondary dystonia comprises a group of diverse dystonia syndromes, including hereditary diseases with a clinical phenotype exceeding dystonia as well as acquired dystonia. The first step in the classification of dystonic symptoms should be according to its clinical characteristics – age at onset, body distribution, temporal pattern and associated features. Limb dystonia in adulthood, as well as craniocervical dystonia presentation in childhood and young adolescence, point away from primary dystonia causes. Associated clinical features such as oculomotor disturbances, parkinsonism, cognitive and neuropsychiatric symptoms, and systemic involvement can be instructive for identifying the underlying dystonia syndrome. The diagnostic workup in patients with secondary dystonia depends on the suspected dystonia syndrome and can include laboratory tests in serum and cerebrospinal fluid (CSF), magnetic resonance (MR) imaging, genetic testing, etc. It is important to identify potential treatable causes, e.g., dopa-responsive dystonia, Wilson’s disease, Niemann–Pick Type C, infectious and autoimmune diseases, etc. Symptomatic treatments are also available.

Keywords

dystoniasecondary dystoniadopa-responsive dystoniaWilson’s diseaseacquired dystoniabotulinum toxindeep brain stimulation
Type
Chapter
Information
International Compendium of Movement Disorders , pp. 445 - 461
Publisher: Cambridge University Press
Print publication year: 2025

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