- Secondary Dystonia-Clinical Clues and Syndromic Associations
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Susanne A Schneider, Kailash P Bhatia
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J Mov Disord. 2009;2(2):58-63.
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DOI: https://doi.org/10.14802/jmd.09016
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Background:
Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes.
Purpose:
The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia.
Methods:
We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment.
Results:
Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded.
Conclusions:
When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.
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Citations
Citations to this article as recorded by  - Oromandibular dystonia – A systematic review
Udit Saraf, Mitesh Chandarana, KP Divya, Syam Krishnan
Annals of Indian Academy of Neurology.2022; 25(1): 26. CrossRef - Oromandibular Dystonia: A Clinical Examination of 2,020 Cases
Laura M. Scorr, Stewart A. Factor, Sahyli Perez Parra, Rachel Kaye, Randal C. Paniello, Scott A. Norris, Joel S. Perlmutter, Tobias Bäumer, Tatiana Usnich, Brian D. Berman, Marie Mailly, Emmanuel Roze, Marie Vidailhet, Joseph Jankovic, Mark S. LeDoux, Ric
Frontiers in Neurology.2021;[Epub] CrossRef - Clinical characteristics and diagnostic clues to Neurometabolic causes of dystonia
Chun Seng Phua, Kishore Raj Kumar, Stanley Levy
Journal of the Neurological Sciences.2020; 419: 117167. CrossRef - Botulinum toxin A in the treatment of dystonia
Lenka Hvizdošová, Pavel Otruba, Martin Nevrlý, Petr Kaňovský
Neurologie pro praxi.2020; 21(1): 21. CrossRef - Sensory trick in upper limb dystonia
Sabino Dagostino, Tommaso Ercoli, Angelo F. Gigante, Roberta Pellicciari, Laura Fadda, Giovanni Defazio
Parkinsonism & Related Disorders.2019; 63: 221. CrossRef - Dystonia in Patients With Spinocerebellar Ataxia Type 2
Vladana Markovic, Natasa T. Dragasevic‐Miskovic, Iva Stankovic, Igor Petrovic, Marina Svetel, Vladimir S. Kostić
Movement Disorders Clinical Practice.2016; 3(3): 292. CrossRef - Drug-induced movement disorders
Dénes Zádori, Gábor Veres, Levente Szalárdy, Péter Klivényi, László Vécsei
Expert Opinion on Drug Safety.2015; 14(6): 877. CrossRef - Novel GNAL mutation with intra-familial clinical heterogeneity: Expanding the phenotype
Miryam Carecchio, Celeste Panteghini, Chiara Reale, Chiara Barzaghi, Valentina Monti, Luigi Romito, Francesco Sasanelli, Barbara Garavaglia
Parkinsonism & Related Disorders.2015;[Epub] CrossRef - A CASE SUPPORTING THE ROLE OF THE CEREBELLUM IN DYSTONIA
Anisha Doshi, Jonathon Rohrer, Tom Warner
Journal of Neurology, Neurosurgery & Psychiatry.2013; 84(11): e2.61. CrossRef
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