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Susanne A Schneider 1 Article
Secondary Dystonia-Clinical Clues and Syndromic Associations
Susanne A Schneider, Kailash P Bhatia
J Mov Disord. 2009;2(2):58-63.
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  • 9 Citations
AbstractAbstract PDF

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.


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.


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.


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.


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.


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
    Anisha Doshi, Jonathon Rohrer, Tom Warner
    Journal of Neurology, Neurosurgery & Psychiatry.2013; 84(11): e2.61.     CrossRef

JMD : Journal of Movement Disorders