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JMD : Journal of Movement Disorders



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Review Article
Secondary Dystonia-Clinical Clues and Syndromic Associations
Susanne A Schneider, Kailash P Bhatia
J Mov Disord. 2009;2(2):58-63.
  • 18,360 View
  • 423 Download
  • 10 Crossref
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  
  • Polioencephalopathy in Eurasier dogs
    Faye Rawson, Matthias Christen, Jeremy Rose, Emilie Paran, Tosso Leeb, Angela Fadda
    Journal of Veterinary Internal Medicine.2024; 38(1): 277.     CrossRef
  • 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
Case Report
Generalized Chorea Induced by an Unilateral Anterior Cerebral Artery Territorial Infarction
Jae Hong Chang, Woo-Keun Seo, Moon-Ho Park, Jong-Mun Lee, Do-Young Kwon, Seong-Beom Koh
J Mov Disord. 2009;2(1):37-39.
  • 9,029 View
  • 85 Download
  • 3 Crossref
AbstractAbstract PDF

Generalized chorea caused by unilateral cerebral infarction has rarely been reported. A 58-year-old woman presented involuntary movement in her all extremities after acute cerebral infarction on her right anterior cerebral artery territory. The involuntary movements were diagnosed as generalized chorea. We didn’t find any cause of generalized chorea except the acute cerebral infarction. Here, we described the case of generalized chorea after unilateral cerebral infarction discussing the possible mechanisms.


Citations to this article as recorded by  
  • Cerebral infarction in centrum semiovale presenting with hemichorea: a case report and literature review
    Jingjing Yi, Lingru Zhang, Tao Zhang, Jianfeng Li, Yifan Zhang, Meining Zhou
    Frontiers in Neurology.2023;[Epub]     CrossRef
  • Movement Disorders Associated With Cerebral Artery Stenosis: A Nationwide Study
    Kye Won Park, Nari Choi, Eungseok Oh, Chul Hyoung Lyoo, Min Seok Baek, Han-Joon Kim, Dalla Yoo, Jee-Young Lee, Ji-Hyun Choi, Jae Hyeok Lee, Seong-Beom Koh, Young Hee Sung, Jin Whan Cho, Hui-Jun Yang, Jinse Park, Hae-Won Shin, Tae-Beom Ahn, Ho-Sung Ryu, So
    Frontiers in Neurology.2022;[Epub]     CrossRef
  • Acute Hemichorea as an Unusual Presentation of Internal Carotid Artery Stenosis
    Dong Wook Kim, Youngchai Ko, Sang Hyun Jang, Soo Jin Yoon, Gun-Sei Oh, Soo Joo Lee, Dong Joo Yun
    Journal of Movement Disorders.2013; 6(1): 17.     CrossRef

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