Secondary Dystonia-Clinical Clues and Syndromic Associations
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 s...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Korean Movement Disorder Society
2009-10-01
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| Series: | Journal of Movement Disorders |
| Subjects: | |
| Online Access: | http://e-jmd.org/upload/jmd-2-2-58-2.pdf |
| _version_ | 1797760913714970624 |
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| author | Susanne A Schneider Kailash P Bhatia |
| author_facet | Susanne A Schneider Kailash P Bhatia |
| author_sort | Susanne A Schneider |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-12T19:05:23Z |
| format | Article |
| id | doaj.art-8ef8e1bb4aa14ab99e43561553cbdfc1 |
| institution | Directory Open Access Journal |
| issn | 2005-940X 2093-4939 |
| language | English |
| last_indexed | 2024-03-12T19:05:23Z |
| publishDate | 2009-10-01 |
| publisher | Korean Movement Disorder Society |
| record_format | Article |
| series | Journal of Movement Disorders |
| spelling | doaj.art-8ef8e1bb4aa14ab99e43561553cbdfc12023-08-02T06:17:01ZengKorean Movement Disorder SocietyJournal of Movement Disorders2005-940X2093-49392009-10-0122586310.14802/jmd.0901657Secondary Dystonia-Clinical Clues and Syndromic AssociationsSusanne A SchneiderKailash P BhatiaBackground: 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.http://e-jmd.org/upload/jmd-2-2-58-2.pdfChoreaBrain infarctionAnterior cerebral artery |
| spellingShingle | Susanne A Schneider Kailash P Bhatia Secondary Dystonia-Clinical Clues and Syndromic Associations Journal of Movement Disorders Chorea Brain infarction Anterior cerebral artery |
| title | Secondary Dystonia-Clinical Clues and Syndromic Associations |
| title_full | Secondary Dystonia-Clinical Clues and Syndromic Associations |
| title_fullStr | Secondary Dystonia-Clinical Clues and Syndromic Associations |
| title_full_unstemmed | Secondary Dystonia-Clinical Clues and Syndromic Associations |
| title_short | Secondary Dystonia-Clinical Clues and Syndromic Associations |
| title_sort | secondary dystonia clinical clues and syndromic associations |
| topic | Chorea Brain infarction Anterior cerebral artery |
| url | http://e-jmd.org/upload/jmd-2-2-58-2.pdf |
| work_keys_str_mv | AT susanneaschneider secondarydystoniaclinicalcluesandsyndromicassociations AT kailashpbhatia secondarydystoniaclinicalcluesandsyndromicassociations |