Document Detail

Secondary dystonia-clinical clues and syndromic associations.
MedLine Citation:
PMID:  24868358     Owner:  NLM     Status:  PubMed-not-MEDLINE    
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.
Susanne A Schneider; Kailash P Bhatia
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Publication Detail:
Type:  Journal Article; Review     Date:  2009-10-30
Journal Detail:
Title:  Journal of movement disorders     Volume:  2     ISSN:  2005-940X     ISO Abbreviation:  J Mov Disord     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2014-05-28     Completed Date:  2014-06-24     Revised Date:  2014-06-24    
Medline Journal Info:
Nlm Unique ID:  101527867     Medline TA:  J Mov Disord     Country:  Korea (South)    
Other Details:
Languages:  eng     Pagination:  58-63     Citation Subset:  -    
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