Document Detail


Rare causes of dystonia parkinsonism.
MedLine Citation:
PMID:  20694531     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The list of genetic causes of syndromes of dystonia parkinsonism grows constantly. As a consequence, the diagnosis becomes more and more challenging for the clinician. Here, we summarize the important causes of dystonia parkinsonism including autosomal-dominant, recessive, and x-linked forms. We cover dopa-responsive dystonia, Wilson's disease, Parkin-, PINK1-, and DJ-1-associated parkinsonism (PARK2, 6, and 7), x-linked dystonia-parkinsonism/Lubag (DYT3), rapid-onset dystonia-parkinsonism (DYT12) and DYT16 dystonia, the syndromes of Neurodegeneration with Brain Iron Accumulation (NBIA) including pantothenate kinase (PANK2)- and PLA2G6 (PARK14)-associated neurodegeneration, neuroferritinopathy, Kufor-Rakeb disease (PARK9) and the recently described SENDA syndrome; FBXO7-associated neurodegeneration (PARK15), autosomal-recessive spastic paraplegia with a thin corpus callosum (SPG11), and dystonia parkinsonism due to mutations in the SLC6A3 gene encoding the dopamine transporter. They have in common that in all these syndromes there may be a combination of dystonic and parkinsonian features, which may be complicated by pyramidal tract involvement. The aim of this review is to familiarize the clinician with the phenotypes of these disorders.
Authors:
Susanne A Schneider; Kailash P Bhatia
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Current neurology and neuroscience reports     Volume:  10     ISSN:  1534-6293     ISO Abbreviation:  Curr Neurol Neurosci Rep     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-09-21     Completed Date:  2011-01-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100931790     Medline TA:  Curr Neurol Neurosci Rep     Country:  United States    
Other Details:
Languages:  eng     Pagination:  431-9     Citation Subset:  IM    
Affiliation:
Sobell Department for Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, UK. susanne.schneider@neuro.uni-luebeck.de
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MeSH Terms
Descriptor/Qualifier:
Diagnosis, Differential
Dystonic Disorders / complications,  etiology*,  genetics
Humans
Parkinsonian Disorders / complications,  etiology*,  genetics
Phenotype

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