Document Detail

A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy.
MedLine Citation:
PMID:  17588239     Owner:  NLM     Status:  MEDLINE    
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by a mutation in the Bruton agammaglobulinemia tyrosine kinase gene that results in severe B-cell deficiency. So far, neurological complications of XLA have been primarily related to acute and/or chronic central nervous system enteroviral infections. In the last few years a progressive neurodegenerative syndrome of unknown etiology has been described in XLA patients. We describe and present a video of an XLA patient who developed a fatal dementing, dystonia-Parkinsonism syndrome 14 years into his immune disorder. Physician awareness of this rare syndrome may lead to its better characterization and management.
Spiridon Papapetropoulos; Jennifer Friedman; Craig Blackstone; Gary I Kleiner; Brian C Bowen; Carlos Singer
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Movement disorders : official journal of the Movement Disorder Society     Volume:  22     ISSN:  0885-3185     ISO Abbreviation:  Mov. Disord.     Publication Date:  2007 Aug 
Date Detail:
Created Date:  2007-09-05     Completed Date:  2007-11-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8610688     Medline TA:  Mov Disord     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1664-6     Citation Subset:  IM    
Copyright Information:
Copyright (c) 2007 Movement Disorder Society.
Department of Neurology, University of Miami, Miller School of Medicine, Miami, Florida 33136, and Massachusetts General Hospital, Boston, USA.
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MeSH Terms
Brain / pathology
Dystonic Disorders / complications,  drug therapy*,  pathology
Immunoglobulins, Intravenous / therapeutic use*
Immunologic Factors / therapeutic use*
Magnetic Resonance Imaging / methods
Parkinsonian Disorders / complications,  drug therapy*,  pathology
X-Linked Combined Immunodeficiency Diseases / complications,  drug therapy*,  pathology
Reg. No./Substance:
0/Immunoglobulins, Intravenous; 0/Immunologic Factors

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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