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A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy.

A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Research Abstract Details 

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  • A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Abstract Text:

    spiridon papapetropoulosSpiridon Papapetropoulos,jennifer friedmanJennifer Friedman,craig blackstoneCraig Blackstone,gary i kleinerGary I Kleiner,brian c bowenBrian C Bowen,carlos singerCarlos Singer,

    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.

    A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Publishing Authors By Initials

    s papapetropoulosS Papapetropoulos,j friedmanJ Friedman,c blackstoneC Blackstone,gi kleinerGI Kleiner,bc bowenBC Bowen,c singerC Singer,

    For similar abstracts research abstracts see: abstracts research

    PUBMED ID PMID:

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    A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Journal Published:

    PUBLICATION TYPE: Research Support, Non-U.S. Gov

    Journal: Movement disorders : official journal of the Movem

    VOLUME: 22

    Page Numbers: 1664-6

    Journal Abbreviation: Mov. Disord.

    ISSN: 0885-3185

    DAY: 15

    MONTH: Aug

    YEAR: 2007

    A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Information

    Number of References:

    LANGUAGE: eng

    NlmUniqueID: 8610688

    A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Keywords Mesh Terms:

    KEYWORDS: X-Linked Combined Immunodeficiency Disea

    MESH TERMS: pathology

    Chemical & Substance for Abstract: A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy. Information

    Substance Name: Immunologic Factors

    Registry Number: 0

    Grant and Affiliation Information for A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy.

    AFFILIATION: Department of Neurology, University of Miami, Miller School of Medicine, Miami, Florida 33136, and Massachusetts General Hospital, Boston, USA. spapapetropoulos@med.miami.edu

    Country: United States

    United States Research PublicationUnited States Research Publication

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    MEDLINETA: Mov Disord

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    A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy Related Publications

     

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