MovDisordV3, Main, Merge, bibRecord, 003F73

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

Identifieur interne : 003F73 ( Main/Merge ); précédent : 003F72; suivant : 003F74

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

Auteurs : Spiridon Papapetropoulos [États-Unis] ; Jennifer Friedman [États-Unis] ; Craig Blackstone [États-Unis] ; Gary I. Kleiner [États-Unis] ; Brian C. Bowen [États-Unis] ; Carlos Singer [États-Unis]

Source :

Movement Disorders [ 0885-3185 ] ; 2007-08-15.

RBID : ISTEX:0B712CF686756E31717AA32DCDC2C6AD9AEA9A53

English descriptors

KwdEn :
- Adolescent, Brain (pathology), Dystonic Disorders (complications), Dystonic Disorders (drug therapy), Dystonic Disorders (pathology), Humans, IVIG, Immunoglobulins, Intravenous (therapeutic use), Immunologic Factors (therapeutic use), Magnetic Resonance Imaging (methods), Male, Parkinsonian Disorders (complications), Parkinsonian Disorders (drug therapy), Parkinsonian Disorders (pathology), X-Linked Combined Immunodeficiency Diseases (complications), X-Linked Combined Immunodeficiency Diseases (drug therapy), X-Linked Combined Immunodeficiency Diseases (pathology), X‐linked agammaglobulinemia, dystonia, extrapyramidal syndrome.
MESH :
- chemical , therapeutic use : Immunoglobulins, Intravenous, Immunologic Factors.
- complications : Dystonic Disorders, Parkinsonian Disorders, X-Linked Combined Immunodeficiency Diseases.
- drug therapy : Dystonic Disorders, Parkinsonian Disorders, X-Linked Combined Immunodeficiency Diseases.
- methods : Magnetic Resonance Imaging.
- pathology : Brain, Dystonic Disorders, Parkinsonian Disorders, X-Linked Combined Immunodeficiency Diseases.
- Adolescent, Humans, Male.

Abstract

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. © 2007 Movement Disorder Society

Url:

https://api.istex.fr/document/0B712CF686756E31717AA32DCDC2C6AD9AEA9A53/fulltext/pdf

DOI: 10.1002/mds.21631

Links to Exploration step

ISTEX:0B712CF686756E31717AA32DCDC2C6AD9AEA9A53

Le document en format XML

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<term>Parkinsonian Disorders (complications)</term>
<term>Parkinsonian Disorders (drug therapy)</term>
<term>Parkinsonian Disorders (pathology)</term>
<term>X-Linked Combined Immunodeficiency Diseases (complications)</term>
<term>X-Linked Combined Immunodeficiency Diseases (drug therapy)</term>
<term>X-Linked Combined Immunodeficiency Diseases (pathology)</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Immunoglobulins, Intravenous</term>
<term>Immunologic Factors</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Dystonic Disorders</term>
<term>Parkinsonian Disorders</term>
<term>X-Linked Combined Immunodeficiency Diseases</term>
</keywords>
<keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Dystonic Disorders</term>
<term>Parkinsonian Disorders</term>
<term>X-Linked Combined Immunodeficiency Diseases</term>
</keywords>
<keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Magnetic Resonance Imaging</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term>
<term>Dystonic Disorders</term>
<term>Parkinsonian Disorders</term>
<term>X-Linked Combined Immunodeficiency Diseases</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Humans</term>
<term>Male</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">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.</div>
</front>
</TEI>
</PubMed>
</double>
</record>

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Movement Disorders (revue)

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

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