| Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies. | |
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MedLine Citation:
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PMID: 22130491 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Autoimmune autonomic ganglionopathy (AAG) is a rare disorder that presents with pandysautonomia typically in middle age and elderly patients. AAG is typically associated with serum autoantibodies that bind to the alpha-3 subunit of the ganglionic acetylcholine receptor (α3-AChR Ab). We report a 13 year old girl who presented with gut pseudo-obstruction, bladder dysfunction and dilated pupils unresponsive to pilocarpine. She had positive α3-AChR Ab plus other autoantibodies suggesting an autoimmune diathesis. Our patient was initially resistant to steroid therapy but responded to the addition of azathioprine resulting in a near complete clinical remission. We conclude that pandysautonomia associated with α3-AChR Ab can occur in children and has multi-organ involvement. |
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Authors:
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Russell C Dale; Bethan Lang; Fabienne Brilot; Yann Polfrit; Grahame H H Smith; Melanie Wong |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-11-28 |
Journal Detail:
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Title: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society Volume: - ISSN: 1532-2130 ISO Abbreviation: - Publication Date: 2011 Nov |
Date Detail:
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Created Date: 2011-12-1 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9715169 Medline TA: Eur J Paediatr Neurol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Crown Copyright © 2011. Published by Elsevier Ltd. All rights reserved. |
Affiliation:
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Neuroimmunology Group, Institute of Neuroscience and Muscle Research, Children's Hospital at Westmead, University of Sydney, Australia. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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