Document Detail


Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.
MedLine Citation:
PMID:  22130491     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Russell C Dale; Bethan Lang; Fabienne Brilot; Yann Polfrit; Grahame H H Smith; Melanie Wong
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-11-28
Journal Detail:
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:
Created Date:  2011-12-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9715169     Medline TA:  Eur J Paediatr Neurol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Crown Copyright © 2011. Published by Elsevier Ltd. All rights reserved.
Affiliation:
Neuroimmunology Group, Institute of Neuroscience and Muscle Research, Children's Hospital at Westmead, University of Sydney, Australia.
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