Document Detail

Isolated sympathetic failure with autoimmune autonomic ganglionopathy.
MedLine Citation:
PMID:  20837310     Owner:  NLM     Status:  MEDLINE    
A 16-year-old boy had a gradual onset of post-exercise myalgia with progressive fatigue and dizziness. He had bradycardia (37 beats/minute) with low supine and normal standing norepinephrine levels (56 and 311 pg/mL, respectively). He had absent sympathetically mediated vasoconstrictor responses during Valsalva maneuver testing. Circulating ganglionic acetylcholine receptor antibodies were identified. Response was gradual to treatment with intravenous immunoglobulin combined with aggressive symptomatic interventions (permanent pacemaker implantation and treatment with pyridostigmine, midodrine, and modafinil). After the intravenous immunoglobulin treatment, his autoantibody levels decreased and the autonomic abnormalities resolved. After a reconditioning exercise program and eventually undetectable antibody titers, he achieved complete recovery. The patient continued to do well after his pacemaker was removed and his medications were discontinued. Thus, severe isolated sympathetic nervous system failure can occur in adolescents with autoimmune autonomic ganglionopathy, and multifaceted treatment can be effective.
Philip R Fischer; Paola Sandroni; Sean J Pittock; Co-burn J Porter; Lenora M Lehwald; Satish R Raj
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric neurology     Volume:  43     ISSN:  1873-5150     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-14     Completed Date:  2011-01-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  287-90     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier Inc. All rights reserved.
Division of General Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
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MeSH Terms
Autoantibodies / immunology*
Autonomic Nervous System Diseases / diagnosis,  immunology*,  therapy
Immunoglobulins, Intravenous / therapeutic use
Midodrine / therapeutic use
Norepinephrine / therapeutic use
Pacemaker, Artificial
Receptors, Cholinergic / immunology*
Sympathetic Nervous System / immunology*
Treatment Outcome
Reg. No./Substance:
0/Autoantibodies; 0/Immunoglobulins, Intravenous; 0/Receptors, Cholinergic; 42794-76-3/Midodrine; 51-41-2/Norepinephrine

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