Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.
Eur J Paediatr Neurol
; 16(4): 396-8, 2012 Jul.
Article
in En
| MEDLINE
| ID: mdl-22130491
ABSTRACT
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Autoimmune Diseases
/
Autonomic Nervous System Diseases
/
Receptors, Nicotinic
/
Ganglia, Autonomic
/
Immunosuppressive Agents
/
Antibodies
Type of study:
Etiology_studies
Limits:
Adolescent
/
Female
/
Humans
Language:
En
Journal:
Eur J Paediatr Neurol
Journal subject:
NEUROLOGIA
/
PEDIATRIA
Year:
2012
Document type:
Article
Affiliation country:
Australia