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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.

Original publication




Journal article


Eur J Paediatr Neurol

Publication Date





396 - 398


Abdominal Pain, Adolescent, Anti-Inflammatory Agents, Antibodies, Autoimmune Diseases, Autonomic Nervous System Diseases, Azathioprine, Bridged Bicyclo Compounds, Heterocyclic, Female, Ganglia, Autonomic, Humans, Immunosuppressive Agents, Intestinal Obstruction, Nicotinic Agonists, Prednisolone, Pyridines, Radiopharmaceuticals, Receptors, Nicotinic