Cookies on this website
We use cookies to ensure that we give you the best experience on our website. If you click 'Continue' we'll assume that you are happy to receive all cookies and you won't see this message again. Click 'Find out more' for information on how to change your cookie settings.

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

DOI

10.1016/j.ejpn.2011.11.001

Type

Journal article

Journal

Eur J Paediatr Neurol

Publication Date

07/2012

Volume

16

Pages

396 - 398

Keywords

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