AI Article Synopsis
- Autoimmune autonomic ganglionopathy (AAG) is a rare condition mainly affecting older adults, leading to widespread autonomic dysfunction.
- A case study highlights a 13-year-old girl experiencing severe symptoms such as gut issues, bladder problems, and unresponsive dilated pupils, with positive autoantibodies indicating an autoimmune condition.
- The girl initially did not improve with steroids but showed significant recovery after adding azathioprine to her treatment, showcasing that AAG can affect children and impact multiple organ systems.
Article 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.
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| http://dx.doi.org/10.1016/j.ejpn.2011.11.001 | DOI Listing |
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Neuroimmunology Group, Institute of Neuroscience and Muscle Research, Children's Hospital at Westmead, University of Sydney, Australia.
Article Synopsis
- Autoimmune autonomic ganglionopathy (AAG) is a rare condition mainly affecting older adults, leading to widespread autonomic dysfunction.
- A case study highlights a 13-year-old girl experiencing severe symptoms such as gut issues, bladder problems, and unresponsive dilated pupils, with positive autoantibodies indicating an autoimmune condition.
- The girl initially did not improve with steroids but showed significant recovery after adding azathioprine to her treatment, showcasing that AAG can affect children and impact multiple organ systems.
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