Overview
Autoimmune autonomic ganglionopathy (AAG) is a rare disorder characterized by antibody-mediated impairment of autonomic ganglia function, leading to severe autonomic failure including orthostatic hypotension and cognitive dysfunction 1.Diagnosis
Presence of orthostatic hypotension and other autonomic symptoms 12
Elevated titers of nicotinic acetylcholine receptor (nAChR) antibodies 12
Neuropsychological testing to assess cognitive domains such as executive function, sustained attention, and working memory 1
Autonomic function tests including plasma norepinephrine concentration and quantitative sweat testing 2Management
First-line treatments: Plasma exchange to reduce antibody titers 1
Adjunctive treatments: Rituximab for sustained reduction of nAChR antibody titers, often requiring maintenance therapy 2
Symptomatic management for orthostatic hypotension and other autonomic symptoms as needed 2Special Populations
Comorbidities: Patients with prior lymphoma may require tailored rituximab dosing and monitoring 2Key Recommendations
Initiate plasma exchange to address elevated antibody titers and improve autonomic symptoms (Evidence: Moderate) 1
Consider rituximab as a maintenance therapy to sustain reduction in nAChR antibody titers and symptom improvement (Evidence: Weak) 2
Regularly assess cognitive function through neuropsychological testing, especially in domains affected by orthostatic hypotension (Evidence: Moderate) 1References
1 Gibbons CH, Centi J, Vernino S, Freeman R. Autoimmune autonomic ganglionopathy with reversible cognitive impairment. Archives of neurology 2012. link
2 Hollenbeck R, Black BK, Peltier AC, Biaggioni I, Robertson D, Winton EF et al.. Long-term treatment with rituximab of autoimmune autonomic ganglionopathy in a patient with lymphoma. Archives of neurology 2011. link