RT Journal
A1 Gibbons CH, Centi J, Vernino S, Freeman R
T1 AUtoimmune autonomic ganglionopathy with reversible cognitive impairment
JF Archives of Neurology
JO Archives of Neurology
YR 2012
FD April 1
VO 69
IS 4
SP 461
OP 466
DO 10.1001/archneurol.2011.2372
UL http://dx.doi.org/10.1001/archneurol.2011.2372
AB Background 
                  Autoimmune autonomic ganglionopathy (AAG) is a rare disorder of antibody-mediated impaired transmission across the autonomic ganglia resulting in severe autonomic failure. Some patients with AAG report cognitive impairment of unclear etiology despite treatment of autonomic symptoms.Objective 
                  To investigate the relationship between orthostatic hypotension, antibody titers, and cognitive impairment in patients with AAG.Design 
                  Prospective cohort.Setting 
                  Academic medical center.Participants 
                  Three patients with AAG underwent neuropsychological testing before and after cycles of plasma exchange in both the seated and standing positions.Main Outcome Measures 
                  Patients' responses to neuropsychological tests were measured by percentage change from baseline in the seated and standing positions before and after plasma exchange to determine the effects of orthostatic hypotension and antibody titers on cognition.Results 
                  Orthostatic hypotension and elevated antibody titer were associated independently with neuropsychological impairment (P < .05), particularly in domains of executive function, sustained attention, and working memory. Cognitive dysfunction improved, even in the seated normotensive position, after plasmapheresis and consequent reduction in antibody levels.Conclusion 
                  Reversible cognitive impairment is independently associated with both orthostatic hypotension and elevated nicotinic acetylcholine receptor autoantibodies, thereby expanding the clinical spectrum of autonomic ganglionopathy and, in so doing, providing an additional treatable cause of cognitive impairment.