title: Autoimmune autonomic ganglionopathy with reversible cognitive impairment.
abstract: 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.
  
  
  METHODS
  Prospective cohort.
  
  
  METHODS
  Academic medical center.
  
  
  METHODS
  Three patients with AAG underwent neuropsychological testing before and after cycles of plasma exchange in both the seated and standing positions.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, Beth Israel Deaconess Medical Center, 1 Deaconess Rd., Boston, MA 02215, USA.
authors.forename: Christopher H
authors.initials: CH
authors.lastname: Gibbons
authors.forename: Justin
authors.initials: J
authors.lastname: Centi
authors.forename: Steven
authors.initials: S
authors.lastname: Vernino
authors.forename: Roy
authors.initials: R
authors.lastname: Freeman
mesh_terms: D000704:Analysis of Variance; D000906:Antibodies / Q000097:blood; D001288:Attention / Q000502:physiology; D020274:Autoimmune Diseases of the Nervous System / Q000097:blood / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D001794:Blood Pressure / Q000502:physiology; D060825:Cognitive Dysfunction / Q000097:blood / Q000209:etiology* / Q000628:therapy; D015331:Cohort Studies; D005260:Female; D005725:Ganglia, Autonomic / Q000473:pathology*; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D007024:Hypotension, Orthostatic / Q000150:complications* / Q000628:therapy; D008875:Middle Aged; D009483:Neuropsychological Tests; D010951:Plasma Exchange / Q000379:methods; D011597:Psychomotor Performance / Q000502:physiology; D011950:Receptors, Cholinergic / Q000276:immunology
chemical_list: D000906:Antibodies; D011950:Receptors, Cholinergic