Rinsho Shinkeigaku (Clinical Neurology)


Autoimmune autonomic ganglionopathy

Shunya Nakane, M.D., Ph.D.1)

1)Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital

Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder of widespread autonomic failure. Approximately half of the patients with AAG have the autoantibodies against the neuronal nicotinic acetylcholine receptor (AChR) in autonomic ganglia. These ganglionic AChR antibodies have the potential to mediate the synaptic transmission in sympathetic, parasympathetic, and enteric ganglia. Therefore, seropositive AAG patients exhibit various autonomic symptoms. Extra-autonomic manifestations (coexistence with brain involvement, sensory disturbance, endocrine disorders, autoimmune diseases and tumors) are present in many patients with AAG. The nicotinic AChRs comprise a family of abundantly expressed ligand-gated cation channels found throughout the central and peripheral nervous systems. Moreover, limited manifestations of autoimmune dysautonomia including autoimmune gastrointestinal dysmotility are newly recognized clinical entity. Although combined immunomodulatory therapy is beneficial for almost all patients with AAG, several case reports of some AAG patients with small benefit exist. This review focuses on the recent progress in the clinical approaches of AAG and its related disorders involving the role of autoantibodies and clinical practice.
Full Text of this Article in Japanese PDF (1300K)

(CLINICA NEUROL, 59: 783|790, 2019)
key words: autoimmune autonomic ganglionopathy (AAG), acetylcholine receptor (AChR), anti-ganglionic acetylcholine receptor (gAChR) antibody, autonomic dysfunction, autoimmune gastrointestinal dysmotility (AGID)

(Received: 14-Aug-19)