title: Autoimmune autonomic ganglionopathy in a pediatric patient presenting with acute encephalitis.
abstract: Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure. Autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR) are detected in 50% of AAG patients. We report the first pediatric case of AAG presenting with acute encephalitis. The patient was a 13-year-old boy who presented with orthostatic hypotension, followed by rapidly progressing disturbance of consciousness. Cerebrospinal fluid analysis revealed significant pleocytosis and increased neopterin concentration. Head MRI showed hyperintensities in bilateral caudate nuclei, putamen, hippocampus, and insula cortex. Severe autonomic dysfunctions such as severe orthostatic hypotension, bradycardia, dysuria, prolonged constipation and vomiting appeared. These symptoms were successfully controlled by repeated immunomodulating therapy with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin. Autoantibodies to the α3 subunit of gAChR were detected at neurological onset, but were undetectable five months later. This observation indicates that AAG should be suspected in patients manifesting acute encephalitis characterized by preceding and prolonged autonomic symptoms, and immunomodulating therapy from an early stage can be effective.
authors.affiliation: Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan. Electronic address: gpkichiro0926@yahoo.co.jp.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Kuki
authors.affiliation: Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Kawawaki
authors.affiliation: Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan.
authors.forename: Shin
authors.initials: S
authors.lastname: Okazaki
authors.affiliation: Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Hattori
authors.affiliation: Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan.
authors.forename: Asako
authors.initials: A
authors.lastname: Horino
authors.affiliation: Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors.forename: Osamu
authors.initials: O
authors.lastname: Higuchi
authors.affiliation: Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors.forename: Shunya
authors.initials: S
authors.lastname: Nakane
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D001323:Autoantibodies / Q000378:metabolism; D020274:Autoimmune Diseases of the Nervous System / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D001921:Brain / Q000000981:diagnostic imaging; D004660:Encephalitis / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D056747:Immunomodulation; D008279:Magnetic Resonance Imaging; D008297:Male; D011950:Receptors, Cholinergic / Q000276:immunology
chemical_list: D001323:Autoantibodies; D011950:Receptors, Cholinergic
keywords: Acute encephalitis; Autoantibody; Autonomic dysfunction; Children; Ganglionic nicotinic acetylcholine receptor; Orthostatic hypotension; Treatment