[Over 5 years follow-up of three cases of autoimmune autonomic ganglionopathy].

Autor: Kurono H; Department of Neurology, Saiseikai Kanagawaken Hospital., Torikai Y; Department of Neurology, Saiseikai Kanagawaken Hospital., Hara H; Department of Neurology, Saiseikai Kanagawaken Hospital.; Wellcare Hara Neurology Clinic., Okamura M; Department of Neurology, Saiseikai Kanagawaken Hospital., Kunimoto M; Kunimoto Life Support Clinic.
Jazyk: japonština
Zdroj: Rinsho shinkeigaku = Clinical neurology [Rinsho Shinkeigaku] 2022 Nov 26; Vol. 62 (11), pp. 860-864. Date of Electronic Publication: 2022 Oct 26.
DOI: 10.5692/clinicalneurol.cn-001793
Abstrakt: We report the clinical course of three cases of anti-ganglionic acetylcholine receptor (gAChR) antibody positive auto-immune autonomic ganglionopathy (AAG) that have been followed for over 5 years. In all three cases, the symptoms improved by acute treatment, but ultimately relapsed. The first case was a female in her 20s who had a chronic history of photophobia, constipation and amenorrhea. The symptoms almost disappeared by plasma exchange, and menstruation resumed. During the course, it relapsed once after a cold. There was no recurrence of AAG during the two pregnancies. The second case was a male in his 60s who visited a hospital for the acute onset of orthostatic hypotension (OH) and psychological symptoms (infantilization and psychogenic pseudosyncope). Although IVIg was effective, it recurred frequently and was difficult to treat. However, all the symptoms disappeared eight years after the onset without any particular reasons. The third case was a female in her 80s who had a chronic history of OH. Acute treatment was effective, but AAG recurred repeatedly. Additionally, it was difficult to judge relapse because of the residual sequelae. During the course, cerebral hemorrhage due to supine hypertension or short-time blood pressure variability and femoral neck fracture caused by OH occurred. She eventually became a wheelchair. This report is clinically important because there are few reports of long-term follow-up of AAG.
Databáze: MEDLINE