Rinsho Shinkeigaku (Clinical Neurology)

Case Report

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

Hiroko Kurono, M.D.1), Yuko Torikai, M.D.1), Hajime Hara, M.D., Ph.D.1)2), Masaya Okamura, M.D.1) and Masanari Kunimoto, M.D., Ph.D.3)

1) Department of Neurology, Saiseikai Kanagawaken Hospital
2) Wellcare Hara Neurology Clinic
3) Kunimoto Life Support Clinic

We report the clinical course of three cases of anti-ganglionic acetylcholine receptor (gAChR) antibody positive autoimmune 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.
Full Text of this Article in Japanese PDF (945K)

(CLINICA NEUROL, 62: 860－864, 2022)
key words: autoimmune autonomic ganglionopathy, autonomic neuropathy, long term follow-up

(Received: 18-Jul-22)