Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of chronic postural tachycardia syndrome with positive anti-ganglionic acetylcholine receptor (gAChR) antibody

Yuya Goto, M.D.1), Yoko Sunami, M.D.1), Keizo Sugaya, M.D., Ph.D.1), Shunya Nakane, M.D., Ph.D.2) and Kazushi Takahashi, M.D., Ph.D.1)

1) Department of Neurology, Tokyo Metropolitan Neurological Hospital
2) Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital

Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance characterized by symptoms such as lightheadedness, fainting, and brain fog that occur with a rapid elevation in heart rate when standing up from a reclining position. The etiology of POTS has yet to be established. However, a growing body of evidence suggests that POTS may be an autoimmune disorder such as autoimmune autonomic ganglionopathy, an acquired, immune-mediated form of diffuse autonomic failure. Many patients have serum antibodies that bind to the ganglionic acetylcholine receptors (gAChRs) in the autonomic ganglia. Herein, we describe a 39-year-old female patient with an eight-year history of orthostatic intolerance. POTS was diagnosed based on the findings of a head-up tilt test, in which a rapid increase in the patientfs heart rate from 58 bpm in the lying position to 117 bpm in the upright position without orthostatic hypotension was observed. The POTS symptoms were refractory to various medications except for pyridostigmine bromide, which resulted in a partial resolution of her symptoms. Her serum was found to be strongly positive for anti-gAChR (4 subunit) autoantibody (2.162 A.I., normal range: below 1.0). Based on these findings, a limited form of autoimmune POTS was diagnosed. After obtaining written informed consent, she was treated with intravenous immunoglobulin (IVIg) 400 mg/kg/day for five days, which led to clinical improvement by reducing her heart rate increase in the upright position. She was able to return to work with IVIg treatment at regular intervals. Our case provides further evidence of a potential autoimmune pathogenesis for POTS. Aggressive immunotherapy may be effective for POTS even in chronic cases.
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(CLINICA NEUROL, 61: 547|551, 2021)
key words: postural tachycardia syndrome, anti-ganglionic acetylcholine receptor antibody, autoimmune autonomic ganglionopathy

(Received: 4-Feb-21)