1. Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM)-like Symptoms Associated with Anti-ganglionic Acetylcholine Receptor Antibodies
- Author
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Yuki Kitazaki, Shunya Nakane, Masamichi Ikawa, Tadanori Hamano, Yasunari Nakamoto, Tomoko Kamisawa, and Toru Kishitani
- Subjects
Myoclonus ,autoimmune autonomic ganglionopathy ,Encephalomyelitis ,Case Report ,Autoimmune autonomic ganglionopathy ,030204 cardiovascular system & hematology ,progressive encephalomyelitis with rigidity and myoclonus ,03 medical and health sciences ,0302 clinical medicine ,Internal Medicine ,medicine ,Humans ,Receptors, Cholinergic ,Pure autonomic failure ,Autoantibodies ,Acetylcholine receptor ,Autoimmune encephalitis ,autonomic failure ,business.industry ,Dysautonomia ,General Medicine ,Middle Aged ,medicine.disease ,autoimmune encephalitis ,Muscle Rigidity ,Nicotinic agonist ,Immunology ,anti-ganglionic acetylcholine receptor antibodies ,Female ,030211 gastroenterology & hepatology ,medicine.symptom ,business - Abstract
This report describes a 59-year-old woman who presented with progressive encephalomyelitis with rigidity and myoclonus (PERM)-like symptoms and severe dysautonomia, including orthostatic hypotension, sinus bradycardia, dysuria, and prolonged constipation. Her neurological symptoms improved after immunotherapy, but the dysautonomia persisted. Anti-ganglionic acetylcholine receptor (gAChR) α3 subunit antibodies, which are frequently identified in patients with autoimmune autonomic ganglionopathy, were detected in the pre-treatment serum. The central distribution of the nicotinic acetylcholine receptors, a target of anti-gAChR antibodies, and immunotherapeutic efficacy observed in this case indicate that anti-gAChR α3 subunit antibodies are associated with the PERM-like features accompanied by autonomic manifestations.
- Published
- 2021