• Yin-Yin Xie, Hong-Mei Meng, Feng-Xiao Zhang, Buajieerguli Maimaiti, Ting Jiang, and Yu Yang.
• Department of Neurology.
• Medicine (Baltimore). 2021 Jan 22; 100 (3): e24312.

RationaleStiff-person syndrome (SPS) is a rare neurological immune disorder characterized by progressive axial and proximal limb muscle rigidity, stiffness, and painful muscle spasms. Amphiphysin antibodies are positive in approximately 5% of SPS patients. To date, there have been no relevant reports on involuntary movement in cases of SPS with amphiphysin antibodies.Patient ConcernsWe describe the case of a 69-year-old man with a 2-year history of progressive stiffness in the neck, bilateral shoulders, and chest muscles, and a more-than-a-year history of dyspnea accompanied by mandibular involuntary movement. The patient was a vegetarian and had good health in the past. The family's medical history was unremarkable.DiagnosesHe was diagnosed with SPS based on the progressive muscle stiffness, the amphiphysin antibody seropositivity, the continuous motor activity on electromyography, and the effective treatment with benzodiazepines.InterventionsThe patient was orally administered clonazepam and baclofen, and corticosteroid IV followed by prednisone orally.OutcomesIn the hospital, after treatment with methylprednisolone, clonazepam, and baclofen, the patient's rigidity, stiffness, and dyspnea significantly improved. The involuntary movement of the mandible persisted throughout the treatment process. Currently, under oral treatment with baclofen and clonazepam, the patient's symptoms of muscle stiffness and dyspnea exist, and follow-up is continued.LessonsWe report a rare and novel case of involuntary movement in SPS with amphiphysin antibodies. The present report explores the relationship between SPS and involuntary movement and expands the spectrum of clinical manifestations of SPS.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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