Isaacs syndrome with CASPR2 antibody: A series of three cases |
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| Affiliation: | 1. Department of Neurology, Huashan Hospital, Fudan University, Shanghai, China;2. Neurosciences Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford OX3 9DS, UK;3. Department of Neurology, Jing''an District Centre Hospital of Shanghai, Shanghai, China;4. Department of Clinical Electrophysiology, Institute of Neurology, Huashan Hospital, Fudan University, Shanghai, China;5. Department of Radiology, Putuo District People''s Hospital of Shanghai, Shanghai, China;1. Department of Neurology, Jing''an District Centre Hospital of Shanghai, China;2. Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China;3. Department of Thoracic Surgery, Huashan Hospital, Shanghai Medical College, Fudan University, China;4. Myasthenia Gravis Division, Yunnan Wenshan Hospital of Traditional Chinese Medicine, Yunnan, China;1. Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China;2. Department of Neurology, Jing''an District Centre Hospital of Shanghai, China;1. Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China;2. Department of Neurology, Jing''an District Centre Hospital of Shanghai, China;3. Institute of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China;4. Department and Institute of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China;1. Kidney Research Center and Department of Nephrology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan;2. Division of Nephrology, Department of Internal Medicine, Ministry of Health and Welfare, Tao Yuan General Hospital, Taoyuan, Taiwan |
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| Abstract: | Isaacs syndrome is a form of peripheral nerve hyperexcitability, characterized by spontaneous muscle twitching and stiffness. Some patients are reported to be positive for CASPR2 antibody that may be one of the pathogenic autoantibodies in Isaacs syndrome. We reported a series of three patients with Isaacs syndrome, including their clinical features, electrophysiologic findings, laboratory parameters and therapeutic responses. All the three patients were positive for CASPR2 antibodies examined on transfected human embryonic kidney 293 cells by indirect immunofluorescence method. One patient had invasive thymoma. Symptomatic treatment was not sufficient for them, while immunotherapies including corticosteroids, double filtration plasmapheresis and rituximab provided favorable outcomes. The titers of CASPR2 antibody decreased after immune modulating therapy in parallel to clinical improvements in two patients. |
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| Keywords: | Isaacs syndrome CASPR2 antibody Immunotherapy |
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