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Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis Current Status

오신중
대한신경과학회 (Korean Neurological Association)|2009.06|Journal of Clinical Neurology|제5권 제2호|pp. 53-64

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참고문헌

키워드

myasthenia gravisseronegative myasthenia gravismuscle-specific tyrosine-kinase-antibody

초록

Muscle-specific tyrosine-kinase-antibody-positive myasthenia gravis (MuSK-MG) has emerged as a distinct entity since 2001. This disease has been reported worldwide, but with varying rates among patients with generalized acetylcholine-receptor-antibody-negative MG. MuSK-MG was detected in approximately 37% of generalized acetylcholine receptor antibody-negative MG. MuSK-MG patients were predominantly female with more prominent facial and bulbar involvement and more frequent crises. Disease onset tended to be earlier. Patients tended to have a relatively poor edrophonium response but showed prominent decrement in the repetitive nerve stimulation test in the facial muscles. Patients were more likely to display poor tolerance of, or a lack of improvement with, anticholinesterase agents. Somewhat better response was observed with steroids and plasma exchange. Most were managed successfully with aggressive immunomodulatory therapies, although a higher proportion of MuSK-MG patients had a refractory course when compared with other forms of generalized MG. I present here an up-to-date overview on MuSK-MG based on our experience at the University of Alabama at Birmingham and the existing literature.

Abstract

Muscle-specific tyrosine-kinase-antibody-positive myasthenia gravis (MuSK-MG) has emerged as a distinct entity since 2001. This disease has been reported worldwide, but with varying rates among patients with generalized acetylcholine-receptor-antibody-negative MG. MuSK-MG was detected in approximately 37% of generalized acetylcholine receptor antibody-negative MG. MuSK-MG patients were predominantly female with more prominent facial and bulbar involvement and more frequent crises. Disease onset tended to be earlier. Patients tended to have a relatively poor edrophonium response but showed prominent decrement in the repetitive nerve stimulation test in the facial muscles. Patients were more likely to display poor tolerance of, or a lack of improvement with, anticholinesterase agents. Somewhat better response was observed with steroids and plasma exchange. Most were managed successfully with aggressive immunomodulatory therapies, although a higher proportion of MuSK-MG patients had a refractory course when compared with other forms of generalized MG. I present here an up-to-date overview on MuSK-MG based on our experience at the University of Alabama at Birmingham and the existing literature.

참고문헌 (65)

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관련 논문AI추천

Repetitive Nerve Stimulation in MuSK-Antibody-Positive Myasthenia Gravis김승민, 김승우, 신하영, 선우문경, 선우일남대한신경과학회 · Journal of Clinical Neurology2017 · 제13권 제3호MuSK항체 양성 중증근무력증권기한대한근전도전기진단의학회 · 대한근전도 전기진단의학회지2012 · 제14권 제1호Clinical and Electrophysiologic Responses to Acetylcholinesterase Inhibitors in MuSK-Antibody-Positive Myasthenia Gravis: Evidence for Cholinergic Neuromuscular Hyperactivity김승민, 박형준, 신하영, 이효은, 최영철대한신경과학회 · Journal of Clinical Neurology2014 · 제10권 제2호Muscle-specific receptor tyrosine kinase (MuSK) myasthenia gravis associated with castleman disease양우익, 오지영, 조정훈, 선우일남대한임상신경생리학회 · Annals of Clinical Neurophysiology2017 · 제19권 제1호Subgroup Classification and Therapeutic Guidance for Myasthenia Gravis이윤진대한소아신경학회 · 대한소아신경학회지2017 · 제25권 제1호Evaluating an In-House Cell-Based Assay for Detecting Antibodies Against Muscle-Specific Tyrosine Kinase in Myasthenia Gravis김민기, 김민주, 김승민, 김승우, 신하영, 최영철대한신경과학회 · Journal of Clinical Neurology2021 · 제17권 제3호

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