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Clinical features and outcomes of patients with muscle-specific kinase antibody-positive myasthenia gravis in Japan

2023; Elsevier BV; Volume: 385; Linguagem: Inglês

10.1016/j.jneuroim.2023.578241

1872-8421

Manato Yasuda, Akiyuki Uzawa, Satoshi Kuwabara, Shigeaki Suzuki, Hiroyuki Akamine, Yosuke Onishi, Yukiko Ozawa, Naoki Kawaguchi, Tomoya Kubota, Masanori Takahashi, Yasushi Suzuki, Genya Watanabe, Takashi Kimura, Takamichi Sugimoto, Makoto Samukawa, Naoya Minami, Masayuki Masuda, Shingo Konno, Yuriko Nagane, Kimiaki Utsugisawa,

Parkinson's Disease and Spinal Disorders

This study included 51 patients with muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG) from a Japanese multicenter survey to examine clinical features and outcomes. Median onset age was 37 years and female predominance was observed. All patients developed generalized symptoms and almost all (50/51) patients had bulbar symptoms. About half of the patients met the criteria for refractory MG. The refractory group had a lower age of onset, higher severity scores, and higher maximum daily doses of oral prednisolone compared to the nonrefractory group. The outcomes for MuSK-MG patients in Japan are not favorable, indicating the need for more aggressive treatment.