Marked Respiratory Failure in an Ambulant Patient with Immune-mediated Necrotizing Myopathy and Anti-Kv1.4 and Anti-titin Antibodies

Atsuhiko Sugiyama; Yosuke Onishi; Kimiko Ito; Kazumoto Shibuya; Keigo Nakamura; Fumiko Oda; Ichizo Nishino; Shigeaki Suzuki; Satoshi Kuwabara

doi:10.2169/internalmedicine.6834-20

CASE REPORTS

Marked Respiratory Failure in an Ambulant Patient with Immune-mediated Necrotizing Myopathy and Anti-Kv1.4 and Anti-titin Antibodies

Atsuhiko Sugiyama, Yosuke Onishi, Kimiko Ito, Kazumoto Shibuya, Keigo Nakamura, Fumiko Oda, Ichizo Nishino, Shigeaki Suzuki, Satoshi Kuwabara

Author information

Atsuhiko Sugiyama
Department of Neurology, Graduate School of Medicine, Chiba University, Japan
Yosuke Onishi
Department of Neurology, Graduate School of Medicine, Chiba University, Japan
Kimiko Ito
Department of Neurology, National Hospital Organization Chibahigashi National Hospital, Japan
Kazumoto Shibuya
Department of Neurology, Graduate School of Medicine, Chiba University, Japan
Keigo Nakamura
Department of Neurology, Graduate School of Medicine, Chiba University, Japan
Fumiko Oda
Department of Neurology, National Hospital Organization, Chiba Medical Center, Japan
Ichizo Nishino
Neurological Research Institute, National Center of Neurology and Psychiatry, Japan
Shigeaki Suzuki
Department of Neurology Keio University School of Medicine, Japan
Satoshi Kuwabara
Department of Neurology, Graduate School of Medicine, Chiba University, Japan

Keywords: respiratory paralysis, muscular diseases, creatine kinase, blepharoptosis

JOURNAL OPEN ACCESS

2021 Volume 60 Issue 16 Pages 2671-2675

DOI https://doi.org/10.2169/internalmedicine.6834-20

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Abstract

We herein report a case of seronegative immune-mediated necrotizing myopathy (IMNM) concurrent with anti-Kv1.4 and anti-titin antibodies. A 72-year-old Japanese woman presented with a 29-year history of fluctuating high serum creatine kinase (CK) levels followed by intermittent ptosis and respiratory muscle weakness. This case highlights the fact that marked respiratory muscle weakness requiring intubation can be seen in an ambulant patient with IMNM. Marked respiratory muscle weakness, rhabdomyolysis-like acute elevation of CK levels, and anti-striational muscle antibodies may be a characteristic constellation of findings in a distinct subgroup of patients with inflammatory myopathy with myasthenia gravis or similar symptoms.

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