Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of anti-acetylcholine receptor antibody-positive ocular myasthenia gravis with anti-titin antibody and anti-Kv1.4 antibody positive inflammatory myopathy

Kenshiro Fuse, M.D.1), Amane Araki, M.D., Ph.D.1), Saori Morozumi, M.D., Ph.D.1), Keizo Yasui, M.D., Ph.D.1), Tomoyuki Kazuta, M.D.2)3), Seiya Noda, M.D., Ph.D.2)4) and Masahisa Katsuno, M.D., Ph.D.2)5)

1) Department of Neurology, Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital
2) Department of Neurology, Nagoya University Graduate School of Medicine
3) Department of Neurology, Chutoen General Medical Center
4) Department of Neurology, National Hospital Organization Suzuka Hospital
5) Department of Clinical Research Education, Nagoya University Graduate School of Medicine

An 84-year-old man was diagnosed with anti-acetylcholine receptor (AChR) antibody-positive ocular myasthenia gravis (OMG) at the age of 77 and received treatment. The patient was referred to our department with swelling and pain in his right upper arm, which had spread to other limbs. His serum anti-AChR antibody and creatine kinase levels were elevated, and MRI of the limbs displayed signal changes suggesting inflammation in the several muscles. Despite showing no sign of thymoma, he was positive for serum anti-titin and anti-Kv1.4 antibodies. We performed a muscle biopsy, which led to a diagnosis of inflammatory myopathy (IM). IM associated with OMG is relatively mild. Age-related immune dysregulation may cause both OMG and IM. Evaluation of disease activity with serum anti-AChR antibody levels, and assessment of prognosis with examining anti-striational antibodies are necessary for appropriate management of IM associated with MG.
Full Text of this Article in Japanese PDF (2023K)

(CLINICA NEUROL, 63: 830−835, 2023)
key words: ocular myasthenia gravis, inflammatory myopathy, anti-acetylcholine receptor antibody, anti-titin antibody, anti-Kv1.4 antibody

(Received: 20-Jul-23)