Prevalence and clinical profiles of anti-myelin-associated glycoprotein neuropathy in Japan: A nationwide survey study of 133 patients

• Published in: European journal of neurology Vol. 31; no. 5; p. e16249
• Main Authors: Aotsuka, Yuya, Misawa, Sonoko, Suichi, Tomoki, Shibuya, Kazumoto, Nakamura, Keigo, Kano, Hiroki, Otani, Ryo, Morooka, Marie, Ogushi, Moeko, Nagashima, Kengo, Sato, Yasunori, Kuriyama, Nagato, Kuwabara, Satoshi
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01-05-2024
• Subjects: Aged; Aged, 80 and over; Ataxia; Autoantibodies; Child; Epidemiology; Female; Glycoproteins; Health services; Humans; Immunoglobulin M; Japan - epidemiology; Macroglobulinemia; Male; Multiple myeloma; Myelin; Myelin-Associated Glycoprotein; Neuralgia - epidemiology; Neurology; Neuropathy; Patients; Pediatrics; Polyneuropathies - drug therapy; Polyneuropathy; Prevalence; Questionnaires; Rituximab; Rituximab - therapeutic use; Surveys; Japan; epidemiology; prevalence; neuropathy; anti‐myelin‐associated glycoprotein; nationwide survey
• ISSN: 1351-5101; 1468-1331; 1468-1331
• DOI: 10.1111/ene.16249

The aim of this study was to determine the prevalence of anti-myelin-associated glycoprotein (MAG) neuropathy and the current status of such patients in Japan.
We conducted a nationwide survey in 2021 using established epidemiological methods. Questionnaires were sent to all neurology and pediatric neurology departments throughout Japan to identify patients with anti-MAG neuropathy. An initial questionnaire was used to determine the number of patients, with a second one used to collect detailed clinical information.
The estimated number of patients with anti-MAG neuropathy was 353, with a prevalence of 0.28 per 100,000 and an incidence of 0.05 per 100,000. The detailed clinical profiles of 133 patients were available. The median (range) age of onset was 67 (30-87) years, with a prominent peak in the age range 66-70 years, and the male-to-female ratio was 3.6. Most patients had distal sensory-predominant polyneuropathy, and neuropathic pain (50%), or sensory ataxia (42%), while 18% had Waldenström's macroglobulinemia or multiple myeloma. Intravenous immunoglobulin was the most frequently used treatment (65%), but the response rate was <50%, whereas rituximab was given in 32% of patients, and 64% of these showed improvement. At the last visit, 27% of patients could not walk independently.
This study on anti-MAG neuropathy provides updated insights into the epidemiology of this disease, clinical profiles, and treatment approaches in Japan. Rituximab therapy, used for only one-third of the patients, demonstrated efficacy. During the final visit, a quarter of the patients were unable to walk independently. Further studies are warranted to determine the optimal management of this rare and intractable disorder.