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. 2024 May;31(5):e16249.
doi: 10.1111/ene.16249. Epub 2024 Feb 20.

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

Yuya Aotsuka  1 , Sonoko Misawa  1 , Tomoki Suichi  1 , Kazumoto Shibuya  1 , Keigo Nakamura  1 , Hiroki Kano  1 , Ryo Otani  1 , Marie Morooka  1 , Moeko Ogushi  1 , Kengo Nagashima  2   3 , Yasunori Sato  2   3 , Nagato Kuriyama  4   5 , Satoshi Kuwabara  1
Affiliations

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

Yuya Aotsuka et al. Eur J Neurol. 2024 May.

Abstract

Background and purpose: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: anti‐myelin‐associated glycoprotein; epidemiology; nationwide survey; neuropathy; prevalence.

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Conflict of interest statement

None declared.

Figures

FIGURE 1
FIGURE 1
Survey profiles, number of responding hospital departments and number of cases.
FIGURE 2
FIGURE 2
Age at onset and gender distribution of anti‐myelin‐associated glycoprotein neuropathy patients.
See this image and copyright information in PMC

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