Overview of Immunotherapy Administration for Miller Fisher Syndrome in Japan: A Nationwide Retrospective Cohort Study

Satoshi Kodama; Mitsuhiro Kainaga; Shotaro Aso; Taisuke Jo; Yohei Hashimoto; Hiroki Matsui; Hideo Yasunaga; Yuichiro Shirota; Masashi Hamada; Kiyohide Fushimi; Tatsushi Toda

doi:10.37737/ace.26002

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Overview of Immunotherapy Administration for Miller Fisher Syndrome in Japan: A Nationwide Retrospective Cohort Study

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Overview of Immunotherapy Administration for Miller Fisher Syndrome in Japan: A Nationwide Retrospective Cohort Study

Satoshi Kodama, Mitsuhiro Kainaga, Shotaro Aso, Taisuke Jo, Yohei Hashimoto, Hiroki Matsui, Hideo Yasunaga, Yuichiro Shirota, Masashi Hamada, Kiyohide Fushimi, …

Annals of clinical epidemiology, Vol.8(1), pp.10-17

01/01/2026

DOI: 10.37737/ace.26002

PMID: 41798215

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Abstract

Miller Fisher syndrome (MFS) is typically associated with a benign course and spontaneous recovery. However, immunotherapies such as intravenous immunoglobulin (IVIG) and plasma exchange (PE) are often administered for this condition because of the potential for clinical deterioration. We aimed to evaluate the real-world use of immunotherapies in MFS management using a nationwide Japanese database. We conducted a retrospective cohort study between April 2014 and March 2020 using the Japanese Diagnosis Procedure Combination database. Patient demographics and treatment modalities were described. Outcomes included length of hospital stay, in-hospital mortality, hospitalization cost, and activities of daily living defined by the Barthel index score. We identified 1,595 patients with MFS. Of these, 999 (62.6%) received immunotherapy (including IVIG for 908 [56.9%] patients, intravenous methylprednisolone for 219 [13.7%] patients, and PE for 18 [1.8%] patients). In patients with and without immunotherapy, the median length of hospital stay was 18 and 12 days, in-hospital mortality was 0.7% and 0.2%, median total hospitalization costs were ¥1,660,200 and ¥549,375, and the proportions of Barthel index score of 95-100 at discharge were 75.3% and 75.2%, respectively. Our findings highlight the widespread use of immunotherapies for MFS in Japan despite its generally favorable natural course.

Guillain–Barré syndrome

Bickerstaff’s brainstem encephalitis

plasma exchange

intravenous immunoglobulin

Details

Title: Subtitle: Overview of Immunotherapy Administration for Miller Fisher Syndrome in Japan: A Nationwide Retrospective Cohort Study
Creators: Satoshi Kodama - University of Iowa
Mitsuhiro Kainaga - The University of Tokyo
Shotaro Aso - The University of Tokyo
Taisuke Jo - The University of Tokyo
Yohei Hashimoto - The University of Sydney
Hiroki Matsui - The University of Tokyo
Hideo Yasunaga - The University of Tokyo
Yuichiro Shirota - The University of Tokyo
Masashi Hamada - The University of Tokyo
Kiyohide Fushimi - Department of Health Policy and Informatics, Institute of Science Tokyo Graduate School, Tokyo, Japan
Tatsushi Toda - The University of Tokyo
Resource Type: Journal article
Publication Details: Annals of clinical epidemiology, Vol.8(1), pp.10-17
DOI: 10.37737/ace.26002
PMID: 41798215
NLM abbreviation: Ann Clin Epidemiol
ISSN: 2434-4338
eISSN: 2434-4338
Language: English
Date published: 01/01/2026
Academic Unit: Neurology
Record Identifier: 9985177939102771

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