Rinsho Shinkeigaku (Clinical Neurology)

Invited Review

The clinical practice guideline for the management of amyotrophic lateral sclerosis in Japan-update 2023

Makoto Urushitani, M.D., Ph.D.1), Hitoshi Warita, M.D., Ph.D.2), Naoki Atsuta, M.D., Ph.D.3),Yuishin Izumi, M.D., Ph.D.4), Osamu Kano, M.D., Ph.D.5), Toshio Shimizu, M.D., Ph.D.6),Yuki Nakayama, R.N., Ph.D.7), Yugo Narita, M.D., Ph.D.8), Hiroyuki Nodera, M.D., Ph.D.9),Takuji Fujita, M.D., Ph.D.10), Koichi Mizoguchi, M.D., Ph.D.11), Mitsuya Morita, M.D., Ph.D.12) and Masashi Aoki, M.D., Ph.D.2)＊

1）Department of Neurology, Shiga University of Medical Science
2）Department of Neurology, Tohoku University Graduate School of Medicine
3）Department of Neurology, Aichi Medical University School of Medicine
4）Department of Neurology, Tokushima University Graduate School of Biomedical Sciences
5）Department of Neurology, Toho University Faculty of Medicine
6）Department of Neurology, Tokyo Metropolitan Neurological Hospital
7）Unit for Intractable Disease Care Unit, Tokyo Metropolitan Institute of Medical Science
8）Department of Neurology, Mie University Graduate School of Medicine
9）Department of Neurology, Tenri Hospital
10）Takumi Medical Corporation, Neurology Clinic
11）Taiheikai Medical Corporation, Johsai Clinic
12）Division of Neurology, Department of Internal Medicine, Jichi Medical University

Amyotrophic lateral sclerosis (ALS) is an adult-onset intractable motor neuron disease characterized by selective degeneration of cortical neurons in the frontotemporal lobe and motor neurons in the brainstem and spinal cord. Impairment of these neural networks causes progressive muscle atrophy and weakness that spreads throughout the body, resulting in life-threatening bulbar palsy and respiratory muscle paralysis. However, no therapeutic strategy has yet been established to halt ALS progression. Although evidence for clinical practice in ALS remains insufficient, novel research findings have steadily accumulated in recent years. To provide updated evidence-based or expert consensus recommendations for the diagnosis and management of ALS, the ALS Clinical Practice Guideline Development Committee, approved by the Japanese Society of Neurology, revised and published the Japanese clinical practice guidelines for the management of ALS in 2023. In this guideline, disease-modifying therapies that have accumulated evidence from randomized controlled trials were defined as “Clinical Questions,“ in which the level of evidence was determined by systematic reviews. In contrast, “Questions and Answers“ were defined as issues of clinically important but insufficient evidence, according to reports of a small number of cases, observational studies, and expert opinions. Based on a literature search performed in February 2022, recommendations were reached by consensus, determined by an independent panel, reviewed by external reviewers, and submitted for public comments by Japanese Society of Neurology members before publication. In this article, we summarize the revised Japanese guidelines for ALS, highlighting the regional and cultural diversity of care processes and decision-making. The guidelines cover a broad range of essential topics such as etiology, diagnostic criteria, disease monitoring and treatments, management of symptoms, respiration, rehabilitation, nutrition, metabolism, patient instructions, and various types of care support. We believe that this summary will help improve the daily clinical practice for individuals living with ALS and their caregivers.
Full Text of this Article in Japanese PDF (708K)

(CLINICA NEUROL, 64: 252−271, 2024)
key words: ALS, amyotrophic lateral sclerosis, guideline, motor neuron disease, systematic review

(Received: 4-Nov-23)