Japanese

Title	筋萎縮性側索硬化症の標準的治療と治療開発の展望
Subtitle	特集 / 教育講演9
Authors	熱田直樹**
Authors (kana)
Organization	**名古屋大学医学部附属病院脳神経内科
Journal	神経治療学
Volume	36
Number	4
Page	337-340
Year/Month	2019 /
Article	報告
Publisher	日本神経治療学会
Abstract	「はじめに」 筋萎縮性側索硬化症(amyotrophic lateral sclerosis : ALS)患者は進行性の全身骨格筋の萎縮, 筋力低下をきたし, 発症から平均3-4年で死亡もしくは永続的な人工換気を要する状態となる. 現在もなお根治的治療は無く, 代表的な神経難病であるが, 診療・ケアにおいてできること, 行うべきことは数多く示されてきている. 標準的な診療・ケアとして, 進行抑制薬の投与, 摂食嚥下障害への対応, 呼吸機能障害への対応, その他の対症療法, リハビリテーション, コミュニケーション支援, 多職種での支援体制構築を行っていく必要がある. ただし, ALS患者の臨床像, 経過は非常に多彩であることが示されており, 個別の患者の病像や経過に応じた対応が求められる. また, ALS患者における神経変性の病態を変化させるような, より有用な根本治療法(disease-modifying therapy)の開発が精力的に行われている.
Practice	臨床医学：内科系
Keywords	amyotrophic lateral sclerosis, disease modifying therapy, nutritional support, multidisciplinary team, disease-related genes

English

Title	Perspectives on standard care and treatment development for ALS
Subtitle
Authors	Naoki ATSUTA
Authors (kana)
Organization	Department of Neurology, Nagoya University Hospital
Journal	Neurological Therapeutics
Volume	36
Number	4
Page	337-340
Year/Month	2019 /
Article	Report
Publisher	Japanese Society of Neurological Therapeutics
Abstract	Patients with amyotrophic lateral sclerosis (ALS) develop progressive generalized skeletal muscle atrophy, weakness, and die on average 3-4 years from onset or require permanent ventilation. Even now, we have no curative treatment for ALS, but there is much to do in medical care. As standard care, we will administer progression inhibitors, manage nutritional and respiratory status, provide symptomatic treatments, introduce rehabilitation, support communication disorder and build a multidisciplinary support team. The clinical courses and prognoses of ALS patients are shown to be very diverse, and the treatment and care plan for each patient should be determined according to the status and progress of the individual patient. The development of disease-modifying treatments that alter the pathophysiology of neurodegeneration in ALS patients is being vigorously carried out. The background is that many genes and molecules related to the pathogenesis of ALS have been identified since the 2000s. Therapeutic development of ALS is approaching the stage of developing disease-modifying therapy by elucidating pathogenic genes and molecular pathogenesis. There has been great progress in elucidating the molecular pathogenesis of ALS, and it is expected to become an active area of drug discovery.
Practice	Clinical internal medicine
Keywords	amyotrophic lateral sclerosis, disease modifying therapy, nutritional support, multidisciplinary team, disease-related genes

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