Japanese

Title	8 末梢神経疾患の治療の進歩
Subtitle	特集 神経疾患治療の進歩2022
Authors	関口縁**, 三澤園子***
Authors (kana)
Organization	**JR東京総合病院脳神経内科, ***千葉大学大学院医学研究院脳神経内科学
Journal	神経治療学
Volume	40
Number	5
Page	717-721
Year/Month	2023 /
Article	報告
Publisher	日本神経治療学会
Abstract	「はじめに」 末梢神経疾患には様々な疾患が含まれるが, 本稿では近年 治療の変化が起きている4つの疾患について, 治療と今後の 展望について述べる. 「I. Guillain-Barre症候群 (Guillain-Barre syndrome: GBS)」 「1. 疾患概要」 GBSは, 感染などを契機に発症する自己免疫性末梢神経障害であり, 単相性で比較的予後が良いと考えられている. しかし国際的な多施設共同の前向きコホート研究(InternationalGBS Outcome Study: IGOS)でも, 経過中の呼吸器装着率は19%, 死亡率は7%, 発症後1年の独歩不能な率は19%と予後が良いとは言い難く, 重症例に対する治療が課題である. 「2. 治療の現状」 GBSの標準治療は血漿交換または免疫グロブリン大量静注療法(intravenous immunoglobulin therapy: IVIg)であり, 臨床現場ではその簡便性からIVIgが選択されることが多い.
Practice	臨床医学：内科系
Keywords	Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, ATTRv amyloidosis

English

Title	Peripheral neuropathy
Subtitle	Review / Advances in Neurological Therapeutics (2022).
Authors	Yukari SEKIGUCHI*, Sonoko MISAWA**
Authors (kana)
Organization	*Department of Neurology, JR Tokyo General Hospital, **Department of Neurology, Graduate School of Medicine, Chiba University
Journal	Neurological Therapeutics
Volume	40
Number	5
Page	717-721
Year/Month	2023 /
Article	Report
Publisher	Japanese Society of Neurological Therapeutics
Abstract	Peripheral neuropathies are very common neurological disorders that are caused by various etiologies. This review focuses on four neuropathies which substantial advances have been made recently. In Guillain-Barre syndrome, treatment for severe cases is still a challenge. Clinical trials of several anti-complement drugs are currently underway and results are awaited. In CIDP (Chronic inflammatory demyelinating polyneuropathy), subcutaneous immunoglobulin has been added as a standard maintainace therapy. The efficacy of rituximab or neonatal Fc receptor (FcRn) for CIDP patients has been investigated. It is also interesting from the point of view of elucidating the pathology, and the publication of the results is awaited. Currently, the treatment for the patients with MMN (Multifocal motor neuropathy) is only IVIg and novel treatments for advanced cases are desired. Based on the findings that IgM anti-GM1 antibodies, which are highly positive in MMN patients, bind to motorneurons and activate complement pathway, a trial of AGX722, an antibody against C2, is currently underway. Therapeutic advances in ATTR amyloidosis have been remarkable : in addition to stabilizers of transthyretine, interference therapeutic agents are becoming the main treatment. Phase I trials with gene-editing therapeutics have also been underway and further developments are expected. Compared to other autoimmune diseases such as Myathenia Gravis or Neuromyelitis Optica, autoimmune peripheral neuropathies currently have limited treatment options. However, the development of these novel therapies may lead to new treatment options. While keeping a close eye on the development of new treatments, efforts should be made to accurately diagnose these diseases at an early stage and provide appropriate treatment to patients.
Practice	Clinical internal medicine
Keywords	Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, ATTRv amyloidosis

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