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<title>非定型パーキンソニズム | 株式会社文光堂</title>


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<meta name="description" content="非定型パーキンソニズムは主に進行性核上性麻痺，大脳皮質基底核変性症，レヴィ小体型認知症，多系統萎縮症などのパーキンソン病に似た症状を示す疾患群で，この領域は基礎，臨床ともに進歩が著しい．本書は，非定型パーキンソニズムの最新の研究動向，現在の診療における課題を提示しつつ，日常診療に役立つ内容となっている．本領域の専門以外の神経内科医も知っておくべき最新の情報がふんだんに盛り込まれている．">

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		<h1 class="catchCopy">神経内科医必携！本邦初の非定型パーキンソニズムの成書</h1>
		

		<h1 class="title">非定型パーキンソニズム<span class="version"></span></h1>
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					編集：下畑享良（岐阜大学大学院医学系研究科脳神経内科学分野教授）
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				<ul class="property"><li class="disc">B5判・272頁</li><li class="disc">ISBN&nbsp;978-4-8306-1547-4</li><li class="disc">2019年5月18日発行</li></ul>

				
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		<div class="headers"><h3 class="current content">内容</h3><h3 class="preface">序文</h3><h3 class="indexes">主要目次</h3></div><section class="current content">非定型パーキンソニズムは主に進行性核上性麻痺，大脳皮質基底核変性症，レヴィ小体型認知症，多系統萎縮症などのパーキンソン病に似た症状を示す疾患群で，この領域は基礎，臨床ともに進歩が著しい．本書は，非定型パーキンソニズムの最新の研究動向，現在の診療における課題を提示しつつ，日常診療に役立つ内容となっている．本領域の専門以外の神経内科医も知っておくべき最新の情報がふんだんに盛り込まれている．</section><section class="preface">序　文<br><br>　脳神経内科医として歩みはじめた頃，私は2つの疾患に強い衝撃を受けた．進行性核上性麻痺と多系統萎縮症である．何の抵抗もなく後方に転倒してしまう患者さんの姿を見たとき，また夜間病棟に大きく響くイビキを聞き，退院後に睡眠中の突然死の知らせを受けたとき，なぜこのようなことが生じてしまうのかと畏怖の念を抱いた．その後も現在に至るまで，主治医として有効な治療薬を処方できることはできず，悔しい思いをしてきた．<br>　しかし近年の研究の進歩により，進行性核上性麻痺，大脳皮質基底核変性症，多系統萎縮症といった非定型パーキンソニズムに対し，タウ蛋白やαシヌクレインが有望な治療標的になりうることが明らかとなり，状況は大きく変わった．すでに複数の臨床試験が行われ，さらに新規試験も計画され，確実に新しい時代に突入した．しかしその成功のために乗り越えるべきいくつもの課題があることも同時に明らかになった．具体的には，さまざまな臨床表現型を呈しても見落とすことなく適切にその背景病理を診断すること，神経変性が可逆的なステージにある，より早期の段階で臨床診断し，病態抑止療法を開始すること，そして臨床表現型が類似する別の疾患（mimics）を適切に除外することが不可欠である．このためには病理学的に診断が確定した症例を対象として，それぞれの疾患の臨床，画像，遺伝子所見を見直し，さらにそれらを踏まえた新しい臨床診断基準を作成する必要がある．また希少疾患であるため，臨床試験の成功を目的としたコンソーシアムを構築する必要もある．すなわち非定型パーキンソニズムの治療の実現のためには，臨床，病理学，放射線医学，基礎研究などのさまざまな領域のエキスパートの相互理解と連携が不可欠である．言い換えるとこれらの疾患に立ち向かうために必要なものは，チームとしての力量といえるだろう．<br>　本書は各領域のエキスパートの先生方に「将来，非定型パーキンソニズムに取り組みたいと思う臨床医，基礎研究者が増えることに貢献するような書籍を作りたい」と執筆を依頼し，ご快諾を得てできたものである．第I章総論では詳細な症候の理解や，疫学，バイオマーカー，リハビリテーション等について議論し，第II章各論では疾患ごとの歴史，診断基準，mimics，画像・病理所見，治療をご提示いただいた．さらに第III章では病態解明と治療法の確立に向けた最新情報をまとめていただいた．いずれの項目においても，今後の課題をご提示いただき，わが国から新たな知見やエビデンスの発信に貢献することを目指した．本書が非定型パーキンソニズムで苦しむ患者さんの診療と，未来の治療開発に役立つことを心より期待したい．<br><br>2019年4月<br>下畑　享良<br>岐阜大学大学院医学系研究科脳神経内科学分野 教授</section><section class="indexes">I　総　論<br>1．本領域における概念の変化<br>　　はじめに／治療標的分子と治療戦略／臨床試験の成功を目指した取り組み／各疾患の臨床像<br>2．症候の理解と電気生理<br>　　非定型パーキンソニズムの特徴的な神経症候／神経生理学的アプローチ<br>3．疫学，疫学研究の方法<br>　　はじめに／臨床研究における疫学研究／非定型パーキンソニズムと疫学研究／JALPAC研究／おわりに<br>4．非定型パーキンソニズムの主な症候<br>　a．運動前症状と意義<br>　　はじめに／多系統萎縮症（MSA）／4リピートタウオパチー（進行性核上性麻痺・大脳皮質基底核変性症）／おわりに<br>　b．眼球運動障害<br>　　はじめに／パーキンソン病の眼球運動障害／多系統萎縮症（MSA）の眼球運動障害／進行性核上性麻痺（PSP）の眼球運動障害／大脳皮質基底核変性症（CBD）の眼球運動障害<br>　c．高次脳機能障害<br>　　はじめに／診断に必要な高次脳機能障害のポイント／非定型パーキンソニズム（CBD，PSP，MSA，FTLD）にみられる高次脳機能障害<br>　d．精神症状<br>　　はじめに／精神症状／DLBの精神症状／PSPの精神症状／CBDの精神症状／MSAの精神症状<br>　e．睡眠障害/覚醒障害<br>　　はじめに／多系統萎縮症（MSA）／不眠や日中の眠気／睡眠構築／レストレスレッグス症候群（RLS）／周期性四肢運動／睡眠関連呼吸障害／レム睡眠行動異常症（RBD）／進行性核上性麻痺（PSP）／レヴィ小体型認知症（DLB）／大脳皮質基底核変性症（CBD）<br>　f．嚥下障害<br>　　はじめに／嚥下障害の特徴／嚥下障害への対策／おわりに<br>　g．コミュニケーション障害<br>　　はじめに／MSAのコミュニケーション障害の特徴／コミュニケーション支援の方法／公的支援制度の種類<br>5．非定型パーキンソニズムの現状と課題<br>　a．脳脊髄液・血液バイオマーカー<br>　　はじめに／アルツハイマー病関連CSFバイオマーカー／CSFαシヌクレイン／CSFニューロフィラメント軽鎖／末梢血中NfL／併存病理による限界／おわりに<br>　b．PET研究<br>　　はじめに〜PET研究とその臨床応用の意義／脳糖代謝イメージングが持つ可能性／ドパミン神経系だけではない神経伝達機能イメージング／脳内異常蓄積蛋白を可視化する神経病理イメージング／おわりに<br>　c．リハビリテーション<br>　　非定型パーキンソニズムにおけるリハの現状／各症候に対するリハ（理学療法）<br>II　各　論<br>1．多系統萎縮症<br>　a．歴史，診断基準，臨床特徴，mimics<br>　　はじめに／歴史／診断基準／臨床特徴／多彩な表現型<br>　b．画像診断（コネクトームを含む）<br>　　はじめに／MSAの画像診断／MSAの新しい解析方法を用いた頭部MRI所見（コネクトームを含む）／おわりに<br>　c．病　理<br>　　MSAの特徴的組織所見／MSAの基本的病理所見／MSAの呼吸・循環障害にかかわる組織所見／MSAの認知機能障害にかかわる組織所見<br>　d．治　療<br>　　はじめに／パーキンソニズム／小脳性運動失調／自律神経障害／その他の症状／これまでに行われた無作為化比較試験／家族性MSAの病因遺伝子の発見とCoQ10補充療法の試み／一般のMSA患者に対するCoQ10補充療法の試み<br>2．進行性核上性麻痺<br>　a．歴史，臨床像，診断基準，mimics<br>　　はじめに／歴史／臨床像／NINDS-SPSPの診断基準から新診断基準へ／新基準の内容と主な臨床像／異型PSP症候群 variant PSP syndromes（vPSP）／PSP mimics／患者会の役割／おわりに<br>　b．画像診断<br>　　はじめに／PSPの多様な画像所見／画像診断におけるPSP mimics<br>　c．病　理<br>　　病理学的所見／PSPの病理像のスペクトラム／鑑別疾患<br>　d．治　療<br>　　はじめに／従来から行われている対症療法／PSPに対する最新の臨床試験情報／これまでに行われた主な臨床試験／従来の臨床試験から学ぶべきこと／現在進行中のタウを標的とする治療法<br>3．大脳皮質基底核変性症<br>　a．臨床像，診断基準，病型，mimics<br>　　はじめに／CBD原著の臨床症候／CBSの疾患概念の成立／CBSの臨床症候と診断基準／CBDの真の臨床像と臨床病型／CBDの診断基準（Armstrong基準）／診断基準の検証／認知症を呈するCBDとADの鑑別／診断基準の使用法／おわりに<br>　b．画像診断・検査所見<br>　　はじめに／大脳皮質基底核変性症の画像所見／おわりに<br>　c．病　理<br>　　はじめに／CBDの病理像／おわりに<br>　d．治　療<br>　　はじめに／大脳皮質基底核変性症の各症状に対する薬物による対症療法／リハビリテーション療法／大脳皮質基底核変性症に対する治療における最新知見／まとめ<br>4．神経変性タウオパチーの分子遺伝学と臨床病理<br>　　はじめに／進行性核上性麻痺（PSP）の遺伝子変異／大脳皮質基底核変性症（CBD）の遺伝子変異／globular glial tauopathy（GGT）の遺伝子変異／Pick病の遺伝子変異／その他のタウオパチーの遺伝子変異／おわりに<br>5．Globular glial tauopathy<br>　　はじめに／GGTの歴史／GGTの臨床像／GGTの病理所見／タウの生化学的所見／おわりに<br>6．レヴィ小体型認知症<br>　a．歴史，臨床像，診断基準，mimics<br>　　歴史／臨床像／診断基準／Mimics／おわりに<br>　b．画像診断・検査所見・治療<br>　　はじめに／DLBの診断に有用な指標的バイオマーカー／レヴィ小体型認知症にみられるその他の検査異常／レヴィ小体型認知症の治療／おわりに<br>　c．病　理<br>　　はじめに／PD Braakステージと臨床症状の関連／偶発的レヴィ病理とレヴィ小体病の連続性／偶発的レヴィ病理の脳内分布／DLB病理診断基準／DLBDの臨床像／おわりに<br>7．正常圧水頭症<br>　a．歴史，臨床像，診断基準，画像所見，治療<br>　　歴史／疫学／臨床像／画像所見／診断基準／治療／脳脊髄液の生理的動態と異常<br>　b．病　理<br>　　はじめに／動物モデルでの病理所見／ヒトの病態生理／iNPHと脳の不要物質ドレナージ機構／自験例の組織所見<br>III　病態解明と治療法の確立に向けて<br>1．治療戦略<br>　a．治療戦略オーバービュー<br>　　はじめに／MSAに対する最近の臨床試験／MSAに対する進行中の臨床試験／PSPに対する最近の臨床試験／PSPに対する進行中の臨床試験／まとめ<br>　b．αシヌクレイン<br>　　はじめに／MSA病態におけるαSynの役割／αSynを標的としたMSAの疾患修飾療法／おわりに<br>　c．タウ蛋白<br>　　タウ蛋白の特徴／タウ蛋白を標的にした治療戦略／克服すべき課題／おわりに<br>　d．プログラニュリン<br>　　はじめに／GRN変異とパーキンソン病／GRN変異と大脳皮質基底核症候群／GRN rs5848遺伝子多型とPD／GRN rs5848遺伝子多型とCBS／脳内における異常蛋白の蓄積とPGRN／PGRNを標的とした治療戦略／おわりに<br>　e．自己免疫<br>　　パーキンソン病の病態機序と自己免疫／パーキンソン病と自己抗体／非定型パーキンソニズムと抗神経抗体／非定型パーキンソニズムと抗IgLON5抗体／おわりに<br>2．動物モデル<br>　a．αシヌクレイン<br>　　はじめに／MSAにおける神経変性機構／GCI形成による遺伝子改変モデルマウスの作出／分子レベルの発病機構の解析と神経病理所見への回帰／治療標的から創薬研究へ<br>　b．タウ蛋白<br>　　はじめに／タウ蛋白とタウオパチー／タウオパチーマウスモデル／マウスモデルを用いた創薬プラットフォーム開発／マウスモデル研究の留意点／おわりに<br>3．臨床試験デザイン<br>　　はじめに／多系統萎縮症に対する臨床開発／進行性核上性麻痺に対する臨床開発／レヴィ小体型認知症に対する臨床開発／非定型パーキンソニズムの臨床開発における問題点／おわりに<br>索　引</section></article>
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</html>