primary central nervous system vasculitis

原発性中枢神経系血管炎

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• 1. 成人における中枢神経系の原発性血管炎primary angiitis of the central nervous system in adults [show details]
  … The cause of primary angiitis of the central nervous system (PACNS) is unknown. Several potential etiologic agents or mechanisms have been proposed, but understanding of the cause of PACNS remains highly …
• 2. 小児期の中枢神経系原発性血管炎childhood primary angiitis of the central nervous system [show details]
  …vasculitis is discussed in detail separately, as is PACNS in adults. cPACNS criteria are based upon the modified Calabrese criteria for adult PACNS and include : Newly acquired, otherwise unexplained …
• 3. 成人における血管炎の概要およびアプローチoverview of and approach to the vasculitides in adults [show details]
  … vasculitis. The involved organ and vessel type should be included in the name (eg, primary central nervous system vasculitis [CNSV], cutaneous small vessel vasculitis, isolated aortitis). Some patients initially …
• 4. 小児血管炎：発生率および分類vasculitis in children incidence and classification [show details]
  …Africa ), granulomatosis with polyangiitis (GPA), microscopic polyangiitis, primary angiitis of the central nervous system (PACNS), and polyarteritis nodosa (PAN) . Eosinophilic GPA (formerly Churg-Strauss …
• 5. 可逆性脳血管れん縮症候群reversible cerebral vasoconstriction syndrome [show details]
  …diagnoses. In the past, patients with RCVS have been misinterpreted as having primary angiitis of the central nervous system (PACNS) or aneurysmal subarachnoid hemorrhage due to overlapping features such as …

English Journal

• PMID 31960097
• PMID 32622763
• PMID 32062770

Japanese Journal

• 臨床研究・症例報告 Childhood primary angiitis of the central nervous systemが原因として考えられた小児脳梗塞の1例

• 植田 綾子,池田 尚広,山崎 雅代,小坂 仁,山形 崇倫
• 小児科臨床 = Japanese journal of pediatrics 73(1), 33-38, 2020-01
• NAID 40022100716

• 脳梗塞と脊髄出血を同時期に発症した原発性中枢神経系血管炎と考えられた1例

• 和田 隆史,北口 浩史,進藤 克郎
• 臨床神経学 59(5), 268-273, 2019
• <p>症例は61歳女性．突然の激烈な腰背部痛と左下肢の脱力を自覚し，同日中に頭部全体の拍動性頭痛も出現した．初診時には左下肢の筋力低下に加えて，左下方の四分の一盲と左下肢の振動覚低下も認めた．血液検査で凝固系や自己抗体の異常はなく，髄液検査で血性髄液と炎症所見を認めた．頭部MRIで複数の脳血管狭窄を伴う脳梗塞と脳表のクモ膜下出血，脊髄MRIで頸髄，胸髄，一部腰髄に多発する脊髄出血を認め …
• NAID 130007655294

• A Long Interval from a Spinal Cord Lesion to a Subsequent Brain Lesion in Primary Central Nervous System Vasculitis

• Kon Tomoya,Funamizu Yukihisa,Suzuki Chieko,Sato Tsugumi,Kurotaki Hidekachi,Kurihara Aiichiro,Kurose Akira,Wakabayashi Koichi,Tomiyama Masahiko
• Internal Medicine 58(10), 1485-1489, 2019
• … <p>Primary central nervous system vasculitis (PCNSV) is an uncommon vasculitis restricted to the small- and medium-sized vessels in the brain and spinal cord. …
• NAID 130007649802

Related Links

• Central Nervous System Vasculitis - Vasculitis Foundation

Primary angiitis of the CNS (PACNS) is vasculitis confined specifically to the brain and spinal cord, which make up the central nervous system. It is not associated with any other systemic (affecting the whole body) disease.

• The diagnosis of primary central nervous system vasculitis ...

In primary central nervous system (CNS) vasculitis, cerebral angiography without histology is not diagnostic. CNS biopsy is diagnostically important and relatively safe. We propose binary diagnostic criteria, categorising cases as either ‘definite’ or ‘possible’, and abandoning any ‘probable’ category.

• Central Nervous System Vasculitis (CNS Vasculitis)

What is central nervous system vasculitis? Central nervous system (CNS) vasculitis is inflammation of blood vessel walls in the brain or spine. (The brain and the spine make up the central nervous system.) CNS vasculitis often occurs in the following situations:

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★リンクテーブル★

リンク元	「血管炎」「原発性中枢神経系血管炎」
関連記事	「primary」「nervous」「central」「system」

「血管炎」

　　[★]

英

angiitis

関

脈管炎 vasculitis　vasculitides、血管内膜炎、血管炎症候群

分類

CHCC2012

large vessel vasculitis LVV 大型血管炎	Takayasu arteritis TAK 高安動脈炎
	giant cell arteritis GCA 巨細胞性動脈炎
medium vessel vasculitis MVV 中型血管炎	polyarteritis nodosa PAN 結節性多発動脈炎
	Kawasaki disease KD 川崎病
small vessel vasculitis SVV 小型血管炎	antineutrophil cytoplasmic antibody-associated vasculitis AAV 抗好中球細胞質抗体関連血管炎	microscopic polyangiitis MPA 顕微鏡的多発血管炎
		granulomatosis with polyangiitis GPA 多発血管炎性肉芽腫症(Wegener肉芽腫症)
		eosinophilic granulomatosis with polyangiitis EGPA 好酸球性多発血管炎性肉芽腫症(Churg-Strauss症候群)
	immune complex SVV 免疫複合体性小型血管炎	anti-glomerular basement membrane disease 抗糸球体基底膜抗体病(抗GBM病)
		cryoglobulinemic vasculitis CV クリオグロブリン血症性血管炎
		IgA vasculitis IgAV IgA血管炎(Henoch-Schonlein紫斑病)
		hypocomplementemic urticarial vasculitis HUV 低補体血症性蕁麻疹様血管炎(抗C1q血管炎)
variable vessel vasculitis VVV 多様な血管を侵す血管炎	Behcet's disease BD Behcet病
	Cogan's syndrome CS Cogan症候群
single-organ vasculitis SOV 単一臓器血管炎	cutaneous leukocytoclastic angiitis 皮膚白血球破砕性血管炎
	cutaneous arteritis 皮膚動脈炎
	primary central nervous system vasculitis 原発性中枢神経系血管炎
	isolated aortitis 限局性大動脈炎
vasculitis associated with systemic disease 全身性疾患関連血管炎	lupus vasculitis ループス血管炎
	rheumatoid vasculitis リウマトイド血管炎
	sarcoid vasculitis サルコイド血管炎
vasculitis associated with probable etiology 推定病因を有する血管炎	hepatitis C virus-associated cryoglobulinemic vasculitis C型肝炎ウイルス関連クリオグロブリン血症性血管炎
	hepatitis B virus-associated vasculitis B型肝炎ウイルス関連血管炎
	syphilis-associated aortitis 梅毒関連大動脈炎
	drug-associated immune complex vasculitis 薬剤関連免疫複合体性血管炎
	drug-associated ANCA-associated vasculitis 薬剤関連ANCA関連血管炎
	cancer-associated vasculitis がん関連血管炎

参考

• 血管炎症候群 - 日本内科学会雑誌第103巻第10号

• 1. 血管炎症候群の診療ガイドライン

http://www.j-circ.or.jp/guideline/pdf/JCS2008_ozaki_h.pdf

• 血管炎・血管障害診療ガイドライン 2016 年改訂版 - 日本皮膚科学会

https://www.dermatol.or.jp/uploads/uploads/files/vasculitisGL.pdf

「原発性中枢神経系血管炎」

　　[★]

英

primary central nervous system vasculitis

「primary」

　　[★]

• adj.

• 一次の、第一次の、主要な、(化学)第一級の、一級の、初生の、(病名)原発性の、原発の、プライマリーの

関

cardinal、chief、first-order、foremost、main、mainly、major、master、primarily、principal、principally

　 　 　 　 　 　

「nervous」

　　[★]

• adj.

• 神経の、心配な、神経質な

関

anxiety、anxious、anxiously、care、concern、fear、nerve、nervi、nervousness、nervus、neural、neuro、neuron、neuroticism、trouble、uneasy、worrisome、worry

　 　 　

「central」

　　[★]

• adj.

• 中心の、中心的な、中心性の、中枢的な、中枢性の、中枢の、中枢神経性の

関

center、centrally、centrally acting、centre、centro、centrum、hub、medial、pivotal、pivotally

　 　 　

「system」

　　[★]

• n.

• 系、システム、体系、体制、方式

関

method、series、strain