関: central nervous system angiitis、central nervous system vasculitis、cerebral angiitis、cerebral vasculitis、granulomatous angiitis、granulomatous arteritis

WordNet

the 3rd letter of the Roman alphabet (同)c
(music) the keynote of the scale of C major
a general-purpose programing language closely associated with the UNIX operating system
inflammation of a blood vessel

PrepTutorEJDIC

carbonの化学記号

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/11/01 16:42:22」(JST)

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Cerebral vasculitis or central nervous system vasculitis (sometimes the word angiitis is used instead of "vasculitis") is vasculitis (inflammation of the blood vessel wall) involving the brain and occasionally the spinal cord. It may produce a wide range of neurological symptoms, such as headache, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behaviour, as well as the phenomena of a mass lesion in the brain leading to coma and herniation. Some of its symptoms may resemble multiple sclerosis.^[1] 10% have associated cerebral hemorrhage.^[2]

Causes[edit]

"Primary" CNS vasculitis is said to be present if there is no underlying cause. More commonly, the disease occurs in the setting of other medical conditions: infections, other forms of systemic vasculitis such as Wegener's granulomatosis or polyarteritis nodosa, connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, particular medications and drugs (amphetamine, cocaine and heroin), certain forms of cancer (particularly lymphomas, leukemia and lung cancer) and Behçet's disease. It may imitate, and is in turn imitated by, a number of other diseases that affect the blood vessels of the brain diffusely such as fibromuscular dysplasia and thrombotic thrombocytopenic purpura.^[2]

Diagnosis and treatment[edit]

Cerebral angiography and magnetic resonance imaging, and ultimately biopsy of the brain, are often required for its diagnosis. Once confirmed, various tests are often performed to establish whether the vasculitis is primary or the result of an underlying cause. Treatment is with glucocorticoids, often with additional immunosuppression with medication such as cyclophosphamide to decrease the immune system's attack on the body's own tissues.^[2] Cerebral vasculitis is a very rare condition that is difficult to diagnose, and as a result there are significant variations in the way it is diagnosed and treated.^[3]

References[edit]

^ Scolding NJ, Jayne DR, Zajicek JP, Meyer PA, Wraight EP, Lockwood CM (January 1997). "Cerebral vasculitis--recognition, diagnosis and management". QJM 90 (1): 61–73. PMID 9093590.
^ ^a ^b ^c Rehman HU (November 2000). "Primary angiitis of the central nervous system" (PDF). J R Soc Med 93 (11): 586–8. PMC 1298150. PMID 11198690.
^ Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I, Gilhus N (July 2002). "The recognition, diagnosis and management of cerebral vasculitis: a European survey". Eur. J. Neurol. 9 (4): 343–7. doi:10.1046/j.1468-1331.2002.00422.x. PMID 12099915.

External links[edit]

Central Nervous System Vasculitis at the Johns Hopkins Hospital vasculitis centre
Primary angiitis of the central nervous system from the Cleveland Clinic

UpToDate Contents

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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
2. 成人の血管炎の分類とそれに対するアプローチ classification of and approach to the vasculitides in adults
3. Early-onset dementia in adults
4. 小児期の中枢神経系原発性血管炎 childhood primary angiitis of the central nervous system
5. 関節リウマチの神経学的症状 neurologic manifestations of rheumatoid arthritis

English Journal

ANCA-associated vasculitis in patients with primary Sjögren's syndrome: Detailed analysis of 7 new cases and systematic literature review.

Guellec D1, Cornec-Le Gall E2, Groh M3, Hachulla E4, Karras A5, Charles P6, Dunogué B3, Abad S7, Alvarez F8, Gérard F1, Devauchelle-Pensec V9, Pers JO10, Puéchal X3, Guillevin L3, Saraux A9, Cornec D11; CRI (Club Rhumatismes et Inflammation) and the French Vasculitis Study Group.
Autoimmunity reviews.Autoimmun Rev.2015 Aug;14(8):742-750. doi: 10.1016/j.autrev.2015.04.009. Epub 2015 Apr 24.
OBJECTIVES: To describe the clinical presentation, management and prognosis of patients diagnosed with both primary Sjögren's syndrome (pSS) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).METHODS: French nation-wide survey completed by a systematic literature review.RES
PMID 25916811

Clinical presentations of Behçet's disease among Nigerians: a 4-year prospective study.

Ajose FO1, Adelowo O1, Oderinlo O2.
International journal of dermatology.Int J Dermatol.2015 Aug;54(8):889-97. doi: 10.1111/ijd.12554. Epub 2014 Dec 12.
BACKGROUND: Behçet's disease (BD) is a chronic, multisystem, inflammatory pan-vasculitis of unknown etiology, with heterogeneous presentations in different parts of the world. It commonly involves the mucosae, skin, joints, eyes, and central nervous system. Behçet's disease is considered to repres
PMID 25494839

Ischemic and Hemorrhagic Strokes due to Eosinophilic Granulomatosis with Polyangiitis.

Aly Z1, Libman R2, Seidman R3, Arora R1, Siller K1, Salamon E1, Katz JM1.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.J Stroke Cerebrovasc Dis.2015 Jul 16. pii: S1052-3057(15)00352-3. doi: 10.1016/j.jstrokecerebrovasdis.2015.06.027. [Epub ahead of print]
BACKGROUND: We report a case of ischemic and hemorrhagic strokes occurring almost simultaneously in a patient diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) previously known as Churg-Strauss vasculitis. To our knowledge, this is the first known case.METHODS: A 59-year-old man pr
PMID 26190306

Japanese Journal

ε4/ε2のアポリポ蛋白E遺伝子型を有したAmyloid-β-related cerebral angiitisの1例

小倉礼,守吉秀行,中井紀嘉,西田卓,北川諭,吉田眞理,安田武司,伊藤泰広
臨床神経学 55(8), 561-566, 2015
症例は53歳男性である．歩行障害・認知機能障害が半年の経過で進行し，頭部MRIで右後頭葉脳表の造影効果をともなうT2WI高信号病変をみとめた．脳生検でAβ関連血管炎（amyloid-β-related angiitis; ABRA）と診断した．生検後に痙攣を反復したため早期にステロイド治療を導入し，プレドニゾロン単独で良好に経過した．ApoE遺伝子型はまれなε4/ε2を示した．ε4はABRAでもっ …
NAID 130005094163

ε4/ε2のアポリポ蛋白E遺伝子型を有したAmyloid-β-related cerebral angiitisの1例

小倉礼,守吉秀行,中井紀嘉,西田卓,北川諭,吉田眞理,安田武司,伊藤泰広
臨床神経学 advpub(0), 2015
症例は53歳男性である．歩行障害・認知機能障害が半年の経過で進行し，頭部MRIで右後頭葉脳表の造影効果をともなうT2WI高信号病変をみとめた．脳生検でAβ関連血管炎（amyloid-β-related angiitis; ABRA）と診断した．生検後に痙攣を反復したため早期にステロイド治療を導入し，プレドニゾロン単独で良好に経過した．ApoE遺伝子型はまれなε4/ε2を示した．ε4はABRAでもっ …
NAID 130005078354

症例報告くりかえす脳梗塞が診断の契機となった中枢神経限局型ANCA関連血管炎の1例

脇坂佳世,萩原のり子,金澤有華 [他]
臨床神経学 = Clinical neurology 54(5), 429-433, 2014-05
NAID 40020092755

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リンク元	「granulomatous angiitis」「granulomatous arteritis」「cerebral angiitis」「cerebral vasculitis」「central nervous system angiitis」
関連記事	「C」「CNS」