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

WordNet

of or relating to the cerebrum or brain; "cerebral hemisphere"; "cerebral activity"
involving intelligence rather than emotions or instinct; "a cerebral approach to the problem"; "cerebral drama" (同)intellectual
inflammation of a blood vessel

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大脳の,脳の / 頭脳的な,知的な

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/08/26 14:28:06」(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 source | 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 source | 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 source | 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 source | 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. Overview of and approach to the vasculitides in adults
3. 小児期の中枢神経系原発性血管炎 childhood primary angiitis of the central nervous system
4. 可逆性脳血管攣縮症候群 reversible cerebral vasoconstriction syndromes
5. ベーチェット症候群の臨床症状および診断 clinical manifestations and diagnosis of behcets syndrome

English Journal

Recognising and diagnosing inflammatory brain disease.

Scolding N.
Journal of neurology, neurosurgery, and psychiatry.J Neurol Neurosurg Psychiatry.2014 Aug;85(8):e3. doi: 10.1136/jnnp-2014-308883.12.
In contrast to 'conventional' neuro-inflammatory or neuro-immune diseases like multiple sclerosis, CNS involvement in systemic inflammatory disorders can be very difficult to recognise, and even more difficult to confirm. Vasculitis, lupus, sarcoidosis and other disorders all may involve the brain a
PMID 25009322

A young boy with affected heart and mind: arterial involvement in a patient with undiagnosed Behçet's disease.

Yalcin U1, Gurses KM, Kocyigit D, Isik M, Ozer N.
Clinical and experimental rheumatology.Clin Exp Rheumatol.2014 Jul 8. [Epub ahead of print]
Behçet's disesase is a vasculitis that manifests with oral and genital ulcerations, skin lesions, uveitis, vascular, central nervous system and gastrointestinal involvement. While most patients develop mucocutaneous and genital ulcers along with eye disease, other patients may also present with art
PMID 25005759

Brain involvement in patients with inflammatory bowel disease: a voxel-based morphometry and diffusion tensor imaging study.

Zikou AK1, Kosmidou M, Astrakas LG, Tzarouchi LC, Tsianos E, Argyropoulou MI.
European radiology.Eur Radiol.2014 Jul 8. [Epub ahead of print]
OBJECTIVES: To investigate structural brain changes in inflammatory bowel disease (IBD).METHODS: Brain magnetic resonance imaging (MRI) was performed on 18 IBD patients (aged 45.16 ± 14.71 years) and 20 aged-matched control subjects. The imaging protocol consisted of a sagittal-FLAIR, a T1-wei
PMID 25001084

Japanese Journal

臨床経験精神症状を呈した再発性多発軟骨炎の1例

精神科治療学 31(2), 247-253, 2016-02
NAID 40020927156

原発性シェーグレン症候群に合併した雷鳴頭痛を伴わないreversible cerebral vasoconstriction syndrome の1 例

脳卒中 advpub(0), 2016
NAID 130005164958

中枢神経血管炎の画像診断 (特集中枢神経の血管炎)

Brain and nerve : 神経研究の進歩 67(3), 249-260, 2015-03
NAID 40020386159

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リンク元	「脳血管炎」「CNS vasculitis」「granulomatous angiitis」「granulomatous arteritis」「cerebral angiitis」
関連記事	「cerebral」