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Primary Central Nervous System Vasculitis Yukio Takeshita 1 1Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine Keyword: primary central nervous system vasculitis , 脳生検 , アンギオグラフィー , シクロホスファミド , 免疫抑制薬 , cerebral biopsy , angiography , cyclophosphamide , immunosuppressants pp.511-515
Published Date 2021/5/1
DOI https://doi.org/10.11477/mf.1416201793
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Abstract

Primary central nervous system (CNS) vasculitis is an uncommon disorder of unknown etiology that is restricted to the brain and spinal cord. Patients do not present with specific clinical features or a classical clinical course, and no blood or imaging investigations are available for diagnostic confirmation. Cerebral biopsy and angiography are the gold standards for diagnosis. No randomized clinical trials have described a therapeutic regimen effective for primary CNS vasculitis; therefore, treatment of primary CNS vasculitis is based on therapeutic strategies used for other types of vasculitis. Early diagnosis is important because corticosteroid-based treatment with or without concomitant cyclophosphamide administration can often prevent serious outcomes and may be followed by a favorable response. Several immunosuppressants such as mycophenolate mofetil, tumor necrosis factor-α blockers, and rituximab may be useful options for patients refractory to the aforementioned regimen. We describe some diagnostic and therapeutic approaches for the management of this condition, with a focus on the importance of obtaining tissue and angiographic evaluation (formal contrast-enhanced or magnetic resonance/computed tomography angiography).


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電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

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