Adult Primary Central Nervous System Vasculitis
Autor: | Carlo Salvarani, Robert D. Brown, Caterina Giannini, Gene G. Hunder |
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Rok vydání: | 2016 |
Předmět: |
Vasculitis
Pathology medicine.medical_specialty Central nervous system 03 medical and health sciences Magnetic resonance imaging 0302 clinical medicine Biopsy medicine Cyclophosphamide Glucocorticoids Stroke 030203 arthritis & rheumatology Angiography Cerebral biopsy medicine.diagnostic_test business.industry medicine.disease Giant cell arteritis medicine.anatomical_structure Differential diagnosis business 030217 neurology & neurosurgery Cerebral angiography Cerebral vasculitis |
Zdroj: | Systemic Vasculitides: Current Status and Perspectives ISBN: 9783319401348 Large and Medium Size Vessel and Single Organ Vasculitis ISBN: 9783030671747 |
DOI: | 10.1007/978-3-319-40136-2_22 |
Popis: | Primary central nervous system vasculitis is an uncommon disorder of unknown cause that is limited to the brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but commonly include headache, altered cognition, focal weakness, or stroke. Serological markers of inflammation are usually normal. Cerebrospinal fluid is abnormal in approximately 80–90 % of the cases. The diagnosis is unlikely in the presence of a normal magnetic resonance imaging (MRI) of the brain. Biopsy of central nervous system tissue showing vasculitis is the only definitive test, however angiography has often been used for diagnosis even though it has only moderate sensitivity and specificity. The size of the vessels involved is varied and influences outcomes and response to treatment. Early recognition is important because treatment with corticosteroids, with or without cytotoxic drugs, may prevent serious outcomes. The differential diagnosis includes reversible cerebral vasoconstriction syndromes and secondary cerebral vasculitis. |
Databáze: | OpenAIRE |
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