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  • Title: Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease.
    Author: Benseler SM, deVeber G, Hawkins C, Schneider R, Tyrrell PN, Aviv RI, Armstrong D, Laxer RM, Silverman ED.
    Journal: Arthritis Rheum; 2005 Jul; 52(7):2159-67. PubMed ID: 15986347.
    Abstract:
    Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.
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