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  • Title: Catastrophic primary central nervous system vasculitis.
    Author: Salvarani C, Brown RD, Morris JM, Huston J, Hunder GG.
    Journal: Clin Exp Rheumatol; 2014; 32(3 Suppl 82):S3-4. PubMed ID: 24854370.
    Abstract:
    Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV. These patients are characterised by bilateral, multiple, large cerebral vessel lesions on angiograms and multiple bilateral cerebral infarctions.
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