Catastrophic primary central nervous system vasculitis

CER7310
2014 Vol.32, N°3 ,Suppl.82
PI 0003, PF 0004
Imaging

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PMID: 24854370 [PubMed]

Received: 02/02/2014
Accepted : 12/03/2014
In Press: 15/05/2014
Published: 16/05/2014

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.