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Catastrophic primary central nervous system vasculitis.

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.

journal_name

Clin Exp Rheumatol

journal_title

Clinical and experimental rheumatology

authors

Salvarani C,Brown RD Jr,Morris JM,Huston J 3rd,Hunder GG

subject

Has Abstract

pub_date

2014-05-01 00:00:00

pages

S3-4

issue

3 Suppl 82

eissn

0392-856X

issn

1593-098X

pii

7965

journal_volume

32

pub_type

杂志文章

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