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Article

Primary necrotizing lymphocytic central nervous system vasculitis due to perforin deficiency in a four-year-old girl.

Arthritis and rheumatism - 1 Mar 2007

Moshous Despina, Feyen Oliver, Lankisch Petra, Schwarz Klaus, Schaper Jörg, Schneider Marion, Dilloo Dagmar, Laws Hans-Jürgen, Schwahn Bernd C, Niehues Tim

Abstract excerpt

We report the case of a 4-year-old girl who presented with headaches, ataxia, and visual disturbances. Cranial magnetic resonance imaging showed multiple supra- and infratentorial lesions with peripheral contrast enhancement and central necrosis. Brain biopsy revealed necrotizing lymphocytic vasculitis of undetermined etiology. Perforin expression was found to be significantly reduced in the patient's peripheral...

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Share to Lymphocytes

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Lymphohistiocytosis, Hemophagocytic

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Source

Identifiers

PMID: 17328077
DOI: 10.1002/art.22442

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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