Towards a histological diagnosis of childhood small vessel CNS vasculitis.

ABSTRACT:

Background

Primary small vessel CNS vasculitis (sv-cPACNS) is a challenging inflammatory brain disease in children. Brain biopsy is mandatory to confirm the diagnosis. This study aims to develop and validate a histological scoring tool for diagnosing small vessel CNS vasculitis.

Methods

A standardized brain biopsy scoring instrument was developed and applied to consecutive full-thickness brain biopsies of pediatric cases and controls at a single center. Stains included immunohistochemistry and Hematoxylin & Eosin. Nine North American neuropathologists, blinded to patients' presentation, diagnosis, and therapy, scored de-identified biopsies independently.

Results

A total of 31 brain biopsy specimens from children with sv-cPACNS, 11 with epilepsy, and 11 with non-vascul

SUBMITTER: Nouri MN 

PROVIDER: S-EPMC11682624 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature