Document Detail

Brain biopsy in children with primary small-vessel central nervous system vasculitis.
MedLine Citation:
PMID:  21031577     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Primary angiitis of the central nervous system in childhood (cPACNS) is an immune-mediated inflammatory process directed toward blood vessels in the central nervous system. It has been associated with variable clinical and radiological presentations, and devastating consequences without treatment. Brain biopsy is required for definitive diagnosis. The objective of this study was to characterize the clinical and histopathological features of brain biopsies in small-vessel cPACNS (SVcPACNS).
METHODS: A single-center prospective cohort study of children diagnosed with cPACNS from 1998 to 2008 was performed. All patients with negative cerebral angiography and brain biopsy were included. Patient data were reviewed for clinical, laboratory, and radiological characteristics at presentation. Standardized brain biopsy review protocols were established, with independent analysis by 2 neuropathologists. Histopathology was correlated with collected clinical data.
RESULTS: A total of 13 SVcPACNS patients were included. Ages ranged from 5 to 17 years. Presenting features included seizures (85%), headache (62%), and cognitive decline (54%). Brain biopsy confirmed SVcPACNS in 11 patients with intramural lymphocytic infiltrate. Two had nonspecific perivascular inflammation only. All 6 nonlesional biopsies yielded a diagnosis of SVcPACNS. Lack of specific histological features correlated with prolonged time to biopsy, prior steroid treatment, and inadequate specimen sampling.
INTERPRETATION: In children presenting with new onset severe headaches, seizures, or cognitive decline, SVcPACNS and brain biopsy should be considered. Lesional biopsies are preferred; however, nonlesional biopsies may succeed in yielding the diagnosis. Steroid treatment prior to biopsy and inadequate biopsy sampling may obscure the diagnosis in true cases of SVcPACNS.
Jorina Elbers; William Halliday; Cynthia Hawkins; Clare Hutchinson; Susanne M Benseler
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Annals of neurology     Volume:  68     ISSN:  1531-8249     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-10-29     Completed Date:  2010-11-29     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  602-10     Citation Subset:  IM    
Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
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MeSH Terms
Biopsy / methods
Brain / pathology*
Child, Preschool
Diagnostic Techniques, Neurological
Prospective Studies
Vasculitis, Central Nervous System / diagnosis,  pathology*
Comment In:
Ann Neurol. 2010 Nov;68(5):573-4   [PMID:  21031572 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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