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Diagnosing central nervous system vasculitis in children
Tania Cellucci1, Susanne M Benseler
1Division of Rheumatology, Department of Pediatrics, Research Institute, The Hospital for Sick Children, and Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.
Insights
Childhood central nervous system vasculitis (cPACNS) requires a tailored diagnostic and treatment approach. Early diagnosis and aggressive therapy improve outcomes for this serious inflammatory brain disease.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Vascular Neurology
Background:
- Childhood primary angiitis of the central nervous system (cPACNS) is an inflammatory brain disease with severe neurological consequences.
- Accurate diagnosis is challenging due to overlapping features with other neurological conditions.
- Recent evidence highlights the importance of a personalized diagnostic strategy.
Purpose of the Study:
- To review current literature on childhood central nervous system vasculitis.
- To discuss a tailored approach to diagnosis and treatment based on recent evidence.
- To improve understanding and management of cPACNS.
Main Methods:
- Literature review of current evidence on cPACNS.
- Analysis of diagnostic yield of brain biopsies in children.
- Evaluation of outcomes based on treatment strategies.
Main Results:
- Elective brain biopsies in children show higher diagnostic yield for cPACNS compared to adults.
- A tailored diagnostic approach is crucial given the expanding spectrum of differential diagnoses.
- Early diagnosis and aggressive treatment are associated with improved neurological outcomes and reduced mortality.
Conclusions:
- Improved understanding of cPACNS facilitates a tailored diagnostic approach.
- Earlier diagnosis and initiation of therapy are critical for this potentially reversible condition.
- This review provides updated insights for managing cPACNS.
Purpose Of Review:
To review the current literature of childhood central nervous system vasculitis, and to discuss a tailored approach to diagnosis and treatment based on recent evidence.
Recent Findings:
Primary angiitis of the central nervous system in children (cPACNS) is an increasingly recognized inflammatory brain disease with potentially devastating neurological consequences. The diagnostic approach should be tailored to the clinical presentation of the child with suspected cPACNS and should address the expanding spectrum of inflammatory and noninflammatory brain diseases with overlapping clinical features. New evidence has confirmed that elective brain biopsies in children have a higher diagnostic yield than in adults and improve our ability to diagnose angiography-negative cPACNS. Finally, observational studies have shown that early diagnosis and aggressive treatment lead to improved neurological outcomes and lower mortality rates in patients with cPACNS.
Summary:
This review summarizes the recent data on diagnosis, classification, treatment, and outcomes in cPACNS. Our improved understanding of cPACNS facilitates a tailored diagnostic approach that results in earlier diagnosis and initiation of therapy for this potentially reversible condition.
