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Central nervous system vasculitis in adults and children
Marinka Twilt1, Susanne M Benseler2
1Division of Rheumatology, Department of Pediatrics, Aarhus University Hospital and Faculty of Medicine, University of Aarhus, Aarhus, Denmark; Division of Rheumatology, Department of Paediatrics, Alberta Children's Hospital, Alberta Children's Hospital Research Institute, Cumming School of Medicine, University of Calgary, Alberta, Canada.
Handbook of Clinical Neurology
|April 27, 2016
Summary
Primary angiitis of the central nervous system (PACNS) is a treatable inflammatory brain condition. Early diagnosis and tailored treatments improve outcomes, but more research on long-term effects is needed.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare inflammatory condition affecting brain blood vessels.
- It presents with diverse neurological, cognitive, and psychiatric symptoms.
- Early diagnosis and intervention are crucial for potentially reversible inflammation.
Purpose of the Study:
- To review the diagnosis, classification, and management of PACNS.
- To highlight the importance of differentiating subtypes and age-specific presentations (childhood vs. adult PACNS).
- To discuss the role of biomarkers and tailored treatment strategies.
Main Methods:
- Review of existing literature on PACNS.
- Analysis of diagnostic criteria, including neuroimaging and histopathology.
- Evaluation of current and novel biomarkers (e.g., von Willebrand factor, cytokines).
- Assessment of treatment approaches and outcomes.
Main Results:
- PACNS diagnosis requires excluding mimics and considering age-specific differentials.
- Subtypes of PACNS exhibit distinct clinical, imaging, and pathological features.
- Biomarkers aid in diagnosis, subtyping, and assessing disease activity.
- Treatment should be individualized, combining immunosuppression with symptomatic management.
Conclusions:
- PACNS management requires a subtype-specific and patient-centered approach.
- While mortality has decreased, further research into PACNS morbidity and its determinants is essential.
- Optimizing rehabilitation through symptom control is key for patient recovery.
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