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Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
Published on: March 26, 2019
Primary angiitis of the central nervous system
1Department of Internal Medicine, Hôtel-Dieu, Centre Hospitalier Universitaire de Nantes, France.
Clinical and Experimental Rheumatology
|August 4, 2009
Summary
Primary angiitis of the central nervous system (PACNS) is rare but increasingly understood. Advances in diagnosis and treatment, including immunosuppressants, have improved outcomes, though optimal therapy requires further study.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare, challenging condition with variable, non-specific clinical presentations.
- Distinguishing PACNS from secondary vasculitis and mimickers is crucial for accurate diagnosis and management.
- Recent large retrospective analyses and recognition of reversible cerebral vasoconstriction syndrome have advanced the field.
Purpose of the Study:
- To review current understanding and recent advances in the diagnosis and management of PACNS.
- To highlight the diagnostic challenges, including the role of neuroimaging and biopsy.
- To discuss current therapeutic strategies and identify areas for future research.
Main Methods:
- Review of recent retrospective analyses of large PACNS patient cohorts.
- Analysis of diagnostic criteria, including neuroimaging and histopathology.
- Evaluation of current treatment regimens and their impact on prognosis.
Main Results:
- Neuroimaging can suggest vasculitis, but leptomeningeal biopsy is definitive, though not always feasible.
- Improved prognosis is attributed to corticosteroids and immunosuppressants like cyclophosphamide.
- Optimal therapeutic regimens and duration for PACNS remain undetermined.
Conclusions:
- PACNS diagnosis requires awareness of differential diagnoses and mimickers.
- While prognosis has improved, further multicenter studies and prospective trials are needed to optimize therapy and understand physiopathogenesis.
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