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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
Julius Birnbaum1, David B Hellmann
1Division of Molecular and Clinical Rheumatology, Department of Medicine, The Johns Hopkins University School of Medicine, The Johns Hopkins Bayview Medical Center, Baltimore, Maryland 21224, USA.
Archives of Neurology
|June 10, 2009
Summary
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis often presenting with headache and encephalopathy. Diagnosis requires brain biopsy, with treatment involving cyclophosphamide and prednisone.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare, idiopathic vasculitis affecting CNS blood vessels.
- Typically seen in individuals around 50 years old, with a 2:1 male-to-female ratio.
- Headache and encephalopathy are the most common initial symptoms, while stroke is less frequent at onset.
Purpose of the Study:
- To summarize the key features, diagnostic challenges, and treatment of PACNS.
- To differentiate PACNS from conditions with similar presentations, such as reversible cerebral vasoconstriction syndrome (RCVS).
Main Methods:
- Review of clinical presentation, diagnostic findings, and treatment outcomes for PACNS.
- Comparison of PACNS characteristics with those of RCVS.
Main Results:
- PACNS diagnosis is confirmed via brain biopsy; imaging and CSF analysis are often nonspecific.
- Serological markers of inflammation are usually normal, and extracranial vasculitis is rare.
- RCVS differs from PACNS, often affecting younger women with thunderclap headaches and normal CSF.
Conclusions:
- PACNS is a challenging diagnosis requiring a high index of suspicion and definitive brain biopsy.
- Cyclophosphamide and prednisone are standard treatments for biopsy-proven PACNS.
- Distinguishing PACNS from RCVS is crucial for appropriate management and patient outcomes.
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