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Published on: March 26, 2019
Primary angiitis of the central nervous system
Rula A Hajj-Ali1, Leonard H Calabrese
1Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA. hajjalr@ccf.org
Primary angiitis of the central nervous system (PACNS) diagnosis remains challenging despite advances. This review highlights clinical presentation, diagnosis, and mimics like reversible cerebral vasoconstriction syndromes (RCVS).
Area of Science:
- Neurology
- Vascular Neurology
- Immunology
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare condition affecting blood vessels in the brain and spinal cord.
- Accurate diagnosis of PACNS is often difficult due to its varied presentation and overlap with other neurological disorders.
Purpose of the Study:
- To review the current understanding of PACNS, focusing on diagnostic challenges and recent advancements.
- To emphasize the importance of differentiating PACNS from its mimics, particularly reversible cerebral vasoconstriction syndromes (RCVS).
- To summarize current treatment strategies for PACNS.
Main Methods:
- Literature review of primary angiitis of the central nervous system (PACNS).
- Analysis of clinical presentations, diagnostic criteria, and imaging findings.
- Comparison with differential diagnoses, including reversible cerebral vasoconstriction syndromes (RCVS).
Main Results:
- Despite progress, PACNS diagnosis presents significant challenges.
- Reversible cerebral vasoconstriction syndromes (RCVS) are a key differential diagnosis to consider.
- A comprehensive approach to diagnosis and treatment is crucial for effective management.
Conclusions:
- Early and accurate diagnosis of PACNS is critical for patient outcomes.
- Awareness of mimics like RCVS improves diagnostic accuracy.
- Further research is needed to refine treatment protocols for PACNS.
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