The New England Journal of MedicinePrimary Central Nervous System Vasculitis

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Primary central nervous system (CNS) vasculitis is a rare inflammatory condition affecting the brain and spinal cord. This review, published in the New England Journal of Medicine, provides an updated overview of the epidemiology, clinical manifestations, pathology, diagnosis, and treatment of this complex disorder. The authors emphasize the importance of early recognition and appropriate management to improve patient outcomes.

Key Points:

• Primary CNS vasculitis has an estimated annual incidence of 2.4 cases per 1 million person-years, affecting all ages with similar prevalence among males and females.
• Clinical presentations vary widely, including focal neurologic deficits, headaches, cognitive decline, and encephalopathy. Two subsets are identified: small-vessel and medium-to-large-vessel involvement.
• Diagnosis remains challenging, often requiring a combination of clinical, laboratory, imaging, and histopathological findings. Brain biopsy remains the gold standard for definitive diagnosis.
• Cerebral angiography, while commonly used, has low sensitivity (15-43%) and may not detect small-vessel involvement.
• Treatment typically involves high-dose glucocorticoids, often combined with cyclophosphamide for severe cases. Mycophenolate mofetil may be an effective alternative.
• Maintenance therapy with mycophenolate mofetil or azathioprine is associated with lower relapse rates and better long-term outcomes.
• Factors associated with poor prognosis include older age at diagnosis, cognitive dysfunction at presentation, and medium-to-large-vessel involvement with cerebral infarctions.

HCN Medical Memo
Primary CNS vasculitis remains a diagnostic and therapeutic challenge. Clinicians should maintain a high index of suspicion in patients presenting with unexplained neurological symptoms, particularly when accompanied by inflammatory CSF findings. Early diagnosis and appropriate immunosuppressive therapy are crucial for improving patient outcomes.

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