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Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome

  • Cardiovascular Disease and Stroke (D Leifer and JE Safdieh, Section Editors)
  • Published:
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Abstract

Primary angiitis of the central nervous system (PACNS) is one of the most devastating pathologic processes that affect the central nervous system (CNS). It results in exclusive inflammation and destruction of CNS blood vessels. Progressive debilitating unexplained neurological deficit associated with abnormal cerebrospinal fluid (CSF) analysis findings is the typical picture of the disease. CNS biopsy is the gold standard diagnostic test. Immunosuppressive therapy is the core treatment. Reversible cerebral vasoconstriction syndrome (RCVS) is a main mimic of PACNS. RCVS is characterized clinically by recurrent thunderclap headache with or without neurological deficit and normal CSF analysis findings and angiographically by reversible diffuse segmental vasospasm of intracranial vessels. A stepwise diagnostic approach should be followed to differentiate PACNS from RCVS and exclude the other clinical, radiographic, and angiographic mimics.

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Conflict of Interest

Tariq A. Hammad declares no conflict of interest.

Rula A. Hajj-Ali declares no conflict of interest.

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Authors and Affiliations

  1. Orthopedic and Rheumatologic Institute, Rheumatologic and Immunologic, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Tariq A. Hammad

  2. Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Center for Vasculitis Care and Research, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Rula A. Hajj-Ali

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Correspondence to Rula A. Hajj-Ali.

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Hammad, T.A., Hajj-Ali, R.A. Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome. Curr Atheroscler Rep 15, 346 (2013). https://doi.org/10.1007/s11883-013-0346-4

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