Primary angiitis of the central nervous system

Research output: Contribution to journalArticle

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis of unknown cause. The mean age of onset is 50 years, and men are affected twice as often as women. Headache and encephalopathy are the most frequent initial symptoms. Stroke or focal symptoms develop in less than 20% of patients at the onset of disease and are uncommon in the absence of headache or encephalopathy. Symptoms or signs of vasculitis outside of the central nervous system are rare; serologic markers of inflammation are typically normal. Magnetic resonance imaging of the brain is abnormal in more than 90% of patients, but the pattern of abnormal findings is not specific. Cerebrospinal fluid analysis is also usually abnormal because of modest, nonspecific elevations in total protein level or white blood cell count. Angiography has a low sensitivity and low specificity. Most patients suspected of having PACNS have another disease. The diagnosis of PACNS is established by brain biopsy. The differential diagnosis of PACNS is broad and includes reversal cerebral vasoconstriction. In contrast to patients with PACNS, patients with reversal cerebral vasoconstriction are more often young women who experience a thunderclap headache and have a normal cerebrospinal fluid analysis. Patients with biopsyproven PACNS are treated with cyclophosphamide and prednisone.

Original languageEnglish (US)
Pages (from-to)704-709
Number of pages6
JournalArchives of Neurology
Volume66
Issue number6
DOIs
StatePublished - Jun 2009

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Brain Diseases
Vasoconstriction
Headache
Cerebrospinal Fluid
Primary Headache Disorders
Central Nervous System Vasculitis
Brain
Vasculitis
Prednisone
Leukocyte Count
Age of Onset
Cyclophosphamide
Signs and Symptoms
Primary angiitis of the central nervous system
Central Nervous System
Angiography
Differential Diagnosis
Stroke
Magnetic Resonance Imaging
Inflammation

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Primary angiitis of the central nervous system. / Birnbaum, Julius; Hellmann, David B.

In: Archives of Neurology, Vol. 66, No. 6, 06.2009, p. 704-709.

Research output: Contribution to journalArticle

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