Diagnosis of intracranial vasculitis: A multi-disciplinary approach

Charleen T. Chu, Linda Gray, Larry B. Goldstein, Christine M. Hulette

Research output: Contribution to journalArticlepeer-review

141 Scopus citations

Abstract

Intracranial vasculitis, or primary angiitis of the central nervous system (PACNS), is an uncommon, often fatal disorder that frequently responds to aggressive immunosuppressive therapy. Magnetic resonance imaging (MRI), cerebral angiography, and brain biopsy are diagnostic modalities that vary in invasiveness and diagnostic accuracy. The purpose of this study was to determine whether certain clinical or radiologic features were predictive of a diagnostic biopsy. Thirty consecutive patients undergoing brain biopsy to 'rule out vasculitis' were studied. Nine patients demonstrated granulomatous or lymphocytic vasculitis, 1 had lymphocytic vasculitis and encephalitis secondary to arbovirus infection, 5 had thickened vessels consistent with hypertensive changes, 5 had amyloid angiopathy and/or changes of Alzheimer disease, 5 demonstrated no pathologic abnormalities, and 1 each had acute infarct, vascular malformation, aneurysm, acellular fibrinoid necrosis, and demyelination. The spectrum of MRI and angiographic changes associated with PACNS were nonspecific, overlapping extensively with changes of chronic hypertension and amyloid deposition. The predictive values of brain biopsy (90-100%) were significantly higher than those of angiography (37-50%) or MRI (43-72%). In this study, morbidity associated with aggressive immunosuppression was significantly greater than that associated with cerebral angiography or brain biopsy. Thus, wedge biopsy of cortical and leptomeningeal tissues is central to the multi-disciplinary approach to a patient with clinical suspicion of PACNS.

Original languageEnglish
Pages (from-to)30-38
Number of pages9
JournalJournal of Neuropathology and Experimental Neurology
Volume57
Issue number1
DOIs
StatePublished - Jan 1998

Keywords

  • Angiography
  • Granulomatous angiitis
  • Intracranial vasculitis
  • Isolated angiitis
  • Magnetic resonance imaging
  • Pathology
  • Primary angiitis of the central nervous system

ASJC Scopus subject areas

  • General Medicine

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