Immune reconstitution inflammatory syndrome of the brain: Case illustrations of a challenging entity

Elisabeth J. Rushing, Angelike Liappis, James D. Smirniotopoulos, Alice B. Smith, James M. Henry, Yan Gao Man, Ann M. Nelson

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

Abstract

Immune reconstitution inflammatory syndrome represents a spectrum of clinicopathologic entities encountered in human immunodeficiency virus-infected patients who have received highly active anti-retroviral therapy. The diagnosis is often challenging, treatment options are limited, and the prognosis is variable. To increase awareness and define the clinicopathologic features, we present our experience with 6 probable cases involving the brain, including 1 autopsy. Clinicopathologic review was supplemented by immunohistochemical analysis. There were 5 men and 1 woman, ranging in age from 34 to 47 (mean, 41; SD, 5.39) years. All patients experienced neurologic deterioration (focal deficits in 5/6) after highly active anti-retroviral therapy. All specimens showed a predominance of CD8+ lymphocytic inflammation. Concurrent CNS infections included human immunodeficiency virus encephalitis, progressive multifocal leukoencephalopathy, cryptococcal meningitis, and syphilis. One patient died, 1 was lost to follow-up, 2 improved, and 2 showed no substantial clinical changes. Subtle and overlapping features may preclude a definitive diagnosis. To capture all suspected cases, it is important to consider the possibility of this entity. In this study, the degree of CD8+ inflammation was more pronounced in the single fatal example, and mast cells were not identified in the infiltrates. Although nonspecific, imaging findings may offer clues to early diagnosis.

Original languageEnglish (US)
Pages (from-to)819-827
Number of pages9
JournalJournal of Neuropathology and Experimental Neurology
Volume67
Issue number8
DOIs
StatePublished - Aug 2008

Keywords

  • Brain
  • CD8
  • Highly active anti-retroviral therapy
  • Human immunodeficiency virus
  • Immune reconstitution inflammatory syndrome
  • Lymphocytes
  • Mast cells

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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