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Related Experiment Videos

Central nervous system vasculitis in the intensive care unit.

Rula A Hajj-Ali1, Shekhar Ghamande, Leonard H Calabrese

  • 1Department of Internal Medicine, Cleveland Clinic Foundation, 9500 Euclid Avenue-G62, Cleveland, OH 44195, USA.

Critical Care Clinics
|November 7, 2002
PubMed
Summary

Primary angiitis of the central nervous system (PACNS) presents with unexplained neurologic defects in ICU patients. Early diagnosis and high-dose steroid treatment are crucial to prevent cognitive dysfunction and prolonged ICU stays.

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Area of Science:

  • Neurology
  • Critical Care Medicine
  • Immunology

Background:

  • Intensivists encounter unexplained neurologic defects in intensive care unit (ICU) patients.
  • A subacute presentation over weeks to months, including headache, altered mental status, and focal deficits, warrants suspicion for primary angiitis of the central nervous system (PACNS).
  • Delayed diagnosis of PACNS can lead to increased morbidity, prolonged ICU stays, and a risk of permanent cognitive dysfunction.

Purpose of the Study:

  • To highlight the diagnostic challenges and management strategies for primary angiitis of the central nervous system (PACNS) in ICU patients.
  • To emphasize the importance of early recognition and treatment to improve patient outcomes.

Main Methods:

  • Review of clinical presentations suggestive of PACNS in ICU settings.

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  • Discussion of diagnostic findings including cerebrospinal fluid (CSF) analysis, MR imaging, and cerebral angiography.
  • Consideration of brain biopsy for definitive diagnosis.
  • Evaluation of treatment protocols involving high-dose steroids and immunosuppressive agents.

    • Main Results:

    • While reactive CSF findings, ischemic changes on MR imaging, and angiographic alterations support PACNS, they are not diagnostic.
    • Brain biopsy may be necessary for confirmation.
    • High-dose steroid therapy, with a prolonged course and gradual taper, effectively manages the condition in most patients.
    • Some patients require additional immunosuppressive therapy.

    Conclusions:

    • Primary angiitis of the central nervous system (PACNS) is a rare but critical consideration in ICU patients with unexplained neurologic deficits.
    • Prompt diagnosis and aggressive treatment, primarily with high-dose steroids, are essential to mitigate severe neurologic sequelae and optimize ICU patient recovery.