Cryptococcal meningoencephalitis
Cryptococcal meningoencephalitis is the most common cause of fungal meningitis worldwide, driven by CNS dissemination of inhaled Cryptococcus in an immunosuppressed host, typically HIV with a CD4 count under 50; treatment is induction amphotericin B plus flucytosine, consolidation and maintenance fluconazole, and aggressive lowering of elevated intracranial pressure.
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What this article covers
- Overview
The most common cause of fungal meningitis worldwide: cryptococcal meningoencephalitis results from central nervous system dissemination of Cryptococcus, an encapsulated yeast that is ubiquitous in the environment.
- Epidemiology
The most common manifestation of C. neoformans disease in human immunodeficiency virus (HIV)-infected patients, usually occurring when the helper T-cell (CD4) count is less than 50/microliter.
- Risk factors
HIV infection with a CD4 cell count under 50/microliter: the dominant risk factor and the population in whom disease is most severe.
- Pathophysiology
Inhalation of aerosolized Cryptococcus spores into the respiratory tract is the initial event, producing asymptomatic or mild pulmonary infection in most exposed persons.
- Clinical features
Insidious onset over days to weeks: fever, headache, and malaise with a waxing and waning course, unlike the acute onset of bacterial meningitis.
- Differential diagnosis
Tuberculous meningitis: a subacute course with CSF lymphocytic pleocytosis, elevated protein, and low glucose overlapping cryptococcal meningitis, but acid-fast smear is insensitive and culture…
- Diagnosis
Cryptococcal meningoencephalitis is confirmed by a positive CSF fungal culture (the gold standard) or a positive CSF cryptococcal antigen test, which has a sensitivity of 93% to 99%; severity and…
- Management
First, start induction antifungal therapy immediately for at least 2 weeks, which is effective in more than 90% of patients; extend induction to 4-6 weeks total in non-HIV, non-transplant patients or…
- Complications
Sudden blindness, deafness, or coma from uncontrolled elevated intracranial pressure: the principal driver of morbidity and the reason opening pressure must be documented and treated at every lumbar puncture.
- Prognosis
Adverse prognostic factors identified at diagnosis include altered mental status, visual abnormalities, a CSF leukocyte count under 20/microliter, a CSF cryptococcal antigen titer greater than…
- Prevention
No vaccine exists against Cryptococcus; prevention centers on maintaining immune competence and prompt treatment of at-risk hosts.
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