Table of ContentsView AllTable of ContentsCausesRisk FactorsSymptomsTreatmentComplicationsPrevention
Table of ContentsView All
View All
Table of Contents
Causes
Risk Factors
Symptoms
Treatment
Complications
Prevention
The diagnosis of cryptococcal meningitis involves a spinal tap to examine cerebrospinal fluid (CSF) extracted from between the bones of the lower spine. Treatment involves the aggressive use of antifungal drugs to clear the infection and alleviate symptoms.
This article explains cryptococcal meningitis, who is vulnerable to infection, and how to recognize the signs of the disease. It also describes how the infection is diagnosed, treated, and prevented, as well as the consequences of untreated cryptococcosis.
Oleg Breslavtsev / Getty Images
Cryptococcal Meningitis Contagiousness and Fungus Sources
Without the means to stem the infection, the fungus can rapidly grow and spread, causing a potentially serious lung infection calledpulmonary cryptococcosis. Symptoms include coughing, shortness of breath, chest pain, and fever.
If not recognized and treated,C. neoformanscan disseminate (spread) into the bloodstream and establish an infection in themeninges, causinginflammationreferred to asmeningitis. At this stage, the risk of death is significantly increased even with treatment.
Less Common Causes
Cryptococcus gattii(C. gattii)is a less common cause of cryptococcal meningitis, mainly isolated to tropical and subtropical regions of the planet. In sub-Saharan Africa,C. gattiiis the primary cause of cryptococcosis.
Although the rate of infection remains low, the fungus’s appearance in North America concerns public health officials.C. gattiiis known to cause disease in people with intact immune systems, not just in immunocompromised people.
Isolated cases ofC. gattiihave been seen as far south as Southern California, primarily among people with HIV/AIDS.
How Fungal Meningitis Is Different
Who Is More Likely to Acquire Cryptococcal Meningitis?
During the height of the AIDS crisis in the 1980s and 1990s, cryptococcal meningitis was a leading cause of death amongpeople living with HIV, not only in the United States but around the world.
Even so, around 152,000 cases of cryptococcal meningitis are diagnosed in people with HIV each year, mainly in sub-Saharan Africa, resulting in around 112,000 deaths annually.
All told, around 90% of cryptococcal infections are in people with HIV, typically those who are untreated anddiagnosed with AIDS(the most advanced stage of HIV infection).
Other people with severely weakened immune systems are also at risk of cryptococcal meningitis, including:
First Symptoms of Cryptococcal Meningitis
In those who develop symptoms, the infection is generallysubacute. This means that the symptoms are less profound and, therefore, more easily dismissed or ignored.
For this reason, many people with HIV will only experience illness whenC. neoformanshas disseminated and causes meningitis ormeningoencephalitis(inflammation of the meninges and brain).
Most cases start relatively mildly, usually around two weeks after an infection has been established, causing generalized and nonspecific symptoms (those that could be attributed to a number of conditions) like:
These symptoms can develop over many weeks before the so-called classic signs of meningitis appear.
Other Symptoms Over the Course of Infection
The classic signs of cryptococcal meningitis develop in between one-quarter and one-third of people with cryptococcosis. The people most likely to experience this are those with a severely suppressed immune system. In those with HIV, this is defined as having aCD4 lymphocyte count(a measurement of a type of white blood cell) of under 100.
Classic signs and symptoms of cryptococcal meningitis include:
If meningoencephalitis develops, a person may also experience lethargy (tiredness), personality changes, memory loss,hallucinations, and reduced consciousness due toincreased pressure inside the brain.
As a disseminated infection, additional symptoms can develop if other organ systems are affected. The skin and lungs are most commonly affected, causing skin ulcers or a pox-like rashor breathing problems likeacute respiratory distress.
Signs and Symptoms of Meningitis
How to Get a Meningitis Diagnosis
Neck stiffness and photophobia are the two telltale signs of meningitis that lead many people to seek a diagnosis. Because cryptococcal meningitis can sometimes be the first symptom of HIV,the diagnostic process can take time.
If meningitis is suspected, the healthcare provider will obtain a sample ofcerebrospinal fluid (CSF)using alumbar puncture(also known as a spinal tap).
The sample is then sent to the lab to check for signs ofC. neoformansusing the following tests:
If the HIV status of the person is unknown, anHIV testwould also be ordered.
How Meningitis Is Diagnosed
Cryptococcal Meningitis Treatment and Infection Time
If you have cryptococcal meningitis, the infection will not clear on its own. You will need immediate, urgent treatment with a combination ofantifungal drugswith strong action againstC. neoformansandC. gattii.
The treatment of cryptococcal meningitis consists of three phases: the induction, consolidation, and maintenance phases.
In Hospital
According to guidelines from the Infectious Diseases Society of America (IDSA) and the World Health Organisation (WHO), recommended drug therapies for the induction phase include:
Upon completion of the induction phase, most people can return home to continue treatment.
At Home
Theconsolidation phasestarts immediately upon your release from the hospital. The IDSA and WHO recommend the following treatment:
After two weeks of the consolidation phase, another spinal tap is performed. If there is no evidence of fungus in your CSF, the fluconazole dose can be dropped to 400 mg once daily for the remainder of this phase.
If fungus is found, fluconazole will continue to be taken at the prescribed dose until sterilization (complete fungal clearance) is achieved. This may take far longer than eight weeks.
Ongoing Maintenance Therapy
Once sterilization is achieved, themaintenance phasebegins. This involves taking a daily dose of fluconazole to prevent you from gettingC. neoformansor any other opportunistic fungal infection.
The IDSA and WHO recommend the following:
This strategy, also known asprophylactic therapy, helps prevent fungal opportunistic infections until your immune system is strong enough to defend itself.
Maintenance therapy with daily low-dose fluconazole would continue until your immune system reaches a “safe zone” (typically a CD4 count of over 100) and aviral load testshows that HIV levels are undetectable in your blood for at least three months.
Once these goals are met, maintenance therapy can be stopped. However, some people may need ongoing and even permanent fluconazole treatment if their immune systems cannot be adequately restored.
Can Cryptococcosis Be Cured?With appropriate treatment, cryptococcal meningitis can be cured. On the other hand, HIV cannot be cured, but with proper treatment, you can live a long, healthy life and avoid cryptococcosis and other serious opportunistic infections.
Can Cryptococcosis Be Cured?
With appropriate treatment, cryptococcal meningitis can be cured. On the other hand, HIV cannot be cured, but with proper treatment, you can live a long, healthy life and avoid cryptococcosis and other serious opportunistic infections.
Secondary Complications From Cryptococcal Meningitis
Cryptococcal meningitis rarely occurs in the absence of a severe breakdown of your immune system. Without the means to stop the disease from spreading, cryptococcus meningitis is invariably fatal if left untreated.
Regardless of your immune status, cryptococcal meningitis can lead tocerebral infarctions(obstruction of blood flow to the brain) and permanent brain damage if not treated appropriately.
This can lead to irreversible and potentially devastating complications like:
What Is the Risk of Death?
Even with treatment, cryptococcal meningitis carries a high risk of death. According to a 2019 study in theJournal of Acquired Immune Deficiency Syndrome,around 26% of people with HIV who get cryptococcal meningitis will die as a result of the infection.
Fungal Infections of the Nervous System
IRIS and Risks of Delayed HIV Therapy
IRIS occurs in people with very low CD4 counts who suddenly start antiretroviral therapy. Freed of the burden of HIV, the immune system can sometimes overreact to an organism likeC. neoformansand launch a potentially deadly, whole-body inflammatory assault.
Studies suggest that 1 in 4 people co-infected with HIV with cryptococcal meningitis will experience IRIS within four months of starting HIV therapy. Of these, 1 out of 10 will die as a result of respiratory failure and damage to thecentral nervous system.
To reduce the risk, health experts recommend starting antiretroviral therapy four to six weeks after antifungal therapy. Your immune system is less likely to overreact if there are fewer fungi in your blood.
How HIV Is Treated Today
How to Lower the Infection Risk
As 9 out of 10 cases of cryptococcal meningitis involve HIV, the first and most effective way to avoid cryptococcosis is to start antiretroviral therapy if you test positive for the virus. The sooner you start therapy, the lower your risk of this and other opportunistic infections.
By startingantiretroviralsearly, when your immune system is intact, your chances of living a normal to near-normal life expectancy are good.Even if you start late, antiretroviral therapy can bolster your immune defenses and greatly reduce your risk of opportunistic infections.
Other things you can do to help reduce your risk of cryptococcosis until your immune system is restored include:
No vaccines or specific precautions can preventCryptococcusexposure in areas where the fungus is in the environment. As such, it is more important to know the signs of cryptococcal meningitis and seek treatment if symptoms appear.
If you don’t know your HIV status, there is no better time than now to get tested. The U.S. Preventive Services Task Force (USPSTF) currently recommends once-off HIV testing for all people aged 15 to 65 as part of a standard medical visit.
What to Do If Your HIV Test Is Positive
Summary
The diagnosis of cryptococcal meningitis involves a lumbar puncture (spinal tap) to check for the presence of the fungus in spinal fluids. The treatment involves the aggressive use of antifungal drugs, delivered first in hospital and later at home. If HIV is involved, antiretroviral therapy would also be started.
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