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Naegleria Infection (cont.)

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What is the treatment for a Naegleria fowleri infection?

Because Naegleria meningoencephalitis is so uncommonly diagnosed and rapidly progresses to death, there are no studies comparing one treatment regimen to another, and performing comparative human studies would be unethical. This makes all uses of medications against N. fowleri "off label." Treatment is currently very intensive, and based on prior successful regimens, combinations of drugs, and advances in managing traumatic brain injuries.

Amphotericin B is an intravenous (IV) drug usually used for fungal infections. It is the drug of choice but often fails if given alone. In addition to intravenous treatment, amphotericin B can be instilled directly into the spinal fluid (intrathecally). Other antifungal drugs that have shown success include fluconazole or miconazole; these may be given via IV and intrathecally. Antibiotics that kill Naegleria include azithromycin (Zithromax, Zmax, AzaSite) and rifampin (Rifadin) and are given via IV, as well.

In addition to antimicrobials, anti-inflammatory and other drugs are used to reduce brain swelling. Brain swelling may be relieved by insertion of a shunt tube (ventriculostomy) to drain excess spinal fluid. The body may be cooled to 93 F (hypothermia). The use of artificial respiration techniques such as "hyperventilation" and induced coma also help reduce swelling and protect brain function while the amoebae are killed.

The most important advance in treatment is the availability of miltefosine in the U.S. This drug, which is highly active against amoebae, was not available outside of Europe until the FDA approved its use by the CDC under an experimental treatment protocol. This made it possible to stock the drug at the CDC, from where it could be shipped within hours to a hospital. In 2013, miltefosine was used in two cases, and both patients survived. One recovered with minimal brain damage and was discharged home after two months hospitalization. The other (who was treated late into illness) suffered permanent disability. Miltefosine has been provided in other cases since without success, however, it is usually days into the infection.

In 2016, miltefosine was approved by the FDA for the treatment of a parasitic infection, leishmaniasis, and it is now commercially available. Recognizing the critical need, the pharmaceutical company, Profunda, Inc., has made a treatment supply of miltefosine available to hospitals on a consignment basis. The hospitals can stock the drug on-site in the event it is needed to treat PAM; they are charged for the drug only if it is used, and the company will restock it when it expires.

It is strongly recommended that an infectious-disease doctor and the CDC Emergency Operations Center be consulted immediately to guide therapy. Again, the CDC Emergency Operations Center is available 24/7 at 770-488-7100.

Medically Reviewed by a Doctor on 8/18/2016


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