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Granulomatous Amebic Encephalitis

By Richard D. Pearson, MD, Emeritus Professor of Medicine, University of Virginia School of Medicine

Granulomatous amebic encephalitis is a rare, usually fatal infection of the central nervous system caused by Acanthamoeba species or Balamuthia mandrillaris. It usually occurs in people with a weakened immune system or generally poor health.

  • The amebas probably enter through the skin or lungs and spread to the brain through the bloodstream.

  • Symptoms of granulomatous amebic encephalitis begin gradually and include confusion, headache, blurred vision, seizures, and skin sores.

  • Imaging tests and a spinal tap can help doctors rule out other causes, and when a skin sore is present, a sample is taken and examined under a microscope to look for amebas.

  • Doctors typically use a combination of drugs including miltefosine to treat the infection.

The amebas that cause this infection live in water, soil, and dust throughout the world. Many people are exposed, but few are infected. Infection usually occurs in people whose immune system is weakened or whose general health is poor, although Balamuthia mandrillaris may infect healthy people.

Amebas probably enter through the skin or lungs and spread to the brain through the bloodstream.

Symptoms

Granulomatous amebic encephalitis symptoms begin gradually. Confusion, headache, and seizures are common. People may have a low-grade fever, blurred vision, changes in personality, and problems with speaking, coordination, or vision. One side of the body or face may become paralyzed.

Balamuthia mandrillaris may cause skin sores in addition to the symptoms above.

Most infected people die, usually 7 to 120 days after symptoms begin.

Diagnosis

  • Computed tomography or magnetic resonance imaging

  • A spinal tap

  • Biopsy of skin sores

Computed tomography (CT) or magnetic resonance imaging (MRI) and a spinal tap (lumbar puncture) are usually done to diagnose granulomatous amebic encephalitis. These tests help exclude other possible causes but usually cannot confirm the diagnosis.

Skin sores typically contain amebas and, if present, are biopsied.

Granulomatous amebic encephalitis is often diagnosed only after death.

Treatment

  • A combination of drugs

Granulomatous amebic encephalitis and any skin sores are typically treated with a combination of drugs including

  • Miltefosine

and one or more of the following:

  • Pentamidine (typically used to treat fungal infections—an antifungal drug—or protozoa infections)

  • Sulfadiazine or trimethoprim/sulfamethoxazole (antibiotics)

  • Flucytosine (an antifungal drug)

  • Fluconazole or the related drugs voriconazole or itraconazole (antifungal drugs)

  • Amphotericin B (an antifungal drug)

  • Azithromycin or clarithromycin (antibiotics)

Some of these drugs are taken by mouth, and others are given by injection. Some can be given in more than one way.

Sometimes surgery is also needed.

Skin sores, if present, are cleaned.