Clinical Information

Several free-living amebae can infect the central nervous system (CNS) and cause devastating, usually fatal, disease. The route of entry and clinical course of infection varies with the type of ameba involved. Naegleria fowleri typically causes rapidly progressive primary amebic meningoencephalitis (PAM) in previously healthy children or adults. Infection is acquired during contact with contaminated water, including swimming and diving in warm stagnant freshwater lakes and by nasal irrigation with nonsterile water. During contact, the amebae enter the nasal sinuses and travel along the olfactory nerve through the cribriform plate of the skull and into the CNS. PAM is almost uniformly fatal within several days of exposure. Because of the rarity of the infection and difficulty in initial detection, about 75% of diagnoses are made after the death of the patient. In contrast, Acanthamoeba species and Balamuthia mandrillaris usually cause a subacute CNS illness, usually in adults who are immunocompromised, called granulomatous amebic encephalitis (GAE). The presentation of GAE can mimic a brain abscess, aseptic or chronic meningitis, or CNS malignancy. The amebae usually disseminate to the CNS from the lungs or a primary skin lesion.

These amebae are usually identified by microscopic examination of cerebrospinal fluid or brain tissue and agar culture. Culture is more sensitive than microscopy alone but takes up to 7 days to produce a positive result. Also, B mandrillaris will not grow in routine culture. Real-time polymerase chain reaction assays offer a rapid and sensitive alternative to microscopy and culture.