Primary amoebic meningoencephalitis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Natural History
Naegleria fowleri enters the nose and then migrates to the brain through the cribiform plate along the olfactory nerve. People do not become infected from drinking contaminated water. After the organisms have multiplied and largely consumed the olfactory bulbs, the infection rapidly spreads through the mitral cell axons to the rest of the cerebrum, resulting in onset of frank encephalitic symptoms, including cephalgia (headache), nausea, and rigidity of the neck muscles, progressing to vomiting, delirium, seizures, and eventually irreversible coma. Symptoms start 1-7 days (median 5 days) after swimming exposure and people die 1-12 days (median 5.3 days) after symptoms begin.
Complications
Complications may vary among the rare survivors of these infections and depend on the extent of CNS involvement. Respiratory failure occurs when the infection spreads to the brain stem, destroying the autonomic nerve cells of the medulla oblongata.
Prognosis
The disease is both exceptionally rare and highly lethal. The high mortality rate of this disease is largely blamed on the unusually non-suggestive symptomology of the early-stage disease compounded by the necessity of microbial culture of the cerebrospinal fluid to effect a positive diagnosis. The parasite also demonstrates a particularly rapid late-stage propagation through the nerves of the olfactory system to many parts of the brain simultaneously (including the vulnerable medulla). For those reasons, it has been suggested that physicians should give an array of antimicrobial drugs, including the drugs used to treat amoebic encephalitis, before the disease is actually confirmed in order to increase the number of survivors. However, administering several of those drugs at once (or even some of them known to treat the condition) is often very dangerous and unpleasant for the patient and people usually die 1-12 days after symptoms begin.