13-Year-Old Boy Unaware of His Surroundings

Paul P. Rega, MD


September 14, 2023

N fowleri meningoencephalitis has a death rate of as much as 97%.[11] Only three survivors have been reported in the United States. The sole chance a patient has for survival is an early diagnosis and initiation of the proper therapy.[9,12] The usual outcome, death within 6-17 days, is typically related to increased intracranial pressure and brain herniation.[6,9]

Delay of the patient in seeking medical attention and delay in making the correct diagnosis explain, in part, why the mortality rate is so high.[11] Without an in-depth questioning of the patient and/or the family, the historical clue of nasal exposure to warm, untreated fresh water will be overlooked. Similarly, when the results of a lumbar puncture demonstrate cloudy fluid with an elevated CSF WBC count, a high CSF protein value, and a low CSF glucose value, the logical diagnosis will be a bacterial meningitis despite a negative Gram stain result.

However, in this case, an elevated index of suspicion for N fowleri meningoencephalitis, in addition to the ordering of appropriate testing, allowed for an early, accurate diagnosis. This led to the activation of the pipeline of therapeutics that have been deemed essential for any hope of survival. The drug regimen recommended by the Centers for Disease Control and Prevention includes amphotericin B, intravenously and intrathecally; azithromycin; fluconazole; rifampin; miltefosine; and dexamethasone.[12] It may be difficult to acquire some of these medications, so making an early, accurate diagnosis is even more critical in order to obtain the drugs as quickly as possible. Other interventions that have been reported in the literature include inducing therapeutic hypothermia (89.6 °F [32 °C] to 93.2 °F [34 °C]) and stabilizing intracranial pressures (eg, with hyperventilation, hyperosmolar therapy, and phenobarbital-induced coma).[6,9,15]

Amazingly, through the aggressive use of induced hypothermia, invasive monitoring of intracranial pressures, and a medley of pharmaceutical measures, this patient made a virtually complete recovery following a week in the intensive care unit and 1 month in a rehabilitation center. The key to success was the timely diagnosis.


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