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A devastating case of a Balamuthia mandrillaris pediatric brain infection
Kathryn A Szymanski1, Michael S Kuwabara2, Neil Friedman3
1Creighton University School of Medicine, 3100 N Central Ave, Phoenix, AZ 85012, USA.
Abstract:
Balamuthia mandrillaris is an amoeba that causes an uncommon but deadly encephalitis, referred to as granulomatous amoebic encephalitis (GAE). The highest incidence reported worldwide has occurred in America, and within the United States, it has been highest in the Southwest affecting predominantly children and young men of Hispanic ethnicity. Clinical presentation of GAE includes fever, headache, nausea, vomiting, lethargy, irritability, stiff neck, hallucinations, photophobia, and seizures. Our patient was a Hispanic male child living in Arizona. The patient presented at 3 years of age for severe encephalitis. Symptoms included difficulty with balance, gait, and sitting up and seizure-like activity. Initial CT showed an area of decreased density consistent with edema in the right frontal and left frontoparietal lobes. Rapid progression was seen on further imaging over the length of the patient's hospital stay revealing diffusion restriction, necrosis/blood products, edema, and hemorrhage. The patient expired three weeks after onset of symptoms and one week after admission to our institution. While there are multiple biochemical techniques that can test for B. mandrillaris, they are rarely employed for multiple reasons stemming from the rare occurrence of this infection. Because of the fatal nature of this infection, we propose (1) testing should be considered if a patient presents with progressing encephalitis on imaging and other pathogenic etiologies are ruled out and (2) the threshold to treat empirically should be low due to the fatal nature of the infection.
Insights
Balamuthia mandrillaris causes deadly granulomatous amoebic encephalitis (GAE), particularly in the Southwestern US. Early consideration for GAE and prompt empirical treatment are crucial due to its fatal nature.
Area of Science:
- Neurology
- Infectious Diseases
- Amoebiasis
Background:
- Granulomatous amoebic encephalitis (GAE) is a rare, fatal central nervous system infection caused by the amoeba Balamuthia mandrillaris.
- The Southwestern United States reports the highest incidence, predominantly affecting Hispanic children and young men.
Observation:
- A 3-year-old Hispanic male in Arizona presented with severe encephalitis, including balance issues, gait disturbance, and seizure-like activity.
- Imaging revealed rapidly progressing edema, diffusion restriction, necrosis, and hemorrhage in the brain.
- Despite medical intervention, the patient expired three weeks after symptom onset.
Findings:
- Balamuthia mandrillaris infection is challenging to diagnose due to its rarity and the limited use of biochemical testing.
- The clinical presentation and rapid neuroimaging changes are critical indicators.
Implications:
- Consider Balamuthia mandrillaris in cases of progressive encephalitis with negative workups for other pathogens.
- A low threshold for empirical treatment is recommended given the high fatality rate of GAE.
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