September 2004: a 6-year-old girl with headache and stiff neck.

Free-living amebas in the genera Naegleria, Acanthamoeba and Balamuthia are known to cause CNS infections. Here we report a case of fatal granulomatous amebic meningoencephalitis (GAE) caused by Balamuthia mandrillaris in a 6-year-old previously healthy girl who presented with headache and stiff neck. She was treated medically for brain abscess after a CT scan identified a ring-enhancing lesion in the right temporo-parietal area. A brain biopsy showed necrosis and granulomatous inflammation. Subsequently, multiple new lesions appeared in the brain bilaterally. A second brain biopsy revealed viable amebic trophozoites that were most abundant in perivascular spaces, accompanied by neutrophils, macrophages and eosinophils. Immunofluorescence study confirmed the amoeba as Balamuthia mandrillaris. This case demonstrates that making diagnosis of GAE pre-mortem requires a high index of suspicion. Amebic infection should be included in the differential diagnosis of any granulomatous lesion in CNS; and careful search for amebic parasites should be carried out especially when necrosis predominates in the pathological material.