Granulomatous amebic encephalitis (GAE), an infection of immunocompromised hosts, is almost uniformly fatal. A case of GAE in a patient who failed to mount a serologic response to Acanthamoeba polyphaga is presented. Although Acanthamoeba polyphaga that is sensitive to multiple antimicrobials grew from brain tissue, an inability to make a premortem diagnosis precluded therapy.
CASE REPORTA 70-year-old female presented for medical attention with lethargy and seizures. Symptoms began 36 days earlier when she was hospitalized with syncope. An evaluation at that time included magnetic resonance imaging (MRI), revealing a focal area of edema in the right temporal lobe consistent with an acute infarction, and she was discharged to a rehabilitation facility with a diagnosis of cerebrovascular accident. She was readmitted with worsening ataxia, lethargy, and generalized seizures. Her past medical history included chronic neutropenia secondary to myelodysplastic syndrome, hypogammaglobulinemia treated with monthly intravenous gamma globulin, asplenia, steroid-dependent discoid lupus (prednisone, 20 mg twice a day), and diabetes mellitus.On exam, the patient was afebrile and hemodynamically stable. An ocular exam did not reveal papilledema. Her neck was supple. She was oriented only to person. A neurological examination was otherwise nonfocal. Laboratory studies included a platelet count of 355 ϫ 10 9 /liter, a hematocrit of 32.8%, and a white blood cell count of 2.8 ϫ 10 9 /liter, which was unchanged from her baseline leukopenia. A lumbar puncture (Table 1) was significant for lymphocytic pleocytosis and hypoglycorrhacia. A repeat MRI revealed intense contrast enhancement of the right posterior temporal lobe, with two new contrast-enhancing areas in the medial temporal lobe. A stereotactic biopsy of the right temporal lobe performed on day 42 was nondiagnostic, with results showing a reactive gliosis with lymphocytic infiltrate in the leptomeninges, but no amebae or granulomas, and negative bacterial, fungal, and mycobacterial cultures.The patient's hospital course was significant for progressive obtundation, requiring intubation for airway protection. The results of repeat lumbar punctures are provided in Table 1. Serial MRI revealed persistent abnormal signal intensity in the temporal lobes, with new areas of uptake in the right basal ganglia, pons, and left occiput. Extensive diagnostic evaluation was unrevealing except for a stable elevation in titers of antibodies to Mycoplasma pneumoniae (1.425 in the acute phase and 1.645 in the convalescent phase) and Ehrlichia chaffeensis (1:128 in the acute phase and 1:128 in the convalescent phase) and evidence of prior Epstein-Barr virus infection (viral capsid antigen immunoglobulin G titer of Ͼ10, viral capsid antigen immunoglobulin M titer of Ͻ10, and EBNA titer of Ͼ10). Despite empirical treatment with acyclovir, decadron, and plasmaphoresis, the patient died 100 days after her initial presentation.Hematoxylin and eosin-stained sections of the patient's brain obtained at ...