Granulomatous amoebic encephalitis (GAE) caused by certain species belonging to the genus Acanthamoeba, Balamuthia, or Naegleria presents as a subacute or chronic illness. Amoebic encephalitis caused by Acanthamoeba is seen more often in immunosuppressed individuals. Thus, it may often be associated with human immunodeficiency virus infection and acquired immune deficiency syndrome (HIV/AIDS), organ transplantation, administration of steroids and systemic lupus erythematosus (SLE). The clinical progression is rapid, most often leading on to mortality of the patients. The diagnosis in most of these patients is established on postmortem examination. We describe a case of fatal granulomatous amoebic encephalitis in a patient recently diagnosed to be having SLE, who was receiving corticosteroids, cyclophosphamide, methotrexate, and hydroxychloroquine. The patient presented in an altered sensorium and expired after being hospitalized for 6 days. Postmortem examination of the brain showed extensive areas of necrosis and neutrophilic infiltrate with trophozoites and cysts of Acanthamoeba.
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