ADEM is a potentially severe demyelinating disorder likely to be increasingly diagnosed as more magnetic resonance imaging studies are performed on patients with acute encephalopathy. Further characterization of the central nervous system inflammatory response will be needed to understand ADEM pathogenesis, to improve diagnostic and treatment strategies and to distinguish ADEM from MS.
Case histories are presented of 2 individuals (a 5-year-old girl and 64-year-old man) who developed encephalitis caused by the free-living amoeba Balamuthia mandrillaris. Both individuals survived after diagnosis and initiation of effective antimicrobial therapy. Immunostaining for Balamuthia-specific antibody levels identified the causative agent of the infections. Antimicrobial therapy with flucytosine, pentamidine, fluconazole, sulfadiazine, and a macrolide antibiotic (azithromycin or clarithromycin) was initiated. Phenothiazines (thioridazine and trifluoperazine) were also used. Both patients recovered, and there was no evidence of recrudescence of the disease at 2 and 6 years after onset of symptoms. Awareness of Balamuthia as the causative agent of encephalitis and early initiation of antimicrobial therapy were critical to the recovery of both patients. Although optimal antimicrobial therapy for Balamuthia amoebic encephalitis has yet to be determined, the antimicrobials used in these 2 cases effectively controlled the disease. These 2 individuals are the only known survivors of this otherwise fatal type of amoebic encephalitis.
Interferon-gamma receptor deficiency is a recently described immunodeficiency that is associated with onset of severe mycobacterial infections in childhood. We describe the occurrence of symptomatic and often severe viral infections in 4 patients with interferon-gamma receptor deficiency and mycobacterial disease. The viral pathogens included herpes viruses, parainfluenza virus type 3, and respiratory syncytial virus. We conclude that patients with interferon-gamma receptor deficiency and mycobacterial disease have increased susceptibility to some viral pathogens.
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