Cytomegalovirus encephalitis in a child with adenosine deaminase-deficient severe combined immune deficiency: a neuropathologic study.
Cytomegalovirus (CMV) encephalitis is exceptional in patients with genetically determined immune deficiency syndromes. Neuropathologic findings of CMV encephalitis were present at postmortem examination in a child we treated for severe combined immune deficiency. Cultured skin fibroblasts of this male infant revealed a deficiency of adenosine deaminase (ADA). Lacking a suitable bone marrow donor, we used transfusions of red blood cells as a source of ADA. However, the child developed encephalopathy and died at 16 months. The main neuropathologic findings were numerous, widely distributed inclusion-bearing and CMV antigen-positive cells; a complete lack of inflammatory reaction; and cell-to-cell fusion of infected cells reflecting the child's severely impaired immune response.
Takano
T
T
Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
Zielenska
M
M
Becker
L E
LE
D000243:Adenosine Deaminase / Q000172:deficiency*; D001921:Brain / Q000201:enzymology / Q000473:pathology* / Q000821:virology; D003587:Cytomegalovirus; D003586:Cytomegalovirus Infections / Q000209:etiology / Q000473:pathology*; D018792:Encephalitis, Viral / Q000209:etiology / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000201:enzymology / Q000473:pathology*
D000243:Adenosine Deaminase