Coexistence of hemimegalencephaly and chronic encephalitis. Detection of cytomegalovirus by the polymerase chain reaction.
We report the extraordinary association of hemimegalencephaly with chronic encephalitis and cytomegalovirus (CMV) positivity in a 5-month-old infant with intractable seizures and a left hemisphere resection. Microscopy revealed a severe neuronal migration disorder (NMD) with fusion of gyri, marked disarray of neuronal lamination, neuronal gigantism and extensive neuronal heterotopias. Also widespread were microglial nodules, gliosis and nodular calcifications and some foci of frank necrosis with calcification. Occasional perivascular and leptomentingeal lymphocytic infiltrates were present. No viral inclusions were identifiable. Polymerase chain reaction on multiple specimens showed unequivocal CMV positivity. In intrauterine CMV infection. NMDs such as polymicrogyria are well recognized, but the association of hemimegalencephaly with CMV infection has not previously been described. Our finding of chronic encephalitis with CMV positivity and hemimegalencephaly in the same patient raises questions about the role of CMV in the etiopathogenesis of the NMD.
Jay
V
V
Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
Otsubo
H
H
Hwang
P
P
Hoffman
H J
HJ
Blaser
S
S
Zielenska
M
M
D001921:Brain / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D002908:Chronic Disease; D003586:Cytomegalovirus Infections / Q000175:diagnosis* / Q000473:pathology; D004292:Dominance, Cerebral / Q000502:physiology*; D018792:Encephalitis, Viral / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009474:Neurons / Q000473:pathology; D016133:Polymerase Chain Reaction / Q000379:methods*; D011247:Pregnancy; D011297:Prenatal Exposure Delayed Effects; D012307:Risk Factors