COEXISTENCE OF HEMIMEGALENCEPHALY AND CHRONIC ENCEPHALITIS - DETECTION OF CYTOMEGALOVIRUS BY THE POLYMERASE CHAIN-REACTION

We report the extraordinary association of hemimegalencephaly with chr onic encephalitis and cytomegalovirus (CMV) positivity in a 5-month-ol d infant with intractable seizures and a left hemisphere resection. Mi croscopy revealed a severe neuronal migration disorder (NMD) with fusi on 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 nec rosis with calcification. Occasional perivascular and leptomentingeal lymphocytic infiltrates were present. No Viral inclusions were identif iable. Polymerase chain reaction on multiple specimens showed unequivo cal CMV positivity. In intrauterine CMV infection, NMDs such as polymi crogyria are well recognized, but the association of hemimegalencephal y with CMV infection has not previously been described. Our finding of chronic encephalitis with CMV positivity and hemimegalencephaly in th e same patient raises questions about the role of CMV in the etiopatho genesis of the NMD.