Clinical case of congenital CMV neuroinfection in a child with selective IgA deficiency

Congenital CMV infection is thought to occur in at least 1 % of infants, although recent clinical studies indicate that these lesions account for 8 % of all neonates. The severity of clinical symptoms of nervous system damage of CMV-etiology depends on the duration of intrauterine infection. In early infection, during the first trimester of the fetal period, severe CNS malformations develop, including anencephaly, porencephaly, schizencephaly, lissencephaly, micropolygyria, and pachygyria. At later infection, during the 2nd–3rd trimesters of pregnancy, there are milder manifestations — ventriculomegaly, impaired myelination of the white matter of the brain, cysts in the poles of the temporal lobes, hypogenesis of the corpus callosum, periventricular calcifications, and lesions of the cochleovestibular nerves. The article presents the medical history of a 5-year-old boy with typical clinical and instrumental signs of congenital CMV infection. The child had deep spastic tetraparesis, severe mental retardation, refractory epileptic syndrome with polymorphic seizures, disorders of pelvic organs, inability to move independently. MRI of the brain showed typical radiological signs of congenital CMV infection: cortical atrophy, ventriculomegaly, periventricular gliosis, demyelination fields in the white matter of the hemispheres, cysts in the poles of the temporal lobes, hypoplasia of the corpus callosum. During the neonatal period, specific IgM to CMV in serum was observed. Blood leukocyte PCR revealed the CMV DNA in a 5-year-old child at the time of admission to the clinic. This infection led to genera-lized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, and lymphomonocytosis. The assessment of immune status showed the presence of selective IgA deficiency, which was associated with the development of this opportunistic infection. Typical mistakes in the clinical management of children with congenital CMV infection and ways to avoid them are discussed.