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Query: UMLS:C0025362 (mental retardation)
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Congenital cytomegalovirus infection occurs in about 1% of live births. Although symptomatic congenital infection often results in severe developmental deficits and mental retardation, about 90% have asymptomatic infection. Previous studies of the intellectual development in children with asymptomatic congenital cytomegalovirus have resulted in mixed findings. To control for the effects of hearing impairment (which occurs in about 15% of asymptomatic children) on intelligence scores, we tested 18 prospectively followed, normally hearing, school-aged children with asymptomatic congenital cytomegalovirus (15 black, ten male) and 18 controls matched for age, sex, race, school grade, and socioeconomic status. Children were tested via the Wechsler Intelligence Scale for Children-Revised, the Kaufman Assessment Battery for Children, and the Wide Range Achievement Test. Multivariate analysis revealed no differences between groups on intelligence scores or subscales, achievement scores, or incidence of learning disabilities (defined as significant discrepancy between intelligence and achievement), and mean scores for both groups were very close to national norms. It is concluded that the 25,000 children born in the United States each year with asymptomatic congenital cytomegalovirus and normal hearing are not likely to be at increased risk of mental impairment.
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PMID:Intellectual development in school-aged children with asymptomatic congenital cytomegalovirus infection. 301 52

Some viruses, such as rubella and human cytomegalovirus, are known to cross the placental barrier and infect the fetus. In other cases of maternal viral infections, such as herpes simplex, evidence for transplacental passage is less convincing and fetal damage or neonatal disease may be coincidental or associated with perinatal infection. Certain cases of fetal or neonatal disease following maternal viral infections may be associated with disease in the mother which affects her metabolic processes or the placenta in such a way as to interfere with development of the fetus and infant. The possible effects of transplacental viral infections are several. Fetal loss may occur by means of abortion or stillbirth. There may be infection of the fetus, with clinical manifestations such as rash, or without clinical manifestations. The infant may be born with congenital defects, including such deformities as cataracts, cardiac anomalies, mental retardation or cerebral palsy. Although a number of maternal viral diseases have been etiologically incriminated in congenital defects, only two-rubella and cytomegalovirus infection-are definitely proved to be associated with anomalies or mental retardation in infants.
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PMID:Congenital anomalies and viral infections in infants. The etiologic role of maternal viral infections. 595 31

Thirty-four patients with congenital cytomegalovirus infection who were symptomatic as newborns were followed in a special clinic providing periodic medical and visual examinations as well as psychometric testing and audiometry. All patients had symptoms of congenital infection by 2 weeks of age, and 31 of 34 had virus isolated from urine within the first month of life. Age at latest follow-up varied from 9 months to 14 years with a mean of about 4 years. Ten patients died and 23 surviving patients had adequate follow-up examinations; all but two had evidence of central nervous system or auditory handicaps. Microcephaly was present in 16 (70%), mental retardation in 14 (61%), hearing loss in seven (30%), neuromuscular disorders in eight (35%), and chorioretinitis or optic atrophy in five (22%). Children with symptomatic congenital cytomegalovirus infection are at very high risk for handicaps that will significantly impair development.
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PMID:Outcome of symptomatic congenital cytomegalovirus infection: results of long-term longitudinal follow-up. 615 68

A male patient born to a mother who developed rubella during the tenth week of gestation presented a typical congenital rubella syndrome with mental retardation, neuro-sensory deafness, hypoplasia of the dental enamel and chorioretinitis. Hyperthyroidism occurred at the age of 3 10/12 years and was treated successfully with propylthiouracil for 4 years. The course was complicated by premature craniosynostosis and a craniectomy was performed at the age of 7 years. Overt diabetes mellitus developed at 17 years and was well controlled by insulin therapy. Histocompatilibity (HLA) antigens were A2, B8, B40. Diabetes mellitus and thyroid disorders have previously been reported after congenital rubella, and recently after congenital cytomegalovirus infection. Our patient had both endocrinopaties. It is possible that HLA B8 antigens might be responsible for increased susceptibility to rubella infection.
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PMID:Hyperthyroidism, diabetes mellitus and the congenital rubella syndrome. 736 31

We came across some cases clinically suggestive of intrauterine infection which were confirmed to be congenital cytomegalovirus infection. A clinical profile of these patients is presented. Intracranial calcification was not seen in any of these patients. Mental retardation was profound in all the patients and all had hepatomegaly. Uncommon findings encountered included hydrocephalus, patent ductus arteriosus and corneal opacities. Other clinical findings and investigation are also discussed.
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PMID:Congenital cytomegalovirus infection in Shimla Hills, Himachal Pradesh, India. 763 48

Neuroblastoma is one of the most frequent solid tumors in childhood, rarely recurrent after five years from diagnosis. Cytomegalovirus (CMV), a major pathogen causing congenital birth defects and severe opportunistic diseases, has been shown to have teratogenic, immunodepressive and oncogenic properties. The case of a girl with stage 4S neuroblastoma diagnosed at three months and relapsed as stage 4 five years later is reported. In both circumstances, active CMV infection was revealed by positive CMV-specific IgM and IgA antibodies, CMV-DNAemia and CMV culture. At three months, the patient presented with subcutaneous nodules, hepatosplenomegaly and increased aminotransferase levels, and the opsolonus-myoclonus syndrome. Mental retardation developed later on. At 5 years, relapsed neuroblastoma was preceded by a mononucleosis-like syndrome concomitant with active CMV infection and decreased levels of immune cells and natural killer activity. Clinical, virologic, and immunologic findings suggest an immune-mediated pathogenic role for CMV in this tumor.
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PMID:Cytomegalovirus-associated stage 4S neuroblastoma relapsed stage 4. 783 43

CMV is the most common cause of intrauterine infection in this country, affecting approximately 1% of newborns. As such, CMV infection is a leading cause of deafness and an important contributor to learning disabilities. The virus is transmitted through close contact with infected secretions as well as transplacentally, through sexual contact, and through blood transfusion and organ transplantation. CMV has a high prevalence in the population; antibody to the virus can be detected in 50% to 85% of individuals. Like herpes simplex, the virus can be reactivated and cause recurrent infection. Congenital disease occurs in about 30% to 40% of women with primary infection. Ten percent of infants with congenital infection will have clinical disease at birth. Of these, as many as 30% die. Ninety percent of survivors have sequelae such has deafness, mental retardation, chorioretinitis, and motor deficits. The other 90% of infants with congenital infection are asymptomatic at birth, but 5% to 17% develop long-term sequelae. Congenital disease also occurs with recurrent maternal infection in about 0.2% to 1% of immune women. Congenital infection due to recurrent disease is far less severe, and less than 10% of affected infants have long-term sequelae (Fig 3). The diagnosis of congenital infection can be established using a combination of ultrasound, amniocentesis, and cordocentesis. Viral culture of neonatal urine can confirm the diagnosis after birth. Routine screening of the pregnant population is not currently recommended because present laboratory methods limit the ability to differentiate primary from recurrent infection. Preventive efforts at this time must focus on good hygiene, limited intimate contact with infected children, and responsible sexual practices.
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PMID:Cytomegalovirus infection in pregnancy. 816 23

Infections were considered to be etiological factors in 29 patients (10%) with infantile spasms; congenital CMV (n = 5), congenital or acquired CMV (n = 1), acquired CMV (n = 5), congenital rubella (n = 2), herpes simplex virus (n = 5), enterovirus (n = 1), adenovirus (n = 1), viral encephalitis of unknown agent (n = 3), meningococcus (n = 4), pneumococcus (n = 1) and pertussis (n = 1). The children with congenital infections had long-lasting tremor and convulsions from birth. Early EEG pattern was characteristic for children with herpes encephalitis but not for other patients. Infantile spasms appeared only some weeks after viral encephalitis. One patient with enterovirus and another with probable adenovirus infection had necrotic changes in their brain CT resembling those of herpes encephalitis. The response to ACTH was poor (38%) compared to the whole series (60%). The long-term outcome was also poor compared to the whole series; mental retardation in 90%, convulsions in 62%, abnormal EEG in 89%. Four children died during the follow-up of 7 years. Autopsy showed disseminated CMV infection in one patient and chronic CMV infection in another. The outcome of children with infectious etiology appears to be particularly poor. Thus, the prevention and specific diagnosis and treatment are important. Steroid therapy should be avoided in children with a history of herpes virus encephalitis (CMV, herpes simplex) in the past.
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PMID:Infantile spasms: infectious disorders. 830 17

Approximately 0.5-1% of all newborns are born infected with cytomegalovirus (CMV), but of these only one out of ten show symptoms at birth, most often with hepatosplenomegaly, thrombocytopenia, and/or brain affection. Of the remaining nine, one may later develop sequelae with hearing loss and/or mental retardation. CMV infection may also be acquired perinatally or in the newborn period, and may cause pneumonia and/or sepsis, possibly also gastrointestinal symptoms like blood in the stool, and poor weight-gain. We have diagnosed CMV infection in ten neonates and infants, and describe these patients in terms of symptoms, diagnosis and treatment. Ganciclovir is being tested in clinical trials as a treatment for congenital CMV infection, and was given to two of our patients with apparently good results.
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PMID:[Cytomegalovirus infection in neonates. Diagnosis and therapeutic experiences]. 919 23

Experimental evidence and pathological observation indicate that human cytomegalovirus (HCMV) has a tropism for cells of the nervous system, including both neuronal and glial cells. As demonstrated in animal models, after a viremic phase, the virus may reach the brain, where it may cause mild infection or severe encephalitis. The nervous system is one of the principal target organs in congenital HCMV infections and in HCMV-infected AIDS patients. In the former case, mortality is high and neurological sequelae, such as mental retardation, are frequent; in the latter it may lead to a progressively wasting encephalopathy and death within a few weeks. The diagnosis of the nervous system manifestations due to HCMV can now rely upon the detection of HCMV DNA in cerebrospinal fluid by means of polymerase chain reaction. However, the current antiviral treatments of these complications are of limited effect.
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PMID:Cytomegalovirus infections of the nervous system. 945 Feb 26


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