Prevalence of rubella virus and cytomegalovirus infections in suspected cases of congenital infections

Although rubella virus and cytomegalovirus (CMV) are important causes of congenital infections, information on their prevalence in our country is scarce. We studied a total of 249 infants suspected of having congenital infections from January 1988 to September 1989. Serum samples of these infants were tested for rubella and cytomegalovirus specific IgM antibodies by mucapture ELISA. Thirty (12%) infants were positive for rubella IgM antibody, and 50 (20%) had CMV specific IgM antibody. In the group presenting with hepatosplenomegaly (n=56) rubella and CMV specific IgM antibodies were detected in 1 (1.7%) and 25 (44.6%) infants respectively. In the group presenting with congenital malformations (n=90), 23 (25.5%) were positive for rubella, and only 9 (10%) had CMV IgM antibodies. Of the infants presenting with mental retardation (n=39), only CMV infection was detected in 3 (7.7%) infants, whereas amongst the group showing intrauterine growth retardation (n=16), 5 (31.25%) had CMV specific IgM antibodies and 2 (12.5%) had rubella specific IgM antibodies. In the miscellaneous group (n=48), 4 (8.3%) and 8 (16.6%) infants had rubella and CMV IgM antibodies respectively. CMV infection was prevalent in a significantly higher number of children with hepatosplenomegaly than rubella while in infants with congential malformations a significantly higher number had rubella infection. It is concluded that rubella and CMV infections are commonly seen in children with intrauterine infections in our population.     

Clinical features of cerebral palsy in children with symptomatic congenital cytomegalovirus infection

BackgroundHuman cytomegalovirus is the most common cause of vertically transmitted viral infection, affecting around 1% of liveborns. Infection is symptomatic in nearly 10% of infected children who are at higher risk of development of severe neurological disorders, including cerebral palsy.AimsTo study the clinical profile of children with cerebral palsy caused by symptomatic congenital cytomegalovirus infection in a multicenter study involving six countries from the Surveillance of Cerebral Palsy in Europe (SCPE) Network.MethodsData on 35 children (13 males, 22 females; mean age at last assessment 12y 6mo, age range 14y 6mo, min 4y, max 18y 6mo) on pre/peri/neonatal history and last clinical assessment were collected. Classification of cerebral palsy and associated impairments was performed according to SCPE criteria.ResultsThe majority of children had bilateral spastic cerebral palsy, 85.7%, with a confidence interval (CI) [69.7–95.2], and 71.4% [CI 53.7–85.4] were unable to walk (GMFCS levels IV–V) while fine motor function was severely affected in 62.8% [CI 44.9–78.5] (BFMF levels IV and V). Most of the children with severe CP had severe associated impairments. 11.4% of children had severe visual and 42.8% severe hearing impairment, 77.1% [CI 59.9–89.6] suffered from epilepsy, also 77.1% had severe intellectual impairment, and speech was undeveloped in 71.4%. Female:male ratio was 1.69:1 and 80% of children were term born.ConclusionsCerebral palsy following symptomatic congenital cytomegalovirus infection seems to be in most cases a severe condition and associated impairments are overrepresented.     

Le diagnostic rétrospectif de l’infection congénitale à cytomégalovirus sur le sang séché des cartes de Guthrie : l’expérience en France

Systematic screening for cytomegalovirus congenital infection is not performed in France. For children with hearing loss or other neurological CMV compatible symptoms, retrospective diagnosis is possible by PCR detection of CMV DNA in dried blood spot of neonatal Guthrie cards. We report here the results obtained with this technique in the French national reference laboratory for cytomegalovirus.     

Congenital cytomegalovirus infection - a common cause of hearing loss of unknown aetiology

Aim: The aim of this study was to investigate the role of congenital cytomegalovirus (CMV) infection as a cause of various types of sensorineural hearing loss (SNHL) in a group of nonsyndromic children with otherwise unknown aetiology of hearing loss. Furthermore, the occurrence of combined congenital CMV infection and connexin 26 (Cx26) mutations was investigated. Methods: The dried blood spot (DBS) cards of 45 children with various degrees of hearing deficits and 46 children with severe/profound hearing loss were tested for CMV DNA with polymerase chain reaction (PCR) technique. The DBS cards of the 46 children with severe/profound hearing loss were also analysed for Cx26 mutations. Results: Of the 45 children with various degrees of hearing loss, nine were positive for CMV DNA (20%). The nine children represented severe/profound, mild and unilateral hearing loss. From the 46 children with severe/profound hearing loss, nine of 46 (20%) were positive for CMV DNA. In addition, three of the CMV DNA‐positive children were carriers of mutations of Cx26. Conclusion: Congenital CMV infection is a high risk factor in hearing impairment among children.     

Congenital syphilis: A guide to diagnosis and management

Although congenital syphilis is a rare disease in Canada, infected infants may experience severe sequelae, including cerebral palsy, hydrocephalus, sensorineural hearing loss and musculoskeletal deformity. Timely treatment of congenital syphilis during pregnancy may prevent all of the above sequelae. However, the diagnosis of suspected cases and management of congenital syphilis may be confusing, and the potential for severe disability is high when cases are missed. The present review provides assistance to practitioners in the diagnosis of suspected cases and management of children with presumed or confirmed infection.     

Congenital cytomegalovirus infections

Congenital cytomegalovirus (CMV) infection is one of the most common viral causes of congenital infections in high resource countries and a leading cause of hearing loss as well as an important contributor to neurodevelopmental disabilities in children. During early pregnancy, CMV has a teratogenic potential and may cause malformations such as migrational disturbances in the brain, which can be visualised using neuroimaging methods such as magnetic resonance imaging (MRI) in such children. As a consequence of variation in epidemiology and seropositivity in fertile women, the prevalence of congenital CMV in their offspring varies in different countries between 0.15-2.0%. Some 10-20% of all children with congenital CMV infections exhibit signs of neurological damage when followed up. This is the case in children both with and without symptoms of infection at birth. Until vaccines and non-toxic antiviral agents are available, hygienic measures are important as prophylaxis. Treatment with antiviral agents may have a place in children with central nervous system involvement during the neonatal period.     

Active epilepsy in mentally retarded children. I. Prevalence and additional neuroimpairments

A population-based study of active epilepsy was conducted in 6-13-year-old mentally retarded children born between 1975 and 1986. The population at risk comprised 48 873 children. Ninety-eight children were identified, 35 mildly and 63 severely retarded. The prevalence was 2.0 per 1000; 0.7 per 1000 for mildly and 1.3 per 1000 for severely retarded children. Sixty-nine children had at least one additional neuroimpairment. Cerebral palsy was found in 42 children with a majority of spastic/ dystonic tetraplegias; visual impairment was present in 24 and autism in 24. Thirty-three children had only a mild or no gross motor disability and mild mental retardation, while 23 had IQs <20 and a very severe gross motor disability. This study underlines the fact that active epilepsy in mentally retarded children is often associated with additional neuroimpairments, especially a combination of severe cerebral palsy and severe visual impairment. Children, epilepsy, mental retardation, neuroimpairments, prevalence     

Cerebral palsy and congenital malformations.

AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children with cerebral malformations. The most frequent groups of non-cerebral malformations were cardiac, facial clefts and limb and skeleton malformations. Children born at term had a significantly higher prevalence of cerebral malformations compared to children born before 32 weeks (12.1% versus 2.1%, p<0.001). CONCLUSION: Cerebral malformations were much more frequent among children with CP than among all livebirths in the population. Malformations in organ systems close to the brain (eye, facial clefts) were more frequent in the CP population while malformations in organ systems further from the brain (renal, genital) were more frequent in the general population.     

Congenital CMV infection in symptomatic infants in Delhi and surrounding areas

Many viral infections are associated with significant maternal and fetal consequences during pregnancy among which cytomegalovirus is one of the most important agent, globally. Both primary and recurrent infection due to this virus can result in fetal infection. Samples from Congenital Anoammaled babies are referred to NICD from Delhi based Government hospitals and surrounding areas for diagnosis of congenital infections like Toxoplasm, Rubella, CMV and Herpes. In the present study, accumulated data is presented for the most common teratogenic virus—Cytomegalovirus prevalence as a causative agent for congenital infection in New Born babies at Delhi and surrounding areas. 96 samples from symptomatic babies in the age group of few days to 6 months exhibiting different congenital anomalies, were reported between 1 st Jan 04 to 30 th April/05. All the blood samples were tested for the detection of CMV (IgM) antibodies using μ-capture ELISA technique. 18(18.75%) samples from babies showed positive titres for CMV-IgM antibodies. None of the mothers of positive babies were found positive for CMV-IgM antibodies but all were serologically exposed to CMV virus previously as their serum samples were positive for CMV-IgG antibodies indicating primary infection in the past or reactivation/reinfection with a different strain of CMV in the early pregnancy.     

Human Cytomegalovirus: detection of congenital and perinatal infection in Argentina

Background  

Human cytomegalovirus (CMV) is one of the most commonly found agents of congenital infections. Primary maternal infection is associated with risk of symptomatic congenital diseases, and high morbidity is frequently associated with very low birth weight. Neonates with asymptomatic infection develop various sequelae during infancy. This is the first Argentine study performed in neonates with congenital and postnatal HCMV infection. The purpose of this study was to evaluate the performance of the polymerase chain reaction (PCR) technique with different pairs of primers, to detect cytomegalovirus isolated in tissue cultures and directly in urine and dried blood spot (DBS) specimens. Results were compared with IgM detection.     

Cerebral palsy in southern Sweden I. Prevalence and clinical features

The prevalence, clinical features and gross motor function of children with cerebral palsy in southern Sweden were investigated. The study covered the birth year period 1990-1993, during which 65514 livebirths were recorded in the area. On the census date (1 January 1998), 68366 children born in 1990-1993 lived in the area. The study comprised 167 children, 145 of them born in Sweden and 22 born abroad. The livebirth prevalence was 2.2 per 1000, and the prevalence including children born abroad was 2.4 per 1000. The distribution according to gestational age, birthweight and subdiagnoses was similar to that in earlier Swedish studies, except for a higher rate of dyskinetic syndromes in this study.

Conclusion: The point prevalence of cerebral palsy was 2.4 and the livebirth prevalence was 2.2. Children born abroad had a higher prevalence and were more often severely disabled. Severe disability was often combined with associated impairments such as mental retardation, epilepsy and visual impairment.     

A wider role for congenital cytomegalovirus infection in sensorineural hearing loss

BACKGROUND: Diagnostic problems in identifying congenital infection cases in infancy have thus far impaired the assessment of the role of congenital cytomegalovirus (CMV) infection in the etiology of sensorineural hearing loss (SNHL). OBJECTIVE: To estimate the impact of congenital infection in children with SNHL by detection of CMV DNA in stored samples of neonatal dried blood (dried blood spots test). METHODS: The Guthrie cards of 130 children with hearing loss >40 dB hearing loss were retrieved from the regional screening center. CMV DNA was extracted by thermal shock and amplified by PCR. RESULTS: The percentage of SNHL cases attributable to congenital CMV infection was 10% (9 of 87) in infants whose SNHL had been diagnosed in their first 2 months of life and 34.2% (13 of 38) in children with deafness of unidentified cause that was diagnosed in early childhood. In the latter group 42.7% (12 of 28) of the children with a hearing loss of >70 dB were CMV-positive. CONCLUSIONS: The results suggest that congenital CMV infection has a more relevant role in the etiology of SNHL than previously reported. The data obtained in both groups suggest that 20 to 30% of all deafness cases are caused by CMV. The percent of congenital CMV cases alone appears to account for all the cases previously attributed to all congenital infections. More than 40% of deafness cases with an unknown cause, needing rehabilitation, are caused by congenital CMV.     

Rehabilitation of cerebral palsy in a developing country: the need for comprehensive assessment

Records of 100 children with cerebral palsy from rural India attending a cerebral palsy clinic were analysed to determine the frequency of associated handicaps and to evaluate whether appropriate intervention had been instituted for these handicaps. All the children underwent speech, hearing, psychological, ophthalmologic, neurological, physical, functional and orthopaedic evaluations on their visit to the clinic. History was elicited from the parents of the patients to identify which of the disabilities were recognized either by them or their primary care physician, and whether any intervention had been instituted. The mean age of the patients was 6.9 years. Eighty-two per cent of the children had one or more disabilities apart from locomotor disabilities, visual defects being the commonest (54%). Fifty-four per cent of the children had more than one associated disability. Although 43% of the patients had been referred by a primary care physician, one or more associated disabilities had not been recognized and epilepsy was the only associated problem that had been treated prior to the evaluation. The study emphasizes the need for comprehensive evaluation of all children with cerebral palsy to enable proper rehabilitation.     

Outcome and management of newborns with congenital cytomegalovirus infection

Congenital cytomegalovirus (CMV) infection is the most common non-genetic cause of hearing loss and neurological disorder in children. Its overall prevalence is approximately 0.5% in Europe. In France, systematic screening during pregnancy is not recommended; screening is performed only if there are maternal or fetal symptoms suggestive of this infection. Approximately 90% of infected newborns are asymptomatic at birth, and among them the risk of neurosensory sequelae is 5–15%. By contrast, the prevalence of neurosensory impairment in symptomatic newborns at birth varies from 17% to 60%. Congenital CMV infection must be confirmed at birth before the 21st day of life by polymerase chain reaction (PCR) on saliva or urine samples. A complete clinical examination, blood tests (blood count, liver function test, CMV PCR), hearing tests, brain ultrasound and eye fundus examination should be performed. Neurological and auditory follow-up must be extended well beyond the neonatal period because the occurrence of neurosensory sequelae may be delayed. Oral valganciclovir is the recommended treatment in moderate or severe congenital CMV infections for a period of 6 weeks to 6 months; such treatment requires regular monitoring because of its possible side effects.     

The effect of a child's disability on mother's mental health

The prevalence of maternal depression was investigated in the mothers of 96 children: 30 premature infants at risk for the development of cerebral palsy; 35 premature infants considered not to be at risk for the development of cerebral palsy; and 31 healthy fullterm infants. There were equally high levels of depression in all three groups of mothers, regardless of birth status, prediction of disability, or presence of actual disability, throughout the first year of the children's lives. Depressed mothers were, however, found to have significantly more psychosocial stress. An early physiotherapy intervention had no effect on the prevalence of depression in mothers whose children were at risk for the development of cerebral palsy.     

Neurodevelopmental assessment after congenital cytomegalovirus infection.

The neurodevelopmental state of 41 children with congenital cytomegalovirus infection and their controls was assessed at 2 years using the Griffiths scale. The scores achieved by children with congenital cytomegalovirus but with no associated neurological abnormality (asymptomatic) were similar to those of the control children, whereas the mean score of the five children with congenital infection and neurological impairment (symptomatic) was significantly lower. This study, which has the statistical power to detect differences in developmental quotient as small as five points, gave no evidence that at 2 years cytomegalovirus infection was associated with mental retardation in the absence of other neurological impairment. Thus 90% of children with congenital cytomegalovirus infection at 2 years are neurologically and developmentally normal.     

The effect of a child's disability on mother's mental health.

The prevalence of maternal depression was investigated in the mothers of 96 children: 30 premature infants at risk for the development of cerebral palsy; 35 premature infants considered not to be at risk for the development of cerebral palsy; and 31 healthy fullterm infants. There were equally high levels of depression in all three groups of mothers, regardless of birth status, prediction of disability, or presence of actual disability, throughout the first year of the children''s lives. Depressed mothers were, however, found to have significantly more psychosocial stress. An early physiotherapy intervention had no effect on the prevalence of depression in mothers whose children were at risk for the development of cerebral palsy.     

Epidemiology of cerebral palsy in England and Scotland, 1984-9

AIMS—To report on the epidemiology of cerebral palsy in England and Scotland, to provide information on the prevalence of cerebral palsy and the severity of the disability or any co-morbidity.METHODS—Cerebral palsy registers were compiled from multiple sources of ascertainment covering all of Scotland and the counties of Merseyside, Cheshire, Berkshire, Buckinghamshire, Northamptonshire and Oxfordshire in England. All cases of cerebral palsy born in 1984 to 1989, to mothers resident in the area, were included. Denominator number of live births and neonatal deaths for determining birthweight specific prevalence were obtained from birth and death registrations. Learning, manual, and ambulatory disabilities were graded for severity. Any co-existing sensory (hearing or visual) morbidity was also graded for severity.RESULTS—There were 789 411 live births in 1984-9, with 3651 neonatal deaths (neonatal mortality 4.6 per 1000 live births) and 1649cases of cerebral palsy—a cerebral palsy prevalence of 2.1 per 1000 neonatal survivors. The birthweight specific cerebral palsy prevalence ranged from 1.1 per 1000 neonatal survivors in infants weighing ?2500 g to 78.1 in infants weighing <1000 g. There was no significant time trend in prevalence of cerebral palsy in any of the birthweight groups, in contrast to the fall in neonatal mortality observed in all birthweight groups. Of the 1649 cases of cerebral palsy, 550 (33.4%) had severe ambulatory disability (no independent walking), 390 (23.7%) had severe manual disability (incapable of feeding or dressing unaided), 381 (23.1%) had severe learning disability (IQ <50), 146 (8.9%) had severe visual disability (vision <6/60 in the better eye) and 12 had severe hearing disability (>70 dB loss).CONCLUSIONS—Registers fill an important gap left by the lack of routine data on the prevalence of disability in children. The ability to record trends in the prevalence and the severity of the disability should inform those who have responsibility for providing services for children with disabilities.     

Clinical and prognostic aspects of congenital hydrocephalus

Clinical data of 24 patients with congenital hydrocephalus are presented. 13 children died within the first 7 months of life; the average period of survival was 2 months. Associated malformations of the CNS or other organs, severely raised intracranial pressure in utero, and postnatal complications involving the CNS indicated a poor prognosis. The follow-up of the 11 surviving children showed severe neurologic sequelae and drug resistent epilepsy in 2 of them. Three others had neural tube defects and associated paraplegia. The 6 remaining children had mild or moderate cerebral palsy or were moderately mentally retarded. If possible, birth should not be induced before 36 weeks of gestation, and the hydrocephalus should be shunted soon after birth. Satisfactory results can be obtained in about one half of surviving patients by early shunting of congenital hydrocephalus.     

Bilateral loss of cortical somatosensory evoked potential at birth predicts cerebral palsy in term and near-term newborns

Bilateral loss of cortical somatosensory evoked potential (SEP) is considered the single best indicator of adverse outcome in acute encephalopathy of adult patients and older children. This study determines whether the presence or absence of the neonatal cortical SEP can predict cerebral palsy at two years in survivors of neonatal encephalopathy scored according to Sarnat criteria. We also compare SEPs with visual evoked potentials (VEPs), the EEG and neonatal neurological status.Fifty-nine neonates admitted to the neonatal intensive care unit had SEP, VEP and EEG recordings analysed according to the presence (n = 37, 63%) or absence (n = 22, 37%) of neonatal encephalopathy (score ≥ 1).Cortical SEP was always present in the perinatal period in those surviving without major neurological disability, while it was bilaterally absent in all but one patient with a subsequent diagnosis of cerebral palsy. Multivariate analysis using the logistic regression model showed that bilateral loss of cortical SEP and Sarnat Score correctly classified the neurological outcome in all patients.Bilateral absence of cortical SEP indicates early identification of neonates at risk of cerebral palsy indicating that EPs have a clinical role in the workup of neonatal encephalopathy.