Congenital rubella syndrome: pattern and presentation in a southern Nigerian tertiary hospital

Background  

Congenital rubella syndrome (CRS) resulting from maternal rubella infection can result in miscarriages, still birth and rubella infection of the infant. The aim of this study was to evaluate the pattern and presentation of CRS over an 8-year period as seen in the University of Port-Harcourt Teaching Hospital (UPTH), Nigeria.     

Burden of congenital rubella syndrome after a community-wide rubella outbreak,Rio Branco,Acre, Brazil, 2000 to 2001

BACKGROUND: During 1999 and 2000 rubella outbreaks were reported in 20 of 27 states in Brazil, many among young adults. We investigated a large rubella outbreak in Rio Branco, Acre, in northwestern Brazil, where rubella vaccination targeting children 1 to 11 years old had been introduced in April 2000. Surveillance for congenital rubella syndrome (CRS) was initiated after the outbreak. METHODS: Suspected rubella cases were detected through active and passive surveillance. Confirmed rubella cases were patients with fever, rash and rubella-specific IgM antibodies. Suspected CRS cases were infants born with CRS-compatible defects or born to mothers with a history of rubella during pregnancy. Confirmed cases were infants with CRS-compatible defects and rubella-specific IgM antibodies. RESULTS: From April 1 to December 31, 2000, 391 confirmed rubella cases were reported. The incidence among persons ages 12 to 19 years (3.3 per 1000 population) was increased 3.7-fold relative to children ages 1 to 4 years (95% confidence interval, 2.4 to 5.8). Of 21 infants with suspected CRS cases, 17 (91%) were tested for rubella-specific antibodies, of whom 7 were IgM-positive and 5 had confirmed CRS. The peak incidence of confirmed CRS (4.3 per 1000) was in March 2001, 7 months after the outbreak peak, with an annualized incidence of 0.6 per 1000. CONCLUSIONS: Vaccination among school age children was insufficient to prevent a rubella outbreak among young adults that resulted in the occurrence of at least 5 cases of CRS. To prevent further cases of CRS, outbreak vaccination of young adults was conducted in November 2000 and among women ages 12 to 39 years in 2001 as part of a national campaign, with a coverage of 98% statewide.     

Congenital rubella syndrome after rubella vaccination in 1–4 weeks periconceptional period

Objective  To examine weather exposure to rubella vaccine during 1–4 wk periconceptional period can cause congenital rubella syndrome (CRS). Methods  This prospective study was performed in 60 pregnant women who received rubella vaccine inadvertently 1–4 wk pre or post conception. Time of conception was determined by last menstrual period (LMP) and first trimester sonography. In addition to gathering mother’s obstetric and demographic information, all neonates were evaluated for CRS signs by systemic physical examination and anti rubella IgG and IgM antibody titers in cord blood samples. Results  A total of 60 pregnant women with the median gestational age of 38 weeks were studied. The mean maternal age was 22 years and 58.3% of pregnancies were unintended. In 90% of mothers there were no post vaccination side effects (fever, lymphadenopathy, arthritis, arthralgia). None of the mothers had a history of drug abuse, smoking or teratogenic exposures. Mean neonatal weight was 3100grs and 6.7% of them were premature. No signs of CRS were found in the neonates based on systemic physical exam at birth and one month later. Mean value of cord blood anti rubella IgG titere was 148/28±67/26 lu/ml. cord blood anti rubella IgM was negative in all of the neonates. Conclusion  In this study inadvertent rubella vaccination 1–4 wk before and after conception did not cause CRS in neonates and according to all reasearches pregnancy termination is not indicated in these cases.     

Congenital rubella syndrome with death from interstitial pneumonia

There was a rubella epidemic in Japan in 2012–2013, which led to an increased number infants being born with congenital rubella syndrome (CRS). Symptoms of CRS are varied and include cataracts, congenital heart disease, and hearing impairment, but case reports of CRS complicated by interstitial pneumonia are rare. We report the case of a patient with CRS who died of respiratory failure caused by interstitial pneumonia. Thrombocytopenia had been present for approximately 1 month after birth, and the patient presented with sudden lung hemorrhage at 64 days old. Thereafter, respiratory condition deteriorated, and the patient died at 107 days old. Given that infants with CRS who have thrombocytopenia and interstitial pneumonia have a high risk of death, they should be monitored carefully for potential complications.     

Burden of congenital rubella syndrome (CRS) in India: <Emphasis Type="Italic">A systematic review</Emphasis>

Background

Rubella, though a mild, vaccine-preventable disease, can manifest with severe teratogenic effects in the fetus labeled as congenital rubella syndrome (CRS) due to primary maternal rubella infection. Despite a reduction in disease burden of several vaccine-preventable diseases through childhood immunization, CRS continues to account for preventable severe morbidity including childhood blindness, deafness, heart disease, and mental retardation.

Objective

To conduct a systematic review to describe the prevalence of CRS and its contribution to major long-term handicaps in Indian population. Another objective was to estimate the susceptibility to rubella infection in Indian adolescent girls and women of reproductive age-group. We also explored strategies to decrease CRS in India by identifying the immunogenicity of rubella containing vaccines (RCV) in Indian children and women, as well as their coverage in India.

Methods

Publications reporting ‘CRS prevalence in general population as well as selected subgroups i.e., suspected intra-uterine infection, congenital ocular abnormalities, deafness, congenital heart disease, mental retardation, and congenital malformations’, ‘seroprevalence to rubella (IgG) amongst women and adolescents’, and ‘immunogenicity and coverage of RCVs’ in Indian population were retrieved through a systematic search. Primary databases employed were Medline through PubMed and IndMed, websites of the WHO, and UNICEF. No restrictions were applied in terms of study designs. The primary outcome measure was ‘congenital rubella syndrome’ (CRS) which was further categorized as ‘suspected CRS’ and ‘confirmed CRS’ as defined by World Health Organization (WHO).

Results

Comprehensive evidence about the true burden of CRS in India is not available. Almost all studies have been done in institutional/hospital set-ups and community-based studies are grossly lacking. There are no studies assessing the prevalence of CRS in general population. All studies have evaluated the CRS burden in symptomatic cohorts of children. 1–15% of all infants suspected to have intra-uterine infection were found to have laboratory evidence of CRS. About 3–10% of suspected CRS cases are ultimately proven to have confirmed CRS with the aid of laboratory tests. CRS accounts for 10–15% of pediatric cataract. 10–50% of children with congenital anomalies have laboratory evidence of CRS. 10–30% of adolescent females and 12–30% of women in the reproductive age-group are susceptible to rubella infection in India. RCVs are highly immunogenic in Indian adolescents and women. The coverage data of RCVs in India is not available. However, the coverage of MMR vaccine has been reported as 42%, 30% and 5% from Delhi, Chandigarh and Goa, respectively.

Conclusion

This systematic review identifies and explores factors associated with the prevalence of CRS in India. There is a need for urgent action in terms of revamping the national immunization policy and introduction of RCVs in the national immunization program. Active surveillance of rubella and CRS is needed to redress the burden of CRS in India.

     

Estimating the burden of congenital rubella syndrome in Costa Rica, 1996-2001

BACKGROUND: The epidemiology of rubella in Costa Rica changed during recent decades, shifting the susceptible groups to the reproductive age. This study estimates the burden of congenital rubella syndrome (CRS) from 1996 to 2001 in this country. METHODS: Three methods to calculate CRS incidence were used. A retrospective search ("Observed cases") was conducted using hospital discharge records of children born from 1996 to 2001 with selected codes of ICD9 and ICD10 consistent with CRS and children <3 months of age with a positive serologic test for rubella IgM antibody at the National Children's Hospital (NCH). Cases were classified as either suspected, compatible or confirmed CRS and congenital rubella infection. "Expected" incidence of CRS was calculated using reported cases of rubella (women 15-45 years of age) and fertility rates, assuming CRS probability of 0.9 during the first trimester of pregnancy and 0.5 of asymptomatic rubella cases. "Estimated" CRS cases were calculated using incidence rates reported from modeling analysis during epidemic and endemic years. RESULTS: Of the 577 discharge charts reviewed and the 66 children reported as rubella IgM(+), 40 compatible CRS cases, 45 confirmed, and 4 with congenital rubella infection cases were identified. The range of annual incidence rate of CRS (per 1000 live births) was as follows: "Observed" = 0.00-0.33, "Expected" = 0.00-0.35 and "Estimated" = 0.5-1.5. Compared with the estimated number of CRS cases, only 27.2% of CRS cases were detected from the retrospective search and 10.1% would be expected when calculated using rubella reported cases. CONCLUSIONS: The under-detection of CRS cases using rubella reported cases in women of reproductive age and retrospective search of CRS reinforces the importance of suspecting CRS in the presence of a single compatible manifestation. Laboratory confirmation is indispensable to implement CRS elimination strategies and should be done in every suspected case.     

Molecular epidemiology of rubella virus in Asia: Utility for reduction in the burden of diseases due to congenital rubella syndrome

BACKGROUND: Rubella is a mild disease mainly of infants, involving a rash and a fever. However, when women who have no immunity to rubella are infected during the early stage of pregnancy, their babies are often born with congenital rubella syndrome (CRS), which is characterized by a few disorders including deafness, cataracts and heart malformations. To prevent CRS, several strains of live attenuated rubella vaccine have been developed and introduced into immunization programs in many countries. In most Asian countries except Japan, Singapore and Taiwan, rubella remains uncontrolled, and the burden of diseases from CRS is high. In order to develop a control program to reduce the number of CRS cases in Asian countries, it is necessary to conduct a survey of rubella and CRS cases, and to then determine the genotype of the circulating rubella virus in each country. METHODS: Cases of rubella and CRS, based on national reporting systems or active surveillance in the Asian countries, are summarized. Sequences of the E1 gene of the virus isolates from the Asian countries were compared by phylogenic analysis. RESULTS: Recent studies of the molecular epidemiology of rubella virus worldwide revealed that there are two genotypes, and that genotype I is circulating almost worldwide, while genotype II is an Asian prototype restricted to the Asian continent. Genotype I viruses fall into a number of groups, some of which are geographically localized. Antigenically these two genotypes are cross-reactive and immunization with either virus results in immunity to all rubella viruses. DISCUSSION: The hypotheses that rubella virus has evolved on the Asian continent is proposed. The World Health Organization (WHO) has recognized that a rubella immunization program can be combined with the measles immunization program. Inclusion of rubella in the expanded program of immunization (EPI) of measles would be ideal in Asian countries, as it would be efficient and cost effective to administer one injection containing a three-combined vaccine (MMR). It would also be desirable given that WHO require laboratory tests to confirm the presence of measles or rubella as part of it's measles control project, because rubella is often misdiagnosed as measles.     

A Rubella Epidemic in Sasebo, Japan in 1957, with Various Complications

Though the rubella vaccination programme for adolescent girls was introduced in Japan in 1977, rubella epidemics have occurred repeatedly. Also in Sasebo, Japan in 1987, we experienced various complications as follows: encephalitis (five cases), meningitis (three), thrombocytopenic purpura (four), vascular purpura (four), hemolytic anemia (two), pneumonia (eight), protein-losing gastroenteropathy (one), multiple organ disorder with encephalitis, purpura, myocarditis, hepatic and renal dysfunction (one), and congenital rubella syndrome (CRS: three). Disorders ranging over multiple organs seem to occur in acquired as well as congenital rubella infection. The incidence of encephalitis was estimated to be 1: 1600 cases of rubella and two of five cases were apparently serious. Though the strategy for preventing rubella has been directed only against CRS, we should note the various and severe complications with acquired rubella infection, and should adopt two-stepped protection: vaccination of young children of both sexes and of adolescent girls.     

Unusual encephaloclastic lesions with paraventricular calcification in congenital rubella

We report an unusual case of congenital rubella. The infant was suffering from a serious encephalopathy, and both prenatal echography and neonatal CT scan showed passive ventriculomegaly with a calcified periventricular border. Usually, such lesions are strongly suggestive of cytomegalovirus (CMV) infection and have never previoulsy been reported in congenital rubella. Classic cerebral lesions in rubella are related to a prominent obstructive vasculopathy. Conversely, encephaloclastic lesions in CMV infection are likely related to a necrosis of brain parenchyma following upon an initial ventriculitis, and perhaps also to a disturbance of neuronal proliferation. Recently, Carey described a neonate with proven congenital rubella and cranial ultrasound findings typical of ventriculitis. However, in spite of the close similarity between our patient's lesions and the typical CMV lesions, we think it's impossible to assert similar pathogenic mechanisms. Actually, it's quite conceivable that only a severe or extensive vasculopathy can lead to brain atrophy with periventricular calcification in congenital rubella.     

Incidence, clinical features and estimated costs of congenital rubella syndrome after a large rubella outbreak in Recife, Brazil, 1999-2000

BACKGROUND: During 1998-2000, a large rubella outbreak was reported from Recife, the capital municipality of Pernambuco State, in northeastern Brazil. In 2002, a study was conducted to assess the burden of congenital rubella syndrome (CRS) after this outbreak. METHODS: To describe the rubella outbreak, we analyzed data available from the National Notifiable Disease System. A retrospective record review for CRS was conducted at 6 maternity hospitals where 53% of Recife's resident live births occurred during 1999-2000 and 1 tertiary health care center. Suspected CRS cases were infants with any manifestation of CRS or maternal infection during pregnancy. Standard international definitions for compatible and confirmed CRS cases were used. Direct CRS costs were based on reimbursements by the National Health System. RESULTS: From October 1998 to July 2000, Recife reported 681 confirmed rubella cases. The highest incidence of rubella was among children 5-11 years of age (5.4 per 1000 population). Forty-five suspected CRS cases were identified; 29 were clinically compatible and 2 were laboratory-confirmed. The average annual incidence of CRS was 0.9 per 1000 live births during 1999-2000. Overall costs for the first year follow-up were estimated at 61,824 US dollars in this cohort. CONCLUSIONS: High rubella vaccination coverage is required to prevent the severe congenital disabilities and high economic costs of CRS. Increased clinician awareness is critical for early CRS detection. Complete reporting is essential to evaluate the impact of vaccination programs and to document progress toward the goal of CRS elimination in the Americas by the year 2010.     

Congenital rubella syndrome in Western Australia

The birth prevalence of congenital rubella syndrome (CRS) in Western Australia has been around 20 per 10 000 live births with peaks up to 100 per 10 000 in rubella epidemic years. The rate appears to be falling but it is still too soon to know whether the rubella vaccination programme in Western Australia has made a significant impact. The cohorts of young women who would have received the schoolgirl vaccination programme are only now entering the child bearing age groups. Observed falls may be due also to delayed diagnosis particularly of cases of deafness only. The proportion of nonimmune young women who would have been eligible for the programme in one maternity hospital has fallen. This study needs to be repeated to ascertain whether further epidemics of rubella have resulted in peaks of CRS or whether the vaccination programme has started to have an effect.     

Congenital rubella syndrome in Western Australia

Abstract The birth prevalence of congenital rubella syndrome (CRS) in Western Australia has been around 20 per 10 000 live births with peaks up to 100 per 10 000 in rubella epidemic years. The rate appears to be falling but it is still too soon to know whether the rubella vaccination programme in Western Australia has made a significant impact. The cohorts of young women who would have received the schoolgirl vaccination programme are only now entering the child bearing age groups. Observed falls may be due also to delayed diagnosis particularly of cases of deafness only. The proportion of nonimmune young women who would have been eligible for the programme in one maternity hospital has fallen.
This study needs to be repeated to ascertain whether further epidemics of rubella have resulted in peaks of CRS or whether the vaccination programme has started to have an effect.     

Fetal risk associated with rubella vaccination during pregnancy

BACKGROUND: Costa Rica implemented a nationwide measles-rubella vaccination campaign among men and women (15-39 years old) in May 2001. A protocol was developed to follow-up the vaccinated women who were unknowingly pregnant, to determine the risk of congenital rubella syndrome (CRS) or congenital rubella infection only associated with the administration of the rubella vaccine RA27/3 during pregnancy. METHODS: To classify the prevaccination maternal immune status, a serum sample was taken at the initial evaluation to detect IgM and IgG rubella antibodies (enzyme-linked immunosorbent assay). All pregnancies were followed up and all newborns were evaluated. A cord serum sample of their children was taken at birth. We calculated odds ratio, OR (95% confidence interval, 95% CI) associated with miscarriage, stillbirth, prematurity, low birth weight, and the presence of defects compatible with CRS. RESULTS: The prevaccination immune status was established in 797 women and 1191 mother and child pairs were analyzed. Adjusted OR for miscarriage (OR = 0.60, 95% CI = 0.26-1.39), stillbirth (OR = 1.32, 95% CI = 0.10-16.81), prematurity (OR = 0.25, 95% CI = 0.03-2.39), low birth weight (OR = 0.25, 95% CI = 0.03-2.23) and defects compatible with CRS (OR = 1.09, 95% CI = 0.34-3.54) showed no association between immune and susceptible maternal status. There were no cases of CRS and no children were IgM positive. CONCLUSIONS: No adverse pregnancy outcome such as miscarriages or CRS was documented in women who were vaccinated and unknowingly pregnant. These results support RA27/3 rubella vaccine safety.     

An epidemic of congenital rubella in Barbados

Rubella and congenital rubella syndrome (CRS) are preventable, but epidemics of rubella and CRS are not infrequent in the Caribbean and other developing countries. As a result of a surveillance system initiated after an epidemic of rubella in the Barbadian population in 1996, cases of CRS were identified and investigated. A total of seven cases of CRS were proven to be rubella IgM-positive. The infants were found to have a mean birthweight of 2587 g and a mean gestational age of 38 weeks. The clinical course, complications and outcome of those infants were documented and the cost of acute hospital care for each patient was also recorded. Cataracts in four infants, congenital heart disease in three and central nervous system abnormalities in five were the major clinical abnormalities. In four infants, two or more clinical systems were affected. The combined total hospital stay was 105 days (mean 15, range 0-44). A national effort to immunize all those at risk and a strict surveillance programme are essential to prevent future epidemics. This would lead to a significant reduction in the number of cases of rubella and CRS and could effect substantial savings in the national health budget.     

Rubella seroprevalence among Turkish adolescent girls living in Edirne, Turkey

This study was designed to estimate the rubella seroprevalence in unvaccinated Turkish adolescent girls in urban and rural areas of Edirne, and to create preventive strategies for congenital rubella syndrome (CRS). The sample, representing 12- to 17-year-old adolescent girls, consisted of 1,600 subjects selected from school lists by systematic and random sampling, which was matched by age and urban-rural residency strata proportional to the corresponding distributions in the Edirne population. For each participant, a questionnaire was completed and rubella-specific IgG antibodies were measured. After analysis of samples, seropositivity prevalence, equivocal and seronegative samples of adolescent girls in Edirne were determined as 93.1%, 0.6% and 6.3%, respectively. Data from the present study may indicate that 6.9% of adolescent girls have considerable risk for rubella infection during pregnancy. Eliminating rubella and CRS in Turkey will require national health service efforts, including vaccination of all adolescents and all susceptible women of childbearing age.     

Clinical Studies of Patients with Rubella Syndrome Occurring in a High Incidence in the Ryukyu Islands in 1965: On the Diagnostic Significance of Clinical Manifestations

The 620 cases with a history of maternal rubella and/or with one or more of cataract, CHD and deafness born in the Ryukyu Islands in 1965 were subjected to clinical studies and 360 cases were diagnosed as rubella syndrome. Rubella retinopathy makes a reliable diagnostic basis for rubella syndrome. As for the typical combination of clinical manifestations, the cases with cataract have CHD, deafness and retinopathy; the cases with CHD not accompanied by cataract have deafness and retinopathy; and the cases with deafness not accompanied by cataract and CHD either have or have not retinopathy. The cases with cataract alone or with CHD can extremely rarely be denned as having rubella syndrome. There existed the close relationship between the combination of clinical manifestations and the time of maternal rubella infection. The main clinical manifestations of rubella syndrome are cataract, congenital heart disease (CHD), and deafness appearing singly or two or more together¹⁾²⁾. Etiology of the congenital cataract, CHD, or deafness is mostly not explained; and only a small portion of the incidence is due to intrauterine rubella infection. For the future of the patients in the above conditions and for their parents, clarification as to whether they are or are not due to rubella is very important. Current general practice for the diagnosis of rubella syndrome is laboratory diagnosis that measures rubella hemagglutination inhibition (HI) antibodies³⁾⁴⁾. As the infants grows in age, the above mentioned technique may not assure an absolute judgment⁵⁾⁶⁾. This makes it necessary to arrive at diagnosis from the clinical findings. A prompt, direct diagnosis from the clinical findings alone will be very convenient. In the Ryukyu Islands where there was a high incidence of rubella syndrome in infants in the second half of 1965, we analyzed clinical findings of the affected children with cataract, CHD or deafness on the basis of serological diagnosis. The diagnostic significance of the clinical findings was so studied. Hence our report in this paper.     

Neuroimaging findings in children with Keutel syndrome

Background

Keutel syndrome is a rare autosomal-recessive condition characterized by abnormal cartilage calcification. Neuroimaging findings associated with this condition have been randomly described in the literature.

Objective

To systematically evaluate the neuroimaging findings in a series of children with Keutel syndrome to broaden our base of knowledge.

Materials and methods

Four children with confirmed Keutel syndrome were reviewed for the brain, head and neck imaging findings.

Results

Three of the four children, all siblings, showed evidence of moyamoya syndrome. All four siblings had pinna cartilage calcification.

Conclusion

We propose that Keutel syndrome be considered and included among the secondary causes of moyamoya syndrome. In children with petrified auricle and neurological symptoms, Keutel syndrome should be considered and brain MRI with MRA is required.     

Aicardi-Goutières syndrome: clinical and neuroradiological findings of 10 new cases

AIM: To describe the clinical and neuroimaging findings in new cases with Aicardi-Goutières syndrome (AGS) from Egypt. METHODS: Ten patients with progressive encephalopathy, bilateral calcification of the basal ganglia and spastic quadriplegia were described. Feeding difficulties, irritability, unexplained episodic fever and acrocyanosis were also observed. They were diagnosed as AGS after excluding possible non-genetic causes (especially TORCH) and because of the high interferon-alpha (IFN-alpha) level in cerebrospinal fluid (CSF) in two children who underwent this specific investigation. RESULTS: Six patients had postnatal microcephaly. Putamen was by far the most common site of calcification (nine cases) inside the basal ganglia. Calcifications were extended to the white matter, periventricular and cerebellum in three cases. Brain atrophy and/or white matter demyelination were evident in most of the cases. Further, hypogenesis of corpus callosum was detected in two cases; one of them had in addition cerebellar hypoplasia, atrial septal defect (ASD) and horseshoe kidney. To the best of our knowledge, the association of these congenital abnormalities has not been reported before in AGS. Eight families were consanguineous. CONCLUSION: This paper presents variability in both age of onset, clinical picture and neuroimaging findings even in the same family, comprising new congenital abnormalities associated with AGS and subsequently expanding the spectrum of heterogeneity. The observation of familial cases and both affected males and females emphasized the major role of the single gene inheritance.     

Deep Sensation in the Congenital Rubella Syndrome

Sixty-three deaf children with congenital rubella syndrome (CRS) and 61 non- CRS deaf children were studied for their sensations of vibration and position. Both groups showed normal responses in the sensation of vibration. However, 35 CRS and 22 non-CRS deaf children revealed abnormal responses in the sense of position. This gave a significantly higher ratio in the CRS deaf children.     

Beta2-microglobulin concentrations in cerebrospinal fluid correlate with neuroimaging findings in newborns with symptomatic congenital cytomegalovirus infection

Overview In newborns with symptomatic congenital cytomegalovirus (CMV) infection, neuroimaging is the best available predictor of neurodevelopmental outcome. Cerebrospinal fluid (CSF) findings in congenital CMV infection have seldom been described. Neonates with central nervous system infections present high CSF Beta₂-microglobulin (β₂-m) levels.Objectives The objectives of this study were: (1) to determine whether CSF β₂-m is increased in newborns with symptomatic congenital CMV infection, and (2) to examine its correlation with neuroimaging findings.Materials and methods Fourteen newborns with symptomatic congenital CMV infection admitted to La Paz Hospital from 1990 through 2004 underwent determination of CSF β₂-m. Ninety-three newborns, constituting the comparison group, underwent CSF β₂-m determination as part of a sepsis or meningo/encephalitis work-up, and at discharge had sterile cultures and normal neurological status. Neuroimaging findings were scored according to a semiquantitative system: (0) no abnormalities; (1) single punctate periventricular (PV) calcification and/or hyperechogenic areas in the thalamus and basal ganglia; (2) multiple discrete PV calcifications and/or ventriculomegaly; and (3) extensive PV calcifications and/or brain atrophy.Discussion and conclusion CSF β₂-m was increased in newborns with CMV infection (median 6.21 mg/L) compared with controls (1.68 mg/L) (P<.001). β₂-m showed a correlation with neuroimaging scores (r _s=0.753, P=.002). β₂-m was higher in patients who scored 2–3 (12.83 mg/L) than in patients who scored 0–1 (5.52 mg/L) (P=.028). CSF β₂-m is increased in newborns with symptomatic congenital CMV infection and correlates with neuroimaging abnormalities. β₂-m appears to be an indicator of the severity of brain involvement in congenital CMV infection.