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Year : 2016  |  Volume : 11  |  Issue : 2  |  Page : 137-139

Psychiatric manifestations of congenital rubella syndrome: A case report and review of literature

Department of Psychiatry, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication3-Aug-2016

Correspondence Address:
Sandeep Grover
Department of Psychiatry, Postgraduate Institute of Medical Education and Research, Chandigarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.187643

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Neurodevelopmental disorders are known to have varied etiology. Among known etiologic causes, congenital rubella syndrome (CRS) is reported to be one of the infections associated with neurodevelopmental disorders. CRS has been reported to be associated with large number of psychiatric manifestation. However, data from developing countries on psychiatric manifestations of CRS are nonexistent. In this report, we present the case of a 7-year-old boy, who presented with mental retardation, atypical autism, and attention deficit hyperactivity disorder. Since birth, the child was found to have congenital cardiac defects and was found to have bilateral profound sensorineural hearing loss since the age of 6 months. Magnetic resonance imaging showed multifocal symmetrical T2/fluid attenuated inversion recovery hyperintensities in bilateral cerebral hemisphere suggestive of sequelae of congenital rubella infection.

Keywords: Congenital rubella syndrome, psychiatric manifestations, rubella

How to cite this article:
Chauhan N, Sen MS, Jhanda S, Grover S. Psychiatric manifestations of congenital rubella syndrome: A case report and review of literature. J Pediatr Neurosci 2016;11:137-9

How to cite this URL:
Chauhan N, Sen MS, Jhanda S, Grover S. Psychiatric manifestations of congenital rubella syndrome: A case report and review of literature. J Pediatr Neurosci [serial online] 2016 [cited 2023 Sep 25];11:137-9. Available from: https://www.pediatricneurosciences.com/text.asp?2016/11/2/137/187643

   Introduction Top

Neurodevelopmental disorders are known to have a varied etiology. A host of causes starting right from the antenatal period (maternal viral infections, drug exposure, radiation exposure), perinatal events (such as fetal hypoxia due to difficult/prolonged labor), postnatal causes (bacterial/viral meningitis, encephalitis, metabolic disturbances such as hypocalcaemia, hypoglycemia, hypomagnesaemia) can lead to neurodevelopmental disorders. Besides these, various genetic syndromes (for example, Down's syndrome, fragile X syndrome, tuberous sclerosis) can also lead to neurodevelopmental disorders.

Among the various antenatal infective causes, toxoplasmosis, other infections (syphilis, varicella-zoster, parvovirus B19), rubella, cytomegalovirus, and herpes infection are also important causes of neurodevelopmental disorders. Rubella infection during the first 12 weeks of pregnancy is associated with 80% increase in the risk of congenital abnormalities. [1] Apart from congenital abnormalities, serious pregnancy-related outcomes such as miscarriages and stillbirths are also known to be associated with maternal rubella infection.

Rubella infection spreads through the placenta to the fetus. The virus involves the chorionic tissue of the placenta resulting in necrosis of the epithelium and fragmentation of small blood vessels and thus it may reach fetal tissue through infected emboli or cause direct damage to small fetal blood vessels. The virus is then widely disseminated within the fetal tissues. According to Chess et al. [2] and Desmond, [3] the damage occurs as a result of two processes following rubella infection, namely cell death and decrease in rate of cell growth. Congenital rubella syndrome (CRS) is the term used to describe the group of congenital abnormalities that occur in a child as a result of rubella infection of mother during gestation. The clinical features can be transient, self-limiting, or permanent, and some features may not manifest until adolescence or adulthood (called "late manifestations"). [4] CRS comprises a triad of severe birth defects in the form of sensorineural deafness, ophthalmological abnormalities (retinopathy, cataract, microophthalmia), and congenital heart disease (pulmonary artery stenosis and patent ductus arteriosus). Patients with CRS may additionally have intellectual disability, microcephaly, micrognathia, low-birth weight, thrombocytopenic purpura, hepatomegaly, problems involving spleen, endocrinal problems (diabetes, hypothyroidism, hyperthyroidism, thyroiditis), esophageal, gastrointestinal, and urogenital problems. [1],[5],[6],[7]

CRS can also lead to large number of psychiatric manifestation, and it is reported that up to half of all patients born with CRS have some form of psychiatric manifestations. [2] The commonly seen psychiatric manifestations include developmental delay, mental retardation, and autism.

Rubella infection is still present in developing countries like India. A recent systematic review concluded that there are no studies to assess the prevalence of CRS in general population or in children. The review suggested that laboratory evidence of CRS was present in 1-15% of infants suspected to have CRS and 3-10% of suspected cases of CRS were confirmed by laboratory tests. Most of the studies from India which have attempted to estimate the prevalence of CRS have included subjects with physical defects (congenital ocular abnormalities, hearing impairment, congenital heart disease, and congenital malformations) as the main inclusion criteria. [8] Only four studies from India have evaluated rubella as an etiological factor in subsets of children presenting with mental retardation/developmental delay with suspected intrauterine infections/congenital malformations and reported mental retardation in 7.6-60% of seropositive cases. [9],[10],[11],[12] Overall, it can be said that there is limited data on psychiatric manifestations among patients with CRS from developing countries like India.

In this article, we present the case of a 7-year-old child with CRS who presented with mental retardation, features of atypical autism along with attention deficit hyperkinetic disorder.

   Case Report Top

A 7-year-old boy presented with behavioral problems since infancy. Detailed evaluation of the patient's early developmental history revealed that he was first in birth order, born of a wanted but unplanned pregnancy. During antenatal period, at 36 weeks of pregnancy, mother was found to have oligohydramnios and possible congenital cardiac defects were reported for the fetus. The child was born of a preterm delivery (36 completed weeks) by emergency cesarean section due to fetal bradycardia. The birth weight of the child was 1.7 kg, he had delayed cry and was cyanosed at birth. Cardiac evaluation at birth confirmed cardiac anomalies (patent ductus arteriosus, ventricular septal defect, tricuspid regurgitation, and left atrium to right atrium shunt), and he was started on tablet digitoxin. Thereafter, as he grew, it was observed that he had a global developmental delay and was detected to have bilateral profound sensorineural hearing loss at 6 months of age. Over the years he continued to manifest delayed milestone and developed autistic features in the form of poor eye contact, poor social skills, poor communication skills, engaging in repetitive head rocking (shaking head side to side) and hand flapping (moving fingers and hand like that of a bird, quick, bending from the wrist) movements, and fixed routine (did not like change in place, while visiting relative's place or if any guest came to their house was seen to become irritable). In addition, since the age of 3 years, he was found to have symptoms of attention deficit hyperkinetic disorder (ADHD) (could not sit at one place for more than 2-3 min, difficult to engage him in any activity). Over the years, he became very adamant about the fixed routine and hyperactivity kept on increasing, also started hitting and biting mother, especially if he did not get what he wanted, which led to severe distress and burden on the mother and resultant psychiatry consultation and admission to child psychiatry inpatient unit. There was no history suggestive of head injury, seizures, frequent diarrhea and vomiting, birth marks, failure to thrive. Physical examination of the child revealed abnormalities in the form of small head (head circumference 49 cm) and bilateral flat feet. On the mental status examination, there was lack of eye to eye contact, he was found to be hyperactive, had irritable mood, along with frequent side to side movement of head and flapping of hands. On evaluation, his intelligence quotient was found to be 25. Magnetic resonance imaging showed multifocal symmetrical T2/fluid attenuated inversion recovery hyperintensities in B/L cerebral hemisphere suggestive of sequelae of congenital rubella infection. All other blood investigations were within normal limits except for Vitamin D levels, which were low (<3 ng/ml). Ophthalmological examination showed evidence of pigmentary retinopathy. He was advised to use hearing aids for profound sensorineural hearing loss by the otorhinolaryngologist. Cardiac evaluation confirmed the presence of cardiac defects; however, there was no decompensation.

On the basis of available evidence, he was diagnosed as having with mental retardation, atypical autism, and attention deficit hyperkinetic conduct disorder. All these symptoms possibly represented a clinical sequelae of CRS.

In the inpatient setting, he was managed with behavior therapy (positive reinforcement, ignoring, modeling, and prompting), parental counseling along with tablet atomoxetine 20 mg/day, tablet risperidone 1 mg HS, tablet phenargan 25 mg/day, and Vitamin D supplementation. Over the period of 8 weeks, patient showed significant reduction in hyperactivity, adamant behavior, and interaction with mother improved. This led to significant reduction in burden on the mother. He has been maintaining well with significant improvement in hyperactivity and various adaptive functions.

   Discussion Top

Rubella, also known as "German measles," was first described by two German physicians in as early as mid-18 th century, [13] but in recent times it was in 1941 when Gregg and Banatvala, ophthalmologists described congenital cataracts and other abnormalities in a group of infants whose mothers had rubella infection early in pregnancy, this infection came into fore front. [14]

Case studies have reported different mental and behavioral disorders among individuals with CRS, but no overall characteristics or prevalence has been reported. [8] Mental retardation is seen in about one-third (37%) of the subjects with CRS and autism has been reported in 7.4% of children with CRS. [15] High rate of impulsivity, disruptive activity, tantrums, self-injury, and aggression are also seen. [15] It has been observed that as the number of physical defects in congenital rubella increases, it simultaneously increases the risk of both presence and severity of psychiatric pathology. [15] Accordingly, all patients suspected to have CRS must be routinely screened for psychiatric manifestations and early intervention must be instituted to improve the outcome. Index case had features of both mental retardation and atypical autism. The index case also reflects that although the child was found to have congenital defects consistent with CRS, the diagnosis was not confirmed till he presented to us. Accordingly, no psychiatric intervention was started, which led to a loss in opportunity for early intervention. Surprisingly, there is a lack of information about ADHD among subjects with CRS. We could not find ADHD as a clinical manifestation of CRS in the reported literature. A thorough internet search of literature on CRS did not reveal the prevalence rate of ADHD among children with CRS. There could be many reasons for the same. First, most of the literature on CRS from developed countries are 2-3 decades old, when ADHD was not a well-recognized clinical entity, as it is today. Second, it is quite possible that many patients with CRS actually do not receive proper psychiatric evaluation and hence, various psychiatric manifestations are missed.

Presence of mental retardation, atypical autism, and ADHD in the present case suggests that there is a need to understand the behavioral phenotype of CRS. Although behavioral phenotype is a term used to describe observable characteristics that occur more often in individuals with a specific genetic syndrome (for example, over eating in Prader-Willi syndrome), a similar approach can also be tried for individuals with CRS. Considering the fact that ocular abnormalities (cataract, pigmentary retinopathy), sensorineural hearing loss, and congenital heart defects, along with multiple psychiatric syndromes (mental retardation, autism and ADHD) co-occur in individuals with CRS, even this can be considered as a behavioral phenotype of CRS.

The present case highlights the fact that many patients with CRS do not receive proper psychiatric evaluation. This case also suggests that CRS must always be considered as an etiological possibility among infants and children presenting with mental retardation. Further, this case highlights the fact that CRS may manifest with a specific behavioral phenotype which requires proper characterization.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Best JM. Rubella. Semin Fetal Neonatal Med 2007;12:182-92.  Back to cited text no. 1
Chess S, Korn SJ, Fernandez PB. Psychiatric Disorders of Children with Congenital Rubella. New York: Brunner/Mazel; 1971.  Back to cited text no. 2
Desmond MM. Medical Aspects of the Congenital Rubella Syndrome. Baylor College of Medicine, Houston, Texas; Samuel P. Hayes Research Library, Perkin School for the Blind, Watertown, Mass; 1975.  Back to cited text no. 3
O'Donnell N. A Report on a Survey of Late Emerging Manifestations of Congenital Rubella Syndrome. New York: Helen Keller National Center; 1991.  Back to cited text no. 4
Reef S, Redd S. Congenital rubella syndrome. In: Centers for Disease Control and Prevention. Manual for the surveillance of vaccine-preventable diseases. Atlanta, GA: Centers for Disease Control and Prevention; 2008.  Back to cited text no. 5
Oster ME, Riehle-Colarusso T, Correa A. An update on cardiovascular malformations in congenital rubella syndrome. Clin Mol Teratol 2010;88:1-8.  Back to cited text no. 6
Dammeyer J. Mental and behavioral disorders among people with congenital deafblindness. Res Dev Disabil 2011;32:571-5.  Back to cited text no. 7
Dewan P, Gupta P. Burden of congenital rubella syndrome (CRS) in India: A systematic review. Indian Pediatr 2012;49:377-99.  Back to cited text no. 8
Ballal M, Shivananda PG. Prevalence of rubella virus in suspected cases of congenital infections. Indian J Pediatr 1997;64:231-5.  Back to cited text no. 9
Broor S, Kapil A, Kishore J, Seth P. Prevalence of rubella virus and cytomegalovirus infections in suspected cases of congenital infections. Indian J Pediatr 1991;58:75-8.  Back to cited text no. 10
Chakrabarty MS, Das BC, Gupta B, Sarkar JK. Rubella as an aetiological factor of congenital malformation in Calcutta - A serological study. Indian J Med Res 1975;63:1438-45.  Back to cited text no. 11
Chaturvedi UC, Tripathi BN, Mathur A, Singh UK, Mehrotra RM. Role of rubella in congenital malformations in India. J Hyg (Lond) 1976;76:33-40.  Back to cited text no. 12
Forbes JA. Rubella: Historical aspects. Am J Dis Child 1969;118:5-11.  Back to cited text no. 13
Gregg NM, Banatvala JE. Congenital cataract following German measles in the mother. Trans Ophthalmol Soc Aust 1941;3:35-6.  Back to cited text no. 14
Chess S, Fernandez P, Korn S. Behavioral consequences of congenital rubella. J Pediatr 1978;93:699-703.  Back to cited text no. 15

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