Mental Retardation

1.1 Introduction
Mental retardation (MR) is a generalized disorder appearing before adulthood, characterized by significantly impaired cognitive functioning and deficits in two or more adaptive behaviors. It has historically been defined as an Intelligence Quotient score under 70.[1] Once focused almost entirely on cognition, the definition now includes both a component relating to mental functioning and one relating to individuals' functional skills in their environment. As a result, a person with a below-average intelligence quotient may not be considered mentally retarded. Syndromic mental retardation is intellectual deficits associated with other medical and behavioral signs and symptoms. Non-syndromic mental retardation refers to intellectual deficits that appear without other abnormalities. Mental retardation is not a disease but a condition in which the intellectual faculties are never manifested or have never been developed sufficiently to enable the retarded person to acquire such an amount of knowledge as persons of his own age and placed in similar circumstances with him-self are capable of receiving.

1.2 Definition
Mental retardation is defined as: Significantly sub-average general intellectual functioning (i.e.2 standard deviation below the mean). IQ below 70. Significant deficit or impairment in adaptive functioning (ie.person's ability to meet the responsibilities of social, personal, interpersonal and occupational areas of life according to his age and sociocultural and educational background.

1.3 Epidemiology:
There are about 15 million mentally retarded in India (2-3% children of general population). The highest incidence in school age children with peak at ages 10 to 12. It is twice as common in boys and girls. Classification of mental retardation: There are four types of mental retardation depending on IQ (+ 5 points in all types) and adaptive behavior. Mild Mental Retardation: (IQ 50 to 70). This constitutes about 85% of the total mentally retarded. Usually their appearance is unremarkable and any motor or sensory deficits are slight. Most people in this group develop more or less normal language abilities and social behavior during the preschool years and their mental retardation may not be detected until the start of schooling. Moderate Mental Retardation: (IQ 35 to 50) about 10% of all the mentally retarded come under this category. In the educational classification, this group was earlier
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called 'trainable', although many of these persons can be educated. They can be trained to speak and support themselves by performing semiskilled or unskilled work under supervision. Severe Mental Retardation: (IQ 20-35) People with severe mental retardation account for about 7% of the mentally retarded. In the preschool years, their development is usually greatly slowed. Eventually, many of them can be trained to look after themselves under close supervision and to communicate in a simple way. As adults, they can undertake simple tasks and engage in limited activities. In the earlier educational classification, they were called dependent. Profound Mental Retardation: (IQ below 20). Less than 1% mentally retarded, only few of them learn to care of themselves completely. Some eventually achieve some simple speech and social behavior.

1.4 Causes of Mental Retardation

I. Prenatal Causes Infections (rubella, cytomegalovirus, syphilis, toxoplasmosis) Physical Damage (injury, hypoxia, radiation) Intoxications (lead, certain drug) Placental dysfunction (toxemia, nutritional growth retardation) Endocrine disorders (hypothyroidism, hypoparathyroidism) 2. Intranatal Causes Birth asphyxia Prolonged or difficult birth Prematurity (complications or excessive oxygen) Kernicterus Instrumental-delivery (head injury: intra-ventricular haemorrhage) 3. Postnatal Damage Injury (accident, child abuse) Infection (encephalitis, meningitis) Intoxication (Lead) 4. Genetic
a) Chromosomal Abnormalities

 Down's syndrome Klinefelter's syndrome Fragile-X- syndrome Trisomy-21 Turner's syndrome

b) Metabolic Disorders Affecting

Amino acids (e.g. Phenylketonuria, Maple syrup urine disease) Lipids (Taysach's, Gaucher's, Niemann-Picks). Carbohydrates - Galactosemia, Gargoylism. Purines - Lesch-Nyan syndrome. Urea cycle - Crystallinuria, aminosuccinic aciduria. Mucopolysaccharides-Hurler's, Hunter's, Sanfillipo's, and Morquio's disease Miscellaneous- Wilson's disease (copper), Tonifanconi syndrome ( cysteine)
c)Gross Disease of Brain

Tuberous sclerosis Neurofibromatosis Epilepsy

d) Cranial Malformations

Hydrocephaly Microcephaly 5. Sociocultural Causes Deprivation of sociocultural stimulation 6. Psychiatric Conditions Autistic disorder Rett's syndrome Childhood-onset schizophrenia Asperger's syndrome
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1.5 Clinical Picture:

It is made up of a number of features any of which can occur in a normal person. Four of these features are generally accepted as strong evidence for the syndrome. The most characteristic signs are Mouth - small mouth and teeth, furrowed tongue, high arched palate. Eyes - oblique palpebral fissures, epicanthic folds.

Head - flat occiput. Hands - short and broad, curved fifth fingers single transverse crease. Joints - hyperextensibility or hyperflexibility, hypotonia, poor Moro feflex. Others - Congenital heart disease (especially arterioventricular communes, ventricular septal defect. patent ductus arteriosus) in about 40% cases, Burchfield Spots (Whitish specking of the iris), flat facieses, small dysplastic ear, impaired hearing and intestinal abnormalities (especially duodenal obstruction).IQ generally between 20 and 50, Hypothyroidism, epilepsy, ocular disturbances, reduced fertility etc.

1.6 Diagnosis of mental retardation

1)History Family history, especially family history of all inherited disorders, or abnormalities in pregnancy and delivery, developmental milestone and associated behavioural disorders. 2) General Physical Examination Height, weight, head circumference, physical signs of specific disorders e.g., heart, kidney etc. 3) Detailed Neurological Examination: especially vision, hearing or specific signs. 4) Mental Status Examination: to detect associated psychiatric disorders and also level of intelligence 5) Investigations. Routine Urine examination, e.g., phenylketonuria, Alkeptonuria, Maple Syrup urine Disease Blood Test: Levels or phenylalanine or specific metabolites in inborn metabolic disorders. Chromosomal Studies: Amniocentesis, Chorionic villi biopsy.
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 Endocrinal: Thyroid function tests. Liver Function tests and biopsy e.g., in Wilson's disease. EEG to detect seizure disorder e.g., in Tuberous sclerosis. CT Scan of Brain e.g., Tuberous sclerosis, neurological syndromes, skull configuration anomalies, focal seizures etc. 6) Developmental Assessment It is based on a combination of clinical experience and standardized methods of measuring intelligence Stanford Binet, Binet Kamath, Wechsler scales for children (6Y2 to 16 years) Wechsler preschool and primary scale of intelligence, Bhatia's Battery performance tests, Seguin Form Board test, Raven progressive matrices etc. Language, motor performance and social skills (Vineland social maturity scale, Denver Development Screening Test, Gessell's Development schedule etc.). Differential Diagnosis Delayed maturation (Specific Developmental Disorders). Blindness or other sensory defects. Childhood psychosis (Childhood onset Schizophrenia). Childhood autism Severe neuroses. Systemic disorders with physical handicap. Deprived children with insufficient stimulation. Epilepsy. States due to the side effects of drugs (e.g., antipsychotics, anticonvulsants etc.).

1.7 Management of mental retardation:

No satisfactory treatment is available till today. No drugs are available to increase the level of intelligence. Most of the mentally retarded children brought for treatment can only be benefited only to a limited extent. Management of mentally retarded patients is directed at the following levels:

1. Primary Prevention: (a) Health Promotion is directed at Good antenatal care and encouraging deliveries in hospitals under proper supervision and care. Improving the socioeconomic status of the country. Education of the public to help in early detection of mental retardation and also, to remove various misconceptions about its causes and treatment. Facilitating research to identify the causes, and to invent new methods of treatment. (b) Specific Protection Good parental, natal and postnatal care the pregnant mothers at risk. Genetic counseling to at risk patients e.g., in phenylketonuria. Avoiding childbirths in late age of the mother (e.g., to prevent Down's syndrome). Avoiding consanguinal marriages in case the hereditary factor is' operative. Avoiding marriages of mentally retarded (especially to mentally retarded) where strong inheritable factors are operating e.g., Tuberous sclerosis. Vaccination of girls with rubella foetus due to rubella. Avoid giving pertusis vaccine to children with history of convulsions or neurological abnormalities 2. Secondary Prevention (Early Diagnosis and Treatment) Early detection and treatment of the preventable disorders (metabolic, endocrinal and nutritional disorders) e.g., Cretinism (thyroxine is given), Phenylketonuria (restrict Phenylalanine in diet), Maple Syrup Disease (Diet with low branched chain amino acids). Amniocentesis and medial termination of pregnancy on medical grounds. Early detection of correctable disorders e.g., nutritional deficiencies (replacement), infections (antibiotics), hydrocephalus and skull configuration disorders (surgery) or situations (understimulation) and their treatment. Prevent them against abuse e.g., (physical or sexual abuse) by legal or by medical measures (e.g., tubectomy of severely retarded girls).

3.Tertiary Prevention (a) Disability Limitation Treatment of physical and psychological problems (by drugs, behavior modification). Institutionalization of severe mentally retarded or those with psychological problems. Education (if educable) and training to avoid handicaps. Physiotherapy to treat the associated deficits. (b) Rehabilitation: It depends on the patient's level of intelligence and his aptitude. These patients need warmth, love; appreciation and discipline. Rehabilitation is aimed at physical (appliances for handicaps), social (social skills training) and occupational areas (e.g., by teaching and training the patients to make them self sufficient). Day care centres and schools, integrated schools, vocational training centres, sheltered farms and workshops are useful. c) Counselling to Parents: Parents should be explained about the causation, and prognosis of mental retardation (to allay their misconceptions, fear and unwarranted expectations of miraculous cure). To educate mothers and families in caring for the mentally handicapped (e.g., training mentally retarded girls in household activities). Special supervision for the physically handicapped or those severely and profoundly mentally retarded. Treatment of psychological problems in parents. (eg: depression in mother resulting in under stimulation of child results in retardation.)

1.8 Conclusion
Although there is no specific medication for mental retardation, parental support with proper medical attention will make marked improvement in functioning level of mentally challenged children. Nurses requires specialised managerial skills to meet the daily needs of these children and have an important role in their rehabilitation programme.
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