mental retardation

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DR.SANDIP GUPTA PGT,PEDIATRICS B.S.M.C.H. APPROACH TO A CASE OF MENTAL RETARDATION

INTRODUCTION:

INTRODUCTION Mental retardation is one of most common chronic neurologic disabiity of childhood. MR affects about 1-3% of population. Majority of cases are idiopathic. Most common mild MR(75-90%) often goes unrecognised . More common in lower socio-economic groups.

DSM IV – TR Definition:

DSM IV – TR Definition Significantly below average intellectual functioning: IQ of below 70 on an individually administered IQ test. Accompanied by significant limitations in adaptive functioning in at least 2 skill areas: Communication, self-care, home living, social/interpersonal skills, use of community resources, self-direction, functional academic skills, work & leisure, health and safety. Onset before age 18yr.

Signs & Symptoms:

Signs & Symptoms Delay in language development. Find it hard to remember things. Difficulty in learning social rules. Have trouble learn specific subject Have trouble solving problems Lack of social inhibitors. Lack of self care skills. Persistence of infantile behaviour . Unable to take higher education.

Grades of MR ::

Grades of MR : According to IQ : Borderline intellectual functioning70--84 Mild mental retardation 50 – 69 Moderate mental retardation 35 – 49 Severe mental retardation 20 – 34 Profound mental retardation Below 20

Mild MR:

Mild MR May not be obvious in early childhood, untill they go to school. In School - have poor academic performance (difficult to differentiate from learning disability or emotional & behavioral disorder.) can learn reading & mathematical skills to level of a typical 9-12 yr child. Learn self care &practical skills to live independently & earn for them self.

Moderate MR:

Moderate MR obvious within 1 st year of life Problem with social work Behaviourable age -8 yrs Academic skills – 2 nd grade level will face difficulty in school, at home, and in the community Need special school, but they can still progress to become functioning members of society. As adults they may live with their parents, in a supportive group home.

Severe & Profound MR:

Severe & Profound MR Little or no speech Limited abilities to manage self care. Require high supervision . Behaviourable age – 3 yrs. They may learn some daily activities but will require full time care –taker.

ASSOCIATED PROBLEMS:

ASSOCIATED PROBLEMS Self injury Pica CP Epilepsy Toilet probs Sleep disorders Eating problems Poisoning Sexual abuse Learning disorders Behavior problems

Developmental assesment:

Developmental assesment Denver Development Screening Test-II Bayley Scales of Infant Development BARODA PHATAK DST Trivendrum Developmental Screening Chart Test for intelligence Binet kamat test Weschler’s intelligence scale for children Goodenough draw a man test.

Denver II Developmental Screening Test:

Denver II Developmental Screening Test Most widely used test for quick routine screening, for children upto 6yrs. Assesses child development in all four domains (gross motor , fine motor adaptive, language, personal social behavior),taking 10-30 min. The milestones are shown in a graphical manner & items through which the chronological age passes are tested.

BAYLEY SCALE OF INFANT DEVELOPMENT:

BAYLEY SCALE OF INFANT DEVELOPMENT Based on motor scale , mental scale and infant behaviour Up to 30 months of age Takes 30-60 minutes 67 motor scale ,107 mental scales BARODA DEVELOPMENT SCREENING TEST Based on BSID, developed by Dr. phatak according to baroda norms, suitable for Indian children. Does not require standard equipment, domains evaluated are gross motor ,fine motor, cognitive, takes 10 min.

Wechsler Intelligence Scale for children:

Wechsler Intelligence Scale for children for children from 5-15yrs Mean score of 100 with standard deviation of 15 Gives verbal and performance scores, takes 45-60 min. Broken into 12 subtests 6 each for verbal & performance abilities. MALIN INTELLIGENCE SCALE FOR INDIAN CHILDREN : - Indian adaptation of WISC - It may not give real capabilities in non school going children as mostly influenced by formal schooling system  

STANFORD BINET INTELLIGENCE SCALE:

STANFORD BINET INTELLIGENCE SCALE for children>2yr. I nclude verbal ability ,perceptual skills , short term memory & hand and eye co-ordination takes 45-60 minutes BINET – KAMAT TEST - indian adaptation of stanford - binet scale - also available in hindi

Goodenough harris ‘draw a man test’:

Goodenough harris ‘draw a man test’

APPROACH TO A CHILD WITH MR:

APPROACH TO A CHILD WITH MR Detailed development history. Antenatal history. Perinatal history. Any neurological problem: seizure, spasticity, motor deficit, abnormal movements, vision& hearing. Features s/o of IEM: abdominal distension. F/o of hypothyroidism. Past history of febrile encephalopathy.

Cont..:

Cont.. Emotional deprivation. Level of indipendence . Scholastic performance. Consangiunity . Sibling history. H/o temper tantrums, hyperkinesis , self destructive behaviour . Any h/o physical & psychological abuse. Lead exposure.

Physical examination:

Physical examination Anthropometry : OFC, shape, frontanelle sutures, monitor OFC. Dysmorphic features: f/o MPS,f /o down’s syndrome, fragileXsyndrome , Neurocutaneous disorder. Detailed neurological examiation : tone, power, primitive reflexes, symmetry. Assesment of hearing & vision.

Investigations :

Investigations Individualised approach Vision & hearing assesment R/o hypothyroidism. Cytogenetic study: high resolution g banding karyogram screening for numeric& structural anomalies, FISH analysis for subtelomeric rearrangement. Neuroimaging : MRI & CT scan. IEM EVAUATION:24 hr urinary screening for aminoacids , organic acids, GAGs, pl.aminoacids , Enzymatic study in fibroblasts & lymphoblasts . EEG Xray Fragile X syndrome screening

Management Treatable /Preventable causes of MR:

Management Treatable /Preventable causes of MR Hypothyroidism Severe PEM Perinatal asphyxia Preterm /LBW Meningitis, encephalitis Bilirubin encephalopathy IEM: Galactosemia ,PKU

Prevention :

Prevention Primary prevention : improvement in perinatal care, iodistion of salt,immunisations,detection &care of high risk pregnancies, penatal screening &genetic counselling . 2 nd ary prevention : neonatal screening, screening of “high risk babies” & early intervention measures. Tertiary prevention : stimulation, training,& education, vocational oppertunities . Mainstreaming Support for families Parenteral support groups.

Drug Therapies:

Drug Therapies No specific drugs but some symptoms can be controlled. Neuroleptic drugs to reduce aggressive and antisocial behavior ( phenothiazines ). antipsychotic drugs( risperidone ). Antidepressant drugs can improve sleep, possibly help reduce self-injurious behavior, reduce depression.

TAKE HOME MASSAGE:

TAKE HOME MASSAGE Mental retardation is preventable in some cases. Most cases are idiopathic & are mild. Diagnosis is clinical. Examination for dysmorphology & detailed neurology is of essence. Care rather than cure is the way of management in most of the cases.

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GROSS MOTOR: 180 degree flip examination in infant < 8 months and gait for > 1 year:

GROSS MOTOR: 180 degree flip examination in infant < 8 months and gait for > 1 year Supine: Note posture, abnormal ATNR, involuntary movements with CP. paucity of movements for hemiplegia. Pull to sit: head lag. Sitting: Head and trunk control. Back is straight or rounded. Weight bearing: scissoring, hypotonia, advanced weight bearing (CP) Ventral suspension: Describe posture, low tone, increase extensor tone. Prone: Observe ability to raise head, trunk above horizontal,

Primitive reflexes::

Primitive reflexes : 1 . Sucking/Rooting :( 0-4,6mths), 2. Palmer grasp; (0-3 months). 3. Placing, stepping: (0-6weeks) 4. ATNR: 2-6 Months. 5. Landau: on ventral suspension, normally extend head, trunk, and hip. Flex head and neck, response is flexion of hip, trunk.0-6 month). 6. Neck righting reflex: rotation of trunk 6mths-2 years. 7. Moro: 0-4 months. 8. Parachute: 6-12 months persist. Prone position, move rapidly, face down. Will extend both upper limbs.

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