الفرقان : سورة174

Mental Retardation: There's a Way

Dr: Samir MM Al-MinshawyLecturer of Neuropediatrics

Minia University

Mental disorders have been recognised in Egypt for millennia; 5000 years ago and described in the Ebers and Kahun papyri. These disorders carried no stigma, as there was no demarcation then between psyche and soma.

In the 14th century – 600 years before similar institutions were founded in Europe – the first psychiatric unit was established, in Kalaoon Hospital in Cairo.

History

Listening to the story :The earliest reference to MR dates to the Egyptian Papyrus of Thebes in 1552 B.C. (Harris 2006).The ancient Greeks and Romans believe that children with ID were born because the gods were angry. Infanticide (thrown from a cliff ) or sale in Sparta (Sheerenberger, 1983)In middle ages, many children were sold into slavery, abandoned, or left out in the cold.• Johann G- established the first known residential facility in 1841 in SwitzerlandIn 1905, Alfred Binet produced the first standardized test for measuring intelligence in children..The Mental Deficiency Act of 1913 made marriage illegal for persons with mental retardation.(Eugenics) “The 1950's was an era of compulsory surgical sterilization (NRCFCPP Information Packet: Parents with Mental Retardation and their Children: 2003).

Declaration on the rights of mentally retarded persons in 1977

The mentally retarded person has the same rights as other human beings.

He has a right to a proper medical care He has a right to economic security and to a decent

standard of living and to perform productive work. He should live with his own family or with foster parents

and participate in different forms of community life. The family with which he lives should receive assistance.

He has a right to a qualified guardian when this is required to protect his personal well-being and interests.

He has a right to protection from exploitation, abuse and degrading treatment.

Whenever he is unable, because of the severity of their handicap, to exercise all their rights in a meaningful way or it should become necessary to restrict or deny some or all of these rights, the procedure used for that restriction or denial of rights must contain proper legal safeguards against every form of abuse.

DefinitionA group of disorders

Mental retardation is stigmatizing .Intellectual disability.In Europe, learning disabilityGlobal developmental delay Diagnostic and Statistical Manual of Mental Disorders, fourth Edition

Intelligence quotient:

It is calculated according to the formula

Grading of intellectual disability:

Mild intellectual disability: IQ 50-55 to 70

Moderate intellectual disability IQ 35-40 to 50-55

Severe intellectual disability IQ 20-25 to 35-40

Profound intellectual disability IQ below 20-25

Intellectual disability, Severity unspecified, when there is strong

presumption of intellectual disability but the person’s intelligence is

untestable by standard tests.

Developmental periods is defined as the period of time between conception & the 18th birthday.

Adaptive behavior is defined as the degrees with which the person meets the standards of personal independence & social responsibility expected of his age & cultural group.

1- Genetic factors:Metabolic and/or neurodegenerative disorder: PKU,

Tay-Sachs

Chromosomal disorder: Down syndrome, Klinefelter syndromeGenetic syndromes: Fragile X and Prader-Willi

syndromeNonsyndromic autosomal mutations: SYNGAP1, GRIK2,

TUSC3

Developmental brain abnormality: Hydrocephalus ± meningomyelocele, lissencephaly

Etiology (Cause versus Risk factor)

2-Prenatal causes:Infection  Rubella  Cytomegalovirus Syphilis  Toxoplasmosis, herpes

simplexEndocrine disorders  Hypothyroidism  Hypoparathyrodism

 �Diabetes mellitus

Intoxication  Lead & certain drugSubstance abuse

Physical damage & disorders  InjuryHypoxia Radiation Hypertension Anemia Emphysema 

Placental dysfunctionToxemia of pregnancyPlacenta previaCord prolapseNutrition growth

retardation

3- Perinatal FactorsBirth asphyxia  Prolonged or difficult birth Prematurity  Kernicterus Instrumental delivery

4- Postnatal FactorsInfectionsi. Encephalitis ii. Measles iii. Meningitis iv. Septicemia AccidentsLead poisoning 

5- Environmental & socio-cultural FactorsCultural deprivation Low socio-economic status Inadequate caretakers Child abuse

6- Familial intellectual disability: Environment, syndromic, or genetic

7- Unknown

Epidemiology15% worldwide have physical or mental disabilityhttp://www.cbsnews.com/news/15According to DSM-IV-TR, 2.5% of the population have

ID

ID occurs more in boys than in girls: 2 : 1 in mild intellectual disability and 1.5 : 1 in severe intellectual disability (X-linked disorders.

من فضلك التستخدم كلمة )متخلفة( أنا طفلة جميلة

Egypt has a national census every 10 years since 1907

Egypt: overall prevalence of 3.9%(Assiut Governorate, representing the Egyptian population). Temtamy et al 1994

Percent distribution of Disabled Persons by Type of Disability in 1996

Diagnosis

DiagnosisHistory taking from parents &

caretakers  Physical examination  Neurological examination Developmental assessmentPsychological assessmentInvestigations: Laboratory studiesImaging studiesElectrophysiologic studies Histologic Findings

Psychological tests

History taking: Signs and symptomsPatients may demonstrate the following:Language delay. Fine motor/adaptive delay: self-feeding, and dressing,,,Cognitive delay: memory, problem-solving, and logical

reasoning Social delays: Lack of interest in age-appropriate toys

and delays in imaginative play and reciprocal play with age-matched peers

Gross motor developmental delayBehavioral disturbances: hyperactivity, disordered

sleep, aggression, self-injury,. Neurologic and physical abnormalities.Extensive Family history (a pedigree of 3 generations or more)

Developmental assessmentAt regular intervals. Parents' Evaluation of Developmental Status (PEDS),

Ages and Stages Questionnaires (ASQ) and Child Development Inventories (CDI). Bayley Infant Neurodevelopmental Screener, Battelle Developmental Inventory and Early Language Milestone Scale.

Behavioral observations should focus on the child's communicative intent, social skills, eye contact, compliance, attention span, impulsivity, and style of play. Physical examinationHead circumferenceHeight and weightNeurological exam Visual impairment and hearing deficits Skin: Extremities

Laboratory studies ( according to….)1-Genetic study karyotypingFragile X testing FISH probes 2-Metabolic screening: Urinary organic acids (organic acidopathies) Urinary mucopolysaccharides and oligosaccharides

(mucopolysaccharidoses) Plasma 7-DHC (Smith-Lemli-Opitz syndrome) Thyroid function tests Very-long-chain fatty acids (peroxisomal disorders) Creatine kinase (in the assessment of profound central

hypotonia versus myopathy) 3-lead testingImaging studiesBrain CT and MRISkeletal films

Electrophysiologic studiesAuditory evoked potentials Visual evoked potentials EEG is not recommended as part of the routine

work-up of MR unless the history is suggestive of seizures or a specific epileptic syndrome

Histologic FindingsDendritic spines number, maturity and morphology.

Psychological testsBayley Scales of Infant Development Stanford-Binet Intelligence Scale Wechsler Preschool and Primary Scale of

Intelligence-Revised (WPPSI-R) Wechsler Intelligence Scale for Children–IV (WISC-

IV) Vineland Adaptive Behavior Scales-II

Dr Hussein AbdeldayemProf of Pediatric Neurology, Alex University

False MR (PSEUDO MR)

Dual Diagnosis

Management

Aim: Early recognition and intervention

Dimensions:1- Prevention 2- Treatment: improvement over time but rarely “cured”. Early and accurate intervention Rehabilitation Dealing with comorbidities Prevent further complications and deterioration

3- follow- up

Prevention - More important and of low cost- - Levels:

- Primary: prevent the occurrence of the disease (immunization , genetic counseling. etc.. Prevent

- Secondary Prevention (early detection (prenatal diagnosis and newborn screening) Stop or reverse

- Tertiary: Prevent complications - Quaternary : (set of health activities that diminish or avoid the consequences

of unnecessary or excessive interventions in the health system) less cost

Examples: PKU, Congenital hypothyroidism, Galactosemia, Congenital rubella syndrome ,

Fetal Alcohol syndrome, Maternal diseases and infection , Perinatal asphyxia. Iron and iodine deficiency etc,,,,,,,,,,,,,,,,,,,,,,,,

Developmental screening tests : Infants at high risk

Treatment?KEY COMPONENTS OF SUCCESSFUL TREATMENT PROGRAMS Time FactorTeam Approach Individualized (patient) Planning Family involvementCultural Considerations Community commitmentPeer Interaction

Developmental pediatrician or psychologistGenetic specialist and counselorPsychiatristDentistSpecial education/educational therapistOccupational, speech and/or physical therapistBehavioristPharmacistDurable medical equipment providersSocial services agencies/social workers

TreatmentsDevelopmental and Educational Services: Special education programs: An Individualized Education Program (IEP)

Behavioral Techniques as family therapy, individual child behavior therapy, cognitive–behavioural therapy, psychodynamic therapies, parent training, and group therapy: preventative and therapeutic for children and parents.

Pharmacological Treatment : Improvement not cure Psychopharmacological Intervention: Medications are targeted to specific

comorbid psychiatric disease or behavioral disturbances like psychostimulants for ADHD and neuroleptic for aggression and self-injury.

No specific pharmacologic treatment is available for cognitive deficiency. Although the pharmacologic enhancement of cognition is an area of interest, research on it is limited.

Diet: Dietary restrictions (such as yeast and gluten-free regimens) vitamin and mineral supplements is generally not supported by research).

Activity: Obesity is prevalent , regular physical activity should be included in the management plan. participation in peer activities, sports gain self-esteem .

Encourgement of evidence based medicine and minimize the gap between research and practice

Smart drugs “Nootropics" Intelligence enhancers, are drugs, supplements, nutraceuticals, and functional foods that improve one or more aspects of mental function, such as working memory, motivation, and attention. Intense marketing may not correlate with efficacy; while scientific studies support the beneficial effects of some compounds

Rationale of use: poorly understood mechanisms of action, not be as a substitute for appropriate services and possibly dangerous side effecs may be presentIncluding: Stimulants : Amphetamine, methylphenidate, caffeine, nicotine,

armodafinil and modafinilOmega-3 fatty acidsNutraceuticals: Bacopa monnieri, panax ginseng.salvia officinalis.

ginkgo biloba and soflavonesRacetams: pramiracetam, oxiracetam, coluracetam, and

aniracetam

Follow up Outpatient CareRegular evaluation at least annually:

Treatment of associated impairmentsPharmacotherapyBehavior managementEducational servicesFamily counseling

Health EducationFamily support and education around the issues of

MR can be obtained from the following: The Arc American

Association on Intellectual and Developmental Disabilities

Exceptional Parent MagazineNational Organization for Rare Disorders (NORD)Brain and Nervous System Center.

Triad

Abuse

PhysicalSexualPsychological FinancialPeer abuse  

Family

Prognosis Mild to moderate mental retardation

has good prognosis.severe to profound ID has a

decreased life expectancy. Comorbid psychiatric and physical

conditions Down syndrome may prdispose to Alzheimer's disease in later life

Pascal Duquenne won the Best Actor Award at the 1996 Cannes Film Festival for his role in The Eight Day.

Special Olympian, swimmer and president of the disability rights organization the Karen Gaffney Foundation. She is the first living person with Down syndrome to receive an honorary doctorate degree.

DISABILITY: IN THE BRAIN, IN THE PUBLIC MIND

Ethi

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SkillsRaise the suspicionDetect earlyMimickers and dual diagnosisTeam bonding Discover patient characteristicsCommunicate effectively: patient, family and othersRespect and sympathy

Thank you

Intellectual Disability: There's a Way

By Dr: Samir MM Al-MinshawyLecturer of Neuropediatrics

Minia University

2nd Scientific Day including EEG Workshop: 17 Sept 2015

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لهال

Thanks: Omega Fit