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Intellectual Disability PowerPoint Presentation

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Description : Check out this medical PowerPoint presentation titled "Intellectual Disability" by Jess P. Shatkin, MD, MPH.
This medical PowerPoint presentation talks about 4 levels of severity of mental retardation, the primary comorbid Axis I disorders, 6 risk factors for mental retardation, the 3 most common causes of mental retardation, and behavioral phenotypes for 5 “common” mental retardation syndromes.

Tags : Intellectual Disability, mental retardation, the primary comorbid Axis I disorders, mental retardation syndromes, Child Neurology PowerPoint presentation, Neurology PowerPoint presentation

Published on : Mar 21, 2023 | Views : 2043 | Downloads : 4

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Slide 1-

Intellectual Disability Jess P. Shatkin, MD, MPH Vice Chair for Education NYU Child Study Center New York University School of Medicine
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What’s in a Name? Idiot Moron Feeble Minded Mentally Retarded Intellectual Disability AAMR American Assn on Intellectual and Developmental Disabilities (AAIDD)
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Learning Objectives Participants will be able to: Define 4 levels of severity of mental retardation. Identify the primary comorbid Axis I disorders. Describe 6 risk factors for mental retardation. Identify the 3 most common causes of mental retardation. Define behavioral phenotypes for 5 “common” mental retardation syndromes.
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Definition Deficits in IQ and adaptive functioning IQ of 70 or below Measured by standard scales Wechsler, Stanford-Binet, Kaufman Impairments in Adaptive Functioning Effective coping with common life demands Ability to meet standards of independence Measured by standard scales Vineland, AAMR Adaptive Behavior Scale
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Degrees of Severity Mild Mental Retardation IQ: 50-55 to approximately 70 Moderate Mental Retardation IQ: 35-40 to 50-55 Severe Mental Retardation IQ: 20-25 to 35-40 Profound Mental Retardation IQ: Less than 20-25
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AAIDD Proposed Classification Based upon the intensity of supports needed, as opposed to IQ (the traditional system): Intermittent Support Limited Support Extensive Support Pervasive Support
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Mild Mental Retardation Previously referred to as “educable” Largest segment of those with MR (85%) Typically develop social/communication skills during preschool years, minimal impairment in sensorimotor areas, often indistinguishable from “typicals” until later age By late teens acquire skills up to approximately the 6th grade level
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Moderate Mental Retardation Previously referred to as “trainable” About 10% of those with MR Most acquire communication skills during early childhood years Generally benefit from social/vocational training and with moderate supervision can attend to personal care Difficulties recognizing social conventions which interferes with peer relations in adolescence Unlikely to progress beyond the 2nd grade academically Often adapt well to life in the community in supervised settings (performing unskilled or semiskilled work)
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Severe Mental Retardation 3 – 4% of those with MR Acquire little or no communicative speech in childhood; may learn to talk by school age and be trained in elementary self-care skills Can master sight reading “survival” words Able to perform simple tasks as adults in closely supervised settings Most adapt well to life in the community, living in group homes or with families
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Profound Mental Retardation 1 – 2% of those with MR Most have an identifiable neurological condition that accounts for their MR Considerable impairments in sensorimotor functioning Optimal development may occur in a highly structured environment with constant aid
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Prevalence 1% (1 – 3% in developed countries) The prevalence of MR due to biological factors is similar among children of all SES; however, certain etiological factors are linked to lower SES (e.g., lead poisoning & premature birth) More common among males (1.5:1) In cases without a specifically identified biological cause, the MR is usually milder; and individuals from lower SES are over-represented
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Psychiatric Features No specific personality type Lack of communication skills may predispose to disruptive/aggressive behaviors Prevalence of comorbid Axis I disorders is 3-4 times that of the general population The nature of Axis I disorders does not appear to be different between “typicals” and those w/MR Patients with MR and comorbid Axis I disorders respond to medications much the same as those without MR
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Most Commonly Associated Axis I Disorders ADHD Mood Disorders Pervasive Developmental Disorders Stereotypic Movement Disorders Mental Disorders due to a GMC
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Predisposing Factors No clear etiology can be found in about 75% of those with Mild MR and 30 – 40% of those with severe impairment Specific etiologies are most often found in those with Severe and Profound MR No familial pattern (although certain illnesses resulting in MR may be heritable)
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Predisposing Factors (2) Heredity (5% of cases) Autosomal recessive inborn errors of metabolism (e.g., Tay-Sachs, PKU) Single-gene abnormalities with Mendelian inheritance and variable expression (e.g., tuberous sclerosis) Chromosomal aberrations (e.g., Fragile X) Early Alterations of Embryonic Development (30% of cases) Chromosomal changes (e.g., Downs) Prenatal damage due to toxins (e.g., maternal EtOH consumption, infections)
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Predisposing Factors (3) Environmental Influences (15-20% of cases) Deprivation of nurturance, social/linguistic and other stimulation Mental Disorders Autism & other PDDs Pregnancy & Perinatal Problems (10% of cases) Fetal malnutrition, prematurity, hypoxia, viral and other infections, trauma General Medical Conditions Acquired in Infancy or Childhood (5% of cases) Infections, trauma, poisoning (e.g., lead)
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Disability Low birth weight is the strongest predictor of disability Male children and those born to black women and older women in the USA are at increased risk for ID Lower level of maternal education is also independently associated with degree of disability
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Etiology At least 500 causes now known Over 150 MR syndromes have been related to the X-chromosome Most common cause of MR: Down’s Syndrome (most common genetic cause) Fragile X Syndrome (accounts for 40% of all X-linked syndromes; most common inherited cause) Fetal EtOH Syndrome (most common attributable cause) together these 3 account for 30% of all identified cases of MR
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Down’s Syndrome Most common chromosomal abnormality leading to MR (1.2/1000 births) Nondysjunction of chromosome 21 Relative strengths: Visual (vs. auditory processing) Social functioning Relative weaknesses: Language expression and pronunciation Generally viewed to suffer less severe psychopathology than other developmentally delayed groups After 40 years of age, affected individuals nearly always demonstrate postmortem neuronal defects indistinguishable from Alzheimer’s Disease
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Behavior & Psychiatric Illness in Downs Recent population based survey of social and healthcare records found: Females had better cognitive abilities and speech production compared with males Males had more behavioral troubles ADHD symptoms were often seen in childhood across gender Depression was diagnosed more often in adults with mild/moderate intellectual impairment Autistic behavior was most common in those with profound intellectual disability Elderly often showed a decline in adaptive behavior consistent with Alzheimer’s Maatta et al, 2006
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Down’s Syndrome
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Fragile X Syndrome FMR-1 gene (>200 trinucleotide CGG repeats, Xq27.3) An example of a “dynamic mutation” where more mutations occur with successive generations General problems: MR, mild CT dysplasia, & macro-orchidism Only 50% of females with the full mutation demonstrate IQs in the borderline/mild MR range (vs. 100% of males) Increases the risk for ADHD, autism (20-60%) & social phobia Increasing deficits in adaptive and cognitive functioning with age Relative strengths: Verbal long-term memory Relative weaknesses: ST memory, VM integration, sequential processing, math & attn
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Fragile X Syndrome
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Fragile X Syndrome
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Fetal EtOH Syndrome Incidence > 1:1000 Irritable as infants, hyperactive as children (ADHD) Teratogen amount: 2 drinks/day (smaller birth size), 4-6 drinks/day (subtle clinical features), 8-10 drinks/day (full syndrome) General problems: prenatal onset of growth deficiency, microcephaly, short palpebral fissures Syndrome can include: Facial deformities (ptosis of eyelid, microphthalmia, cleft lip [+/- palate], micrognathia, flattened nasal bridge and filtrum, & protruding ears) CNS deformities (meningomyelocele, hydrocephalus) Neck deformities (mild webbing, cervical vertebral & rib abmormalities) Cardiac deformities (tetralogy of Fallot, coarctation of aorta) Other abnormalities (hypoplastic labia majora, strawberry hemangiomata)
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Fetal EtOH Syndrome
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Prader-Willi Syndrome Deletion in chromosome 15 (15q11-13); freq 1:15000 60-80% w/microscopic deletion on paternal 15; remaining PWS have 2 copies of maternal chromosome w/no paternal chromosome (“uniparental disomy”) Infantile hypotonia, hyperphagia/food seeking, morbid obesity, small hands/feet, mild to moderate MR Relative stability in adaptive functioning during adolescence and early adulthood Relative strengths: Expressive vocabulary, LT memory, visual/spatial integration and visual memory (unusual interest in jigsaw puzzles) Relative weaknesses: Temper tantrums, emotional lability, mood symptoms (dx?), anxiety, skin picking, OCD symptoms (>50% OCD)
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Prader-Willi Syndrome
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Prader-Willi Syndrome
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Angelman Syndrome Severe MR, seizures, ataxia & jerky arm movements (puppet-like gait), absence of speech, and bouts of laughter (aka “happy puppet”) Deletion in chromosome 15 (15q11-13) In contrast to PWS, all identified cases of deletion traced to maternal chromosome 15 Illustrating “genomic imprinting,” (the fact that the parent of origin of the deletion at the same locus impacts the phenotype; that is, deletion of paternal 15q11-13 results in Prader-Willi but deletion of maternal 15q11-13 results in Angelman.)
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Angelman Syndrome
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Williams Syndrome MR, supravalvular aortic stenosis, “elfin-like” facies, infantile hypercalcemia, and growth deficiency Deletion of elastin gene (7q11.23) Relative strengths: Remarkable facility for recognizing facial features Loquacious, pseudo-mature “cocktail party speech” Relative weaknesses: Increased risk for ADHD, Anxiety D/O
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Williams Syndrome
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Psychotropic Medications No medications identified to treat MR nor to address specific symptoms No medications are FDA approved Rates of medication use vary from 12 – 40% in institutions vs. 19 – 29% in community settings amongst current studies (excl anticonvulsants) Singh et al, 1997 More recent review found that 22.8% of MR persons in group homes in the Netherlands were prescribed psychotropic medications Stolker et al, 2002
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Stimulants ADHD is the most widely diagnosed psychiatric disorder amongst children and adolescents with MR Prevalence rates estimated to be 8.7 – 16% (Emerson, 2003; Stromme & Diseth, 2000) At least 20 RDBPC trials published involving MTP with persons with MR; positive results range from 45 – 66%; lower than the rates found with non-MR population Positive predictors of response include IQ>50 and higher baseline scores on parent/teacher ratings of inattention and activity level Limited data on other treatments for ADHD symptoms Handen et al, 2006
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Antidepressants: Sertraline/Zoloft ® No DBPC studies w/Sertraline in patients w/MR One open label study of children with PDD noted improvements in anxiety and agitation (Steingard et al, 1997) Luiselli et al (2001) noted a case of one adult w/severe MR who showed improvement in SIB with Sertraline In the adult MR/PDD population, Sertraline has been found to result in clinically significant improvement of SIB and aggression (Hellings et al, 1996; McDougle et al, 1998)
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Antidepressants: Fluoxetine/Prozac® Among 15 published case reports and 4 prospective open label trials involving children and adults with MR and/or PDD, decreases in SIB, irritability, or depressive symptoms were noted (with the exception of two studies) for the majority of subjects treated with fluoxetine (Aman et al, 1999) Among the negative studies, some individuals discontinued fluoxetine due to increased aggression, agitation, and hypomanic behavior One open label study of fluoxetine in 128 children with MR/PDD, 3-8 y/o, reported an excellent response in 17%, a good response in 52%, and a fair/poor response in 31% (DeLong et al, 2002)
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Antidepressants: Fluvoxamine/Luvox® One open label study of 60 adults w/MR (200-300mg/d) reported a significant reduction in ratings of aggression after 3 weeks of treatment (La Malfa et al, 2001) McDougle et al (1996) conducted a DBPC study of fluvoxamine in 30 adults w/PDD and found significantly reducted aggression and repetitive thoughts/behavior McDougle (1998) also reported significant side effects and minimal clinical improvement in a DBPC study of children with PDD and symptoms of ritualistic and repetitive movements Fukuda et al (2001) conducted a DBPC trial in 18 children w/PDD where clinical global ratings improved for half of the subjects and significant gains were noted in eye contact and language use
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Antidepressants: Paroextine/Paxil® Davanzo et al (1998) demonstrated reductions in aggression (but not SIB) in 15 adults with MR in an open label study, but effects did not last beyond a one month period A retrospective chart review of 12 adults with MR found only 1/3 of subjects were “minimally” or “much” improved in domains of aggression, property destruction, or SIB (Branford et al, 1998) Masi et al (1997) treated 7 adolescents with MR and MDD; after 9 weeks of treatment, 4 subjects no longer met DSM-IV criteria for MDD
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Antidepressants: Citalopram/Celexa® Verhoeven et al (2001) found citalopram effective in an open label trial of 20 adults with MR and MDD, demonstrating a moderate to marked improvement in 12 of 20 patients on CGI after 6 months
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Antipsychotics in the Treatment of MR The typical antipsychotics have long been prescribed for disorders other than psychosis in patients with MR, including aggression, hyperactivity, antisocial behavior, sterotypies, and SIB The atypical antipsychotics are now being increasingly used b/c of the belief that they carry a decreased side effect profile
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Antipsychotic: Clozapine/Clozaril® Found effective in treating resistant psychosis in adults with MR (Antochi et al, 2003)
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Antipsychotic: Risperidone/Risperdal® Efficacious in both children and adults with MR in controlling hyperactivity, irritability, aggressive behavior, SIB, and repetitive behaviors (Aman & Madrid, 1999; Hellings, 1999; Turgay et al, 2002; Van Bellinghen & DeTroch, 2001) A DBPC trial in 118 children w/MR, 5-12 y/o, found 53.8% were responders vs. 7.9% w/placebo (Aman et al, 2002) Similarly, McCracken et al (2002) reported a 69% response rate (vs. 12% w/placebo) among 101 children w/PDD, most of whom had comorbid MR
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Antipsychotic: Olanzapine/Zyprexa® McDonough et al’s (2000) open label study of 7 adults w/MR documented improvement in SIB in 57% of subjects and worsening effects in 14% Similarly, a chart review of 20 adults w/MR found significant decreases in global challening behaviors and specific target behaviors, such as aggression, SIB, and destructive behaviors (Barnhill & Davis, 2003) Handen & Hardan (2006) conducted a prospective open label trial in 16 adolescents w/MR and found 12 of 15 experienced a 50% or greater decrease on behavior ratings assessing irritability Robust clinical effects noted in Friedlander et al’s chart review of adolescents and young adults w/MR (2001)
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Antipsychotic: Quetiapine/Seroquel® Hardan et al (2005) reported efficacy in the treatment of hyperactivity, inattention, and conduct problems in 10 children and adolescents w/MR Martin et al (1999) found quetiapine poorly tolerated in a study of boys with autism
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Antipsychotic: Ziprasidone/Geodone® A case series of children and adolescents w/PDD reported decreased aggression and irritability (McDougle et al, 2002) Cohen et al (2003) switched 40 adults w/MR to ziprasidone from other antipsychotics and noted an improved side effect profile w/either no change or improvement in maladaptive behavior in 72% of subjects
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Antipsychotic: Aripiprazole/Abilify® Stigler et al (2004) found aripiprazole beneficial in treating aggression, agitation, and SIB in five children w/PDD Staller (2003) reported decreased irritability, anxiety, and preoccupations in an adult w/Asperger’s D/O
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Alpha-2 Agonists: Guanfacine/Tenex® & Clonidine/Catapres® Frankhauser et al (1992) demonstrated the efficacy of clonidine in the treatment of hyperactivity in children w/PDD Posey et al (2004) conducted a chart review of 80 children w/PDD who had been treated with guanfacine; 24% of the sample evidenced decreased hyperactivity, inattention, and tics