disorders of cognition

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  • 2007 PREP SA on CD-ROM

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    Question: 43

    An 8-year-old boy is inattentive at home and school, has difficulty completing his homework, and is failing reading. Physical examination findings are normal, he has friends at school, and the family has been living in their newly built home for the past 3 years. You begin to discuss a diagnosis of attention-deficit/hyperactivity disorder, and his mother asks you what tests you will perform to try to determine the cause of the problem.

    Of the following, your BEST response is that you will order

    A. a lead level

    B. an electroencephalogram

    C. computed tomography scan of the brain

    D. no tests at this time

    E. thyroid studies

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    Critique: 43 Preferred Response: D

    Attention-deficit/hyperactivity disorder (ADHD) is a common, yet heterogeneous disorder that has a complex etiology; genetic, environmental, and biologic factors all play roles. For example, ADHD is associated with both genetic syndromes such as fragile X and intrauterine toxic exposures such as fetal alcohol syndrome. It also has been shown in family and twin studies that ADHD is more common in close family members of those diagnosed with the disorder. Research continues into the neurobiologic basis of ADHD. Evidence is increasing that alterations in the frontal lobe and frontal subcortical connections play a significant role in the disorder. Affected children also have difficulties with executive functioning, such as organization, impulse control, and inattention, that are common in other disorders involving impaired frontal lobe function. Although not necessary for all children, a full psychoeducational evaluation likely is necessary before ADHD can be diagnosed in the boy in the vignette. The boy also may have a learning disability or an environmental cause of his academic difficulty, such as a chaotic home environment or significant social stresses. ADHD is diagnosed clinically using American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders-4th ed (DSM-IV) criteria; routine medical testing is not necessary. Lead intoxication is associated with cognitive dysfunction, including inattention and hyperactivity, but it is not a significant cause of ADHD. A lead level should be obtained if any concerns for lead exposure are established. Thyroid dysfunction also may cause cognitive changes, but other information from the history or physical examination should prompt the request of these studies. Electroencephalography or computed tomography scan of the brain is unlikely to provide clinically useful information in a child who has no history of seizure, significant brain injury, or neurologic findings on examination.

    References:

    American Academy of Pediatrics Committee on Quality Improvement, Subcommittee on Attention-Deficit/Hyperactivity Disorder. Clinical practice guideline: diagnosis and evaluation of the child with attention-deficit/hyperactivity disorder. Pediatrics. 2000;105:1158-1170. Available at: http://pediatrics.aappublications.org/cgi/content/full/105/5/1158

    Reiff MI, Tippins S, LeTourneau AA. ADHD: A Complete and Authoritative Guide. Elk Grove Village, Ill: American Academy of Pediatrics; 2004

    Sims MD. Attention-deficit/hyperactivity disorder. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders Co; 2004: 107-110

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    Question: 59

    An 8-year-old boy has difficulty with reading; he reads slowly and makes many mistakes. He has a history of a febrile seizure at 1 year of age and a fall at age 2 years that resulted in a brief loss of consciousness. His mother read an article about interventions for improving vision function in children who have reading difficulties and would like your opinion. Findings on his physical examination are normal, except for vision of 20/40 bilaterally.

    Of the following, your BEST response is that you would like to

    A. order brain magnetic resonance imaging

    B. order electroencephalography

    C. refer him for optometric evaluation

    D. refer him for a functional vision assessment

    E. request a psychoeducational evaluation

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    Critique: 59 Preferred Response: E

    The child described in the vignette has symptoms that are concerning for a reading learning disability. The most helpful intervention is further evaluation of his academic difficulty by requesting a psychoeducational evaluation. Reading is a complicated endeavor that involves the integration of multiple neurologic pathways. Although vision is necessary for reading, complex visual processing facilitates reading and comprehension of written language. Children who have common ophthalmologic disorders may have academic difficulty only when there is an inability to see adequately what is written on the page. Mild or correctable vision deficits rarely cause reading or academic problems. Of note, there is no evidence to support the value of eye exercises or the use of special tinted lenses as therapy for reading difficulties. Brain magnetic resonance imaging and electroencephalography are unlikely to provide useful information in a child who has normal findings on physical examination and a single febrile seizure. He should receive routine eye evaluations, but given the near-normal vision documented on your office screening, he is unlikely to have a vision disorder that is causing his reading difficulty. For children who have vision loss, a functional vision assessment, which evaluates how the child uses vision to perform routine tasks, can be useful.

    References:

    Committee on Children With Learning Disabilities American Academy of Pediatrics (AAP), and American Academy of Ophthalmology (AAO), and American Association for Pediatric Ophthalmology and Strabismus (AAPOS). Learning disabilities, dyslexia, and vision: a subject review. Pediatrics. 1998;102:1217-1219. Available at: http://pediatrics.aappublications.org/cgi/content/full/102/5/1217

    Galaburda AM, Duchaine BC. Developmental disorders of vision. Neurol Clin. 2003;2:687-707

    Olitsky SE, Nelson LB. Reading disorders in children. Pediatr Clin North Am. 2003;50:213-224

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    Question: 75

    You diagnose attention-deficit/hyperactivity disorder in a 10-year-old girl. She is growing well, and physical examination findings are normal.

    Of the following, the MOST appropriate test to confirm the diagnosis is

    A. brain computed tomography

    B. brain magnetic resonance imaging

    C. electroencephalography

    D. no test at this time

    E. positron emission testing

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    Critique: 75 Preferred Response: D

    Attention-deficit/hyperactivity disorder (ADHD) is a common, yet heterogeneous disorder that has a complex etiology; genetic, environmental, and biologic factors all play roles. For example, ADHD is associated with both genetic syndromes such as fragile X and intrauterine toxic exposures such as fetal alcohol syndrome. It also has been shown in family and twin studies that ADHD is more common in close family members of those diagnosed with the disorder. Research continues into the neurobiologic basis of ADHD. Evidence is increasing that alterations in the frontal lobe and frontal subcortical connections play significant roles in the disorder. Affected children have difficulties with executive functioning, such as organization, impulse control, and inattention, that are common in other disorders involving impaired frontal lobe function. Neurodiagnostic studies such as magnetic resonance imaging, electroencephalography, computed tomography scan, and positron emission testing may be used in research settings, but they are not used routinely in the evaluation of children who have ADHD. Such studies may be appropriate if there are specific concerns regarding risk for seizure, brain malformation, or brain injury.

    References:

    American Academy of Pediatrics Committee on Quality Improvement, Subcommittee on Attention-Deficit/Hyperactivity Disorder. Clinical practice guideline: diagnosis and evaluation of the child with attention-deficit/hyperactivity disorder. Pediatrics. 2000;105:1158-1170. Available at: http://pediatrics.aappublications.org/cgi/content/full/105/5/1158

    American Academy of Pediatrics Subcommittee on Attention-Deficit/Hyperactivity Disorder and Committee on Quality Improvement. Clinical practice guideline: treatment of the school-aged child with attention-deficit/hyperactivity disorder. Pediatrics. 2001;108:1033-1044. Available at: http://pediatrics.aappublications.org/cgi/content/full/108/4/1033

    Reiff MI, Tippins S, LeTourneau AA. ADHD: A Complete and Authoritative Guide. Elk Grove Village, Ill: American Academy of Pediatrics; 2004

    Sims MD. Attention-deficit/hyperactivity disorder. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders Co; 2004:107-110

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    Question: 91

    You and your colleagues are discussing implementation of routine developmental screening in your office.

    In your research, you have found that

    A. early identification is effective in improving educational outcome

    B. most developmental screening tests have a sensitivity of approximately 90%

    C. screening for behavioral and developmental concerns requires separate questionnaires

    D. subsequent screening is not necessary after children pass two screening tests

    E. the use of developmental screening tools requires extensive staff training

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    Critique: 91 Preferred Response: A

    Developmental or behavioral disabilities are seen in approximately 15% to 18% of children in the United States. The American Academy of Pediatrics recommends that pediatricians use validated screening tools at each health supervision visit, but time constraints and other pressing issues can make it difficult to comply with this recommendation. However, early intervention has been shown to be effective in improving long-term educational and vocational outcomes for children who have developmental or behavioral disabilities as well as preventing teen pregnancy and criminality. The hundreds of assessment measures available in the United States can add to practitioners confusion when choosing a screening test. Different tools are used in varying age groups and have different screening focuses (behavioral versus developmental versus psychosocial). However, children who have disabilities and are not identified on an initial screening are likely to be identified at subsequent screenings, and children who are overidentified likely have unique needs even though they might not qualify for special services. Although separate questionnaires are available for behavioral and developmental screening, tools also are available to screen for both areas in the same questionnaire. Screening should be performed at all health supervision visits, even if the child has passed several screening tests. Developmental screening is simple and can be completed by parent questionnaire or administered by staff who have minimal training (Item C91A).

    References:

    American Academy of Pediatrics Committee on Children with Disabilities. Developmental surveillance and screening of infants and young children. Pediatrics. 2001;108:192-196. Available at: http://pediatrics.aappublications.org/cgi/content/full/108/1/192

    Glascoe FP. Early detection of developmental and behavioral problems. Pediatr Rev. 2000;21:272-280. Available at: http://pedsinreview.aappublications.org/cgi/content/full/21/8/272

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    Critique: 91

    Item C91A: Table

  • 2007 PREP SA on CD-ROM

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    Question: 107

    A 10-year-old child recently had cognitive testing that showed a full scale intelligence quotient of 105. On achievement testing, he performed at the 3rd percentile in reading and at the 50th percentile in math.

    Of the following, these findings MOST likely represent

    A. attention-deficit/hyperactivity disorder

    B. mental retardation

    C. poor testing conditions for the achievement test

    D. specific learning disability

    E. vision impairment

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    Critique: 107 Preferred Response: D

    Learning disabilities are characterized by an unexpected difficulty in one academic area in children who otherwise have the intelligence, motivation, and educational opportunity to learn. The child described in the vignette has normal cognition, with a full scale intelligence quotient of 105 and a normal math performance, but subnormal reading performance on achievement testing, a pattern that is concerning for a specific learning disability. For the boy in the vignette, the disability appears to be in reading. Children who have learning disabilities may appear inattentive in academic situations in which they do not understand the material, and attention-deficit/hyperactivity disorder (ADHD) may be comorbid with learning disability. However, ADHD is unlikely to be the primary diagnosis for the boy in the vignette. Children who have mental retardation have intelligence quotients of less than 70 plus associated limitations in adaptive functioning. Testing conditions for the boy in the vignette are unlikely to have been poor, given his performance on cognitive testing and the math portion of the achievement testing. Only severe vision impairment would affect the ability to read and would have been obvious through history or physical examination.

    References:

    Lyon GR, Shaywitz SE, Shaywitz BA. Specific reading disability (dyslexia). In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders Co; 2004:110-112

    Silver L. Developmental learning disorders. In: Lewis M, ed. Child and Adolescent Psychiatry: A Comprehensive Textbook. 3rd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2002:621-629

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    Question: 123

    You diagnose attention-deficit/hyperactivity disorder in a 10-year-old boy and recommend treatment with methylphenidate. His mother asks about potential adverse effects of the treatment.

    Of the following, the MOST common adverse effect of treatment is

    A. delayed sleep onset

    B. depression

    C. hallucinations

    D. tics

    E. weight gain

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    Critique: 123 Preferred Response: A

    Most children who receive medication for treatment of attention-deficit/hyperactivity disorder (ADHD) are prescribed stimulant medications, such as methylphenidate or dextroamphetamine. Stimulants generally are considered safe, with generally mild and short-lived adverse effects. The most common adverse effects are decreased appetite, irritability, and delayed sleep onset. Approximately 15% to 30% of children experience motor tics, most of which are transient and do not represent an absolute contraindication to continuing with the medication. Although stimulants do not cause Tourette syndrome, they may unmask the tic symptoms. The safety profile of stimulants is being reviewed, and in February 2006, the United States Food and Drug Administration placed a black box warning for cardiovascular risk, including sudden death and stroke, especially in children who have underlying heart disease. Other medications for ADHD include atomoxetine and tricyclic antidepressants. Atomoxetine is a norepinephrine reuptake inhibitor that had a similar adverse effect profile to stimulants in clinical trials, but has been associated with nausea, hepatic injury, and risk of suicidal ideation. Tricyclic antidepressants typically cause sedation and related anticholinergic adverse effects such as dry mouth and constipation. If the child in the vignette experiences delayed sleep onset, the condition can be managed by changing the timing of the medication dose or decreasing an afternoon dose. Depression and hallucinations are rare adverse effects of stimulant medication. Tics are less common than changes in sleep onset. A child who receives stimulant medication for treatment of ADHD is more likely to have weight loss or lack of weight gain related to decreased appetite than weight gain.

    References:

    American Academy of Pediatrics Committee on Quality Improvement, Subcommittee on Attention-Deficit/Hyperactivity Disorder. Clinical practice guideline: diagnosis and evaluation of the child with attention-deficit/hyperactivity disorder. Pediatrics. 2000;105:1158-1170. Available at: http://pediatrics.aappublications.org/cgi/content/full/105/5/1158

    American Academy of Pediatrics Subcommittee on Attention-Deficit/Hyperactivity Disorder and Committee on Quality Improvement. Clinical practice guideline: treatment of the school-aged child with attention-deficit/hyperactivity disorder. Pediatrics. 2001;108:1033-1044. Available at: http://pediatrics.aappublications.org/cgi/content/full/108/4/1033

    MTA Cooperative Group. National Institute of Mental Health multimodal treatment study of ADHD follow-up: 24-month outcomes of treatment strategies for attention-deficit/hyperactivity disorder. Pediatrics. 2004;113:754-761. Available at: http://pediatrics.aappublications.org/cgi/content/full/113/4/754

    Reiff MI, Tippins S, LeTourneau AA. ADHD: A Complete and Authoritative Guide. Elk Grove Village, Ill: American Academy of Pediatrics; 2004

    Sims MD. Attention-deficit/hyperactivity disorder. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders Co; 2004:107-110

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    Question: 139

    You diagnose attention-deficit/hyperactivity disorder in an 8-year-old girl and initiate therapy with a daily morning dose of long-acting methylphenidate. Her mother asks about using stimulant medication after school, on weekends, and during the summer.

    Of the following, your BEST response is that

    A. an after-school dose is not necessary with a long-acting form of methylphenidate

    B. dosing outside school hours allows parents to monitor medication effect

    C. immediate-release medication should be used during holidays

    D. summer dosing increases the risk of tolerance to medication

    E. weekend dosing should be decreased by 50%

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    Critique: 139 Preferred Response: B

    Children who have attention-deficit/hyperactivity disorder (ADHD) commonly have difficulty attending to and completing tasks both at home and school. Families may resist giving medication outside of school hours because they assume that the same degree of attention needed in a school setting is not necessary in the childs other settings. However, best results from stimulant medication therapy have been noted when children receive medication in all of their typical settings. Additionally, parents will be more aware of medication adverse effects and adequacy of dosing if they observe the child when he or she is taking the medication. An after-school dose of medication may be necessary for the child to perform successfully in after-school and home activities if the medication effects have worn off at the end of the school day. There is no need to change the type or the dosing of medication used during weekends and holidays. Continuous dosing throughout the year does not make a child more tolerant to stimulant medications, and drug holidays are not necessary.

    References:

    American Academy of Pediatrics Subcommittee on Attention-Deficit/Hyperactivity Disorder and Committee on Quality Improvement. Clinical practice guideline: treatment of the school-aged child with attention-deficit/hyperactivity disorder. Pediatrics. 2001;108:1033-1044. Available at: http://pediatrics.aappublications.org/cgi/content/full/108/4/1033

    Reiff MI, Tippins S, LeTourneau AA. ADHD: A Complete and Authoritative Guide. Elk Grove Village, Ill: American Academy of Pediatrics; 2004

    Wender EH. Managing stimulant medication for attention-deficit/hyperactivity disorder. Pediatr Rev. 2001;22:183-190. Available at: http://pedsinreview.aappublications.org/cgi/content/full/22/6/183

    Wender EH. Managing stimulant medication for attention-deficit/hyperactivity disorder: an update. Pediatr Rev. 2002;23:234-236. Available at: http://pedsinreview.aappublications.org/cgi/content/full/23/7/234

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    Question: 155

    You are evaluating a 10-year-old boy who has behavior problems. He hits his younger brother, talks back to his parents, and refuses to perform his assigned household chores. He does well in school, but is oppositional in the classroom. His parents ask what they can do to improve his behavior.

    Of the following, your BEST response is that

    A. he needs cognitive testing

    B. he should be referred for individual counseling

    C. medication is the preferred first-line treatment

    D. school-based behavior programs are preferred to home-based programs

    E. the parents should be trained in behavior modification techniques

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    Critique: 155 Preferred Response: E

    Behavior modification is a general category of therapy that refers to principles and techniques based on learning theory and used to change behavior. Behavioral techniques are used to either strengthen or maintain desired behaviors or to decrease or eliminate undesired behaviors. For example, parental praise or token economies are examples of techniques used to reinforce behavior, and time-out is a technique used to decrease or eliminate a behavior. The child described in the vignette has oppositional behavior that may respond to behavioral management. He is doing well in school and, therefore, probably does not need cognitive testing. Individual counseling may be used as an adjunct, but he is more likely to respond to consistent behavior management. Behavioral approaches should be tried before using medication to control behavior. The behavioral plan should be instituted both at home and in school, with communication between teacher and parents to maintain consistency.

    References:

    Boris NW, Dalton R. Disruptive behavioral disorders. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders Co; 2004:88-90

    Vitulano LA. Child and adolescent behavior therapy. In: Lewis M, ed. Child and Adolescent Psychiatry: A Comprehensive Textbook. 3rd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2002:998-1014

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    Question: 171

    You are evaluating a 6-year-old boy who has Duchenne muscular dystrophy. He is doing well in a regular classroom and will be attending second grade in a different school next year. On physical examination, you note a healthy-appearing boy who has pseudohypertrophy of the calf muscles and uses a Gower maneuver (Item Q171A) to rise from the floor.

    In gathering information to help this child's transition to a new school, you are MOST likely to ask about

    A. augmented communication resources

    B. recent pulmonary function testing

    C. signs of sleep apnea

    D. the number of floors in the school

    E. wheelchair use

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    Critique: 171 Preferred Response: D

    The time of diagnosis and medical, social, and educational transitions are common stress points for parents in the life of their child who has a developmental disability. The need for increasing medical equipment and assistance, such as braces, a wheelchair, or a gastrostomy tube, may cause significant stress. Similarly, entering kindergarten, middle school, junior high school, and high school each present challenges. A child likely needs re-evaluation of school services provided at those times and may require significant testing or accommodations for differing physical facilities. For the ambulatory boy in the vignette, the most likely difficulty he will encounter is numerous stairs in a multilevel school that may be tiring for him to climb and descend. He is healthy and is unlikely to have problems with sleep apnea or need pulmonary function tests until his disease has progressed further. Likewise, at his present level of function, he does not need a wheelchair or communication device. However, most children who have Duchenne muscular dystrophy need educational support for related learning disabilities that may manifest over time.

    References:

    Perrin JM. Chronic illness in childhood. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders Co; 2004:135-138

    Plauch Johnson C, Kastner TA, and the Committee/Section on Children With Disabilities. Helping families raise children with special health care needs at home. Pediatrics. 2005;115:507-511. Available at: http://pediatrics.aappublications.org/cgi/content/full/115/2/507