Pediatric Neurodegenerative Diseases Flashcards
TEST: ADHD epidemiology:
almost as common as asthma (5% prevalence rate, 5-10% for children under 12, 2-4 for adolescents, 2.5% of adults
half that are diagnosed as kids grow out of it by adolescents, and another half grow out by adulthood (so only have of the 5% that are diagnosed as children actually remain ADHD as adults)
TEST: Side effects of the first line treatment for ADHD:
decreased appetite and trouble sleeping: be aware that there can be growth suppresion and this is most important in males;
TEST: there can also be growth spurts during the summer when taken off their medication
TEST: a patient with ADHD is prescribed methylphenidate for symptoms of inattention and hyperactivity. After an adequate trial dose, the patient does not fully beneefit from the medication. Which of the following actions is the most appropriate?
add a low-dose amphetamine
add an anxiolytic medication
add an antidepressant
discontinue methylphenidate and prescribe a mixed amphetamine compound
discontinue methylphenidate and prescribe an antidepressant
discontinue methylphenidate and prescribe a mixed amphetamine compound
Treatment of ADHD entales methylphenidate (ritalin) or amphedamine (salts). If one doesn’t work (70-80% will respond) then the next step is to try prescribing a different one
Autism epidemic?
the rate of autism has increased exponentially recently, and is now up to 1:68
TEST?: The rate of children that have autism, who are of below normal intelligence IQ is:
Intellectual disability
31% IQ < 70
23% IQ in 70-85 range
FAR MORE BELOW NORMAL IQ THAN GENERAL POPULATION
TEST: Which of the following symptoms is most suggestive of autism spectrum disorder?
9yo gets upset when asked to do homework
6yo kid likes to play with toy trains
4yo kid has no interest in engaging socially with others (peers or adults)
12yo kid stutters and has motor tics
4yo kid has no interest in engaging socially with others (peers or adults)
For ASD Domain 1, in eye gaze studies, the kids that went on to have autism would normally look at ______ instead of ______
specifically at hands or MOUTH instead of face or EYES; in adults it would be directly at the MOUTH and not at the eye contact, etc
for ASD Domain 2, you have to have 2 of the following
Stereotyped activity
Motor: Spinning, rocking, flapping, finger waving; lining up toys
Speech: scripts, echolalia
Inflexibility with routines
Highly restricted, fixated interests that are abnormal in intensity or focus
Clocks, wheels, water
Hyper- or hyporeactivity to sensory input
Adverse response to sounds, textures
Excessive smelling/touching of objects
Visual fascination with lights, movement (spinning)
TEST: Child comes to the classroom for the first time, runs right to toys, and does not say goodbye to his mother, nor even acknowledges she is leaving. Other children attempt to play with him but his response is to either ignore them or get angry at their advances. His focus on spinning objects in play might be considered what type of behavior often seen with ASD?
rigidity stereotyped behavior lack of social reciprocity poor language development obsessional thinking
stereotyped or repetitive motor movements, part of criterion B for ASD
TEST: late teenage boy that has been diagnosed with ASD, an example of Domain 2 characteristics (repetitive interests, behaviors, activities)
ECHOLALIA (repeating whatever someone says back to them to be annoying), and fixated on keys that he has collected, kept on him at all times, etc
TEST: 6 yo girl is obsessed with pet bird- focus on bird information, scripts about them, echopraxia (meaningless repetition or imitation of the movements of others as a symptom of psychiatric disorder)
repetitive interests, behaviors, activities characteristic of ASD
stimming?
Self-stimulatory behavior, also known as stimming and self-stimulation, is the repetition of physical movements, sounds, or repetitive movement of objects common in individuals with developmental disabilities, but most prevalent in people with autism spectrum disorders.
TEST: what medication has shown some mild benefit for the irritability symptoms of autistic spectrum disorders? fluoxetine aripiprazole holdol clonidine methylphenidate
aripiprazole (and risperidone) has been shown to be clinically useful for irritability symptoms such as tantrums or self-injurious behaviors
START WITH THE LOWEST DOSE POSSIBLE (LIQUID DROPPER and increased til efficacy)
in addition to aripiprazole, and resperidone, treatment to help children with ASD learn to function better socially includes:
behavioral interventions; group therapy in social skills
TEST: This is the most common cause of inherited intellectual disability:
Fetal Alcohol Syndrome Down Syndrome Autism Spectrum Disorder Angelman Syndrome Fragile X Syndrome
Fragile X Syndrome
mentally challenged 14 yr old boy has a long face, large ears, micropenis and large testes. Chromosome analysis is likely to demonstrate?
fragile x syndrome
12 yr old boy has repetitive speech, gaze avoidance, and hand flapping, developmental delay, abnormal temperment of unknown cause:
fragile x
Children with ________ disorder are the most likely to engage in behaviors such as vandalism, theft, and destruction of property.
a. autism spectrum
b. conduct
c. oppositional defiant
d. attention-deficit hyperactivity
conduct disorder
Someone with autism spectrum disorder would have deficits in social communication and ________ .
a. emotional labeling
b. their amount of chromosomes
c. social interaction
d. social isolation
c. social interaction
Approximately 60 to 75% of children with autistic disorder have IQs ________, indicating the presence of intellectual disabilities.
a. between 40 and 55
b. below 70
c. between 70 and 80
d. above 80
b. below 70
_______ is a neurodevelopmental disorder with an estimated heritability rate greater than 90%.
a. Attention deficit hyperactivity disorder
b. Reading disorder
c. Fetal alcohol syndrome
d. Autism spectrum disorder
d. Autism spectrum disorder
Early and intensive ________ treatment improves the long-term outcome for children with autistic disorder.
a. Behavioral
b. Pharmacological
c. Psychological
d. Cognitive
a. Behavioral
TEST: This is the most common cause of inherited intellectual disability:
Fetal Alcohol Syndrome Down Syndrome Autism Spectrum Disorder Angelman Syndrome Fragile X Syndrome
Fragile X Syndrome
NOTE: autism is not inherited, it’s most commonly a de neuvo mutation
Talking excessively is considered an element of which part of ADHD?
a. inattention
b. hyperactivity
c. stereotypical behaviors
d. impulsivity
b. hyperactivity
Because boys with ADHD __________ than girls do, they are more likely to receive treatment.
a. articulate their needs better
b. have more outspoken advocates
c. have more severe symptoms
d. develop symptoms earlier
c. have more severe symptoms
Which of the following is one of the factors that likely contributes to the increased prevalence of autism spectrum disorder in recent years?
a. lack of special education programs
b. the measles virus
c. changes in diagnostic criteria
d. MMR vaccines
c. changes in diagnostic criteria
In treating ADHD, ________ teaches parents how to reward positive behaviors and decrease negative behaviors.
a. medication monitoring
b. cognitive restructuring training
c. behavioral parent training
d. communication skills curriculum
c. behavioral parent training
Hand flapping, spinning, and ritualistic pacing are repetitive behaviors that serve no observable social functions. Collectively, these are called
a. compulsions.
b. disorder of verbal expression.
c. coping mechanisms.
d. stereotyped behaviors.
d. stereotyped behaviors.
For every ________ girl(s) diagnosed with autistic disorder, about ________ boys are diagnosed.
a. one; two
b. three; four
c. one; four
d. two ; five
c. one; four
________ is an indication of the neurodevelopmental basis of autistic disorder during the first few years of life.
a. Accelerated head and brain growth
b. Delayed development of fine motor skills
c. Rapid weight gain
d. Delayed skeletal development
a. Accelerated head and brain growth
Which of the following is true of the two symptom clusters of ADHD has two symptom clusters (hyperactive-impulsive and inattentive)?
a. Hyperactive symptoms are always more severe than inattentive symptoms.
b. Children commonly have symptoms from each cluster.
c. Symptoms from one cluster always predominate clearly in cases of ADHD.
d. Hyperactive symptoms persist longer over the lifespan than do inattentive symptoms
b. Children commonly have symptoms from each cluster
As children with ADHD mature, about __________% will continue to have the disorder during adolescence.
a. 30
b. 65
c. 50
d. 25
c. 50
The most commonly inherited cause of mental retardation is ________, which occurs when a DNA series makes too many copies of itself and “turns off” a gene on the X chromosome.
a. cultural-familial
b. fragile X syndrome
c. Down syndrome
d. phenylketonuria
b. fragile X syndrome
________ uses shaping and positive reinforcement to improve the social, communicative, and behavioral skills of children with autistic disorder by intensively training and rewarding specific behaviors.
a. Behavioral family training
b. Multisystemic therapy
c. Applied behavior analysis
d. Social psychoanalysis
c. Applied behavior analysis
Behavior patterns of autism spectrum disorder include all of the following EXCEPT
a. adherence to routines.
b. preoccupation with a particular interest.
c. self-injurious behaviors.
d. ability to start but not end a conversation.
d. ability to start but not end a conversation.
The estimated hereditability of autism spectrum disorder is _______.
a. 40%
b. 90%
c. 20%
d. 70%
b. 90%
What is the likelihood that a 42-year-old mother will give birth to an infant diagnosed with Down syndrome?
a. 1 out of 70
b. 1 out of 150
c. 1 out of 1000
d. 1 out of 30
a. 1 out of 70
Children with ADHD have a deficit in ________, which are cognitive abilities to formulate a goal, plan the actions to achieve the goal, and maintain the plan in memory in order to execute it.
a. high-level cognition
b. intelligence quotient
c. affective functioning
d. executive functioning
d. executive functioning
Children with ADHD have a deficit in ________, which are cognitive abilities to formulate a goal, plan the actions to achieve the goal, and maintain the plan in memory in order to execute it.
a. high-level cognition
b. intelligence quotient
c. affective functioning
d. executive functioning
d. executive functioning
