WorksheetsChild Neuro
Total questions: 25
Worksheet time: 47mins
An 11-year-old boy trips and falls walking down a flight of stairs and is diagnosed with a concussion. Prior to his injury, he was a healthy child, although he does have a history of recurrent severe ear infections when he was a toddler. He does well in school, although he did have difficulties with learning to read in the first and second grades. Which of this child’s characteristics puts him at greater risk for the development of persistent postconcussive symptoms?
age less than 13 years
history of ear infections
male sex
non–sports-related cause of his concussion
reading difficulties in early years of school
Which of the following is a typical component of US state concussion legislation?
education for athletes and their parents
evaluation by neuroimaging (CT or MRI) prior to return to play
mandated school accommodations
removal from play for a minimum of 3 weeks
return to play only after evaluation by the team coach
When should the Glasgow Coma Scale score be assessed after traumatic brain injury (TBI) in order to best distinguish mild from moderate TBI?
immediately after the inciting event
within 5 minutes of the inciting event
within 10 minutes of the inciting event
within 15 minutes of the inciting event
30 minutes or later postinjury
What does the author of the article recommend regarding timing of initiation and duration of continuous EEG monitoring in critically
ill children in the intensive care unit who are at risk for nonconvulsive status epilepticus?
initiate after cessation of generalized convulsive status, then maintain for at least 1 hour if normal
initiate after cessation of generalized convulsive status, then maintain for at least 8 hours if normal
initiate after routine EEG, then maintain for at least 1 hour if normal
initiate as soon as possible, then maintain for at least 1 hour if normal
initiate as soon as possible, then maintain for at least 12 to 24 hours if normal
Which of the following statements is most accurate about the determination of competency in the United States?
clinical team assessments are required by all courts to establish competency
competency is automatically assumed at majority, if no process has determined otherwise
the determination of competency can only be made by a licensed psychiatrist
lack of competency is automatically assumed at majority, provided the child has required a formal individualized education program throughout school
the process to legally remove competency is equivalent to the process to determine lack thereof prior to majority
Which of the following is one of the primary goals of the transition from pediatric to adult care for neurologic patients?
avoidance of legal guardianship
empowerment of patients and caregivers
independent living
preservation of relationship with pediatric care team
vocational training and placement
Which of the following is the first step of a successful transition from pediatric to adult neurologic care?
assessment of the patient’s self-management skills
discussion with patient and caregiver of expectations of future transition
encouragement of patient by provider to become more independent
establishment of legal guardianship
formal neuropsychological testing to determine competency
Which of the following medications has been US Food and Drug Administration (FDA)-approved to treat irritability and aggression in children with autism spectrum disorder?
amitriptyline
clonazepam
clonidine
fluoxetine
risperidone
A 7-year-old girl is being evaluated for behavioral concerns. She is described by her parents as a “very bright but very awkward child who has trouble fitting in.” On standardized educational testing, she has consistently performed above grade level in all domains. However, her teachers report that she is “in her own world” and has difficulty adapting to any change in classroom routine. For the past year, she has been highly preoccupied with meteorology, frequently redirecting any conversation back to this subject. Although interested in having friends at school, she has been unable to form any lasting friendships, mostly because of her insistence on doing things her way without compromise and a tendency not to let others have a turn to speak in conversations. She also tends to look to the side of the person with whom she is speaking rather than making eye contact, and her voice has a singsong quality to it. Which of the following diagnoses best fits this child’s overall presentation?
attention deficit hyperactivity disorder
autism spectrum disorder
intellectual disability
obsessive-compulsive disorder
social anxiety disorder
Which of the following chromosomes appears to be enriched with genes mutated in patients with intellectual developmental disorder?
X
Y
4
15
21
A patient with unexplained intellectual developmental disorder without suggestive phenotypic features has a normal chromosomal microarray. Which of the following evaluations would be most appropriate to
do next?
24-hour video EEG monitoring study
first-tier metabolic testing
G-banded karyotype with telomere fluorescence in situ hybridization (FISH)
leukocyte enzyme activity analysis of arylsulfatase A and biotinidase
whole-exome sequencing
Which of the following patients would best be described as having an intellectual disability?
a 1-year-old with trisomy 21 who is not meeting gross motor, fine motor, or language developmental milestones
a 2-year-old who is not meeting appropriate fine motor and language developmental milestones
a 6-year-old with deficits in social communication as well as behavioral abnormalities with repetitive behaviors and restricted interests
a 7-year-old with an IQ of 50 and significant limitations in adaptive behavior
a 10-year-old with difficulties in reading and mathematics requiring additional instructional support
Modafinil and armodafinil are not approved by the US Food and Drug Administration (FDA) to treat narcolepsy in children younger than 17 years because of which of the following adverse effects?
agitation
cardiac arrhythmias
seizures
stevens-johnson syndrome
tremors
A 10-year-old boy presents with a 6-month history of daytime sleepiness, including falling asleep on the school bus, during class, and while watching television in the evenings. He reports always feeling tired and naps for 1 to 2 hours at a time whenever possible. He has had several episodes of sudden loss of tone with subsequent falls to the ground, without alteration of consciousness, that were triggered by laughing with his friends. A definitive diagnosis in this patient could be made based on which of the following findings?
CSF hypocretin-1 concentration of 110 pg/mL or less
consumer-wearable actigraph confirming several daytime naps
positive human leukocyte antigen typing for the DQB1*0602haplotype
presence of a sleep-onset rapid eye movement (REM) periodwithin 2 hours of sleep onset on nocturnal polysomnogram
repeated 3-second change in background rhythm on EEG
A 5-year-old girl has a 1-year history of recurrent vomiting episodes that begin during sleep. She awakens with severe nausea and pallor and then vomits for 10 minutes. Her eyes have been observed to deviate during these episodes, and she retains consciousness. She is an otherwise developmentally normal healthy child. EEG is most likely to show which of the following patterns?
high-amplitude asynchronous slow waves and multifocal spikes and polyspikes
high-voltage sharp discharges involving the right centrotemporal region
nonreactive, discontinuous focal, theta frequency rhythm with intermixed sharp waves
paroxysmal generalized 4 Hz to 6 Hz spike-and-slow-wave activity
shifting multifocal spikes involving the occipital region
An 8-month-old girl has a 3-month history of seizures that began as recurrent hemiconvulsive febrile seizures that alternated sides. She subsequently developed febrile myoclonic, focal, and generalized seizures. More recently, she began to have reflex seizures triggered by her nightly baths. Which of the following treatments is contraindicated?
clobazam
ketogenic diet
oxcarbazepine
stiripentol
valproate
Which of the following epileptic encephalopathies has the best overall prognosis?
continuous spike and wave in slow sleep
early myoclonic epilepsy
epilepsy of infancy with migrating focal seizures
Landau-Kleffner syndrome
Lennox-Gastaut syndrome
A previously healthy 6-month-old boy is referred to the neurology clinic because of a 1-month history of daily stereotyped abnormal movements. These usually occur upon awakening or as he is falling asleep and are characterized by brief axial flexion with simultaneous flexion of the hips, shoulders, and elbows. These episodes of flexion are brief but occur repetitively over 3 to 5 minutes, and the patient cries between them. Also of concern is his development; whereas he met early milestones on time, he now has lost the ability to roll and seems“sleepy and out of it” all the time. On EEG, the interictal background is very high in amplitude and chaotic with very frequent multifocal epileptiform transients and no normal waveforms, and each of his flexion spells is associated with a high-amplitude generalized slow wave followed by a diffuse relative suppression of voltage. Which of the following electroclinical syndromes is the most likely diagnosis in this patient?
early infantile epileptic encephalopathy
early myoclonic encephalopathy
epilepsy of infancy with migrating focal seizures
infantile spasms
Lennox-Gastaut syndrome
A 5-year-old boy presents with a history of seizures that began at the age of 2. Initially, he had nocturnal tonic seizures followed by the emergence of atypical absence seizures during wakefulness and several generalized tonic-clonic seizures. He is now being evaluated for episodes of sudden loss of tone, at times with an isolated head drop or a fall from standing. Although he met his early milestones on time, he did not say single words until the age of 30 months and now has a 50-word vocabulary. He is ambulatory but very uncoordinated. Although delayed, he has not had a developmental regression. His neurologic examination is revealing only for these cognitive impairments with no dysmorphic features or focal findings. Which of the following interictal EEG patterns would most likely be seen in this patient?
diffusely slow background with spike-and-wave bursts at 1 Hz to 2.5 Hz
focal spike-and-wave transients occupying more than 85% of slow-wave sleep
hypsarrhythmia with excessive interhemispheric synchrony
normal background with spike-and-wave bursts at 3 Hz to 4 Hz
suppression burst pattern
Which of the following chemotherapeutic agents can induce antithrombin III deficiency?
asparaginase
cyclophosphamide
cytarabine
5-fluorouracil
ifosfamide
A 15-month-old boy is evaluated for visual and growth impairment. He was born at term after an uncomplicated pregnancy, but it was noted that he did not fix and follow or develop normal visual attentiveness. He is diagnosed with hypopituitarism. Given these symptoms and signs, an MRI will most likely demonstrate findings consistent with which of the following disorders?
agenesis of the corpus callosum
hemimegalencephaly
periventricular nodular heterotopias
septooptic dysplasia
tuberous sclerosis complex
Polymicrogyria is most likely to be caused by a disruption of which of the following processes?
cell proliferation
neuronal differentiation/maturation
neurulation
postmigrational development
prosencephalic development
A 6-month-old girl presents with global developmental delay and severe microcephaly. On examination, the skull bones have folded inward as if the skull has partially collapsed, although the scalp appears normal. The patient has contractures of the elbows, wrists, and knees, which were present from birth, as well as diffuse spasticity. Brain MRI reveals very thin and dysplastic cerebral parenchyma, while an earlier CT scan revealed subcortical calcifications. Given these findings, which of the following causes of the patient’s microcephaly is most likely?
early intrauterine hypoxic-ischemic injury
failure of rostral neural tube fusion
genetic defect in cell proliferation
genetic defect in neuronal migration
intrauterine infection with Zika virus
A 13-year-old boy presents with new-onset epilepsy. He was born at term and has developed normally. His seizures are characterized by behavioral arrest and staring with orofacial automatisms lasting
for 30 to 45 seconds. His general physical and neurologic examination are normal. Brain MRI reveals periventricular nodular heterotopia without any associated cortical abnormalities. A mutation of which of the following genes is most likely responsible for this disorder of neuronal migration?
DCX
FLNA
LIS1
PIK3R2
SHH
A 12-year-old boy presents for evaluation of attention deficit hyperactivity disorder. On examination, he is found to have a head circumference at the 98th percentile, hypertelorism, and eight café au lait spots. He has no axillary or inguinal freckling, and his neurologic examination is normal. He is otherwise healthy but has a long-standing history of mild academic difficulties in school. His mother is also examined and demonstrates five café au lait spots with sparse bilateral axillary freckles without inguinal freckling; she also has a history of mild learning difficulties but has had no medical problems. What is the most likely diagnosis in this patient?
incontinentia pigmenti
Legius syndrome
Noonan syndrome with multiple lentigines
neurofibromatosis type 1
neurofibromatosis type 2
