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Neurology is for the birds Neurology for the boards Jim

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Neurology is for the birds Neurology for the boards Jim Owens Pediatrics, Neurology, and Rodent Medicine You are examining a 2-year-old girl who has a 6-month history ... – PowerPoint PPT presentation

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Title: Neurology is for the birds Neurology for the boards Jim


1
Neurology is for the birds Neurology for the
boards
  • Jim Owens
  • Pediatrics, Neurology, and
  • Rodent Medicine

2
  • You are examining a 2-year-old girl who has a
    6-month history of developmental regression.
    During her first postnatal year, she met all
    motor, language, and social milestones. Her head
    circumference, which currently is at the 3rd
    percentile, was at the 75th percentile at birth.
    On physical examination, she makes poor eye
    contact and repetitively wrings her hands.

3
Small head, wringing hands
  • Of the following, the MOST appropriate diagnostic
    test is
  • arylsulfatase A
  • fragile X
  • hexosaminidase A
  • MECP2 gene testing
  • urine N-acetyl-aspartic acid

4
  • A 17-year-old girl complains of clumsiness over
    the past 3 days. She has had moderate headaches
    for 1 month and neck discomfort for 3 days.
    Physical examination reveals right-sided
    dysmetria and left upper and lower extremity
    numbness to pinprick and weakness, graded as 4/5.
    The remainder of her examination results are
    normal.

5
Headache, numbness, weakness, dysmetria
  • Of the following, the MOST appropriate evaluation
    to establish this patient's diagnosis is
  • computed tomography scan of the brain
  • lumbar puncture
  • measurement of nerve conduction velocities
  • measurement of somatosensory evoked potentials
  • urine toxicology screen

6
  • You are seeing for the first time a 15-month-old
    boy who was born at 28 weeks' gestation. He had
    an afebrile seizure at 12 months, but takes no
    medications. He uses both a cup and a bottle and
    takes most solid foods without choking. He wakes
    frequently at night. He can sit alone, but does
    not crawl or walk. He uses three words other than
    "mama" or "dada." His growth parameters are at
    the 50th percentile for length and head
    circumference and at the 10th percentile for
    weight. On physical examination, you note
    increased tone in his lower extremities and trunk.

7
Preemie, not crawling, increased LE tone
  • Of the following, the MOST likely other
    information that you would expect is a history of
  • constipation
  • developmental regression
  • recurrent rashes
  • tachycardia
  • tendon releases

8
  • A 12-year-old girl presents with her third
    headache in the last 2 months. She describes the
    pain as pounding, sharp, and severe. The pain is
    bifrontal and has been present for 1 hour. Past
    history is notable for motion sickness at age 4
    years. Physical examination results are normal,
    but the girl draws a picture of dots when asked
    if she sees anything prior to pain.

9
Headaches, car sickness, and seeing dots
  • Of the following, the MOST appropriate treatment
    for this child is
  • intramuscular meperidine
  • intranasal butorphanol
  • oral ibuprofen
  • oral zolmitriptan
  • subcutaneous sumatriptan

10
  • A 4-year-old girl is brought to the emergency
    department by her babysitter because the child
    has suddenly become clumsy, and her speech has
    become slurred over the last hour. On physical
    examination, the girl is afebrile and dysarthric.
    She has prominent vertical and horizontal
    nystagmus, along with truncal and appendicular
    ataxia. Deep tendon reflexes are normal, as are
    results of the remainder of the physical
    examination.

11
Acutely ataxic 4 year old
  • Of the following, the MOST likely diagnosis is
  • brain tumor
  • cerebellar hemorrhage
  • Guillain-Barré syndrome
  • Meningoencephalitis
  • toxic ingestion

12
  • A 2-year-old girl is rushed by ambulance to the
    emergency department for sudden-onset ataxia. Her
    parents have yet to arrive. On physical
    examination, the girl is afebrile, yet
    diaphoretic, with some nystagmus on far lateral
    gaze. Her ataxia has resolved. The remainder of
    physical examination findings are normal

13
Acutely ataxic 2 year old
  • Of the following, the MOST likely diagnosis for
    this child is
  • basilar migraine
  • benign paroxysmal vertigo
  • cerebellar hemorrhage
  • phenytoin intoxication
  • seizure

14
  • You have been treating a 2-year-old girl for
    pneumococcal meningitis for the past 5 days. Of
    the following, the MOST likely complication of
    her disease is
  • brain abscess
  • cerebral infarct
  • cranial nerve palsy
  • hearing impairment
  • sagittal sinus thrombosis

15
  • A 4-year-old girl presents to the emergency
    department with a 3-day history of left-sided
    weakness. On physical examination, she has
    temperature of 99F (37.2C), pulse of 50
    beats/min, respiratory rate of 24 breaths/min,
    and blood pressure of 118/78 mm Hg. She cannot
    abduct her right eye, but has normal funduscopic
    examination results. Strength on the left is 4/5
    in the upper and lower extremity and on the right
    is normal.

16
4 year old with left-sided weakness and right
abducens palsy
  • Of the following, the MOST appropriate next step
    in the evaluation of this child is
  • cerebral angiography
  • computed tomography scan of the brain
  • electroencephalography
  • positron emission tomography scan of the head
  • single-photon emission computed tomography scan
    of the brain

17
  • A 7-year-old girl presents with a 4-week history
    of an erythematous rash that involves the eyelids
    and malar areas and now has occurred at the
    elbows and knees. Her mother reports that the
    girl has had a poor appetite, low-grade fevers,
    muscle aches, and weakness during the past week.
    Physical examination confirms the rash. She has
    difficulty rising from a chair. Other findings on
    her physical examination are normal. Serum
    creatine kinase is 677 U/L.

18
7 year old with rash, malaise, weakness
  • Of the following, the test MOST likely to lead to
    this child's diagnosis is
  • erythrocyte sedimentation rate
  • molecular testing for fascioscapulohumeral
    dystrophy
  • muscle biopsy
  • polymerase chain reaction testing for dystrophin
  • serum antinuclear antibody

19
  • The parents of a 4-month-old bring the boy to you
    because of failure to thrive. The baby was born
    after a term gestation, and there were no
    complications at delivery. The mother reports
    that he has a weak suck, and his body seems limp.
    On physical examination, the child's weight is
    4.1 kg, length is 52 cm, and occipitofrontal
    circumference is 40.5 cm. His penis appears small
    for age, and the child exhibits diffuse
    hypotonia. Deep tendon reflexes are normal. Other
    findings on the physical examination are normal.

20
Floppy 4 month old
  • Of the following, the test MOST likely to
    establish this child's diagnosis is
  • arylsulfatase A measurement
  • electromyography with nerve conduction velocities
  • fluorescent in situ hybridization testing
  • magnetic resonance imaging of the brain
  • thyroid-stimulating hormone measurement

21
  • A father brings in his 6-year-old boy who has
    chronic constipation because the child now has
    developed enuresis during both night and day. On
    physical examination, you discern a small, firm
    sacral dimple just to the right of the gluteal
    cleft. The remainder of the physical examination
    findings are normal.

22
Constipated 6 year old with a sacral dimple
  • Of the following, the MOST appropriate next step
    in the evaluation of this child is
  • magnetic resonance imaging of the spine
  • radiographs of the abdomen and pelvis
  • ultrasonography of the kidneys, ureters, and
    bladder
  • urine culture
  • voiding cystourethrography

23
  • A 15-year-old girl is rushed to the emergency
    department after slipping off a diving board and
    striking her head on cement. On physical
    examination, her mental status evaluation results
    are completely normal. She can abduct her upper
    extremities at the shoulder, but cannot flex or
    extend her arms and minimally moves her fingers.
    She cannot move her limp lower extremities. You
    order emergent magnetic resonance imaging of the
    brain and spine.

24
Diving board induced quadraparesis
  • Of the following, the MOST important therapy to
    implement before the patient is sent for imaging
    is
  • fosphenytoin 18 phenytoin equivalents/kg
  • intravenouslydexamethasone 1 mg/kg orally
  • low-molecular weight heparin 1 mg/kg
    subcutaneously
  • mannitol 1 g/kg intravenously
  • methylprednisolone 30 mg/kg intravenously

25
  • An 11-day-old infant presents to the clinic with
    a history of a temperature of 100.8F (38.2C)
    and a 1-day history of poor feeding. Findings on
    physical examination are normal. You initiate a
    sepsis evaluation that includes a lumbar
    puncture. The cerebrospinal fluid results
    demonstrate a white blood cell count of
    6x10³/mcL, with 68 neutrophils, 2 bands, and
    30 lymphocytes. The protein concentration is 200
    mg/dL, and the glucose value is 36 mg/dL. The
    abnormal findings prompt you to order magnetic
    resonance imaging, which demonstrates abnormal
    frontal lobes bilaterally that includes some
    degree of infarction but also abscesses and
    cerebritis

26
Unfortunate 11 d/o with cerebritis and abscesses
  • Of the following, the MOST likely pathogen is
  • Citrobacter koseri
  • Escherichia coli
  • Klebsiella pneumoniae
  • Listeria monocytogenes
  • Streptococcus agalactiae

27
  • A newborn in your neonatal intensive care unit
    has had intermittent seizures for 72 hours. You
    have been unable to control the seizures with
    phenobarbital, diphenylhydantoin, and lorazepam.
    Electrolyte, calcium, magnesium, and glucose
    concentrations are normal. The infant
    subsequently becomes apneic, comatose, and
    unresponsive.

28
Comatose newborn with seizures
  • Of the following, the BEST laboratory test to
    determine the cause of coma in this infant is
  • analysis of whole blood lead concentration
  • evaluation of urine for reducing substances
  • measurement of serum amino acids, organic acids,
    lactate, and ammonia
  • measurement of serum cortisol, thyroxine, and
    thyroid-stimulating hormone
  • measurement of very long-chain fatty acids

29
  • You are called to see a hospitalized 9-year-old
    girl who suddenly has become dystonic, with her
    neck hyperextended, and is unable to move her
    eyes, now superiorly deviated. The nurses relate
    that this girl has non-Hodgkin lymphoma and has
    been receiving highly emetogenic chemotherapy

30
9 year old with hyperextended neck
  • Of the following, the drug MOST likely to have
    caused this girl's symptoms and signs is
  • Aprepitant
  • Diphenhydramine
  • Lorazepam
  • Metoclopramide
  • Ondansetron

31
  • A 7-year-old boy presents for evaluation of
    attention-deficit/hyperactivity disorder,
    following the suggestion of his first-grade
    teacher. The child 's academic and behavioral
    function were described as normal in preschool
    and kindergarten, but this year he constantly
    talks out of turn, does not stay in his chair,
    and has frequent emotional "melt-downs." His
    mother says his memory is poor and grades are
    declining. On physical examination, you note very
    brisk reflexes in the arms and legs, two beats of
    clonus at the ankles, and slow and clumsy
    fine-motor movements.

32
7 year old with acute ADHD
  • Of the following, the most appropriate INITIAL
    diagnostic test is
  • brain magnetic resonance imaging
  • Electroencephalography
  • Electromyography
  • serum creatine kinase measurement
  • serum lactate measurement

33
  • A 14-year-old girl who has a 1-year history of
    migraine headaches presents to the emergency
    department with a severe headache that she calls
    "the worst headache of my life. " The headache
    occurred suddenly after she lifted a heavy box.
    Her mother says that the girl has been holding
    her head stiffly. On physical examination, she
    appears in severe pain and has meningismus. Other
    findings on the physical examination are normal.

34
14 year old migraineur with bad headache
  • Of the following, the MOST appropriate initial
    course of action is
  • noncontrast head computed tomography scan
  • intravenous administration of ceftriaxone
  • intravenous administration of dihydroergotamine
  • lumbar puncture
  • oral administration of sumatriptan

35
  • A 10-year-old boy presents with leg weakness that
    has progressed over 24 hours, bladder and bowel
    incontinence, and back pain. There is no history
    of trauma. On physical examination, leg reflexes
    are diminished, and there is numbness in the legs
    and lower trunk. Rectal examination demonstrates
    decreased tone. Sensory examination shows absent
    pinprick sensation below T6.

36
Incontinence with leg weakness and numbness
  • Of the following, the MOST appropriate initial
    diagnostic test is
  • brain magnetic resonance imaging with contrast
  • electromyography of the legs
  • lumbar puncture
  • nerve conduction velocities
  • spine magnetic resonance imaging with contrast

37
  • During the health supervision visit of a
    10-year-old boy, you note some wasting and
    weakness of his lower leg muscles, with
    diminished patellar and ankle reflexes. You
    examine his parents' legs and feet and notice
    that his mother has a bilateral foot drop and
    deformed feet

38
10 year old with distal LE wasting
  • Of the following, the MOST likely diagnosis is
  • Becker muscular dystrophy
  • celiac disease
  • chronic inflammatory demyelinating polyneuropathy
  • hereditary sensorimotor neuropathy
  • hydrocephalus

39
  • A 5-year-old girl presents with leg weakness of
    12 hours' duration. She is afebrile and describes
    no pain in her back or elsewhere. Findings on
    physical examination include distal leg weakness
    and diminished reflexes in the legs.
    Finger-to-nose testing reveals slight dysmetria.
    Rectal tone is normal. You find an engorged tick
    on her occipital scalp

40
Weak girl, happy tick
  • Of the following, the MOST likely diagnosis is
  • Botulism
  • Guillain-Barré syndrome
  • Hydrocephalus
  • tick paralysis
  • transverse myelitis

41
  • You receive a phone call at your office from a
    resident, who is making rounds in the hospital.
    She tells you that computed tomography scan of
    the 14-year-old girl you admitted last night with
    a history of fever and increasing headaches
    revealed a large brain abscess and asks your
    advice on choice of antimicrobial therapy.

42
14 year old with brain abscess
  • Of the following, the BEST combination of
    antimicrobial agents to start for this patient is
  • cefazolin metronidazole gentamicin
  • clindamycin ceftriaxone
  • nafcillin metronidazole cefuroxime
  • piperacillin-tazobactam gentamicin
  • vancomycin metronidazole ceftriaxone

43
  • You are treating a 16-year-old girl who has a
    history of generalized tonic-clonic seizures, the
    most recent of which occurred 6 months ago. She
    is followed by a neurologist who is treating her
    with phenylhydantoin with good results. During a
    routine physical examination, she confides that
    she is sexually active, and she requests a form
    of birth control. After some discussion, she asks
    to be started on oral contraceptive pills (OCPs).

44
Dilantin and OCPs
  • Of the following, the MOST accurate statement
    about oral contraceptive use in this scenario is
    that
  • OCPs are contraindicated for use in women taking
    antiepileptics
  • only barrier methods should be recommended for
    this girl
  • the girl can be weaned off her antiepileptic
    medication at this time
  • the girl should be switched to a different
    antiepileptic before starting OCPs
  • the OCP estrogen dose should be higher than that
    usually prescribed

45
  • A 10-year-old boy in whom you diagnosed
    attention-deficit/hyperactivity disorder (ADHD)
    has been receiving stimulant medication daily
    since he was 7 years old. About 2 years ago, he
    developed persistent, repetitive throat clearing,
    and subsequently he has had waxing and waning
    motor and vocal tics that include eye rolling,
    grimacing, head bobbing, sniffing, and humming.
    For the past 2 months, his tics have worsened he
    now has a loud squeak and head-jerking tic. His
    mother decided to stop his stimulant medication
    last week, fearing it might be causing his tics
    to worsen. Now he is much more hyperactive and is
    having more behavioral and attentional
    difficulties at school and home. His tics have
    not improved

46
10 y/o with ADHD and worsening tics
  • Of the following, the MOST appropriate treatment
    plan is to prescribe
  • carbamazepine to see if the movement disorders
    are seizures
  • daily penicillin to prevent tic exacerbations
    caused by streptococcal infections
  • haloperidol to treat the tics
  • methylphenidate again to treat the ADHD
  • sertraline to treat the tics

47
  • A 7-year-old patient who has Down syndrome is
    brought to the clinic by her mother, who is
    worried that the child has an increasingly
    abnormal gait and worsening clumsiness. At age 3
    years, she was screened for cervical instability
    with flexion and extension cervical spine films,
    which showed normal results. On physical
    examination today, you note that she has an
    unsteady gait, and she has brisk deep tendon
    reflexes diffusely. These findings represent a
    significant change from 9 months ago when your
    neurologic examination showed only slightly
    diminished tone.

48
Trisomy 21 with new hyper-reflexia
  • Of the following, the MOST likely cause of these
    symptoms and signs in a child who has Down
    syndrome is
  • cerebellar medulloblastoma
  • Chiari I malformation
  • leukemia involving the central nervous system
  • subluxation of the atlantoaxial joint
  • transverse myelitis of the cervical cord

49
  • A 10-year-old boy presents to the emergency
    department with confusion. He is febrile. While
    you are examining him, his eyes glaze over and
    deviate to the right, he has automatic chewing
    movements, and he is completely unresponsive for
    30 seconds, after which he is very sleepy.
    Emergent head computed tomography scan shows low
    density in the right temporal lobe.

50
Febrile 10 y/o with seizure and temporal mass
  • Of the following, the MOST appropriate next step
    is
  • administration of intravenous acyclovir
  • administration of intravenous ampicillin
  • administration of intravenous dexamethasone
  • brain magnetic resonance imaging with contrast
  • emergent electroencephalography

51
  • A 12-year-old girl in your practice had been born
    preterm and presented in early childhood with
    developmental delay. She was diagnosed with
    cerebral palsy and has been given physical,
    occupational, and speech therapy. Magnetic
    resonance imaging at age 3 years showed white
    matter volume loss, particularly adjacent to the
    ventricles. The parents are concerned their
    daughter's condition may be degenerating because
    she has fallen progressively further behind her
    peers, and she has become increasingly anxious
    and oppositional. She has not developed seizures.
    Her recent special education re-evaluation at
    school revealed verbal and performance
    intelligence quotients in the 70s, unchanged from
    3 years ago. On physical examination, you note
    dolichocephaly, hyperreflexia at the knees, and
    two beats of clonus at each ankle.

52
Former preemie with concern for regression
  • Of the following, the MOST appropriate next step
    is
  • follow-up magnetic resonance imaging
  • measurement of urine organic acids
  • referral for behavioral therapy
  • referral to genetics
  • sleep-deprived electroencephalography

53
  • A mother brings her 3-year-old boy to the
    emergency department. She explains that the boy
    suddenly stopped paying attention, stared, and
    had jerking of his arms and legs for about 1
    minute. His lips turned blue, and he became
    incontinent of urine. After the episode, he
    appeared confused and became very sleepy. On
    physical examination, he has a temperature of
    104F (40C). Following administration of
    acetaminophen, his temperature has decreased to
    98.6F (37C). He is alert, interactive with his
    parents, and has normal findings on physical
    examination.

54
3 year old with seizure and fever
  • Of the following, the MOST appropriate next step
    is to
  • begin therapy with carbamazepine
  • obtain magnetic resonance imaging
  • obtain sleep-deprived electroencephalography
  • perform a lumbar puncture
  • provide the family with education

55
  • An 8-year-old girl is brought to the emergency
    department via ambulance. On the playground, she
    suddenly stopped playing, bent forward and fell
    to the ground, and had jerking of her arms and
    legs. She drooled excessively and was
    unresponsive. Afterwards, she was confused, her
    speech was slurred, and she was somewhat
    combative for about 30 minutes. In the emergency
    department, she is responding appropriately, is
    afebrile, and has normal findings on general and
    neurologic examinations. Her mother states that
    she has always been healthy and is an average
    student. Review of systems reveals no headaches
    or recent illness.

56
8 y/o with first time spell
  • Of the following, the MOST appropriate next step
    prior to discharge from the emergency department
    is to
  • educate the family about prognosis and safety
  • obtain a stat electroencephalogram
  • obtain magnetic resonance imaging of the brain
  • order measurement of serum electrolytes
  • perform a lumbar puncture

57
  • An 18-month-old developmentally delayed child
    presents to your office for follow-up after an
    emergency department visit the previous day for a
    first seizure. The seizure began with jerking of
    the left arm, followed quickly by loss of
    responsiveness and jerking of the entire body. It
    lasted about 2 minutes and was followed by
    sleepiness for 4 hours. In the emergency
    department, the child was difficult to arouse.
    Head computed tomography scan showed no acute
    changes, and findings on lumbar puncture were
    normal. The child was afebrile. The examining
    physician diagnosed an ear infection and advised
    the family to follow up with you today. The child
    was born at 26 weeks' gestation. At present, he
    can sit but is not yet standing. Physical
    examination reveals a head circumference of 43
    cm, and the head shape is dolichocephalic. Other
    remarkable findings on physical examination
    include hyperreflexia with crossed adduction at
    both knees and ankle clonus.

58
18 month old former 26 weeker with O.M. and first
seizure
  • Of the following, you are MOST likely to advise
    the parents that
  • electroencephalography should be performed to
    confirm that the incident was a seizure
  • recurrent seizures in their child most likely
    will cause brain damage
  • the risk of seizure recurrence for their child is
    about one in three
  • the risk of seizure recurrence in their child is
    increased because of his developmental delay and
    hyperreflexia
  • their child has epilepsy and needs to begin
    antiseizure medication

59
  • You are seeing a term 4,500-g large-for-gestationa
    l age (LGA) infant in the nursery at 2 hours of
    age. His delivery was complicated by a difficult
    vaginal extraction with forceps assistance, and
    he had a shoulder dystocia. Physical examination
    reveals a large infant who is well perfused and
    in no respiratory distress. There is no crepitus
    along the clavicles. You elicit an asymmetric
    Moro reflex with inability to raise the right arm
    at the shoulder. The infant holds his right arm
    in adduction and internal rotation, with
    pronation of the forearm. His left hand displays
    a normal grasp.

60
Term forceps baby with right UE weakness
  • Of the following, a TRUE statement about this
    infant's condition and prognosis is that
  • brachial plexus injury with palsies affecting the
    lower arm and hand have a poorer prognosis than
    those with isolated upper arm palsy
  • brachial plexus injury with palsies affecting the
    lower arm and hand is due most commonly to in
    utero nerve damage acquired in infants of
    diabetic mothers and will be permanent
  • clavicular fracture often complicates brachial
    plexus injury in LGA infants and results in a
    long-term shoulder drop
  • complete avulsion of the brachial plexus is the
    most common injury and requires microsurgical
    repair by 3 months of age
  • Erb palsy is the least common form of brachial
    plexus injury, involves the lower arm and hand,
    and requires several months to heal

61
  • At 8 AM, your nurse urgently calls you to see a
    child in the waiting room. You come out and
    observe a 5-year-old boy whose eyes are glassy
    and staring off to the right. He is making
    chewing movements and has urinated. He is not
    responding to his mother's calls or touch. He
    then blinks several times and begins to respond,
    but is clearly confused. His mother explains that
    her son has been diagnosed with epilepsy, but she
    ran out of medication 2 days ago

62
Drama in your waiting room
  • Of the following, the MOST appropriate
    maintenance antiseizure medication for this child
    is
  • Carbamazepine
  • Diazepam
  • Ethosuximide
  • Phenobarbital
  • Phenytoin

63
  • An 8-year-old boy presents to the emergency
    department following 3 days of progressive
    difficulty walking. He says that his back hurts.
    Physical examination shows no abnormalities. On
    neurologic examination, he is alert, makes good
    eye contact, and responds to questions
    appropriately, but seems distressed. Cranial
    nerve examination results are normal. On motor
    examination, strength is 4/5 in the arms and
    legs. Reflexes are absent. He cannot rise from
    the floor unassisted. Sensory examination
    findings are normal

64
8 year old with trouble walking
  • Of the following, the MOST important initial
    procedure is
  • brain magnetic resonance imaging
  • Electroencephalography
  • Electromyography
  • forced vital capacity
  • lumbar puncture

65
  • A 5-year-old boy is brought to the office 4 hours
    after falling off his bicycle. His mother reports
    that he was not wearing a helmet, and bystanders
    said that he did not lose consciousness. When his
    friends brought him home, he was tearful and
    sleepy but was answering questions appropriately.
    The mother noted an abrasion on the left side of
    his head and applied ice to a small area of
    swelling on the left temple. Over the last
    several hours, however, he has become
    increasingly confused and has had multiple
    episodes of vomiting. On physical examination, he
    is difficult to arouse, and his right pupil is
    larger than his left.

66
Sleepy child after helmetless bike crash
  • Of the following, a TRUE statement regarding this
    patient's likely diagnosis is that
  • improved survival is associated with prompt
    neurosurgical intervention in symptomatic
    patients
  • intracranial injury is unlikely because there was
    no loss of consciousness
  • mannitol is the initial treatment of choice
  • the gradual onset of symptoms is most consistent
    with a concussion
  • the injury is caused by laceration of the veins
    that bridge the dural sinuses and the brain

67
  • A mother brings in her 4-year-old boy because she
    is concerned about his increasing clumsiness. He
    has been previously healthy and achieved
    developmental milestones on time. His growth
    parameters are normal. On physical examination,
    his mental status is normal, as are results of
    cranial nerve and sensory examinations and
    reflexes. However, he cannot rise from the floor
    without using his hands, and his running looks
    clumsy.

68
Clumsy 4 y/o boy
  • Of the following, the MOST appropriate next test
    to assess the cause of this child's symptoms is
  • Electromyography
  • lumbar puncture
  • measurement of serum creatine kinase
  • muscle biopsy
  • spine magnetic resonance imaging

69
  • A 16-year-old girl presents to the emergency
    department with the complaint of weakness for 3
    weeks. She is having difficulty walking up
    stairs, particularly in the evening, and she has
    had double vision intermittently. She has no
    pain. On physical examination, this slim
    adolescent female appears sad and uninterested,
    with droopy eyelids. Cranial nerve examination
    shows slight limitation of abduction of the right
    eye, with complaints of double vision on the
    right, facial weakness, and a nasal-sounding
    voice. Motor examination documents 4/5 strength
    in the hands, shoulders, and hips. Reflexes are
    1 in arms and legs. Sensory examination results
    are normal.

70
Teen with diplopia and progressive weakness
  • Of the following, the MOST important initial test
    is
  • edrophonium chloride (Tensilon) test
  • forced vital capacity
  • lumbar puncture
  • magnetic resonance imaging of the brain
  • measurement of serum creatine kinase

71
  • A 15-year-old girl presents to the emergency
    department with a 4-week history of nasal
    drainage and face pain and a 2-week history of
    frontal headaches and fatigue. Her mother
    complains that her daughter has an "attitude" and
    has not been respectful or seemed to care about
    anything for the past 2 weeks. The daughter awoke
    this morning with a headache and vomited. On
    physical examination, the adolescent is afebrile
    and has normal vital signs. She responds slowly
    to questions and is not oriented to the date. She
    complains of pain to palpation of her cheeks and
    forehead. She has no nuchal rigidity and no focal
    weakness. The remainder of the physical
    examination findings are normal.

72
Teen with new attitude, headache, and emesis
  • Of the following, the BEST initial diagnostic
    procedure is
  • computed tomography scan of the head with
    intravenous contrast
  • emergent electroencephalography to rule out
    nonconvulsive status epilepticus
  • lumbar puncture to rule out meningitis
  • nasal swab for bacterial culture
  • urine drug screen for barbiturates, amphetamines,
    and cocaine

73
  • A 4-year-old boy presents with headache and
    difficulty walking. On physical examination, he
    is afebrile, all growth parameters are within
    normal limits, and his mentation appears normal.
    The optic discs are clearly visible and appear
    normal. He has normal eye position in primary
    gaze but cannot abduct his right eye fully. He
    has normal tone, strength, and reflexes in his
    upper limbs, but has bilateral hyperreflexia at
    the knees and ankle clonus. On gait examination,
    he toe-walks.

74
Diplopia, hyperreflexia, toe walking
  • Of the following, the MOST important next step is
    to obtain
  • computed tomography scan of the head
  • electromyography/nerve conduction studies of the
    legs
  • lumbar puncture
  • magnetic resonance imaging of the thoracolumbar
    spine
  • visual evoked potentials

75
  • The mother of a 10-month-old child who has mild
    hypotonia brings him to the office after he has
    an unprovoked seizure. On physical examination,
    you note several hypopigmented macules on the
    trunk. Magnetic resonance imaging of the brain
    reveals several thickened areas of cerebral
    cortex, with abnormal signal and abnormalities
    along the walls of the lateral ventricles.

76
Hypotonic 10 m/o with seizures, skin findings,
and abnormal MRI
  • Of the following, the MOST likely diagnosis is
  • incontinentia pigmenti
  • neurofibromatosis type 1
  • Sturge-Weber syndrome
  • tuberous sclerosis
  • von Hippel-Lindau syndrome

77
  • A 16-year-old girl who is new to your practice
    complains of a nearly constant headache for the
    past year. She describes the pain as a band
    around her head that often is throbbing and is
    worse during the middle of the day. She denies
    nausea or vomiting but reports occasional
    fatigue. There is no family history of headaches.
    She has missed more than 20 days of school this
    year because of the headache, and she is
    struggling to maintain a C average. She admits to
    hating school and does not participate in
    extracurricular activities because she "doesn't
    like anything." Findings on her physical
    examination, including complete neurologic and
    funduscopic evaluation, are normal.

78
Missing school with head pain
  • Of the following, the BEST next step in the
    management of this girl's headaches is to
  • advise her to keep a headache diary and return in
    2 months
  • obtain a lumbar puncture
  • obtain computed tomography scan of the brain
  • prescribe oral sumatriptan
  • refer her for psychosocial evaluation and
    counseling

79
  • A 6-year-old boy presents with a sudden-onset
    loss of awareness characterized by staring,
    drooling, and chewing movements for more than 15
    minutes, followed by confusion, then deep sleep.
    On physical examination in the emergency
    department, the child is afebrile and appears to
    be returning to normal. Vital signs and general
    examination findings are normal, and there are no
    focal findings. Head computed tomography scan
    shows a large, contrast-enhancing cerebral mass
    without edema or midline shift.

80
6 y/o with seizure and mass
  • Of the following, the MOST likely diagnosis is
  • arteriovenous malformation
  • brain damage from a seizure
  • Ependymoma
  • glioblastoma multiforme
  • herpes encephalitis

81
  • A 14-year-old girl is brought to the emergency
    department because she has back pain and a sudden
    inability to walk. Neurologic examination shows
    normal upper limb strength. However, her legs are
    flaccid, relatively symmetrically weak,
    areflexic, and numb to pinprick. Vibratory and
    position sense in the legs persists. A sensory
    deficit exists below the sixth thoracic
    dermatome. Rectal examination shows low rectal
    tone. The remainder of her physical examination
    findings, including vital signs, are normal.

82
14 year old with floppy legs
  • Of the following, the MOST appropriate next
    diagnostic study is emergent
  • brain magnetic resonance imaging
  • lumbar puncture
  • nerve conduction/electromyography of the legs
  • somatosensory evoked potentials
  • spine magnetic resonance imaging

83
  • A 4-year-old boy who has neuroblastoma presents
    with back pain and an inability to urinate. He is
    alert, with normal general examination findings
    and normal mental status. Strength and tone in
    the arms are normal, but tone is low in the legs,
    and patellar reflexes are diminished.

84
4 y/o with neuroblastoma, back pain, and trouble
voiding
  • Of the following, the MOST appropriate next step
    for diagnosis is
  • lumbar puncture
  • magnetic resonance imaging with contrast of the
    spine
  • postvoid bladder residual measurement
  • radiograph of the spine
  • voiding cystourethrography

85
  • A 10-year-old boy has double vision and drooping
    eyelids. On physical examination, he is afebrile
    and has normal mentation. Pupillary responses are
    normal, but he has bilateral ptosis. He cannot
    fully adduct his right eye. You note that his
    ptosis increases with sustained upward gaze.
    Bedside forced vital capacity is normal.

86
10 year old with droopy lids
  • Of the following, the test MOST likely to confirm
    the diagnosis is
  • brainstem auditory evoked potentials
  • cold caloric testing
  • edrophonium test
  • lumbar puncture
  • visual evoked potentials

87
  • A mother brings her 8-year-old daughter to your
    office after the girl experiences a first
    unprovoked generalized tonic-clonic seizure at
    school. The child had been seen in an emergency
    department, and results of a head computed
    tomography scan performed there were normal. Her
    development, school performance, and results of
    physical examination are normal. You review
    safety concerns (no unsupervised time in bathtub
    or pools, wearing a bicycle helmet) and seizure
    first aid with the mother. Following published
    guidelines, you obtain routine electroencephalogra
    phy (EEG), which a neurologist interprets as
    normal. The mother asks you about anticonvulsant
    therapy to prevent further seizures.

88
Normal 8 y/o with first seizure
  • Of the following, you are MOST likely to advise
    her that
  • repeat sleep-deprived EEG is needed to select
    medication
  • treatment with carbamazepine should be initiated
  • treatment with phenobarbital should be initiated
  • treatment with phenytoin should be initiated
  • treatment with seizure medication should be
    deferred

89
  • A 6-year-old boy presents in late summer to the
    emergency department with a severe headache and
    muscle pains. He recently returned from a camping
    trip. On physical examination, he is febrile and
    has no focal weakness, but he suffers a prolonged
    tonic-clonic seizure and becomes unresponsive.
    Head computed tomography scan reveals no
    abnormalities. Acyclovir and fosphenytoin are
    administered. Magnetic resonance imaging shows
    subtle, diffuse signal change and thickening in
    the cerebral cortex, no signal changes in
    temporal lobes, and no meningeal enhancement.

90
6 y/o with seizure and cerebritis
  • Of the following, the MOST likely cause of the
    boy's symptoms is
  • Arbovirus
  • Borrelia burgdorferi
  • herpes simplex virus 1
  • Listeria monocytogenes
  • Taenia solium

91
  • During the routine health supervision visit for a
    4-month-old infant, you note low tone and poor
    visual interaction. His head shape is symmetric
    and his head circumference is 36 cm (lt2nd
    percentile). He had been born at term with a head
    circumference of 32 cm (2nd percentile).

92
4 m/o with poor tone and visual interaction
  • Of the following, the MOST helpful initial
    diagnostic test to explain the cause of the
    infant's abnormal examination findings is
  • brain magnetic resonance imaging
  • Electroencephalography
  • head ultrasonography
  • high-resolution karyotyping
  • three-dimensional head computed tomography scan

93
  • A 4-year-old boy recently underwent hematopoietic
    stem cell transplantation for acute myelogenous
    leukemia. Fourteen days after his transplant, he
    experiences a seizure and confusion. He is
    receiving cyclosporine, prednisone, ganciclovir,
    fluconazole, cefotaxime, tobramycin, and
    omeprazole. Magnetic resonance imaging shows
    signal changes in bilateral occipital lobes.

94
4 y/o s/p HSCT with seizure
  • Of the following, the MOST likely cause of the
    seizures is
  • Cyclosporine
  • Fluconazole
  • Ganciclovir
  • Prednisone
  • tobramycin

95
  • The mother of a 7-year-old girl who has epilepsy
    phones your office because her child has
    developed a rash. The mother is worried that the
    rash may be due to her new antiseizure
    medication.
  • Of the following, the MOST appropriate next step
    is to
  • defer evaluation until the next health
    supervision visit
  • examine the child in your office promptly
  • refer the child to a dermatologist
  • refer the child to her neurologist
  • send the child to the laboratory to obtain a
    complete blood count

96
  • A 2-year-old girl is brought to the emergency
    department after being found unconscious at her
    grandparent's home. Her mother reports that she
    was in her usual good health when she was dropped
    off at her grandparents 2 hours ago and that
    there is no history of trauma. Of note, the
    grandmother found a spilled, opened bottle of her
    "blood pressure" medicine in the bathroom. On
    physical examination, the girl is somnolent but
    arouses with stimulation. There is no sign of
    trauma on physical examination. Her temperature
    is 98.0F (37.0C), heart rate is 60 beats/min,
    respiratory rate is 25 breaths/min, and oxygen
    saturation is 93 on room air. Her pupils are 2
    mm and reactive bilaterally. Her mouth and mucous
    membranes are dry, and she has no rashes. You
    order serum electrolyte measurement and a urine
    toxicology screen.

97
Somnolent 2 y/o
  • Of the following, the MOST appropriate additional
    tests to obtain are
  • chest radiography and electrocardiography
  • computed tomography (CT) scan of the abdomen and
    electrocardiography
  • CT scan of the head and electrocardiography
  • CT scan of the head and lumbar puncture
  • electrocardiography and skull radiographs

98
  • The parents of a 6-month-old previously well
    infant bring her to your office. She had been
    developing normally, but she stopped interacting
    with her parents over the last 24 hours. For
    several days prior to this development, she had
    had unusual spells during which her head and chin
    dropped to her chest. Now she is having clusters
    of these spells involving head drop and body
    flexion. On physical examination, there is no
    bruising. The infant is afebrile and alert, her
    tone is low, and she does not make persistent eye
    contact or track visually. You refer her to the
    emergency department, where results of a complete
    blood count, electrolyte panel, urinalysis, and a
    noncontrast head computed tomography scan are
    normal.

99
6 m/o with unusual spells
  • Of the following, the test that is MOST likely to
    reveal the correct diagnosis is
  • Electroencephalography
  • Electroretinography
  • lumbar puncture
  • muscle biopsy
  • serum lactate measurement

100
  • A 16-year-old boy presents to the emergency
    department with an acute change in his mental
    status. According to his parents, he was
    previously healthy and has suffered no recent
    trauma. On physical examination, he is somnolent,
    has pinpoint pupils and mild hypotension, and
    demonstrates shallow breathing.

101
Newly somnolent 16 y/o
  • Of the following, the test that is MOST likely to
    help determine the cause of his altered level of
    consciousness is
  • brainstem auditory evoked response
  • chest radiography
  • Electroencephalography
  • serum amino acid measurement
  • urine toxicology screen

102
  • A term newborn is delivered to a mother who has
    had a 5-day history of a nonspecific
    gastroenteritis, some loose stools, generalized
    malaise, and low-grade fever. The infant had a
    seizure at 6 hours of age and is ill, with an
    inspired oxygen requirement of 0.40, some
    petechiae, and oozing from the umbilicus and
    phlebotomy sites. He is irritable on neurologic
    examination.

103
Term 5 d/o who is ill
  • Laboratory findings include
  • White blood cell count, 7.5x103/mcL (7.5x109/L)
  • Platelet count, 90.0x103/mcL (90.0x109/L)
  • Hematocrit, 45 (0.45)
  • Aspartate aminotransferase, 240.0 U/L
  • Alanine aminotransferase, 300.0 U/L
  • Fibrinogen, 90.0 mg/dL (2.6 mcmol/L)
  • Prothrombin time, 20 seconds
  • Partial thromboplastin time, 60 seconds
  • Internationalized Normalized Ratio (INR), 1.80
  • Serum glucose, 90.0 mg/dL (5.0 mmol/L)

104
Term 5 d/o who is ill
  • A lumbar puncture reveals 35 white blood cells,
    with 50 polymorphonuclear cells and 50
    mononuclear cells 1 red blood cell glucose of
    60.0 mg/dL (3.3 mmol/L) and protein of 100 mg/dL
    (1,000 g/L). No organisms are seen on
    cerebrospinal fluid (CSF) Gram stain.

105
Term 5 d/o who is ill
  • Of the following, a TRUE statement about this
    patient's meningitis is that
  • gram-negative organisms are unlikely to be
    causative
  • group B streptococcal meningitis is likely to be
    the cause
  • infection likely is related to maternal
    enteroviral infection
  • the abnormal CSF glucose and protein values
    indicate bacterial meningitis
  • the abnormal liver function test results and CSF
    cell counts indicate herpes simplex virus
    infection

106
  • A 17-year-old boy who receives carbamazepine for
    epilepsy presents to the emergency department
    after a 40-minute generalized tonic-clonic
    seizure. He has been well, and there is no
    history of trauma. On physical examination, he
    answers a few questions, but he is sleepy and
    confused. He is afebrile, and his vital signs are
    normal. Although he is uncooperative, he moves
    all limbs spontaneously with good strength.

107
17 y/o with breakthrough seizure
  • Of the following, the diagnostic test that is
    MOST likely to explain this seizure is
  • magnetic resonance imaging
  • noncontrast head computed tomography scan
  • prolonged electroencephalography
  • serum anticonvulsant measurement
  • serum electrolyte measurement

108
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