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Pediatric Questions > NEUROLOGY > Flashcards

NEUROLOGY Flashcards

1
Q

An 18-month-old turns cyanotic associated with a brief loss of consciousness that occurred during a temper tantrum

A

Reassurance (cyanotic breath-holding spell)

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2
Q

An 18-month-old turns pale with limp jerking and brief loss of consciousness that occurred after the child was scared by his sibling

A

Reassurance (pallid breath-holding spell)

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3
Q

A 4-month-old infant is having episodes of tonic
neck extension and dystonic posturing of trunk
associated only with feedings. Has a normal
neurologic exam

A

Sandifer syndrome

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4
Q

A premature infant has brief jerking of the right
upper extremity that cannot be suppressed

A

Neonatal seizure

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5
Q

A previously healthy 16-month-old boy has 60 s
generalized seizure in the setting of febrile illness (not involving the CNS) and is now acting normal

A

Simple febrile seizure

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6
Q

A previously healthy 16-month-old boy has had 2 febrile seizures in the last 24 h, and the infant is now acting normal

A

Complex febrile seizures

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7
Q

What is the recurrence risk after the first simple
febrile seizure?

A

Approximately 30%

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8
Q

What is the risk of developing epilepsy in children with simple febrile seizures?

A

Approximately 2%

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9
Q

An 8-year-old boy is having multiple brief daily
episodes of behavioral arrest and eye fluttering
with an EEG showing 3 Hz/s spike-and-wave
discharges

A

Absence seizure (petit mal seizure)

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10
Q

The first-line treatment for absence seizures

A

Ethosuximide

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11
Q

Antiseizure medication that should be avoided in women of child-bearing age due to teratogenicity

A

Valproic acid

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12
Q

A 6-month-old infant having episodes of tonic
flexion of trunk, head, and extremities, occurring in clusters

A

Infantile spasms

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13
Q

Triad of infantile spasms, hypsarrhythmia on EEG, developmental regression

A

West syndrome

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14
Q

A 3-year-old boy with a prior history of infantile
spasms who now has intellectual disability,
multiple seizure types, EEG showing slow spikewave activity

A

Lennox–Gastaut syndrome

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15
Q

An 16-year-old girl who is an excellent student has a generalized tonic-clonic seizure after a sleepover party with her friends. She also reports having jerking movements of her arms in the mornings

A

Juvenile myoclonic epilepsy

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16
Q

A 5-year-old with nighttime seizures involving the face and focal centrotemporal spikes in sleep

A

Rolandic epilepsy with centrotemporal spikes

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17
Q

A 3-year-old with language regression and
continuous spike-wave discharges in slow wave
sleep

A

Landau–Kleffner syndrome

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18
Q

A 9-year-old previously healthy girl with
intractable focal seizures as well as hemiparesis
and cognitive decline. MRI of the brain shows
atrophy of one hemisphere

A

Rasmussen encephalitis O. I. Naga

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19
Q

An infant with rapid head growth, full fontanel,
irritability, vomiting

A

Hydrocephalus

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20
Q

The most common cause of macrocephaly

A

Benign familial macrocephaly

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21
Q

An infant with failure to thrive, developmental
delay, intractable seizures with an MRI showing a “smooth brain”

A

Lissencephaly

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22
Q

Elevated maternal alpha-fetoprotein, an infant born with a large cranial defect, abnormalities of the face and eyes, without a cortex but an intact brainstem

A

Anencephaly

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23
Q

Global intellectual disability, brain MRI showing
bilateral clefts within the cerebral hemisphere

A

Schizencephaly

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24
Q

An infant with a sacral tuft of hair and normal
neurologic exam

A

Spina bifida occulta

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25
Q

MRI showing downward displacement of the
cerebellar tonsils through the foramen magnum

A

Arnold–Chiari malformation

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26
Q

Cystic expansion of the fourth ventricle in the
posterior fossa, associated with hydrocephalus,
cerebellar ataxia, and associated with corpus
callosum agenesis

A

Dandy–Walker syndrome

27
Q

Newborn with a skull defect, a sac-like protrusion containing brain material

A

Encephalocele

28
Q

Infant born with a short neck, very low hairline in the back of the head, and limited range of motion in the neck

A

Klippel–Feil syndrome

29
Q

Child with a stroke-like event has MRI with the
appearance of a “puff of smoke”

A

Moyamoya disease

30
Q

Adolescent girl presents with ptosis and double
vision and is also complaining that she feels
weaker by the end of the day

A

Juvenile myasthenia gravis

31
Q

Preschool age boy has a history of toe walking,
frequent falls, and enlarged calves. O/E: he has
significant proximal muscle weakness, positive
Gower sign, and laboratory evaluation shows
elevated CPK (creatine phosphokinase)

A

Duchenne muscular dystrophy

32
Q

A 10-year-old boy presents frequent falls, and
weakness for 3 months. O/E: he has mild proximal muscle weakness, and calf pseudohypertrophy and his echocardiogram shows cardiomyopathy, laboratory shows elevated serum CPK

A

Becker muscular dystrophy—older onset and
milder form

33
Q

Group of disorders characterized by decreased
deep tendon reflexes, decreased proprioception, and a vibratory sense that is caused by defective peripheral nerve demyelination

A

Charcot–Marie–Tooth syndrome

33
Q

Newborn infant presents with hypotonia,
respiratory distress, and facial muscle weakness. The genetic test is positive for CTG trinucleotide repeat

A

Congenital myotonic dystrophy

34
Q

A 12-year-old girl previously healthy over few
months presents with progressive clumsiness, gait and limb ataxia, recurrent falls, rapid, jerky
movements of both eyes, areflexia, lower extremity weakness, dysarthria, and dysphagia. Positive frataxin gene sequencing, associated with cardiomyopathy and increased risk of diabetes mellitus

A

Friedreich ataxia—GAA trinucleotide repeat
(autosomal recessive and presents in older
children)

35
Q

A 3-year-old girl presents with recurrent
respiratory infections, clumsiness, gait and limb
ataxia, recurrent falls, oculomotor apraxia,
choreoathetosis, dysarthria, and ocular
telangiectasias, and serum α-fetoprotein is elevated

A

Ataxia–telangiectasia (autosomal recessive and
presents in young children)—mutation of the
ATM gene

36
Q

Adolescent with a history of herpes zoster presents with peripheral nerve paralysis, facial pain, and deafness

A

Ramsay Hunt syndrome

37
Q

History of diarrhea followed by progressing
ascending weakness and loss of deep tendon
reflexes with CSF showing elevated protein

A

Guillain–Barré syndrome

38
Q

A 4-month-old infant with severe hypotonia and
feeding difficulty. O/E: the infant is in frog-leg
position and has tongue fasciculations

A

Spinal muscular atrophy

39
Q

Progressive weakness in legs with focal back pain, bowel and bladder dysfunction and sensory level on the exam. Eventually develops into spastic diplegia

A

Transverse myelitis

40
Q

A 6-month-old consistently reaches for toys with
the right hand. What is the most likely underlying cause?

A

Central or peripheral neurologic abnormality of
the opposite side, including hemiparesis

41
Q

An 18-month-old boy with a history of
prematurity, including bilateral intraventricular
hemorrhages, who is brought in for evaluation
because he is not walking and has increased tone in his legs. Scissoring of the legs is noted when he is held in a vertical position

A

Spastic diplegic cerebral palsy

42
Q

A school-age child develops abnormal limb
movements a few weeks after a group A betahemolytic strep infection

A

Sydenham chorea

43
Q

Child with dyskinetic cerebral palsy and a history of elevated bilirubin

A

Kernicterus

44
Q

An 18-month-old girl with acquired microcephaly, language regression, repetitive hand-wringing movements, loss of purposeful hand use. Genetic testing reveals a mutation in the MECP2 gene

A

Rett syndrome

45
Q

Adolescent girl is complaining of right frontal
pulsating headache with photophobia and nausea. She reports that during the headache episodes, she prefers to be in a dark, quiet room. Her father and paternal grandmother also get headaches

A

Migraine headache

46
Q

Adolescent complaining of a mild headache
described as “band-like” around the head.
Headache is responsive to over-the-counter
analgesics

A

Tension headache

47
Q

Adolescent girl with a BMI more than 98th %
presenting with headaches, nausea, vomiting,
double vision, papilledema, and inability to abduct her left eye. What is the most likely underlying cause?

A

Idiopathic intracranial hypertension
(pseudotumor cerebri)

48
Q

A 1-year-old girl presents with an increasing
number of café-au-lait spots all over her body. She has more than 6 macules, each macule measuring more than 5 mm. The rest of the exam is normal. No family history of neurofibromatosis. What is the next best step?

A

Referral to a pediatric ophthalmologist to check
for Lisch nodules annually

49
Q

The child in the previous example is positive for
Lisch nodules. What are the recommendations?

A

Genetic consultation, annual pediatric
ophthalmology evaluation, regular
developmental assessment, and regular blood
pressure monitoring

50
Q

Multiple café-au-lait spots, Lisch nodules on
ophthalmology exam, and presence of multiple
neurofibromas

A

Neurofibromatosis type 1
Autosomal dominant

51
Q

Presents with ringing in the ears; imaging shows
bilateral vestibular schwannomas

A

Neurofibromatosis type 2
Autosomal dominan

52
Q

A 10-month-old infant presents with infantile
spasms and is noted to have multiple
hypomelanotic macules (ash leaf spots). MRI brain shows cortical tubers

A

Tuberous sclerosis complex

53
Q

History of port-wine stain, seizures, and glaucoma

A

Sturge–Weber syndrome

54
Q

Child presenting with chronic back pain, lower
extremity weakness, leg length discrepancy, foot deformities, scoliosis, neurogenic bladder, and recurrent urinary tract infections. There is no acral hair or skin abnormalities in the lower back

A

Tethered cord syndrome

55
Q

The child with a ventriculoperitoneal (V/P) shunt
with a triad of high fever, focal neurologic deficits, and headache

A

Shunt infection—possible brain abscess

56
Q

Toddler refusing to walk or stand, with back
tenderness and elevated ESR

A

Diskitis

57
Q

What is the first-line therapy for tic disorder with no other complications?

A

Behavioral modification

58
Q

Child with recurrent throat clearing, facial grimacing, and grunting, but otherwise acting
normal

A

Tic disorder (Tourette syndrome if symptoms
> 12 months)

59
Q

An infant with nystagmus, titubation, and
torticollis

A

Spasmus nutans

60
Q

A teenager with excessive daytime sleepiness,
sudden episodes of loss of muscle tone and
hallucinations when going to sleep

A

Narcolepsy

61
Q

A 9-year-old has a sudden onset of severe
abdominal pain, becomes pale, feels dizzy, and her vision slowly goes black followed by loss of
consciousness for a few seconds and quick return to baseline

A

Vasovagal syncope

62
Q

The reflex that appears around 8–9 months in
preparation for a child to stand and walk

A

Parachute reflex

Pediatric Questions (31 decks)

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