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A. Apert syndrome
B. Carpenter syndrome
C. Chotzen syndrome
D. Pfeiffer syndrome
E. Crouzon syndrome
29. Which of the following is a factor increasing the risk for deformational
plagiocephaly?
A. Female
B. Postmaturity
C. Sleep position is prone at birth and at 6 wk
D. Breast feeding
E. Tummy time < 3 times/day
30. What is the BEST treatment for a 6-month-old infant with severe
deformational plagiocephaly?
A. Watch-and-wait management
B. Repositioning and physiotherapy
C. Increasing tummy time
D. Helmet therapy
E. Surgery
31. Which of the following is a major risk factor for recurrence of febrile
seizures?
A. Family history of febrile seizures
B. Family history of epilepsy
C. Complex febrile seizure
D. Male gender
E. Age < 1 yr
32. Which of the following risk factors has the highest incidence for occurrence
of subsequent epilepsy after a febrile seizure?
A. Recurrent febrile seizures
B. Fever < 1 hr before febrile seizure
C. Family history of epilepsy
D. Complex febrile seizures (focal)
E. Neurodevelopmental abnormalities
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33. A 6-month-old infant develops high fever followed by prolonged focal
seizure for more than 30 minutes after vaccination, subsequently the seizures
start to occur with lower grade of fever and then without fever. During the
second year of his life, atypical absences occur frequently with developmental
delay.
Of the following, the MOST likely diagnosis is
A. Generalized epilepsy with febrile seizures plus (GEFS+),
B. Dravet syndrome
C. Temporal lobe epilepsy secondary to mesial temporal sclerosis
D. Focal complex febrile seizures
E. Febrile infection–related (or refractory ) epilepsy (FIRES)
35. A 10-month-old boy presented with febrile seizure which lasted about 10
minutes with no clinical signs of meningeal irritation, his immunization status
was unknown.
A medical student asks you about lumbar puncture LP for this baby; your
answer should be
A. No need for LP at time being
B. LP should be done because his immunization status was unknown
C. LP should be done after brain ultrasound
D. LP should be done if he developed another seizure within 24 hour
E. LP should be done because he is a male gender
36. An 18-month-old boy is presenting with the first simple febrile seizure and is
otherwise neurologically healthy; his father read in the net that EEG has a
significant role in the management of his son.
Your proper answer should be
A. EEG should not be performed as part of the evaluation
B. EEG would predict the future recurrence of febrile seizures
C. EEG would predict the future recurrence of epilepsy
D. EEG should be done after 2 wk of a febrile seizure
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E. EEG should be done after second attack of febrile seizure
39. A 5-year-old child wakes up at night due to a focal seizure with throat
tingling and clonic contractions of one side of the face, with drooling and
inability to speak but with preserved consciousness and comprehension. EEG
shows typical wide-based spikes that are markedly increased in frequency
during drowsiness and sleep with normal MRI.
Of the following, the MOST likely diagnosis is
A. atypical benign childhood epilepsy with centrotemporal spikes
B. benign childhood epilepsy with centrotemporal spikes
C. benign epilepsy with occipital spikes
D. nocturnal autosomal dominant frontal lobe epilepsy
E. familial benign epilepsy syndromes
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41. A 7-month-old infant presents with epileptic spasms (that usually occur in
clusters), developmental regression, and a high voltage, slow, chaotic
background with multifocal spikes EEG.
Of the following, the MOST likely diagnosis is
A. Ohtahara syndrome
B. early myoclonic infantile encephalopathy
C. Dravet syndrome
D. West syndrome
E. Lennox-Gastaut syndrome
42. A 5-year-old boy presents with developmental delay, tonic seizures that
occur in sleep with EEG findings of 1- to 2-Hz spike and slow waves, polyspike
bursts in sleep, and a slow background in wakefulness. The seizures are
intractable despite multiple therapies.
Of the following, the MOST likely diagnosis is
A. Ohtahara syndrome
B. Doose syndrome
C. Dravet syndrome
D. West syndrome
E. Lennox-Gastaut syndrome
44. Which of the following drugs is the first choice for focal seizures?
A. Oxcarbazepine
B. Ethosuximide
C. Valproate
D. Lamotrigine
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E. Topiramate
45. Which of the following drugs is MOST often used as initial treatment of
absence seizures?
A. Valproate
B. Lamotrigine
C. Ethosuximide
D. Acetazolamide
E. Clonazepam
47. Which of the following antiepileptic drugs may cause irreversible visual field
deficit?
A. Benzodiazepines
B. Carbamazepine
C. Vigabatrin
D. Levetiracetam
E. Phenobarbital
48. Which of the following antiepileptic drugs may cause aplastic anemia?
A. Benzodiazepines
B. Carbamazepine
C. Vigabatrin
D. Levetiracetam
E. Phenobarbital
49. Hepatic and pancreatic toxicity are serious side effects in which of the
following antiepileptic drugs?
A. Benzodiazepines
B. Valproic acid
C. Vigabatrin
D. Levetiracetam
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E. Gabapentin
50. Which of the following antiepileptic drugs is used in an epileptic patient with
migraine?
A. Benzodiazepines
B. Valproic acid
C. Vigabatrin
D. Levetiracetam
E. Gabapentin
53. Which of the following is the safest antiepileptic drug to use during
pregnancy?
A. Levetiracetam
B. Valproic acid
C. Phenytoin
D. Ethosuximide
E. Gabapentin
54. Which of the following antiepileptic drugs requires frequent (even weekly)
monitoring of liver function and blood counts throughout the therapy?
A. Levetiracetam
B. Valproic acid
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C. Felbamate
D. Ethosuximide
E. Gabapentin
58. Antiepileptic drug AED therapy should be discontinued gradually; often over
a period of
A. 1-2 months
B. 2-4 months
C. 3-6 months
D. 4-8 months
E. 5-10 months
59. Which of the following neonatal seizures is frequently not associated with
electrographic discharges?
A. Spasms
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B. Focal clonic
C. Focal tonic
D. Generalized myoclonic
E. Generalized tonic
65. What is the first-line agent recommended by the American Epilepsy Society
Guidelines to control status epilepticus?
A. Phenobarbital
B. Lorazepam
C. Phenytoin
D. Valproate
E. Levetiracetam
67. How you treat a 1-year-old boy presenting with recurrent severe cyanotic
breath-holding spells?
A. Education and reassurance of the parents
B. Atropine sulfate
C. Anti-seizure drug therapy
D. Cardiac pacemaker
E. Basic cardiopulmonary resuscitation
68. A 9-year-old boy presents with pallor and sweating followed by a gradual
collapse with loss of consciousness triggered by standing for a long time without
movement when taking a hot shower.
What is the MOST likely cause of this event?
A. Complex partial seizure
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B. Vasovagal syncope
C. Temporal lobe epilepsy
D. Exercise-induced anaphylaxis
E. Long QT syndrome
71. A 4-month-old boy infant presents with repeated head drops, hundreds
times per day, without concurrent EEG epileptic activity. His parents asked
about prognosis, your answer is
A. It persists for life
B. It subsides when child walks without support
C. It subsides by 1st year of life
D. It may lead to developmental delay
E. It may convert to epileptic myoclonus
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72. A 2- year-old girl brought by her mother complaining from rhythmic rocking
movements in lying and sometimes sitting position often associated with
perspiration, irregular breathing, and grunting, but no loss of consciousness.
Which of the following is the MOST likely cause of this event?
A. Tic
B. Mannerism
C. Anxiety attack
D. Infantile gratification
E. Hyperventilation spell
80. Definite tuberous sclerosis complex TSC is diagnosed when at least two
major or one major plus two minor features are present.
Which of the following is a minor feature?
A. Subependymal nodules
B. Facial angiofibromas (≥3)
C. Ungual fibromas (≥2)
D. Retinal achromic patch
E. Shagreen patch
82. What is the first-line therapy for infantile spasms in Tuberous Sclerosis
Complex TSC?
A. Adrenocorticotropic hormone (ACTH)
B. Vigabatrin
C. Epilepsy surgery
D. Valproate
E. Everolimus
83. A young mother brought her 6-month-old boy with infantile spasm
secondary to tuberous sclerosis complex TSC with good response to vigabatrin,
his cardiac echo revealed cardiac rhabdomyoma. The mother counsels you
about the fate of this cardiac lesion.
Your best answer should be
A. It usually ends with congestive heart failure
B. It tends to slowly resolve spontaneously
C. It usually ends with arrhythmias
D. If started in fetal life, it is fatal in infancy
E. It usually needs surgical intervention
84. What is the imaging modality of choice for demonstrating the extension of
pial capillary malformation in Sturge-Weber syndrome (SWS)?
A. Brain MRI
B. Brain MRI with contrast
C. Head CT
D. Head CT with contrast
E. Brain US
85. What is the MOST common cause of death in von Hippel–Lindau disease?
A. Pheochromocytoma
B. Renal carcinoma
C. Cerebellar hemangioblastoma
D. Hemangioblastoma of the spinal cord
E. Retinal angiomas
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86. A 9-month-old infant presents with developmental delay, hypotonia,
abnormal eye movements, abnormal respirations, and a distinctive facial
feature. Axial MRI brain demonstrates “molar tooth sign”.
Of the following, the MOST likely diagnosis is
A. agenesis of vermis of the cerebellum
B. Chiari malformation
C. Dandy-Walker syndrome
D. Leigh disease
E. Joubert syndrome
89. A 12-year-old boy presents with slowly progressive ataxia which affect the
lower extremities more than the upper extremities. Examination demonstrates
a positive Romberg test, absent ankle deep tendon reflexes; and extensor
plantar response. The boy has explosive dysarthric speech, nystagmus, with
preserved intelligence. He also has high-arched feet and hammer toes.
Of the following, the MOST likely diagnosis is
A. Roussy-Levy disease
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B. Ramsay Hunt syndrome
C. Friedreich ataxia
D. ataxia-telangiectasia
E. Refsum disease
90. A 9- year-old girl awakened yesterday morning with clumsiness, dropped the
tea cup from her hand on breakfast then she started to develop generalized
abnormal body movement involving the face with crying and unintelligible
speech. When the patient asked to hold the upper limbs overhead, arms and
palms turn outward. Examination of heart is normal.
Of the following, the MOST likely diagnosis is
A. benign hereditary chorea
B. Sydenham chorea
C. chorea secondary to systemic lupus erythematosus
D. chorea secondary to antiphospholipid antibody syndrome
E. paraneoplastic chorea
92. A 5-week-old infant presents with repetitive focal myoclonic jerks occurring
during sleep, more prominent in the upper extremities continued for few
seconds, these jerks began during the 1st week of life, waking the baby causes
the movements to abruptly cease. Neurologic examination is normal.
Of the following, the MOST likely cause of these movements is
A. Opsoclonus myoclonus (ataxia) syndrome
B. Benign neonatal sleep myoclonus
C. Benign myoclonus of early infancy
D. Hypoglycemia
E. Myoclonic epilepsy with ragged red fibers (MERRF)
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97. What is the MOST effective treatment for control of seizures in Rasmussen
encephalitis?
A. High-dose steroids
B. Plasma exchange
C. IVIG
D. Tacrolimus
E. Functional hemispherectomy
98. A 15-month-old child presents with progressive loss of motor and language
milestones beginning at 6 months of age with seizures, macrocephaly, and doll-
like face. Abdominal examination reveals splenomegaly while fundoscopy shows
cherry-red spots, and the auditory brainstem responses shows prolonged
latencies.
Of the following, the MOST likely diagnosis is
A. Sandhoff disease
B. Juvenile GM2 gangliosidosis
C. Tay-Sachs disease TSD
D. Krabbe disease
E. Metachromatic leukodystrophy
99. A 10-month-old boy infant presents with excessive irritability and crying,
unexplained episodes of hyperpyrexia, vomiting, and difficulty feeding,
diagnosed by GP doctor as colic with frequent milk formula change with no
improvement, now he develops generalized seizures , alterations in body tone
with rigidity and opisthotonos and visual inattentiveness as a result of optic
atrophy.
Of the following, the MOST likely diagnosis is
A. Sandhoff disease
B. juvenile GM2 gangliosidosis
C. Tay-Sachs disease
D. Krabbe disease
E. Metachromatic leukodystrophy
100. A 2-year-old boy has insidious onset of gait disturbance, frequent falls,
with feeding and swallowing impairment. The extremities are hypotonic, and
the deep-tendon reflexes are absent. The parents noticed deterioration in
intellectual function and dysarthric slurred speech. Examination of the retina
shows optic atrophy.
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Neurophysiologic evaluation shows slowing of peripheral nerve conduction
velocities. CT and MRI images of the brain indicate diffuse symmetric
attenuation of the cerebellar and cerebral white matter.
Of the following, the MOST likely diagnosis is
A. Sandhoff disease
B. Juvenile GM2 gangliosidosis
C. Tay-Sachs disease
D. Krabbe disease
E. Late infantile metachromatic leukodystrophy
104. A 9-year-old boy presents with periocular pain and headache after a non-
specific viral illness, few days later, he develops bilateral visual loss.
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What is the MOST likely cause of this visual loss?
A. Sarcoidosis
B. Behçet disease
C. Lyme disease
D. Leber hereditary optic neuropathy
E. Optic neuritis
106. Which of the following imaging studies can demonstrate arterial ischemic
stroke (AIS) few minutes following the onset?
A. Brain CT
B. Cerebral MRI
C. Diffusion-weighted MRI
D. MR angiography
E. Ultrasound brain
107. Which of the following may cause hand dominance within the first year of
life?
A. Perinatal stroke
B. Megaloblastic anemia
C. Sickle cell anemia
D. Moyamoya disease
E. Focal cerebral arteriopathy
109. Which of the following CNS infections may have the highest CSF protein
level?
A. Tuberculous meningitis
B. Viral meningitis
C. Acute bacterial meningitis
D. Partially treated bacterial meningitis
E. Fungal meningitis
110. Which of the following viral diseases may cause low glucose in CSF?
A. Herpes simplex virus
B. Arboviruses
C. Mumps
D. Adenoviruses
E. Rabies
113. What are the predominant organisms that cause brain abscesses in
children?
A. Staphylococcus aureus
B. Haemophilus spp.
C. Escherichia coli
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D. Klebsiella pneumoniae,
E. Streptococcus anginosus
114. Which of the following tests can differentiate between bacterial and viral
meningitis?
A. C-reactive protein
B. Erythrocyte sedimentation rate
C. Procalcitonin
D. Blood culture
E. WBC count
117. What is the MOST common neurologic sequelae from bacterial meningitis?
A. Hearing loss
B. Cognitive impairment
C. Recurrent seizures
D. Delay in acquisition of language
E. Visual impairment
120. Which of the following viral encephalitis may have normal CSF glucose,
protein, and cell counts?
A. Herpes simplex virus
B. Parecho viruses
C. Epstein-Barr virus
D. Cytomegalo virus
E. Mumps
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