CNS

Question . 1. To be effective for preventing myelomeningocele, administration of

folic acid needs to begin:
By 3 months of gestation
At the first missed period
Before conception
Explanation: Folic acid supplementation has been a major
public health success in reducing the incidence of neural tube
defects in the United States. Supplementation also reduces the
recurrence rate of neural tube defects in families with a
previously affected child. (See Chapter 585 in Nelson Textbook
of Pediatrics, 17th ed.)
By 30 days of gestation
At 3 months prior to delivery
Question . 2. A newborn with myelomeningocele is being evaluated, and the
parents ask about the likelihood of the development of hydrocephalus. Which of the
following best describes the risk of hydrocephalus in this situation?
The risk increases with higher spine lesions
Explanation: Hydrocephalus is quite common in children with
a myelomeningocele and must be evaluated with a head
ultrasound study or CT. (See Chapter 585 in Nelson Textbook
of Pediatrics, 17th ed.)
The risk decreases with higher spine lesions
The risk is unknown at birth
The risk is less than 25%
The risk is related to the cause of the malformation

Question . 3. A 4-yr-old boy is evaluated for his first generalized tonic-clonic

seizure, which lasted 10 min. There is no history of illness or fever, and findings on
examination an hour after the seizure are completely normal. The most appropriate
management is:
Begin therapy with carbamazepine
Order an EEG
Explanation: An EEG will help define the presence of a
seizure focus and if there is a specific seizure type. A normal
EEG may help the pediatric neurologist determine if
anticonvulsant therapy is needed. (See Chapter 586 in Nelson
Textbook of Pediatrics, 17th ed.)
Order a CT scan of the brain
Order an MRI study of the brain
Order psychometric testing
Question . 4. A 15-mo-old girl is evaluated for a 10-min-long generalized seizure
associated with a temperature of 40oC. Which of the following factors in the history
is most likely to increase the risk of future seizures?
APGAR score of 3 at 5 min
Family history of epilepsy
Explanation: Future nonfebrile seizures can be predicted
when there is a family history of seizures. Up to 30% of children
with febrile seizures have a recurrence when they become
febrile; these are often unpredictable. (See Chapter 586 in
Nelson Textbook of Pediatrics, 17th ed.)
Clinical evidence of roseola
Female gender
Presence of 2 caf-au-lait spots

Question . 5. It has been determined that sodium valproate is the best

anticonvulsant medication for a 12-yr-old boy with epilepsy. His parents ask about
the possibility of fatal liver necrosis if he takes the drug. Which of the following
factors markedly increases the risk of this complication?
Male gender
Age younger than 2 yr
Explanation: Young age is a risk factor for hepatic and
perhaps pancreatic complications of valproic acid. It is much
safer in 12-yr-old children than those younger than 2 yr.
Down syndrome
Family history of epilepsy
Allergic reactions to phenobarbital
Question . 6. The chances of severe life-threatening skin reactions such as
Stevens-Johnson syndrome are greatest with which of the following anticonvulsant
medications?
Phenobarbital
Gabapentin
Carbamazepine
Lamotrigine
Explanation: In addition, phenobarbital and carbamazepine
are also associated with an acute drug hypersensitivity
syndrome involving skin, mucous membranes, and the liver.
Sodium valproate
Question . 7. A 3-yr-old boy is being evaluated after an episode at home during
which he lost consciousness for 5 min; he was brought to the emergency
department an hour later. On examination, which of the following factors is most
helpful in distinguishing whether this episode was a seizure or syncope?
Family history
Temperature
Blood pressure
Level of consciousness
Explanation: Syncope usually produces a loss of tone and a
fall, with rapid recovery of consciousness once the affected
person is in a horizontal position. Occasionally there may be a
few tonic-clonic jerking movements with syncope, thus
confusing the assessment.

Size of pupils
Question . 8. A 5-yr-old child is referred with a 6-wk history of morning headaches,
often associated with vomiting. His parents have noted that during this period he
has become irritable and moody. Which of the following would be most likely to be
identified during the physical examination?
Marked elevation of blood pressure
Tenderness on percussion of frontal sinuses
Papilledema
Explanation: This child has a brain tumor and increased
intracranial pressure. Papilledema takes time to develop, must
be looked for in all patients with headaches, and is an ominous
sign. (See Chapter 588 in Nelson Textbook of Pediatrics, 17th
ed.)
Loud orbital bruit
Significant refractive error
Question . 9. A 6-yr-old child with neurofibromatosis (NF1) is found to have an
optic glioma on a routine MRI study, confined to the right optic nerve. Findings on
the neurologic, physical, and retinal examinations are normal. The visual acuity is
20/20 bilaterally, uncorrected. Which of the following is the correct management?
Surgical removal of the tumor
MRI of the optic nerve every 3 mo
Chemotherapy
Annual examination by a pediatric ophthalmologist
Explanation: Patients with NF1 and an optic glioma as in this
case can be observed over time. In many situations the tumor
is slow growing and produces few problems. (See Chapter 589
in Nelson Textbook of Pediatrics, 17th ed.)
Radiation therapy limited to the right optic nerve

Question . 10. A 10-yr-old girl is being evaluated for new onset of school problems,
obsessive-compulsive behavior, and occasional uncontrolled movements of the
hands. She has been healthy and has not taken any medications. Further
evaluation is most likely to reveal:
Brain tumor in the posterior fossa
Partial complex epilepsy
Evidence of streptococcal infection
Explanation: Poststreptococcal obsessive-compulsive disorder
is a well-recognized clinical entity.
Hydrocephalus
Tardive dyskinesia
Question . 11. A 5-yr-old girl is evaluated for progressive difficulty in walking, which
seems to worsen during the day; her walking is much better after a good night's
sleep. Examination during the afternoon shows that she has rigidity in the leg
muscles and dystonic twisting of her feet. This kind of movement disorder often
responds dramatically to which of the following medications?
Sodium valproate
Lorazepam
C. L-Dopa
Explanation: L-Dopa is the treatment of choice for hereditary
progressive dystonia with marked diurnal variation, also called
Segawa disease. This is one of the dopa-responsive dystonias.
Gabapentin
Lithium
Question . 12. A 2-yr-old boy with the spastic diplegia form of cerebral palsy is
being evaluated. MRI of his brain is most likely to show:
Multicystic encephalomalacia
Periventricular leukomalacia
Explanation: PVL is a common observation in children with
CP. It is first observed in the neonatal period in both term and
preterm infants. If identified in the neonatal period and
extensive, it is a very strong predictor of CP.
Normal anatomy

Basal ganglia abnormalities

Agenesis of the corpus callosum
Question . 13. A 6-mo-old female infant is evaluated after the mother notes that
she is strongly left-handed and delayed in sitting and in using the right hand. MRI of
the brain reveals a large porencephalic cyst in the distribution of the left middle
cerebral artery. Which of the following information is most likely to contribute to
establishing the cause of this disorder?
Results of an EEG
Chromosome analysis
Evaluation for thrombophilic disorders
Explanation: The MRI study reveals the sequelae of a
neonatal stroke. Although neonatal strokes are not always due
to thrombosis, the child must be evaluated for
hypercoagulability. (See Chapter 591 in Nelson Textbook of
Pediatrics, 17th ed.)
History of delivery
History of maternal drug ingestion
Question . 14. Which of the following therapies has been found to be effective in
long-term follow-up for some patients with the X-linked neurodegenerative disease
adrenoleukodystrophy?
Vegetable oil as a dietary supplement
Hyperbaric oxygen therapy
Bone marrow transplantation
Explanation: Performed prior to the onset of severe
symptoms, bone marrow transplantation has had some value in
improving the clinical course in some patients with X-linked
adrenoleukodystrophy. (See Chapter 592 in Nelson Textbook
of Pediatrics, 17th ed.)
Total volume exchange blood transfusion
Plasmapheresis

Question . 15. A 5-yr-old girl is evaluated for severe mental retardation,

microcephaly, hand-wringing movements, poor growth and weight gain, and
seizures. Genetic analysis indicates a mutation in a transcription factor called
MeCP2 that normally functions to silence transcription of numerous genes. This
finding indicates that she has which of the following disorders?
Juvenile Huntington disease
Metachromatic leukodystrophy
Rett syndrome
Explanation: Rett syndrome is classically described by the
features of this case. (See Chapter 592 in Nelson Textbook of
Pediatrics, 17th ed.)
Adrenoleukodystrophy
Menkes disease
Question . 16. An 11-yr-old boy had been fighting and wrestling with another boy at
school and sustained several bruises on the face and neck. The next morning he
woke up with inability to speak clearly and weakness on the right side of his body,
greater in the arm than in the leg. The most likely cause for his neurologic disorder
is:
Left-sided brain tumor
Focal seizure disorder
Carotid artery intimal dissection
Explanation: Strokes due to carotid dissection can occur
spontaneously or with neck twisting or by flexion-extension of
the neck and have been reported following roller coaster rides
or visits to the beautician. In most cases the lesion heals
without residual neurologic deficits. (See Chapter 593 in Nelson
Textbook of Pediatrics, 17th ed.)
Venous sinus thrombosis
Cervical spinal cord injury

Question . 17. A 5-yr-old child has a 6-mo history of increasing difficulty in walking
associated with urinary incontinence. Physical examination shows an alert child
with increased deep tendon reflexes, clonus and bilateral Babinski reflexes in the
lower extremities, and absent deep tendon reflexes in the upper extremities
associated with grade 3/5 weakness symmetrically in all extremities. The most likely
diagnosis is:
Myasthenia gravis
Spinal cord tumor
Explanation: The findings described in this patient are typical
of an upper motor lesion. The bilateral nature and the other
features localize the lesion to the spinal cord. (See Chapter 579
in Nelson Textbook of Pediatrics, 17th ed.)
Guillain-Barr syndrome
Metachromatic leukodystrophy
Peripheral neuritis
Question . 18. A 7-mo-old girl is presented to the emergency department with
gradual onset of fever, lethargy, and irritability. Her immunizations are up to date.
Examination reveals a febrile infant who does not interact with the examiner and
cries inconsolably. A lumbar puncture is performed, and the cerebrospinal fluid
contains 1,500 white blood cells/mm3, 84% of which are granulocytes; a glucose
concentration of 12 mg/dL; and a protein concentration of 70 mg/dL. Gram stain is
negative. The most likely etiologic agent for this infection is:
An enterovirus
Herpes simplex virus
Haemophilus influenzae type b
Neisseria meningitides
Explanation: Both meningococci and even pneumococci
(vaccine covers only some but not all pneumococci) are the
most common causes of non-neonatal bacterial meningitis. The
H. influenzae type b vaccine has almost eliminated this
pathogen as a cause of serious bacterial infections in children.
(See Chapter 594 in Nelson Textbook of Pediatrics, 17th ed.)
Group B streptococci

Question . 19. The greatest risk of meningitis following documented occult

bacteremia is associated with infection caused by:
Streptococcus pneumoniae
Haemophilus influenzae type b
Neisseria meningitides
Explanation: Meningococci may rarely cause transient
bacteremia, but a positive blood culture for this pathogen is
usually associated with a localized infection in the meninges,
lung, joint, or elsewhere. (See Chapter 362 in Nelson Textbook
of Pediatrics, 17th ed.)
Salmonella species
Question . 20. The most common sequela associated with bacterial meningitis is
Mental retardation
Chronic seizure disorder
Impaired vision
Impaired hearing
Explanation: Hearing deficits are most common and are
potentially attenuated by steroids. In addition, a higher
incidence of neuropsychiatric disturbances is noted following H.
influenzae type b infection. (See Chapter 594 in Nelson
Textbook of Pediatrics, 17th ed.)
Behavioral disturbance
Question . 21. The most common cause of viral meningoencephalitis is:
An enterovirus
Explanation: Enteroviral disease is quite common in the
summer and fall, producing an aseptic meningitis. PCR
techniques have helped to identify this organism. (See Chapter
594 in Nelson Textbook of Pediatrics, 17th ed.)
Herpes simplex virus
An arbovirus
Mumps virus

A respiratory virus

Question . 22. A 4-yr-old girl has experienced progressive loss of ambulation over
a 2-yr period. On examination, the child is apathetic and uninterested in her
surroundings. She has horizontal nystagmus and optic atrophy. Her voice is
dysarthric. She is hypotonic, and her deep tendon reflexes are absent. A sibling
died at the age of 6 yr with a similar history. The motor nerve conduction velocities
show marked slowing, and computed tomography (CT) of the head shows diffuse
symmetric attenuation of the cerebral and cerebellar white matter. The most likely
diagnosis is:
Multiple sclerosis
Metachromatic leukodystrophy
Explanation: Metachromatic leukodystrophy is a familial
degenerative disease affecting both the central nervous system
(CNS) and peripheral nervous system white matter?hence the
loss of deep tendon reflexes with CNS symptoms. (See
Chapter 592 in Nelson Textbook of Pediatrics, 17th ed.)
GM2 gangliosidosis (Tay-Sachs disease)
Neuronal ceroid lipofuscinosis
Acute demyelinating encephalomyelitis
Question . 23. A 3-yr-old girl has a 2-wk history of fever associated with bifrontal
headache, lethargy, and vomiting. She has a history of perioral cyanosis and
dyspnea with exertion beginning in infancy. She suddenly has a 10-min focal tonicclonic seizure. The child is obtunded and has a temperature of 100.8F (38.2C),
pulse of 118/min, and blood pressure of 96/70 mm Hg in her right arm, measured
while she is supine. Perioral cyanosis is noted at rest. A harsh pansystolic murmur
is heard best along the left sternal border. Examination of her eye grounds reveals
bilateral papilledema. She has right-sided weakness associated with hyperreflexia
and an extensor plantar reflex. The most likely cause of the hemiparesis is:
Moyamoya disease
A brain tumor
Neurocysticercosis
Methemoglobinemia
A brain abscess
Explanation: This young girl has tetralogy of Fallot and a brain
abscess resulting in part from the right-to-left cardiac shunt.
Predisposing factors for brain abscesses in other patients
include chronic otic and sinus infections. (See Chapter 595 in
Nelson Textbook of Pediatrics, 17th ed.)

Question . 24. Causes of megalocephaly include all of the following except:

Thalassemia
Chronic subdural effusions
Hydrocephalus
Canavan disease
Congenital CMV infection
Explanation: Congenital cytomegalovirus (CMV) infection
usually causes microcephalus, not macrocephalus. Expansion
of the bone marrow (hemolytic anemias), storage diseases
(lysosomal, leukodystrophies), excessive cerebrospinal fluid
(CSF) (hydrocephalus), intracranial bleeding (subdural), and
familial factors contribute to megalocephaly. (See Chapter 585
in Nelson Textbook of Pediatrics, 17th ed.)
Familial factors
Question . 25. A 12-yr-old girl experiences acute monocular blindness of 2 days'
duration. Past medical history reveals that she has had headaches for the past 3 yr
that she cannot characterize, one brief episode of diplopia, and one episode of
paresthesias of the feet. These episodes were not related in time, did not occur in
immediate proximity to the headache, and resolved spontaneously. Findings on
physical examination, including the funduscopic examination, are unremarkable
other than reduced visual acuity. The most important diagnostic step is to perform:
CT
MRI
Explanation: MRI is most useful in confirming the diagnosis of
a possible demyelinating disease such as multiple sclerosis.
MRI demonstrates small 3- to 4-mm plaques compatible with
the disease. The MRI study should include the brain and spinal
cord. (See Chapter 592 in Nelson Textbook of Pediatrics, 17th
ed.)
An electroencephalogram
Peripheral nerve conduction tests
A nerve biopsy

Question . 26. A 12-year-old presents with a history of severe headache, a grand

mal seizure, and sudden collapse with unresponsive flaccid coma. The patient had
a history of intermittent right-sided headaches without an aura and at times without
relief with rest. In addition to coma on physical examination, the patient is afebrile
and has nuchal rigidity. The most likely diagnosis is:
Bacterial meningitis
Tuberculous meningitis
Brain tumor
Arteriovenous malformation
Explanation: Arteriovenous malformation, like an aneurysm,
may rupture, producing hemiplegia or coma. Blood in the
subarachnoid space produces nuchal rigidity and may be
detected by CT or a carefully performed lumbar puncture. (See
Chapter 584 in Nelson Textbook of Pediatrics, 17th ed.)
Pott puffy tumor
Question . 27. Papilledema of acute onset is associated with all of the following
except:
Reduced visual activity
Explanation: Acutely, the visual acuity is usually not reduced
in papilledema. This is in contrast with papillitis noted during
optic neuritis; affected patients have poor visual acuity.
Hyperemia of the optic nerve
Constricted arterioles of the optic disc
Dilated disc veins
Indistinct optic nerve border
Question . 28. The Gowers sign demonstrates:
Poor reflexes
Spinal dysraphism
Tethered cord
Proximal motor weakness

Explanation: Motor weakness, especially of the thighs,

requires the child to stand from a supine position by using the
arms to "climb" up the legs to stand erect.
Hysterical paralysis
Question . 29. Characteristics of simple partial seizures include all of the following
except:
Loss of consciousness
Explanation: There is never a loss of consciousness in simple
partial seizures. Actually, some patients may talk to those
around them during the event. (See Chapter 586 in Nelson
Textbook of Pediatrics, 17th ed.)
Versive seizures
Duration of 10-20 sec
No postictal state
Abnormal EEG findings
Question . 30. Rasmussen encephalitis is characterized by all of the following
except:
Epilepsia partialis continue
Onset before age 10 yr
No sequelae
Explanation: Sequelae may include hemiplegia, hemianopia,
or aphasia. (See Chapter 586 in Nelson Textbook of Pediatrics,
17th ed.)
Abnormal EEG findings
Possible role of CMV infection
Question . 31. Landau-Kleffner syndrome is characterized by all of the following
except:
Onset at age 5 yr
More common in girls
Explanation: The disorder is more common in boys. The
etiology is unknown, and the treatment of choice is valproic
acid. (See Chapter 586 in Nelson Textbook of Pediatrics, 17th
ed.)
Aphasia

Normal hearing
Multiple seizure types
Question . 32. Neurofibromatosis type 1, an autosomal dominant disorder (gene on
chromosome 17), is defined by six or more caf-au-lait macules greater than 5 mm
in diameter in prepubertal children or greater than 15 mm in postpubertal children
plus at least one of the following except:
Axillary or inguinal freckling
Lisch nodules of the iris
Two or more neurofibromas or one plexiform neurofibroma
Ash leaf macule
Explanation: The ash leaf-shaped hypopigmented macule is
most typical of tuberous sclerosis (being present in over 90% of
affected children), another autosomal dominant disorder. (See
Chapter 589 in Nelson Textbook of Pediatrics, 17th ed.)
Osseous lesions (sphenoid dysplasia, scoliosis)
Optic gliomas
An affected first-degree relative
Question . 33. A 19-yr-old girl presents with headache, unsteadiness, and poor
hearing that has worsened over the past 5 yr. Her father's medical history includes
some type of brain surgery, and he has been deaf since the age of 35 yr. The most
likely diagnosis is:
Neurofibromatosis type 2
Explanation: NF2 accounts for 10% of all NF cases, has
distinctive chromosomal sites, and is characterized by bilateral
acoustic neuromas. Caf-au-lait macules may not be present.
(See Chapter 589 in Nelson Textbook of Pediatrics, 17th ed.)
Optic glioma
Neurofibromatosis type 1
Tuberous sclerosis
Late-onset congenital deafness

Question . 34. Physical features of Sydenham chorea include all of the following
except:
Hypertonia
Explanation: Hypotonia is the rule. (See Chapter 590 in
Nelson Textbook of Pediatrics, 17th ed.)
Milkmaid's grip
Choreic hand
Darting tongue
Emotional lability