PEDIATRIC
NEUROLOGY
Harvey Bennett, M.D.
GORYEB CHILDREN’S HOSPITAL
Anita L. Belman, M.D.
SUNY STONY BROOK MEDICAL
CENTER
ANSWERS ARE IN BOLD &/or underlined

Mrs. Smith brings her 5 yr old son Billy to see you
because on two separate occasions (2 mo interval)
Billy had a severe pounding headache associated with
photophobia & vomiting. The headache was finally
relieved by sleep.
All of the following are appropriate questions to ask EXCEPT
1) Are there any family members with a hx of intermittent
headache ?
2) Were there any particular foods that seem to act as
triggers ?
3) Were there any visual symptoms ?
4) Were there any recent psychic stressors ?
5) Was there sensitivity to loud noises at the time of the
headache ?
(continued) Mrs. Smith brings her 5 y o son Billy to see you
because on two separate occasions (2 mo interval) Billy had a
severe pounding headache associated with photophobia &
vomiting. Headache was relieved by sleep.
The best work-up for this patient includes:
1) Lumbar Puncture
2) Computed Tomography
3) Electrolytes and CBC
4) MRI scans
5) None of the above
The preferred treatment for the above patient is:
1)Depakote
2)Topamax
3) Imitrex
4) Ibuprophen
5) None of the above
Mrs. Stone brings her 10 mo old child in for a well baby check up. She
tells you on two separate occasions Jonnie had started to cry (because
of frustration or discomfort) and “passed out”. Although she was
worried the 1st time, her neighbor reassured her not to worry. Now that
it has happened a 2nd time she is concerned and asks you for advice on
this condition. The baby’s examination is normal.
You should:
1) Get Neurology Consult
2) Get an EEG
3) Get an MRI scan
4) Electrolytes and Liver Function Tests
5) None of the above
All of the following are considered reasonable treatment EXCEPT:
1) Reassurance
2) Leave child in lateral recumbent position
3) Ferrous Sulfate
4) Phenobarbital
5) Altering discipline for child’s frustration or anger
Which of the following is a FALSE statement about
Neurofibromatosis type I
It is an autosomal dominant disorder
2. At least three café au lait spots are required
for diagnosis
3. It can be a multi-system disorder with skin,
bone, visceral & nervous system involvement
4. Lisch nodules are pigmented hamartomatous
lesions of the iris that are not present at birth
but become more frequent after puberty
1.
All of the statements below about Tuberous
Sclerosis are true EXCEPT:
A very common cause of Infantile Spasms
Autosomal dominant transmission
Associated with adenoma sebaceum and
hypopigmented lesions
4. The presence of seizures early in life is not a
predictor of Mental Retardation
5. The earliest seen lesions are usually hypopigmented macules (ash leaf spots) which can
be seen in the newborn period
1.
2.
3.
In Sturge Weber Syndrome (Encephalofacial angiomatosis)
all of the following are true EXCEPT:
The facial lesion is usually unilateral
The underlying cortex is often affected from a
leptomeningeal angioma
3. Seizures & hemiparesis are common
manifestations
4. Glaucoma is often a concomitant problem
5. The CNS findings are always present at birth
1.
2.
Mrs. Williams brings her two sons, Evan, age 8y, & Robert, age 5y, for
their first visit to your office. Mrs. Williams states, Evan has been to
different pediatricians in the past. He has also seen numerous
specialists over the years because of his “problems” ( habits ).
An Ophthalmologist because of eye blinking.
An ENT specialist because of throat clearing.
An Allergist because of nasal sniffing.
An Orthopedist because of neck stretching.
Now they are seeing a behavioral psychologist to deal with unusual
behaviors such as lining things up, evening things out etc.
Robert, just a few months ago, had some eye blinking but it totally
resolved in two months.
By definition Evan has:
1) Tourette Syndrome
2) Transient tics of Childhood
3) Chronic Motor Tic Disorder
4) Sydenham’s Chorea
5) None of the above
By definition Evan has:
1) Tourette’s Syndrome
2) Transient tics disorder of Childhood
3) Chronic Motor Tic Disorder
4) Sydenham’s Chorea
5) None of the above
By definition Robert has:
1) Tourette Syndrome
2) Transient Tics of Childhood
3) Chronic Motor Tic Disorder
4) Sydenham’s Chorea
5) None of the above
The two most common associated behavioral S & S of
Tourette syndrome are:
1) ADD and OCD
2) OCD and Rage attacks
3) ADD and sleep disturbances
4) ADD and Learning disabilities

Joey is seen in your office because of a 2 day hx of
difficulty walking. A few days prior to the onset of
gait problems he had some vague muscle pains.
About 2-3 weeks before he had a URI. On your
examination you find he has a wide-based gait and
areflexia. He no longer has muscle pain.
The best diagnostic procedure would be:
1) CSF examination
2) Electromyography and nerve conduction times
3) MRI scan of Brain
4) CT or MRI of Spine
The most likely diagnosis is:
1) Acute inflammatory Myositis
2)Guillan Barre Syndrome
3) Cord compression syndrome
4) Acute Cerebellar Ataxia
Mrs. Williams brings her 5 y o daughter, Dawn, to
your office because for the past day Dawn has had
problems walking. At first she seemed clumsy and had
difficulty with balance, but by this morning she was
unable to stand. Mrs. Williams also says Dawn is having
problems reaching for objects. Dawn has been healthy
except for a recent URI.
The diagnostic procedure that needs to be done:
1) MRI of the brain
2) CSF examination
3) Drug toxicology
4) All of the above
The most likely diagnosis is:
1) Drug intoxication
2) Acute cerebellar ataxia
3) A posterior Fossa mass
4) Complicated Migraine
A 5 year old boy uses this maneuver to stand
Where is his weakness ?
What is maneuver called ?
What is his diagnosis ?
The following statements are true about Duchenne’s
muscular dystrophy (DMD) and Becker’s Muscular
Dystrophy (BMD) EXCEPT:
1) Both are X linked
2) Both have defects in Dystrophin
3) Both have early age of onset
4) Both respond to Steroids
The best way to make a diagnosis in a young male with
myopathic symptoms and a myopathic examination is:
1) Muscle Biopsy
2) MRI of Muscle
3) Mutation Analysis
4) CPK
•
A 12-month-old previously healthy infant develops fever to
39.5ºC (103.1º1F) and suffers a 2-minute generalized tonicclonic seizure. Physical examination reveals an alert child
who has no abnormal neurological findings.
The likelihood that this child will develop epilepsy
is CLOSEST to
1)
2)
3)
4)
5)
1%
10%
25%
33%
50%

A 13-month-old girl has had several brief febrile
seizures. Although the parents would like their child to
receive prophylactic treatment, they do not want you to
prescribe phenobarbital or valproic acid.
Among the following, the BEST alternative home treatment is
1.
2.
3.
4.
5.
An antibiotic, given orally once daily for prophylaxis
Diazepam, given every 8 to 12 hours during fever
Midazolam, given intramuscularly during fever
Phenytoin, given orally twice daily for prophylaxis
Synergistic use of aspirin, acetaminophen, and
ibuprofen, given orally during fever

A 25 month old developmentally delayed girl has a
prolonged generalized tonic-clonic seizure lasting >20
minutes. Her temperature is 39ºC (102.2ºF); there is no
evidence of meningismus. Her siblings are also sick (fever
and URI). Her brother and maternal aunt have epilepsy.
The risk that this child will develop epilepsy is CLOSEST to
1.
2.
3.
4.
5.
2 to 4%, twice the usual risk
9 to 15%, because she has three risk factors
30%, because she experience a prolonged seizure
50%, because she has Down syndrome
90%, because she sustained brain damage during the
prolonged seizure
•
A 1 year old boy had a generalized tonic -clonic seizure
that lasted 5 minutes. History includes non-bloody
diarrhea and upper respiratory tract symptoms for several
days. Findings on physical examination are normal except
for a temperature of 39.6ºC (103.3ºF) and nasal
congestion.
Of the following, the MOST likely cause of this boy’s seizure is
1.
2.
3.
4.
5.
Bacterial meningitis
Fever
Hyponatremia
Idiopathic epilepsy
Shigellosis

A 7 month old boy has clinical & electroencephalographic
evidence of infantile spasms. Extensive investigation
uncovers no cause. The condition is regarded as
idiopathic.
Of the following, the BEST advice to the parents is:
1.
2.
3.
4.
5.
A treatable cause will be found eventually
Since no etiology was found, immunization is the most
likely cause
The prognosis is more favorable in idiopathic cases
than in those caused by an underlying disorder
Treatment with Phenobarbital is indicated when the cause
is unknown
Tuberous sclerosis is likely to become evident in the
future
•
According to her teacher, an 8 year old girl “spaces
out” at school. Lately, the parents have seen similar
spells at the dinner table. At times they have also
noted associated eye blinking or lip smacking.
Of the following, the finding that would be MOST
suggestive of absence (petit mal) rather than partial
complex seizures is
1.
2.
3.
4.
5.
Aura prior to spells
Incontinence during spells
Isolated spikes on electroencephalography
Prompt recovery after spells
Spells that last 15 to 30 seconds
All of the following statements are true EXCEPT
1. Dawn’s EEG shows a 3 per second spike and wave
pattern
2. The most likely diagnosis is absence seizures
3. The anticonvulsant medication(s) of choice would be
either phenobarbital or phenytoin since they are not
expensive and have a long half-life.
4. Dawn’s prognosis for “outgrowing” this seizure disorder is
excellent.
•
A 3 month old boy who has diarrhea is fed only apple
juice for 3 days. Because of increasing lethargy, he is
brought to the hospital where he has a generalized
tonic-clonic seizure lasting 10 minutes. He is treated
with Lorazepam and Phenobarbital and intubated for
apnea. He is noted to be well hydrated and afebrile.
All other findings are normal. The serum glucose level
is 80 mg/dl.
Of the following, the MOST likely diagnosis is
1.
2.
3.
4.
5.
Bacterial meningitis
Fructose intolerance
Hypocalcemia
Hyponatremia
Viral meningoencephalitis
–
1.
2.
3.
4.
5.
In which of the following situations would
discontinuation of prophylactic anticonvulsant
therapy be MOST justified ?
Absence seizures in remission for 1 year
Adolescent-onset myoclonic epilepsy in remission for 3
years
Hypertensive seizures with hemiparesis in remission for 2
weeks
Major motor seizures in remission for 2 years
Partial complex seizures in remission for 6 months
•
The parents of a 4 year old girl report that several
nights per week she comes into their bedroom. While
standing next to their bed, she exhibits right facial
jerking and difficulty swallowing and talking. A cousin
has absence seizures.
Of the following, the MOST likely diagnosis is
1.
2.
3.
4.
5.
Absence seizures
Benign rolandic epilepsy
Conversion reaction
Night terrors
Panic attacks
•
10 y o girl presents to the ER with history of loss of
consciousness. The girl had been in church, singing in
the choir. She remembers feeling hot and “dizzy” and
was sweating. The members of the choir said she fell to
the ground and was notably pale. She “came to” within
a few minutes. By the time EMS arrived, she had
regained consciousness but was a little confused. In the
ER, she was fully oriented with a normal exam.
What is the likely cause of her sudden loss of
consciousness?
1.
2.
3.
4.
5.
Seizure
Migraine
Psychogenic
Syncope
Cardiac arrhythmia
•
A 2-month-old boy has episodes of hypotonia and pallor
with feedings. You suspect gastro-esophageal reflux.
The parents, however, are worried that their child has
epilepsy.
The BEST way to rule out epilepsy would be to document
1.
2.
3.
4.
5.
A negative family history of epilepsy
Abnormal pulse oximetry during a spell
Normal findings on EEG during a spell
Normal findings on an imaging study of the brain
Normal findings on an interictal electroencephalogram
•
A 12-year-old boy had well-controlled epilepsy until
recently (generalized tonic-clonic seizures). He now has
developed seizures characterized by opisthotonic
posturing, jerking of one leg then the other, and writhing.
Seizures are frequent, occurring several times per week.
An electroencephalogram obtained 2 years ago
revealed epileptiform discharges. His parents are in the
process of getting divorced.
The BEST explanation for this boy’s “spells” is
1. Acute psychosis
2. Adverse drug reaction
3. Complex partial seizures
4. Paroxysmal choreoathetosis
5. Pseudoseizures
•
A 7-year-old boy is evaluated in the ER for unsteady
gait. 2 weeks ago, he had a flu-like illness. On
examination, he is unable to abduct his eyes – has
bilateral esotropia. He also appears to have a flat
affect and decreased facial movements. His power is
4/5 in all extremities. His DTR’s are depressed and he
walks holding on.
The MOST LIKELY diagnosis is
1)
2)
3)
4)
5)
Transverse myelitis
Myasthenia gravis
Myotonic dystrophy
Miller-Fisher syndrome
Moebius syndrome
•
An 8-year-old girl is admitted to the hospital for
difficulty swallowing. She had a sore throat 2 weeks
ago. On examination, she has left 6th nerve palsy, with
bilateral facial weakness and absent gag reflex. There
is bilateral lower extremity hyper-reflexia and upgoing
(toes) plantar reflex.
The MOST LIKELY diagnosis is
1.
2.
3.
4.
5.
Myasthenia gravis
Syringomyelia
Guillain-Barre syndrome
Bell’s palsy
Brainstem glioma
 A 6 mo old boy is seen in clinic for his routine check-up &
immunization. His regular PMD is away. You are covering
the clinic.
Your examination shows his head circumference is
>98th % and has been steadily crossing percentiles since
birth. He has tachycardia and hyperdynamic circulation.
- Mom reports there was concern about poor feeding and
congestive heart failure in the neonatal period however his
echocardiogram was normal and he gradually improved.
The MOST LIKELY cause for your concern
1. Bilateral subdural collections
2. Soto’s syndrome
3. Congenital aqueductal stenosis
4. Vein of Galen malformation
5. Familial megalencephaly
 (continued)
6 mo old boy comes to clinic for routine check-up and
immunization. Head circumference (HC) is > 98th %.
Since birth his HC has been crossing percentiles. He is
tachycardia and has hyperdynamic circulation.
Your concern is the possibility of a Vein of Galen malformation
What physical finding will be helpful ?
1. Sunset sign
2. Dysmorphic features
3. Cranial bruit
4. Retinal hemorrhage
5. Cherry red spot
•
A 15-year-old boy is brought to the ER by his family.
They have been unable to arouse him and get him ready
for school. He has a history of seizures and mental
retardation. He is on anticonvulsant medication. For the
past one week, he has been complaining of headache
and vomiting.
Which of the following is UNLIKELY to be a cause of his
coma?
1.
2.
3.
4.
5.
Increased intracranial pressure
Migraine
Post-ictal state
Toxic encephalopathy
Herpes encephalitis
What test will be helpful in his immediate management?
1.
2.
3.
4.
5.
CT scan
EEG
MRI brain
Lumbar puncture
Liver function tests
•
A 3-year-old boy is reported by the baby sitter to be
staggering and confused. EMS was called. By the
time the child arrives in the ER, he is unresponsive.
He is noted to have 4 mm sluggishly reactive pupils.
His vital signs are stable. His grandmother is a
diabetic and is on oral hypoglycemic agents. His
mother is on treatment of depression.
Which of the following test would you request?
1.
2.
3.
4.
5.
CT scan of the head
Lumbar puncture
EEG
EKG
CBC
•
A 7-year-old boy is evaluated in the Emergency Room
for progressively worsening headache with vomiting
and unsteady gait.
Which of the following is NOT suggestive of increased
intracranial pressure?
1.
2.
3.
4.
5.
Papilledema
Diplopia
Paresthesias
Hemiparesis
New onset strabismus
Which of the following brain tumors is NOT likely to cause
early increased intracranial pressure?
1.
2.
3.
4.
5.
Brain stem glioma
Cerebellar astrocytoma
Medulloblastoma
Ependymoma
Choroid plexus papilloma
•
A 15-year-old teenage girl presents with new onset
double vision. She is obese and reports being fatigued
easily. Her vital signs are stable. Her left eye does not
abduct fully, pupils are equal and reactive.
The MOST LIKELY cause of her double vision is
1.
2.
3.
4.
5.
Pseudotumor cerebri
Myasthenia gravis
Migraine
Depression
Miller-Fisher syndrome
What diagnostic procedure will confirm your
diagnosis?
1.
2.
3.
4.
5.
CT scan
MRI brain
Tensilon test
Lumbar puncture
EEG
•
On routine school eye exam, a 15 yr old adolescent boy
is noted to have decreased vision in the left eye. His
neurological exam is normal without focal deficit. His
secondary sexual characteristics are delayed.
Imaging study is LIKELY to show which of the following?
1.
2.
3.
4.
5.
Astrocytoma
Ganglioglioma
Pinealoma
Glioblastoma
Craniopharyngioma
The most appropriate treatment options for this patient are?
1.
2.
3.
4.
5.
Surgery only
Surgery with chemotherapy
Surgery and radiotherapy
Chemotherapy and radiation
Observation only
•
Mrs. Jones brings Anne, age 9, to your office for a
consultation. She is concerned Anne may have to
repeat 4th grade. Anne had been an excellent student
but this year her school performance deteriorated. In
addition, Anne has become clumsy. She even has
trouble tying her shoe-laces. Her older sister is now in
a wheel-chair. (She developed trouble walking one
year ago. Mom reports that both girls are adopted.)
Mom is quite persistent in reporting that Anne is
different compared to her sister. On examination,
Anne has mild spasticity with diminished reflexes and
difficulty with tandem gait.
Which of the following is MOST LIKELY ?
1.
2.
3.
4.
5.
Adrenoleukodystrophy
HIV encephalopathy
Neuronal ceroid lipofuscinosis
Metachromatic leukodystrophy
Krabbe disease
•
A 17-month-old boy is referred for evaluation of
developmental delay. His birth and neonatal history are
normal. Presently he can sit with support. His visual
tracking is poor. He developed seizures – myoclonic
jerks at 11 months of age. He is taking Keppra and
Topamax. On examination, he has significant spasticity
with depressed reflexes.
Which of the following does he NOT have?
1.
2.
3.
4.
5.
Mitochondrial encephalopathy
Fragile X syndrome
Ceroid lipofuscinosis
Leigh’s disease
Alpers disease

A 4 year-old girl with obvious “developmental delay” is
seen in the office for evaluation of seizures. Birth history
was normal as was early development. She was
walking at 15 months, but slowly her gait deteriorated.
She now walks holding on or prefers to sit in the stroller.
Seizures began when she was 11 months old. Since
then she has been on Phenobarbital.
Examination shows: microcephaly (although her head
circumference at birth was normal). She does not reach
for toys. Instead she keeps her hands clasped often with
a wringing movement. She tends to “breathe heavy”.
Which of the following is her MOST LIKELY diagnosis?
1. Cornelia de Lange syndrome
2. Hellers syndrome
3. Rett syndrome
4. Mucopolysaccharidosis
5. Alexander disease