Block 11 Board Review
Part 2 of 4
Neurology/Heme-Onc
18April2014
Chauncey D. Tarrant, M.D.
Chief of Residents 13-14
3% of Initial Certifying Exam!!!
Pediatrics In Review Articles
• Seizures
• Malformations
PIR Quiz
1. A 2-year-old has febrile seizures. Which of the
following factors will increase her risk of subsequently
developing epilepsy?
A. An initial febrile seizure with a relatively low
temperature.
B. Brief duration between onset of the fever and seizure.
C. Complex febrile seizure.
D. Family history of febrile seizures in a first-degree
relative.
E. Younger age at onset of febrile seizures.
1. A 2-year-old has febrile seizures. Which of the
following factors will increase her risk of subsequently
developing epilepsy?
A. An initial febrile seizure with a relatively low
temperature.
B. Brief duration between onset of the fever and seizure.
C. Complex febrile seizure.
D. Family history of febrile seizures in a first-degree
relative.
E. Younger age at onset of febrile seizures.
2. Treatment with antiepileptic drugs is recommended
after 2 or more recurrent afebrile seizures. A common
question from parents in response to this treatment
parameter is, “What percentage of children who
experience a single unprovoked seizure will not have
another?”
A. 10%.
B. 20%.
C. 40%.
D. 60%.
E. 80%.
2. Treatment with antiepileptic drugs is recommended
after 2 or more recurrent afebrile seizures. A common
question from parents in response to this treatment
parameter is, “What percentage of children who
experience a single unprovoked seizure will not have
another?”
A. 10%.
B. 20%.
C. 40%.
D. 60%.
E. 80%.
3. An 8-year-old child with a long history of recurrent
generalized tonic-clonic seizures develops generalized
convulsive status epilepticus. Which of the following is
the first treatment of choice on encountering trained
medical personnel?
A. Diazepam.
B. Fosphenytoin.
C. Lorazepam.
D. Pentobarbital.
E. Phenobarbital.
3. An 8-year-old child with a long history of recurrent
generalized tonic-clonic seizures develops generalized
convulsive status epilepticus. Which of the following is
the first treatment of choice on encountering trained
medical personnel?
A. Diazepam.
B. Fosphenytoin.
C. Lorazepam.
D. Pentobarbital.
E. Phenobarbital.
4. A 7-year-old boy develops spells that consist of a
unilateral contraction of the left side of his face
without impairment of consciousness or speech
arrest. Which of the following is the most likely
diagnosis?
A. Absence seizures.
B. Benign rolandic epilepsy.
C. Juvenile myoclonic epilepsy.
D. Psychomotor seizures.
E. Simple partial seizures.
4. A 7-year-old boy develops spells that consist of a
unilateral contraction of the left side of his face
without impairment of consciousness or speech
arrest. Which of the following is the most likely
diagnosis?
A. Absence seizures.
B. Benign rolandic epilepsy.
C. Juvenile myoclonic epilepsy.
D. Psychomotor seizures.
E. Simple partial seizures.
5. Generalized tonic-clonic seizures in
adolescents are typical of which epileptic region
of the brain?
A. Centrofrontal lobe region.
B. Corpus callosum region.
C. Frontal lobe region.
D. Parietal lobe region.
E. Temporal lobe region.
5. Generalized tonic-clonic seizures in
adolescents are typical of which epileptic region
of the brain?
A. Centrofrontal lobe region.
B. Corpus callosum region.
C. Frontal lobe region.
D. Parietal lobe region.
E. Temporal lobe region.
1. You are seeing a 12-year-old girl who has
myelomeningocele in your office for complaints of radiating
back pain over the past 3 months. She has not changed her
bowel or bladder management program but is
now having new urinary leakage between catheterizations.
The additional symptom that most supports your
presumptive diagnosis is:
A. Declining school performance.
B. Deteriorating gait.
C. Difficulty swallowing.
D. Early morning headache.
E. Worsening handwriting.
1. You are seeing a 12-year-old girl who has
myelomeningocele in your office for complaints of radiating
back pain over the past 3 months. She has not changed her
bowel or bladder management program but is now having
new urinary leakage between catheterizations. The
additional symptom that most supports your presumptive
diagnosis is:
A. Declining school performance.
B. Deteriorating gait.
C. Difficulty swallowing.
D. Early morning headache.
E. Worsening handwriting.
2. An emergency department physician calls you about her
evaluation of a 14-month-old child who has
myelomeningocele and a ventriculoperitoneal shunt. Results
of computed tomography scan and shunt series
are unchanged from previous studies, but the child is not
“normal,” according to the parents. Which of the
following is most concerning for a shunt malfunction?
A. Ankle clonus.
B. Limited upward gaze.
C. Patulous anus.
D. Pes cavus.
E. Swelling on back.
2. An emergency department physician calls you about her
evaluation of a 14-month-old child who has
myelomeningocele and a ventriculoperitoneal shunt. Results
of computed tomography scan and shunt series
are unchanged from previous studies, but the child is not
“normal,” according to the parents. Which of the
following is most concerning for a shunt malfunction?
A. Ankle clonus.
B. Limited upward gaze.
C. Patulous anus.
D. Pes cavus.
E. Swelling on back.
3. During a neonatal consultation for an infant born with
a neural tube defect (neurologic level L3), the
family asks if their child will walk. The most accurate
statement regarding this child’s future walking ability
is that she will walk:
A. Independently.
B. With ankle bracing.
C. With high leg bracing.
D. With hip bracing.
E. With low leg bracing and forearm crutches.
3. During a neonatal consultation for an infant born with
a neural tube defect (neurologic level L3), the
family asks if their child will walk. The most accurate
statement regarding this child’s future walking ability
is that she will walk:
A. Independently.
B. With ankle bracing.
C. With high leg bracing.
D. With hip bracing.
E. With low leg bracing and forearm crutches.
4. You are seeing an 8-month-old boy who was born with
myelomeningocele for a routine follow-up
evaluation in the clinic. His parents have been reading on the
Internet about the Chiari II malformation and
ask if their son has this problem. The clinical finding most
commonly associated with Chiari II malformation
is:
A. Bulging fontanel.
B. Leg pain.
C. Scoliosis.
D. Stridor.
E. Urinary incontinence.
4. You are seeing an 8-month-old boy who was born
with myelomeningocele for a routine follow-up
evaluation in the clinic. His parents have been reading
on the Internet about the Chiari II malformation and ask
if their son has this problem. The clinical finding most
commonly associated with Chiari II malformation is:
A. Bulging fontanel.
B. Leg pain.
C. Scoliosis.
D. Stridor.
E. Urinary incontinence.
Seizures
What are some metabolic causes of
seizures?
What are some metabolic causes of
seizures?
• glucose, sodium, phosphate, magnesium, and
calcium disturbances
Which drugs precipitate or exacerbate
seizures?
Which drugs precipitate or exacerbate
seizures?
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Neuroleptics
Stimulants
Buspirone*
Diphenhydramine
Antibiotics (beta lactams)
Pink Grapefruit Juice (not a drug)
What are the most common causes of
acute seizures?
What are the most common causes of
acute seizures?
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fevers
Infections
head injury
Neonates: hypoxia-ischemia
What are some examples of
nonepileptic events?
What are some examples of
nonepileptic events?
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Breath Holding Spells
Tics
Self Stimulation
Syncope
Gastroesophageal reflux
Psychogenic seizures
Sleep Disturbances (sleepwalking/sleep
apnea)
What are the factors associated with
increased risk of seizure disorder?
What are the factors associated with
increased risk of seizure disorder?
• Family history of epilepsy
• Previous history of seizure
– Modest risk with complex febrile seizures
• History of meningoencephalitis
• History of penetrating traumatic brain injury
• Presence of diseases that lead to electrolyte
disturbances
• Presence of acidosis associated with hypoxia
• History of toxic Ingestion
What are the etiologic and therapeutic
implications of partial versus
generalized seizures?
What are the etiologic and therapeutic
implications of partial versus
generalized seizures?
Partial
Generalized (GTC)
Focal findings with no altered
consciousness (simple)
Altered consciousness (complex)
Multifocal
Prodromeseizurepostictal state
Trileptal, Keppra
Lamictal, Valproic Acid, Topamax, Keppra,
Zonegran
How do you manage a child with a first
seizure?
How do you manage a child with a first
seizure?
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Thorough history and physical
Consider Neurology Consult/EEG
Thorough family history
Seizure diary
Videotape events (if possible)
History of epilepsy?
History pf conditions that cause electrolyte
disturbances?
• Predisposing factors (sleep deprivation, fevers,
illness, or infection)
How do you manage a child with
recurrent seizures?
How do you manage a child with
recurrent seizures?
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MRI
EEG
Consider treatment with AEDs
Others: surgery (brain surgery vs. VNS),
ketogenic diet (low glycemic index diet)
How would you manage a patient with
psychogenic seizures?
How would you manage a patient with
psychogenic seizures?
• Discuss with parents after negative video EEG
• Consultation with child psych
• Treat underlying psychological cause (anxiety,
depression, conversion, somatization etc.)
Common Side Effects and Toxicities in
AEDs… (this article)
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Lamictal
Trileptal
Clonazepam
Ethosuximide
Valproic Acid
Common Side Effects and Toxicities in
AEDs…
• Lamictal
– Rash (SJS) if titration too fast
– Can exacerbate known neurobehavioral symptoms
• Trileptal
– Decrease WBC counts and decreased Na
• Clonazepam
– BZD withdrawal seizures, sedation
• Ethosuximide
– GI upset
• Valproic Acid
– Thrombocytopenia, transaminitis, increased pancreatic
enzymes leading to pancreatitis, weight gain, PCOS, balding
From PREP
Febrile Seizures
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Natural History
Risk Factors Associated with later Epilepsy
Diagnostic Criteria
Evaluation
Management
Febrile Seizures
• Natural History: 6mo-5yrs
• Risk Factors Associated with later Epilepsy:
family hx, complex, devo/neuro
abnormalities
• Diagnostic Criteria: age ≥6mo, associated with
febrile illness, no previous sz history
• Evaluation: None if simple; consider MRI/EEG
if complex
• Management: Reassurance (rule of 1/3s)
Infantile Spasms aka.._______
Syndrome
• Characteristic Clinical Features?
• Treatments?
• Prognosis?
Infantile Spasms aka West Syndrome
• Characteristic Clinical Features?
– http://www.youtube.com/watch?v=aVoJtslvqOU&feat
ure=player_detailpage
– infants ages 3 to 9 months
– spasm like seizures that involve flexion, extension,
mixed flexion-extension of the arms, legs, and trunk
• Treatments? ACTH/ Vigabatrin (Tuberous
Sclerosis)
• Prognosis? Poor
FYI
• Drug selection is based on seizure type
• Treat if patient has 2 or more recurrent seizures
• Discontinue AED therapy after 2yrs seizure free,
and wean off
• Check AM trough levels if there is a concern for
efficacy (fast metabolizers) or compliance
• Trileptal and Tegretol levels can be elevated by
macrolides
• Children with epilepsy have a higher occurrence
of anxiety, depression, ADHD
FYI
• Benign Rolandic Epilepsy
– centrotemporal spikes
– Most common type of partial epilepsy in
childhood,
– usually between 5-10yrs
– Involve unilateral facial sensory-motor and
oropharyngogutteral symptoms, hypersalivation,
and speech arrest
– Usually stop by age 16yrs
– Meds after 3 or more
FYI
• Juvenile Myoclonic Epilepsy
– Age 5-15yrs
– (1) myoclonic jerks on awakening
– (2) GTC seizures in 90% of patients, and
– (3) development of absence seizures in one-third
of all patients.
Malformations
What are common orthopedic
problems associated with Spina Bifida?
What are common orthopedic
problems associated with Spina Bifida?
What is the long term management for
neurogenic bladder/bowel?
What is the long term management for
neurogenic bladder/bowel?
Bladder
Bowel
Self Catheterization
Timed Toileting
Renal Ultrasound
Increased fiber in diet
Urodynamics
Laxatives
Vesicostomy
Enemas (antegrade colonic enema)*
What are the clinical and radiological
features and prognosis of spina bifida
occulta?
What are the clinical features and
prognosis of spina bifida occulta?
• Sometimes called “hidden spina bifida”
• May have no clinical manifestations
• May only see a small gap in the spine, no
opening
• Good prognosis
What is the Differential for a Child with
Spina Bifida with Neurologic
deterioration?
What is the Differential for a Child with
Spina Bifida with Neurologic
deterioration?
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Increased ICP
Shunt Malfunction
Chiari II Malformation
Tethered Cord
What are some signs and symptoms of
hydrocephalus/VP shunt malfunction?
What are some signs and symptoms of
hydrocephalus/VP shunt malfunction?
PREP
During the hottest week of the summer, a 6-monthold previously healthy infant presents to the
emergency department via ambulance in tonicclonic status epilepticus. After two doses (each 0.05
mg/kg) of intravenous lorazepam, the seizure
continues. The bedside glucose measurement is
normal, as is a noncontrast head computed
tomography scan.
Of the following, the MOST likely cause of the
child’s prolonged seizure is
A. hyperthyroidism
B. hypocalcemia
C. hypomagnesemia
D.hyponatremia
E. pyridoxine deficiency
A. hyperthyroidism
B. hypocalcemia
C. hypomagnesemia
D. hyponatremia
E. pyridoxine deficiency
A 4-year-old boy has had two fairly similar, brief episodes
within the past month consisting of abrupt arrest of
ongoing behavior, glassy-eyed staring, and lip smacking,
followed by confusion and sleepiness for 1 hour. He has
had no fevers or other signs of illness at the time of either
event. Findings on his medical and developmental
histories are otherwise normal. Physical and neurologic
examination results are normal. Brain magnetic
resonance imaging yields normal results, and
electroencephalography shows no abnormalities.
Of the following, the MOST appropriate treatment for this
boy is
A. carbamazepine
B. ethosuximide
C. felbamate
D. phenobarbital
E. phenytoin
A. carbamazepine
B. ethosuximide
C. felbamate
D. phenobarbital
E. phenytoin
A 12-year-old girl who has idiopathic partial epilepsy that
has been well controlled with carbamazepine for 1 year
develops an ear infection. Because she is allergic to
penicillin, an urgent care physician prescribes a course of
azithromycin. Three days later, she presents to the
emergency department with vomiting and dizziness. On
physical examination, she has end-gaze nystagmus in
both horizontal directions and a broad-based gait.
Of the following, the MOST likely explanation for this
girl’s clinical findings is
A. carbamazepine toxicity
B. confusional migraine
C. otitic hydrocephalus
D. postictal presentation
E. unilateral labyrinthitis
A. carbamazepine toxicity
B. confusional migraine
C. otitic hydrocephalus
D. postictal presentation
E. unilateral labyrinthitis
A 2-year-old girl presents after a prolonged focal-onset
seizure. She was playing when she suddenly started
crying and her right arm started jerking. Her whole body
then jerked for 10 to 15 minutes, during which time she
could not respond to her mother. The jerking was
subsiding when the paramedics arrived, and the child
slept en route to the emergency department. The girl was
born at 26 weeks’ gestation and has developmental delay.
She sits but does not yet walk.
Of the following, the STRONGEST predictor of seizure
recurrence in this child is
A. exposure to brother’s video games
B. family history of febrile seizures
C. minor trauma the day before the visit
D. preexisting neurologic impairment
E. stress associated with visiting the biological father
A. exposure to brother’s video games
B. family history of febrile seizures
C. minor trauma the day before the visit
D. preexisting neurologic impairment
E. stress associated with visiting the biological father
A 9-year-old boy had 2 generalized tonic-clonic seizures 1
week apart at 7 years of age. Evaluation at that time included
a normal routine electroencephalogram (EEG), sleep-deprived
EEG, 3-day video EEG, and brain magnetic resonance imaging
(MRI). Administration of carbamazepine was initiated and the
boy has not had further seizures. He has done well in school,
receiving average grades. His neurologist recently obtained an
EEG, which was again normal, and is recommending that the
boy discontinue anticonvulsant therapy. The boy’s mother is
concerned that if medication is discontinued, her son might
have another seizure.
Of the following, the MOST accurate statement about
discontinuing anticonvulsants is
A. a follow-up MRI should be obtained before stopping medications
B. it is best to discontinue medications abruptly
C. medications should be continued until after puberty
D. the normal EEG is an indication that seizures will not recur
E. the standard for tapering medications is 2 years of being seizure free
A. a follow-up MRI should be obtained before stopping medications
B. it is best to discontinue medications abruptly
C. medications should be continued until after puberty
D. the normal EEG is an indication that seizures will not recur
E. the standard for tapering medications is 2 years of being seizure free
Two months ago, an otherwise healthy 15-year-old girl
presented to the emergency department (ED) with a
generalized tonic-clonic seizure she had that morning. The
seizure was described as an abrupt stiffening of the body that
was followed by generalized fast rhythmic jerking; her eyes
were open, but she was unaware of her surroundings. The
episode lasted 1 to 2 minutes and was followed by confusion
and sleep. A head computed tomography scan performed in
the ED was normal, and she was discharged on no seizure
medication. Her mother calls your office because she has just
had another morning seizure after a sleepover with her
friends.
Of the following, the MOST appropriate course of action is to
recommend
A. electroencephalography to determine if the child has
epilepsy
B. neurologic consultation for seizure medication
C. psychological evaluation for psychogenic nonepileptic
seizure (pseudoseizure)
D. repeat head computed tomography scan
E. vitamin D concentration to assess for risk of fracture
A. electroencephalography to determine if the child has
epilepsy
B. neurologic consultation for seizure medication
C. psychological evaluation for psychogenic nonepileptic
seizure (pseudoseizure)
D. repeat head computed tomography scan
E. vitamin D concentration to assess for risk of fracture
A 15-year-old boy who has idiopathic partial
epilepsy presents to the emergency department
after a prolonged seizure. His maintenance
medication is oxcarbazepine.
Of the following, the MOST appropriate next
step in managing this patient is to
A. change his medication
B. obtain a serum oxcarbazepine concentration
C. obtain emergent magnetic resonance imaging of the brain
D. obtain serum calcium, magnesium, and phosphorus concentrations
E. schedule outpatient electroencephalography
A. change his medication
B. obtain a serum oxcarbazepine concentration
C. obtain emergent magnetic resonance imaging of the brain
D. obtain serum calcium, magnesium, and phosphorus concentrations
E. schedule outpatient electroencephalography
An 8-year-old girl has a seizure at school. She was sitting at
her desk when suddenly her whole body stiffened and she fell
to the floor and had jerking movements of her limbs. The
event lasted about 2 minutes and then stopped. The girl was
sleepy for about 30 minutes and then returned to normal.
This has never happened before, she has been healthy all her
life, and there is no one in the family who has seizures. You
order an electroencephalogram and receive a report that
describes “right and left centrotemporal spikes, consistent
with benign rolandic epilepsy.” You refer her to a pediatric
neurologist and her appointment is next week.
Of the following, the MOST appropriate advice to provide the
girl’s parents at this time is
A. brain damage is common even after a single seizure
B. children who have epilepsy should not participate in
contact sports
C. children who have epilepsy should not take baths alone
D. risk of sudden death is high in children who have epilepsy
E. their daughter will likely need lifelong seizure medications
A. brain damage is common even after a single seizure
B. children who have epilepsy should not participate in
contact sports
C. children who have epilepsy should not take baths alone
D. risk of sudden death is high in children who have epilepsy
E. their daughter will likely need lifelong seizure medications
You note an upcoming health supervision visit appointment for a 10year-old child who has myelomeningocele complex. You are seeing him
for the first time after your partner, who previously cared for him,
retired. In preparation for the visit, you review the child’s medical
history and some background on this complex condition. The boy had
an open spinal dysraphism repaired at birth. He also had herniation of
the cerebellar vermis (a Chiari II malformation) that required surgical
decompression and hydrocephalus that necessitated a
ventriculoperitoneal shunt. The boy has been stable for 3 years, but
according to your reading, he is at risk for acute deterioration due to
shunt malfunction or new problems in the brainstem or upper or lower
spinal cord.
Of the following, the finding that is MOST indicative of potential
neurological deterioration in this child is
A. double vision
B. facial weakness
C. gait dysfunction
D. impulsive behavior
E.unilateral headache
A. double vision
B. facial weakness
C. gait dysfunction
D. impulsive behavior
E.unilateral headache
A female newborn is noted to have a sacral subcutaneous
lump with a fatty consistency and a slight lateral deviation of
the superior aspect of the intergluteal fold. Her physical
examination findings were otherwise normal, and there was
no patellar hyperreflexia. Magnetic resonance imaging of the
thoracic and lumbar spine was obtained and showed a
lipomeningomyelocele. It is unclear if the cord is tethered.
The infant was referred to a multidisciplinary spina bifida
clinic, where renal ultrasonography was normal and
urodynamic assessment showed no evidence of detrusor
hyperreflexia or bladder-sphincter dyssynergia.
Of the following, the symptom or sign that you are MOST
likely to look for during health supervision visits is
A. chronic progressive diarrhea
B. development of an ataxic gait
C. development of a sacral hemangioma
D. loss of patellar reflexes
E. urinary retention and leakage
A. chronic progressive diarrhea
B. development of an ataxic gait
C. development of a sacral hemangioma
D. loss of patellar reflexes
E. urinary retention and leakage
Quick Associations
Infantile Spasms are associated with
which neurocutaneous disorder?
Infantile Spasms are associated with
which neurocutaneous disorder?
• Tuberous Sclerosis
Kids with Spina Bifida are commonly
allergic to…
Kids with Spina Bifida are commonly
allergic to…
• Latex