Transcript Paediatric Neurology 2012

LMCC Review:
Pediatric Neurology
Asif Doja, MEd, MD, FRCP(C)
March 27th, 2012
Outline
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Seizures
Febrile Seizures
Status Epilepticus
Headache
Seizures
Question 1
Someone can be diagnosed with epilepsy if
they have:
A. More than one febrile seizure
B. More than one afebrile seizure
C. Seizures in the context of hypoglycemia
D. One seizure and a history of brain injury
Question 2
All of the following seizure types are
classified as “generalized” seizures
EXCEPT:
A. Complex partial seizures
B. Absence seizures
C. Tonic-clonic seizures
D. Atonic seizures
Question 3
All of the following are features of Absence
seizures EXCEPT:
A. Lack of an aura or warning
B. Impairment in consciousness
C. Post-ictal drowsiness/lethargy
D. 3 Hz spike and wave on EEG
Question 4
Which of the following is an appropriate first
line treatment for an 8 year old child with
epilepsy?
A. Bromide therapy
B. Ketogenic Diet
C. Carbemazepine
D. Phenobarbital
Question 5
A 9 year old child presents with recurrent episodes of
waking in the morning with facial twitching,
dysarthria and normal level of consciousness. The
most likely diagnosis is:
A. Transient Ischemic Attacks
B. Benign Epilepsy of Childhood with Rolandic
Spikes
C. Juvenile Myoclonic Epilepsy
D. Facial tics
Definitions
• Seizure: Paroxysmal discharge of neurons
resulting in behaviour change, motor or
sensory dysfunction
• Epilepsy: > 1 unprovoked seizure
Was it a Seizure?
• Differential Diagnosis
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Syncope
Breath Holding
Night Terrors
Tics
GERD
etc
Syncope vs Seizure
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Vasovagal reflex
Usually happens when standing up
Lightheaded feeling
Pale, cold, clammy
Loss of consciousness and fall
Tremble but no tonic-clonic movements
No post-ictal lethargy
Focal vs. Generalized Seizures
Focal
• Simple Partial
• Complex Partial
• Partial Seizure with 2O
Generalization
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Generalized
Generalized TonicClonic
Tonic
Clonic
Absence
Atonic
Myoclonic
How to differentiate
“Staring Spells”
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Complex Partial
Aura
~ 30 sec or more
Decr LOC
Automatisms
Post-ictal period
EEG: focal epileptiform
abnormality
Hyperventialtion has no
effect
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Absence
No aura
Lasts few seconds
Decr LOC
May have automatisms
No post-ictal period
EEG: 3 HZ spike and
wave
Provoked by
hyperventialtion
Investigations and Treatment
• Neuroimaging if focal findings present
• May do EEG after first seizure
• Treatment if patient has 2 or more seizures
– Commonly used: Carbemazepine, Valproic Acid,
Phenobarbital
– Many other newer anticonvulsants ie Topiramate,
Levotiracetam
– (For refractory patients: Ketogenic Diet, Epilepsy
surgery)
Epilepsy Syndromes
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West Syndrome
Infantile Spasms
Onset in 1st year
Symmetrical
contractions of
trunk/extremities
EEG: hypsarrythmia
Poor prognosis
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Lennox Gastault
Onset age 3-5
Multiple seizure types
Developmental delay
EEG: slow spike and
wave
Many have history of
infantile spasms
Epilepsy Syndromes
Benign Epilepsy of
Childhood with
Rolandic Spikes
(BECRS)
• 5-10 years
• Simple partial seizures
involving face
• Remits spontaneously,
no treatment
Juvenile Myoclonic
Epilepsy
• 12-16 years
• Myoclonus and GTC
seizures
• Good prognosis, but
requires lifelong
treatment with
Valproic Acid
Question 1
Someone can be diagnosed with epilepsy if
they have:
A. More than one febrile seizure
B. More than one afebrile seizure
C. Seizures in the context of hypoglycemia
D. One seizure and a history of brain injury
Question 2
All of the following seizure types are
classified as “generalized” seizures
EXCEPT:
A. Complex partial seizures
B. Absence seizures
C. Tonic-clonic seizures
D. Atonic seizures
Question 3
All of the following are features of Absence
seizures EXCEPT:
A. Lack of an aura or warning
B. Impairment in consciousness
C. Post-ictal drowsiness/lethargy
D. 3 Hz spike and wave on EEG
Question 4
Which of the following is an appropriate first
line treatment for an 8 year old child with
epilepsy?
A. Bromide therapy
B. Ketogenic Diet
C. Carbemazepine
D. Phenobarbital
Question 5
A 9 year old child presents with recurrent episodes of
waking in the morning with facial twitching,
dysarthria and normal level of consciousness. The
most likely diagnosis is:
A. Transient Ischemic Attacks
B. Benign Epilepsy of Childhood with Rolandic
Spikes
C. Juvenile Myoclonic Epilepsy
D. Facial tics
Febrile Seizures
Question 1
Which of the following is NOT a feature of a
typical febrile seizure?
A. Onset between ages 6 months – 6 years
B. Duration of < 15 minutes
C. Only one seizure in 24 hour span
D. Patients usually have pre-existing
developmental delay
Question 2
Which of the following is FALSE regarding atypical
febrile seizures?
A. They may show clonic jerking on only one side of
the body
B. The patient is at no increased risk for further
febrile seizures.
C. The patient can present in status epilepticus
D. The patient can show focal abnormalities on
neurologic exam.
Question 3
A 8 month old female has one typical febrile seizure,
then 2 months later has another. With respect to
anticonvulsants, you would prescribe:
A. Phenobarbital
B. Carbemazepine
C. Valproic Acid
D. None, as the patient does not require treatment
Question 4
A 7 month old male has a typical febrile seizure.
With respect to doing a lumbar puncture, the
AAP guidelines state that you should:
A. Not do an LP
B. Do an LP if the temperature is > 39 degrees
C. Do an LP only if there are meningeal signs
D. Do an LP irregardless of the physical exam
findings
Question 5
What is the risk of developing epilepsy in a
child with a typical febrile seizure?
A. 1%, the same as the general population
B. 2-3%
C. 10-15%
D. 33%
Febrile Seizures
• 3-5% of all children
• Ages 6 months to 6 years
• Usually GTC
Typical vs Atypical Febrile
Seizures
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Typical
Duration < 15 min
No focality
Does not recur in 24hour period
No hx of
developmental delay
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Atypical
Duration > 15 min
Focal findings during
seizure or after exam
> 1 in 24 hours
Previous History of
Developmental Delay
Risk of Recurrence
• 33% chance of recurrence (75% occur
within 1 year)
• Risk Factors:
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Family history of feb. con. or epilepsy
Short duration of fever prior to seizure
Developmental / Neurological problems
Atypical febrile seizure
Investigations
• History and Physical – determine source of
fever
• EEG and Neuroimaging only needed in
atypical cases
• LP:
– If < 12 months: Do LP
– If 12-18 months: Consider LP
– If > 18 months: Only if meningeal signs present
Management
• Reassurance
• Risk of developing epilepsy is 2-3% (1% in
general population)
• Antipyretics and fluids for comfort (neither
prevent seizures)
• No need for anticonvulsants
Question 1
Which of the following is NOT a feature of a
typical febrile seizure?
A. Onset between ages 6 months – 6 years
B. Duration of < 15 minutes
C. Only one seizure in 24 hour span
D. Patients usually have pre-existing
developmental delay
Question 2
Which of the following is FALSE regarding atypical
febrile seizures?
A. They may show clonic jerking on only one side of
the body
B. The patient is at no increased risk for further
febrile seizures.
C. The patient can present in status epilepticus
D. The patient can show focal abnormalities on
neurologic exam.
Question 3
• A 8 month old female has one typical febrile
seizure, then 2 months later has another. With
respect to anticonvulsants, you would prescribe:
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A. Phenobarbital
B. Carbemazepine
C. Valproic Acid
D. None, as the patient does not require treatment
Question 4
A 7 month old male has a typical febrile seizure.
With respect to doing a lumbar puncture, the
AAP guidelines state that you should:
A. Not do an LP
B. Do an LP if the temperature is > 39 degrees
C. Do an LP only if there are meningeal signs
D. Do an LP irregardless of the physical exam
findings
Question 5
What is the risk of developing epilepsy in a
child with a typical febrile seizure?
A. 1%, the same as the general population
B. 2-3%
C. 10-15%
D. 33%
Status Epilepticus
Question 1
Status Epilepticus is defined as:
A. 30 minutes or > of continuous seizure
activity
B. Recurrent seizures with no intervening
normal level of consciousness for > 30 min
C. A and B
D. None of the above
Question 2
A 5 year old boy presents to the ER with a 45
minute GTC seizure. What is your initial
management?
A. ABC’s
B. Stat CT head
C. Lorazepam 0.1mg IV push
D. Tox screen
Question 3
Which of the following metabolic
disturbances is MOST likely to cause
seizures?
A. High Potassium
B. High Chloride
C. Low urea
D. Low glucose
Question 4
First line anticonvulsant treatment in status
epilepticus should be:
A. Lorazepam
B. Phenytoin
C. Phenobarbital
D. Thiopentol coma
Status Epilepticus
• 30 minutes or > of continuous seizure
activity
• Recurrent seizures with no intervening
normal level of consciousness for > 30 min
Status Epilepticus
• ABC’s
– Oxygen / pulse oximetry
– Bag-valve support or intubation if req’d
– IV access
• Check blood sugar -- give dextrose if low
(2-4 ml/kg of 25% solution)
Status Epilepticus
• Anticonvulsants:
– Benzodiazepines ie Lorazepam (0.1 mg/kg IV),
can repeat X1
– If fails, Phenytoin 20mg/kg (no faster than 1
mg/min)
– If fails, Phenobarbital 20 mg/kg (no faster than
1 mg/min)
– If fails, will need to go to ICU for barbituate
coma (ie thipentol) or midazolam infusion
Question 1
Status Epilepticus is defined as:
A. 30 minutes or > of continuous seizure
activity
B. Recurrent seizures with no intervening
normal level of consciousness for > 30 min
C. A and B
D. None of the above
Question 2
A 5 year old boy presents to the ER with a 45
minute GTC seizure. What is your initial
management?
A. ABC’s
B. Stat CT head
C. Lorazepam 0.1mg IV push
D. Tox screen
Question 3
Which of the following metabolic
disturbances is MOST likely to cause
seizures?
A. High Potassium
B. High Chloride
C. Low urea
D. Low glucose
Question 4
First line anticonvulsant treatment in status
epilepticus should be:
A. Lorazepam
B. Phenytoin
C. phenobarbital
D. Thiopentol coma
Headache
Question 1
• A 7 year old male presents with headache.
Which of the following would NOT be a
“red flag” on history?
A. Early morning vomiting
B. Headache worse after certain foods
C. Vomiting without nausea
D. Focal neurologic symptoms
Question 2
• Which is the following is FALSE regarding
migraine in children
A. The headache can last as little as 1 hour in
children
B. Children do not need to have nausea AND
vomiting to be diagnosed with migraine
C. There is often a family history of migraine
D. MRI is often needed to rule ot other serious
causes of headache.
Question 3
• Which of the following medications has the
best evidence for aborting migraine in
children?
A. Acetaminophen
B. Demerol
C. Sumatripan
D. Ibuprofen
Question 4
• Which of the following is NOT a migraine
variant in childhood?
A. Alice in Wonderland syndrome
B. Paroxysmal Torticollis
C. Cyclic Vomiting Syndrome
D. Benign Paroxysmal Vertigo
E. All of the above are migraine variants in
childhood
Key Questions to ask on H/A Hx
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Duration
Constant or Intermittent
Quality of Pain (ie throbbing, pressure)
Scale 1-10
Location of pain +/- radiation
Nausea or vomitting
Photo or Phonophobia
Aggravating and Alleviating factors
Key Questions to ask on H/A Hx
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Early am waking
Weight loss, fever etc
Aura / Visual changes
Focal neuro symptoms
Change with position / Valsalva
Family Hx of H/A
Key items on Physical
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Temperature
Blood pressure and CVS exam
Cranial Bruits
Scalp tenderness
Fundi
Focal neurological signs
H/A in increased ICP
• Nocturnal or early morning H/A in 15%
• Nx and Vx in 50%
• May be precipitated by change in position /
Valsalva
Other features of Brain Tumours/
H/A in increased ICP
• Personality change, memory problems, poor
concentration
• Seizures in 1/3
• Vomiting NOT preceded by nausea
• Focal neuro findings
• Papilledema – formally seen in 60-70%
– Now seen in ~ 10-20%
– Likely due to better neuroimaging techniques
Migraine
• Epidemiology
– 75% of H/A’s referred for pediatric neurologic
consultation
– prevalence 1.2 – 11% depending on age
• +ve family hx in 70 – 90%
Key Features
• May have previous history of motion
sickness
• Headache is dull then becomes
pulsating/throbbing (NOT maximal at
onset)
• Unilateral (2/3) or bilateral (1/3)
• Can be associated with cutaneous allodynia
Key Features
• Ask re: nausea, vomiting, anorexia, relief
with sleep, “Do they look sick”?
• Triggers: exercise, anxiety, fatigue, head
trauma, menses, foods (chocolate, nitrites,
MSG)
• Auras: visual changes, dysesthesias of
limbs and perioral region
– For auras, ask re: sudden onset vs gradual onset
Diagnostic Criteria
• A. At least 5 attacks
• B. Headache lasting 30 min to 48 hrs
• C. Headache has at least 2 of the following
1. Bilateral (fronto-temporal) or unilateral location
2. Pulsating quality
3. Moderate to severe intensity
4. Aggravation by routine physical activity
• D. During headache, at least 1 of:
– 1. Nausea or vomiting
– 2 . Photophobia or phonophobia
Migraine Treatment
Abortive
• Reference: Neurology, 2004
• Best Evidence (Level A)
– Ibuprofen (10mg/kg)
• Level B
– Acetaminophen (15 mg/kg)
(Often need to tell parents correct dose)
• Intranasal Sumatriptan effective in adolescents
– (5-20 mg at onset of H/A, can repeat X 1)
• Insufficient evidence for oral triptans
Migraine Variants:
With Headache
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Hemiplegic Migraine
Confusional Migraine
Basilar Migraine
Ophthalmoplegic Migraine
Migraine Variants:
No Headache
• Alice in wonderland
syndrome
• Benign Paroxysmal
Vertigo
• Paroxysmal Torticollis
• Cyclic Vomitting
Question 1
• A 7 year old male presents with headache.
Which of the following would NOT be a
“red flag” on history?
A. Early morning vomiting
B. Headache worse after certain foods
C. Vomiting without nausea
D. Focal neurologic symptoms
Question 2
• Which is the following is FALSE regarding
migraine in children
A. The headache can last as little as 1 hour in
children
B. Children do not need to have nausea AND
vomiting to be diagnosed with migraine
C. There is often a family history of migraine
D. MRI is often needed to rule ot other serious
causes of headache.
Question 3
• Which of the following medications has the
best evidence for aborting migraine in
children?
A. Acetaminophen
B. Demerol
C. Sumatripan
D. Ibuprofen
Question 4
• Which of the following is NOT a migraine
variant in childhood?
A. Alice in Wonderland syndrome
B. Paroxysmal Torticollis
C. Cyclic Vomiting Syndrome
D. Benign Paroxysmal Vertigo
E. All of the above are migraine variants in
childhood
Questions?