LMCC Review: Pediatric Neurology Asif Doja, MD, FRCP(C) April 3 rd , 2008
Jan 02, 2016
LMCC Review:Pediatric Neurology
Asif Doja, MD, FRCP(C)
April 3rd, 2008
Outline
• Seizures
• Febrile Seizures
• Status Epilepticus
• Hypotonia in the Newborn and Cerebral Palsy
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 seizuresB. Absence seizuresC. Tonic-clonic seizuresD. 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 therapyB. Ketogenic DietC. CarbemazepineD. 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 AttacksB. Benign Epilepsy of Childhood with Rolandic
SpikesC. Juvenile Myoclonic EpilepsyD. 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– Syncope– Breath Holding– Night Terrors– Tics– GERD– etc
Syncope vs Seizure
• 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
Generalized• Generalized Tonic-
Clonic• Tonic• Clonic• Absence• Atonic• Myoclonic
How to differentiate “Staring Spells”
Complex Partial• Aura• ~ 30 sec or more• Decr LOC• Automatisms• Post-ictal period• EEG: focal epileptiform
abnormality• Hyperventialtion has no
effect
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
West Syndrome• Infantile Spasms• Onset in 1st year• Symmetrical
contractions of trunk/extremities
• EEG: hypsarrythmia• Poor prognosis
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 seizuresB. Absence seizuresC. Tonic-clonic seizuresD. 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 therapyB. Ketogenic DietC. CarbemazepineD. 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 AttacksB. Benign Epilepsy of Childhood with Rolandic
SpikesC. Juvenile Myoclonic EpilepsyD. 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 yearsB. Duration of < 15 minutesC. Only one seizure in 24 hour spanD. 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 epilepticusD. 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
Typical• Duration < 15 min• No focality• Does not recur in 24-
hour period• No hx of
developmental delay
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:– 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 yearsB. Duration of < 15 minutesC. Only one seizure in 24 hour spanD. 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 epilepticusD. 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%
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 BD. 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’sB. Stat CT headC. Lorazepam 0.1mg IV pushD. 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 BD. 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’sB. Stat CT headC. Lorazepam 0.1mg IV pushD. 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
Hypotonia and Cerebral Palsy
Question 1
The child in the preceding picture is alert, has little spontaneous movement and no reflexes. He most likely has:
A. Central HypotoniaB. Peripheral HypotoniaC. Mixed Central and Peripheral HypotoniaD. None of the above
Question 2
Which of the following on obstetrical history is NOT usually associated with hypotonia in the newborn?
A. Decreased fetal movement
B. Breech presentation
C. Jitteriness immediately after birth
D. Polyhydramnios
Question 3
The following would all be considered causes of peripheral hypotonia EXCEPT:
A. Spinal Muscular Atrophy
B. Neonatal Myasthenia Gravis
C. Myotonic Dystrophy
D. Trisomy 21
Question 4
With respect to Cerebral Palsy, which of the following statements is NOT correct?
A. 66% of cases are due to intrapartum asphyxia
B. Prematurity is a leading cause of the spastic diplegic form
C. in 1/3 of cases there is no etiology
D. A majority of patients have spasticity
Question 5
Which of the following is the most common form of cerebral palsy?
A. Spastic
B. Athetoid/Dystonic
C. Ataxic
D. Mixed
Hypotonia
• Decreased resistance to movement• “floppy baby”• Obstetrical/Perinatal History
Fetal movement– Breech presentation– Polyhydramnios– History of miscarriage– Resuscitation at birth
Hypotonia
• Check for resting posture “frog legged position” (indicates peripheral)
• Check infant’s postural tone– Traction response – pull to sit– Vertical (axillary) suspension– Horizontal (ventral) suspension
Hypotonia
Central
-“Floppy and Strong”
- preserved reflexes
-May not be alert
Peripheral
-“Floppy and Weak”
- absent reflexes
-alert
Hypotonia - Central
• Genetic– Trisomy 21, Prader-Willi
• Perinatal Problems– Perinatal asphyxia
• Infections – TORCH
Hypotonia - Peripheral
• Motor Neuron– Spinal Muscular Atrophy (SMA)
• Peripheral Nerve• Neuromuscular junction
– Neonatal mysathenia gravis
• Muscle– Congenital muscular dystrophy, myotonic
dystrophy
Cerebral Palsy
• Nonprogressive impairment of central motor function, caused by insult or anomaly of the immature CNS
• Only 10% due to intrapartum asphyxia• No etiology in 1/3 of cases• Can present with low tone initially then with
spastic tone– Velocity dependent increase in resistance to movement
Cerebral Palsy- Types
• Spastic– 70-80%– Can be spastic diplegia in prems (from
periventricular leukomalacia)– Quadriplegia with hypoxic-ischemic
encephalopathy (asphyxia)
Cerebral Palsy- Types
• Athetoid/Dystonic– 10-15%– Due to damage to basal ganglia
• Ataxic– <5%– Damage to cerebellum or thalamus
• Mixed– 10-15%
Question 1
The child in the preceding picture is alert, has little spontaneous movement and no reflexes. He most likely has:
A. Central HypotoniaB. Peripheral HtypotoniaC. Mixed Central and Peripheral HypotoniaD. None of the above
Question 2
Which of the following on obstetrical history is NOT usually associated with hypotonia in the newborn?
A. Decreased fetal movement
B. Breech presentation
C. Jitteriness immediately after birth
D. Polyhydramnios
Question 3
The following would all be considered causes of peripheral hypotonia EXCEPT:
A. Spinal Muscular Atrophy
B. Neonatal Myasthenia Gravis
C. Myotonic Dystrophy
D. Trisomy 21
Question 4
With respect to Cerebral Palsy, which of the following statements is NOT correct?
A. 66% of cases are due to intrapartum asphyxia
B. Prematurity is a leading cause of the spastic diplegic form
C. in 1/3 of cases there is no etiology
D. A majority of patients have spasticity
Question 5
Which of the following is the most common form of cerebral palsy?
A. Spastic
B. Athetoid/Dystonic
C. Ataxic
D. Mixed
Questions?