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?