lmcc review: pediatric neurology

LMCC Review:Pediatric Neurology

Asif Doja, MEd, MD, FRCP(C)

March 27th, 2012

Outline

• 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 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

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 migraineD. 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 syndromeB. Paroxysmal TorticollisC. Cyclic Vomiting SyndromeD. Benign Paroxysmal VertigoE. All of the above are migraine variants in

childhood

Key Questions to ask on H/A Hx

• 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

• 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

• 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 location2. Pulsating quality3. Moderate to severe intensity4. Aggravation by routine physical activity

• D. During headache, at least 1 of:– 1. Nausea or vomiting – 2 . Photophobia or phonophobia

Migraine TreatmentAbortive

• 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

• 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 migraineD. 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 syndromeB. Paroxysmal TorticollisC. Cyclic Vomiting SyndromeD. Benign Paroxysmal VertigoE. All of the above are migraine variants in

childhood

Questions?