Pediatric Neurology: Seizures--Pediatric Syndromes

Front 1

What is a common presentation of Benign Rolandic Epilepsy?

Back 1

A child wakes up in the morning with UNILATERAL grimacing, facial twitching, drooling, or swallowing, while she/he makes a disturbing "gurgling" noise. Frequently the spell will develop into generalized convulsions.

Front 2

At what ages does benign Rolandic epilepsy present for the first time?

Back 2

From age 3 to 10 years

Front 3

Name 3 features describing the seizures typical of benign Rolandic epilepsy.

Back 3

1. Partial seizures, with or without secondary generalization

2. Early morning or nocturnal

3. Short lasting (< 2')

Front 4

1. What EEG findings are associated with benign Rolandic epilepsy?

2. What is the best way to capture these findings?

Back 4

1. Unilateral Central-temporal (C-T) spikes, rarely bilateral

2. Best discovered using a 24-hour study

Front 5

What are the typical findings on physical evaluation with benign Rolandic epilepsy?

Back 5

None; the exam is normal

Front 6

What is likely to be found on MRI in a patient with benign Rolandic epilepsy?

Back 6

MRI is generally normal.

Front 7

What is the usual outcome for benign Rolandic epilepsy?

Back 7

Patients should outgrow it in the second decade of life, around puberty.

Front 8

What cognitive defects are found with benign Rolandic epilepsy?

Back 8

Generally, they are cognitively normal between spells, but cognitive dysfunction may occur at the time of the spikes.

Front 9

Name 4 signs one might see during the "aura" of benign Rolandic epilepsy .

Back 9

1. Unilateral facial twitching or grimacing

2. "Gurgling" noises, likely related to impaired swallowing.

3. Drooling

4. Inability to speak

Front 10

What are "cryptogenic" infantile spasms? And what is their prognosis?

Back 10

These spasms have a "hidden" cause, or in other words, no brain lesion can be found. The prognosis for normal psychomotor development and cognitive functioning is much better.

Front 11

What vitamin deficiency is related to infantile spasms?

Back 11

Pyridoxine (B6)

Front 12

Name 2 first line meds to treat infantile spasms.

Name 4 second line meds.

Back 12

First line:

1. Vigabatrin

2. ACTH

Second line:

1. Topiramate

2. Valproate

3. Benzodiazepine

4. Zonisamide

Front 13

How should we handle refractory cases of infantile spasms that won't respond to any med?

Back 13

Look carefully for a single focus that might be removed surgically. If a MRI is negative, consider a PET scan looking for focal areas of hypometabolism.

Front 14

Name 3 seizure types that can be observed in a patient with Lennox-Gastaut syndrome.

Back 14

1. Atonic (drop)

2. Myoclonic

3. Tonic

Front 15

Name 3 common features of Lennox-Gastaut syndrome.

Back 15

1. Developmental delay

2. MIXED SEIZURE TYPES

a. Drop

b. Myoclonic

c. Tonic

3. Peculiar EEG finding: "slow" spike and wave patterns

Front 16

How might the kids with Lennox-Gastault syndrome be distinguished from the rest in the waiting room of the neuro clinic?

Back 16

They are the kids wearing helmets to protect them from the head injuries their drop seizures would cause.

Front 17

1. The EEG findings in Lennox-Gastault syndrome resemble those of which other seizure type?

2. How can they be distinguished?

Back 17

1. Absence epilepsy

2. In absence, the spike-and-wave patterns are at a constant 3 Hz, and the patients are cognitively normal. In Lennox-Gastault, the spike-waves are "slower," at 1 to 2.5 Hz.

Front 18

The mixed seizures of Lennox-Gastault are notoriously difficult to control. Name 5 different interventions that have been tried.

Back 18

1. Felbamate

2. Ketogenic diet

3. Vagal nerve stimulator

4. Corpus callosotomy

5. Helmet for atonic seizures

Front 19

What are "cryptogenic" infantile spasms? And what is their prognosis?

Back 19

These spasms have a "hidden" cause, or in other words, no brain lesion can be found. The prognosis for normal psychomotor development and cognitive functioning is much better IF DISCOVERED AND TREATED.

Front 20

Where do infantile spasms fall in the classification of seizures? (2 points)

Back 20

1. Generalized seizures

2. Myoclonic

Front 21

Name 3 features describing the seizures associated with infantile spasms.

Back 21

1. Sudden, brief jerks involving head, trunk, arms, or legs

2. These can flexion spasms, extensor spasms, or both

3. Come in clusters, often on waking up

Front 22

At what age(s) do infantile spasms appear?

Back 22

3-8 months of age

Front 23

Name 5 seizure types that can be observed in a patient with Lennox-Gastaut syndrome.

Back 23

1. Atonic (drop)

2. Myoclonic

3. Tonic

4. Tonic-clonic

5. Atypical absence

Front 24

Often, infantile spasms can be mistaken for something else. Name 4 of these conditions.

How might these benign entities be distinguished from infantile spasms?

Back 24

1. Colic

2. GERD

3. Startle

4. Hiccups

These benign conditions do NOT happen in clusters.

Front 25

1. In infantile spasms, how many short seizures can make up a "cluster"?

2. How many clusters of such seizures can happen in a day?

Back 25

1. Up to 150 seizures can be counted in a cluster.

2. Up to 60 clusters can be counted in a day.

Front 26

What are "symptomatic" infantile spasms?

Back 26

These are spasms that can be related to a brain lesion. The prognosis for normal psychomotor development is bad.

Front 27

Name 4 types of disorders with CNS lesions associated with "symptomatic" infantile spasms.

Back 27

1. Neurocutaneous disorders, including tuberous sclerosis

2. Perinatal injuries

3. Chromosomal disorders, e.g. Down syndrome

4. Brain malformations, notably cortical dysphasias (that, because of age-specific decreased myelinization, might not be visible on MRI in the early stages.

Front 28

In the management of benign Rolandic seizures, what else needs to be monitored?

Back 28

School performance: the seizures can interfere with normal sleep, thus with the processing of what was learned the day before. The effects can manifest as ADD, other cognitive issues requiring meds.

Front 29

What might have happened the night before an episode of Rolandic seizures?

Back 29

Sleep deprivation: they often occur the morning after a wedding or a sleepover.

Front 30

What is the relationship between infantile spasms and Lennox-Gastault syndrome?

Back 30

About 30-40% of babies with infantile spasms will grow up to become kids with Lennox-Gastaut syndrome.

Front 31

Name 3 behavioral problems associated with Lennox-Gastaut syndrome.

Back 31

1. Hyperactivity

2. Psychosis

3. Autistic behaviors

Front 32

Name 4 salient features of the Landau-Kleffner syndrome.

Back 32

1. "Acquired" aphasia after 4-6 years of normal language development

2. Some seizures with epileptiform EEG, notably at night.

3. Appearance of memory and learning problems in early school age

4. "Autistic" features in early school age.

Front 33

Autism and Landau-Kleffner syndrome are easy to confuse. How might history-taking distinguish them?

Back 33

In classic autism, speech regression starts as early as 18 mos; in Landau-Kleffner, the loss of speech development doesn't happen before 4-6 years. (Even so, LK syndrome can appear as early as 18 months of age!)

Front 34

Name 2 important problems in the management of Landau-Kleffner Syndrome.

Back 34

1. Seizure management--relatively easy

2. Memory and learning are the BIG problems here, because the day's learning is integrated into sleep.

Front 35

Considering Landau-Kleffner syndrome:

1. What should you expect on the PE?

2. And on the MRI?

3. What about the EEG?

Back 35

1. The exam is non-focal

2. MRI is normal

3. Best seen during sleep: expect bitemporal wave anomalies and at worst "electric status epilepticus of sleep"

Front 36

Are all aspects of speech equally affected in Landau-Kleffner syndrome? If not, where might there be differences?

Back 36

In general, though not always, receptive speech is worse affected than expressive speech: this goes a long way toward explaining the autistic features.

Front 37

Name 2 good reasons for ordering an EEG for every autistic kid.

Back 37

1. Check for Landau-Kleffner syndrome

2. 1/3 kids with classical autism will have seizures in their own right.

Front 38

Name 2 groups of seizure meds useful in the management of Landau-Kleffner related seizures.

Back 38

1. Prednisone--in courses as needed

2. Benzodiazepines (e.g., Valium)

Front 39

How might Landau-Kleffner syndrome be managed by surgery if drugs don't help? Under what circumstances?

Back 39

If the EEG shows mostly unilateral anomalies, consider multiple subpial transections

Front 40

Name 4 features that distinguish the tonic-clonic and atonic seizures of Panayiotopoulos syndrome.

Back 40

1. Sudden onset of autonomic symptoms resembling migraine

2. Development of seizures afterward

3. Occur during sleep in 2/3 cases

4. Seizures usually last over 10 minutes, 44% develop status lasting over 30 minutes

Front 41

Name 3 kinds of seizures appearing in Panayiotopoulos syndrome. What do they have in common?

Back 41

1. Unilateral tonic-clonic seizures

2. Secondarily generalized tonic-clonic seizures

3. Prolonged atonic seizures (ictal syncope)

All, of course, focal in etiology

Front 42

What is the usual age of onset of Panayiotopoulos syndrome?

Back 42

3-6 years old

Front 43

Name 3 of the autonomic symptoms encountered in a episode of the Panayiotopoulos syndrome.

Back 43

1. Vomiting

2. Facial pallor

3. Eye deviation

Front 44

What EEG findings are associated with Panayiotopoulos syndrome?

Back 44

1. High amplitude sharp-slow wave complexes

2. Locations shift, can be posterior or multifocal

Front 45

Name 4 drugs useful in the treatment of recurrent seizures caused by Panayiotopoulos syndrome.

Back 45

1. Oxcarbazepine

2. Valproate

3. Clobazam

4. Keppra (leviteracetam)

Keep rescue med at hand

Front 46

What is the prognosis for resolution of Panayiotopoulos syndrome?

Back 46

Often resolves within 1-2 years of onset, always before 12 years old.

Front 47

Name 2 of the seizure types seen with the syndrome of "benign infantile seizures

Back 47

May be single isolated seizures or clusters

1. Generalized tonic-clonic

2. Focal with secondary generalization

Front 48

Name the typical age range in which benign infantile seizures first appear?

Back 48

2 mos to 2 years

Front 49

What other symptom has been associated with benign infantile seizures?

Back 49

Mild diarrhea (Japan)

Front 50

What distinguishes "benign" infantile seizures from other seizures affecting infants?

Back 50

These do not develop into epilepsy.

Front 51

What lab study may be useful in managing benign infantile seizures?

Back 51

Chromosome study looking for PRRT2 mutations

Front 52

Name 3 drugs useful in the management of RECURRENT benign infantile seizures.

Back 52

1. Trileptal (oxycarbazepine)

2. Keppra (leviteracetam)

3. Rescue med for prolonged break-through seizures