Seizures in Pediatric Patients-Swartz

Question 1

A (blank) is a phenomenon of heightened neuronal excitability and depolarization which spreads to neighboring neurons, columns, gyri, lobes to produce a loss of function with or without obvious clinical manifestations.

Answer 1

seizure

Question 2

What is epilepsy?

Answer 2

• At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart. (ie have 2 seizures and dont find an abnormality still considered epilepsy)
• One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrency risk after 2 unprovoked seizures (at least 60%), occurring over the next 10 years. (ie if you have a seizure and some sort of abnormality, treat as epilepsy)

Question 3

How do you diagnose epilepsy?

Answer 3

Constellation of signs and symptoms with known etiology, pathophysiology and outcome

Question 4

What seizures are seen in childhood?

Answer 4

• neonatal seizures (unique etiologies and semiologies)
• febrile seizures
• epileptic encephalopathies
• metabolic syndromes
• genetic syndromes-

Question 5

What are semiologies?

Answer 5

progression of clinical signs that occur during the course of a seizure

Question 6

How are seizures in children similiar to seizures in adults?

Answer 6

you need ictal recordings for diagnosis and prognosis-CEEG

• importance of semiology (for diagnosis and prognosis)
• Need neuroimaging
• need for rapid initiation of treatment

Question 7

What neuroimaging do you want to use for epilepsy?

Answer 7

MRI (80% effective)

CT (50%))

Question 8

What is the prevalence of epilepsy in children?

Answer 8

up to 5% overall

Question 9

Epilepsy increases in (blank) years in third world countries

Answer 9

teen

Question 10

What is an acute symptomatic seizure?

Answer 10

seizure following head trauma within 24 hours

Question 11

What is the lifetime incidence of epilepsy?

Answer 11

1/26

Question 12

What is the incidence of epilepsy in children at birth? at three? stabilizes at?

Answer 12

Incidence 0.2% at birth. Drops to 0.1% age three and stabilizes at .05%. (USA)

Question 13

When do neonatal seizures typically occur?

Answer 13

Occur up to 28 days post term but usually in first week of life

Question 14

You can differentiate the motor phenomena of seizures from release phenomenon by whether the movement(s) is induced by (blank) or (blank).
If it is a seizure what should you do?

Answer 14

stimulation (noise, tactile, passive movement) or stopped by repositioning.

treat specifically and with AED

Question 15

What is this:

Repetitive rhythmic jerking of a limb, face or trunk

Answer 15

focal clonic-epileptic

Question 16

What is this:

Sustained posturing of a limb, eye deviation, assymetric trunk

Answer 16

focal tonic-epileptic

Question 17

What does a generalized tonic non-epileptic seizure cause?

Answer 17

sustained, symmetric posturing in the limbs, neck and trunk. Can effect or flexor, extensors or both.

Question 18

What is a generalized tonic non-epileptic seizure commonly caused by?

Answer 18

GERD

Question 19

Is myoclonic epileptic or non epileptic?

Answer 19

can be beoth

Question 20

Are spasms epiletic or nonepileptic?

Answer 20

epileptic

Question 21

What is this:

eye movements, oral – buccal, semipurposeful, complex purposeless – Non-epileptic

Answer 21

motor automatisms

Question 22

What causes neonatal seizures?

Answer 22

• hypoxic-ischemic pre-or perinatal insult
• infection
• intracranial hemorrhage
• congenital CNS abnormality
• electrolyte disturbance
• inborn errors of metabolism
• toxins
• genetics

Question 23

What infections can cause neonatal seizures?

Answer 23

septicemia
meningitis
meningoencephalitis
HIV

Question 24

What electrolyte disturbances can cause neonatal seizures?

Answer 24

Ca, Na, Mg, Glu

Question 25

What inborn error of metabolism can cause neonatal seizures?

Answer 25

pyridoxine

Question 26

What toxins can cause neonatal seizures? | What genetic causes can cause neonatal seizures?

Answer 26

Toxins – bilirubin, medications, drugs in mother | Genetic – BNFC, chromosomal, Leigh’s,

Question 27

How do you treat neonatal seizures?

Answer 27

``` Treat etiology (hypocalcemia, etc) Treat with an AED or ASD (anti-seizure drug) ```

Question 28

What three seizures may not require AED?

Answer 28

- brief and infrequent focal motor | - tonic or myoclonic seizure

Question 29

When can you withdraw AED?

Answer 29

two week after the last seizure if EEG is negative

Question 30

What is the first line seizure med? | What are other ones and what are their downfalls?

Answer 30

Levetiracetam - phenobarbital (IQ depression) - phenytoin (hemorrhagic disease of the newborn)

Question 31

If given drugs and patient is still seizing, what do you give them?

Answer 31

Ativan or midazolam + high dose of phenobarbital or Diphenhydramine

Question 32

What is a new AED?

Answer 32

lacosamide

Question 33

What is the most common seizure of childhood? When age does it occur? How long do they last?

Answer 33

Febrile seizures (a type of generalized tonic clonic seizure) 3 months to 5 years less than 10 minutes

Question 34

Do febrile seizures recur? How long do they last? Are they partial or generalized at onset? How do you treat these? A very small amount of febrile seizures are recurrent, how do you treat these?

Answer 34

``` no 10 minutes generalized Usually self-limitied, Benzo (ativan, midazolam) Home diastat (rectal valium) ```

Question 35

For a child having febrile seizures, when should you do a lumbar puncture?

Answer 35

in children less than 1 yr old.

Question 36

A febrile seizure is a seizure that has a fever (blank) an INFECTION of the CNS

Answer 36

WITHOUT

Question 37

What are risk factors for recurrent FS?

Answer 37

- first seizure at <1 yr - FS following low grade or brief fever - epilepsy in 1st degree relative - complex febrile seizures - neurodevelopmental abnormalities

Question 38

What are risk factors for FS becoming epilepsy?

Answer 38

- abnormal neonatal hx | - family hx of retardation

Question 39

What are syndromes of recurrent seizures associated with regression of development milestones? What do these cause?

Answer 39

``` Ohtahara Syndrome Dravet Syndrome West Syndrome Lennox Gastaut Syndrome Landau Klefner ESES ``` Epileptic encephalopathies We (west) Left (Lennox) Essex (ESES) for the land (Laundau) of the Ohtahara Dragon (Dravet)

Question 40

What is this: - EEG characterized by suppression-burst pattern - seen in neonatal period (10da-3 months) - seizures are tonic spasms, drop attacks and partion (rare myoclonus can also occur) What is the prognosis like? How do you treat it?

Answer 40

Ohtahara's Syndrome or EIEE (early infantile epileptic encephalopathy) Very poor (100% Intellectual disability) Tx: ACTH, steroids, Valproic acid (depakote), ketogenic diet, Topiramate, Clobazam, Felbamate

Question 41

What is this: unilateral clonic seizures, febrile and afebrile in 1st year of life in previously normal infant. Recur at 6-8 wk intervals. May cause STATUS (continual seizing)

Answer 41

Dravet's syndrome (SEI, severe myoclonic epilepsy of infancy)

Question 42

What is will increase you chances of getting Dravets syndrome? Is this severe? What is the molecular defect associated with this?

Answer 42

Family hx of epilepsy or FS YES defects in 3 Na channel subunit genes and a GABA subunit gene

Question 43

What will make Dravet's syndrome worse?

Answer 43

- sodium channel blockers!! DO NOT GIVE THESE - carbamazepine - diphenhydramine - oxcarbazepine - lamotrigine

Question 44

What is this: characterized by infantile spasms-brief bilateral contraction of muscles of neck, trunk and extremities -onset in first 4-8 months of life.

Answer 44

``` West Syndrome (SEEI) Severe encephalopathic Epilepsy in Infants ```

Question 45

What is this: EEG – hypsarrhythmia, Slow waves or slow SW, BS, rarely normal Can be misdiagnosed as Morrow reflex, colic, startle responses 85% symptomatic; 15% cryptogenic (with or without abnormal MRI "jack-knife seizures"

Answer 45

``` West Syndrome (SEEI) Severe encephalopathic Epilepsy in Infants ```

Question 46

What is this: Triad of slow spike-wave on EEG, mental retardation and mixed seizure types – myoclonic jerks, atypical absences and drop attacks (tonic or atonic).

Answer 46

Lennox Gastaut Syndrome

Question 47

What is this: Mean age = 2 y/o, usually preschool (10% of sz up to age 5) but 1-14 y/o = range. Males>females Drop attacks have “electrodecremental response” on EEG

Answer 47

Lennox Gastaut syndrome

Question 48

``` What is this: Ages 3-9 y/o, Males > Females EEG shows multifocal SW Child develops verbal agnosia and decreased spontaneous speech Remits before age 15 Etiology unknown Seizures may not be prominent feature Treatment – steroids, KD, felbamate, early speech therapy. 40-50% recover to a normal life. ```

Answer 48

Landau-Klefner syndrome

Question 49

What is this: EEG – some bursts of SW while awake become continuous in sleep (1.5-2Hz) Seizures – partial, generalized, atypical absence Normal development until onset – may return to near normal with control of Spike waves Rx - ? CBZ, TOP, ACTH, VPA

Answer 49

Electrical Status Epilepticus During Sleep

Question 50

What is this: Bihemispheric initial involvement of 3 Hz GSW lasting 3-10 sec Average age of onset 5.7 yrs (3-9 y/o) Risk factors – febrile sz; family Hx Medications – ETX(Ethosuximide), LTG, LEV, VPA, TOP, ZON Typical = normal IQ, faster SW (3-3.5 Hz), normal background 65% remit by puberty; 15% evolve to JME; Atypical – slower SW – 1.5-3Hz, abnormal background, lower IQ’s,

Answer 50

Absence seizures

Question 51

What are the three types of absence seizures?

Answer 51

``` childhood absence (3-5 y/o) juvenile absence (puberty) Juvenile myoclonic epilepsy (12-18 y/o) ```

Question 52

What is the most common epilepsy syndrome?

Answer 52

Juvenile myoclonic epilepsy

Question 53

What is this: Most common epilepsy syndrome Possibly 20% of all epilepsies Hallmark is myoclonic jerks, fast GSW (4-6 Hz) normal IQ and development Lifelong (maybe 10% remit) Onset 5 – absence in 20%, 8-10 – myoclonus, 15 – GTC -60% Rx – LEV, VPA, LTG, TOP, ZON, CLNZ, LAC(locasamide)? Several associated gene loci – myoclonic gene involved in spindle body directing cell division and neuronal migration

Answer 53

Juvenile myoclonic epilepsy

Question 54

What is this: 13-23 % of all childhood epilepsies = most common When does this remit?

Answer 54

Benign Focal Epilepsy of Childhood (BFEC or Rolandic epilepsy) -by age 16

Question 55

What is this: Onset 3-13 y/o, peak 7-8 years Normal NE, IQ, MRI and EEG background GTC’s occur in sleep, 15% have in day time also Partial seizures are unilateral paresthesias +/- clonus of face, lips, tongue. Also salivation, speech arrest but preserved consciousness. EEG – centro-temporal spikes, high amplitude, triphasic Is it genetic?

Answer 55

BFEC | Yes

Question 56

What is this: Benign occipital; spikes occipital or anterior With autonomic onset, autonomic status,

Answer 56

Panayiotopolus syndrome

Question 57

What is this: CTSW bilateral. Focal ones produce focal atonias, when they generalize produce atonic drops. Nocturnal seizures = Rolandic, GTC or jerks. EEG in sleep = ECSWS. All recover

Answer 57

Atypical Benign Partial Epilsepy of Childhood

Question 58

What is this: Boys > Girls. Peak age 2-3. Seizures can appear throughout adulthood LAUGHTER W/OUT EMOTION 10-30 sec, frequent Can have autonomic, complex partial GTC as well. Can be associated with precocious puberty, behavioral and cognitive difficulties

Answer 58

Hypothalamic Hamartomas (gelastic) Epilepsy. Rare