Emergency managment of the pediatric patient with generalized convulsive status epilepticus

Question 1

What is the definition of convulsive status epilepticus (CSE)?

Answer 1

1. Continuous generalized tonic-clonic seizure activity with loss of consciousness for longer than 30min

OR 2. >2 discrete seizures without a return to baseline mental status

Question 2

What is “early” or “impending” status epilepticus?

Answer 2

Continuous or intermittent seizure lasting longer than 5min without full recovery of consciousness between seizures

Question 3

What is the annual incidence of CSE?

Answer 3

10-73/100 000 children

135-156/100 000 children <2yo

Question 4

What is the mortality of CSE?

Answer 4

2.7-8%

Question 5

What is the overall morbidity (mainly newly diagnosed neurological disorders) of CSE?

Answer 5

10-20%

Question 6

What are common acute symptomatic etiologies (17-52%) of CSE?

Answer 6

1. Acute CNS infection (bacterial meningitis, viral meningitis, or encephalitis)
2. Metabolic derangement (hypoglycemia, hyperglycemia, hyponatremia, hypocalcemia or anoxic injury)
3. Anti-epileptic drug noncompliance or withdrawal
4. Anti-epileptic drug overdose
5. Non-anti-epileptic drug overdose
6. Prolonged febrile convulsion (23-30%)

Question 7

What are common remote etiologies (16-39%) of CSE?

Answer 7

1. Cerebral migrational disorders (lissencephaly or schizencephaly)
2. Cerebral dysgenesis
3. Perinatal HIE
4. Progressive neurodegenerative disorders

Question 8

How common is idiopathic or cryptogenic CSE?

Answer 8

5-19%

Question 9

What are the objectives for the acute management of CSE?

Answer 9

1. Maintenance of ABCs
2. Termination of seizure and prevention of recurrence
3. Diagnosis and initial therapy of life-threatening causes of CSE
4. Arrangement of appropriate referral for ongoing care or transport to a secondary or tertiary care centre
5. Management of refractory status epilepticus

Question 10

When do ischemic and excitotoxic neuronal cell loss start to occur?

Answer 10

After 30 min of seizure

Question 11

At what time are seizures at risk of continuing for at least 30min?

Answer 11

After 5-10min

Question 12

What are pre-hospital management option for status epilepticus?

Answer 12

1. Lorazepam buccal/PR 0.1mg/kg (max 4mg)
2. Midazolam buccal 0.5mg/kg (max 10mg) OR intranasal 0.2mg/kg (max 5mg/nostril)
3. Diazepam PR 0.5mg/kg (max 20mg/dose)

Question 13

What are the first and second line options for a seizing patient with IV access?

Answer 13

1. Lorazepam IV 0.1mg/kg (max 4mg)
2. Midazolam IV 0.1mg/kg (max 10mg)
3. Diazepam 0.3mg/kg (max 5mg <5yo or 10mg >5yo)
   Repeat x1 within 5min

Question 14

What are the first and second line options for a seizing patient without IV access?

Answer 14

1. Lorazepam buccal/PR 0.1mg/kg (max 4mg)
2. Midazolam buccal 0.5mg/kg (max 10mg) OR intranasal 0.2mg/kg (max 5mg/nostril)
3. Diazepam PR 0.5mg/kg (max 20mg/dose)
   Repeat x 1 within 5min

Question 15

What are the third line options for a seizing patient with IV access?

Answer 15

1. Fosphenytoin IV 20mg/kg in NS or D5W (max 1000mg) over 5-10min
2. Phenytoin IV 20mg/kg in NS over 20min (max 1000mg)
3. Phenobarbital 20mg/kg in NS or D5W over 20min (max 1000mg)

Question 16

What are the fourth line options for a seizing patient with IV access?

Answer 16

Use phenobarbital if fosphenytoin/phenytoin already used OR

use fosphenytoin or phenytoin if phenobarbital already used

Question 17

What are the third line options for a seizing patient WITHOUT IV access?

Answer 17

1. Fosphenytoin IM 20mg/kg (max 1000mg)
2. Paraldehyde PR 400mg/kg (0.4mL/kg/dose) max 10g (10mL) dilute 1:1 in oil
3. Phenytoin IO 20mg/kg in NS (max 1000mg)

Question 18

What are the fourth line options for a seizing patient WITHOUT IV access?

Answer 18

Use paraldehyde as above if fosphenytoin/phenytoin used OR

use fosphenytoin or phenytoin if paraldehyde used already

Question 19

What should be used for refractory status epilepticus?

Answer 19

1. RSI
2. Midazolam continuous infusion
3. Thiopental/Pentobarbital bolus, continuous infusion

Question 20

When should hypoglycemia be treated?

Answer 20

<2.6mmol/L

Question 21

What is the recommended management of hypoglycemia in CSE?

Answer 21

1. 2-4mL/kg of D25W IV

2. 5mL/kg D10W IV

Question 22

What are the SE of benzodiazepines?

Answer 22

1. Respiratory depression
2. Hypotension
3. Sedation

Question 23

What are the side effects of fosphenytoin?

Answer 23

1. Hypotension
2. Bradycardia
3. Arrhythmia

Question 24

What are the side effects of phenytoin?

Answer 24

1. Hypotenstion
2. Bradycardia
3. Arrhythmia
4. “Purple glove” syndrome from extravasation

Question 25

What are the SE of phenobarbital?

Answer 25

1. Respiratory depression
2. Hypotension
3. Sedation

Question 26

What are the SE of paraldehyde?

Answer 26

1. Mucosal irritation

Question 27

What other medication can be considered in CSE?

Answer 27

Pyridoxine (vitamin B6) 100mg IV, then 50mg IV/PO BID

Question 28

What investigations should be considered in CSE?

Answer 28

1. Electrolytes
2. Glucose
3. CBC + diff
4. Cultures (if sepsis suspected)
5. Cap or art gas
6. Anticonvulsant levels
7. Urine and blood for tox screen
8. Serum Ca, BUN, Mg, liver enzymes, lactate, NH4
9. LP

Question 29

What are reasons for head CT in the context of CSE?

Answer 29

1. history of trauma
2. evidence of ICP
3. focal neurological signs
4. unexplained LOC
5. suspicion of cerebral herniation

Question 30

When should non-CSE be expected?

Answer 30

If LOC does not recover as expected post convulsion do EEG to exclude non-CSE

Question 31

When should a child be referred to a secondary or tertiary care hospital?

Answer 31

1. Children without history of previous epilepsy or febrile seizures

Question 32

When should children be transported to a PICU?

Answer 32

1. Unstable vital signs

2. Continuing CSE

Question 33

What is the definition of refractory status epilepticus (RSE)?

Answer 33

1. CSE unresponsive to different anti-epileptic medications

2. Duration >30-60min

Question 34

What are other medication options for RSE?

Answer 34

1. Propofol
2. Topiramate
3. Levetiracetam