STATUS EPILEPTICUS IN CHILDRENDr:Abdelwhab M EL Sadany

MD PhD Pediatrics Mansoura G hospital

Definition of SE

The 30 minute definitionIt is a prolonged seizure that last more

than 30 minutes, orRecurrent seizures during which the

patient does not regain consciousness within a 30 min period.

The theory is that neuronal damage may begin after 30 minutes

Classification of SE

According to the presence or absence of motor activity:

Convulsive status epilepticus Nonconvulsive status epilepticusAccording to the cause Symptomatic, when there is a definite

cause Remote symptomatic with acute

precipitant. Idiopathic with unknown cause.

Convulsive SE

Convulsions that are associated with rhythmic jerking of extremities.

Generalized tonic clonic Focal seizures Myoclonic seizures Absence seizures

Non-Convulsive Status Epilepticus (NCSE)

Defined as seizure activity seen on electroencephalogram (EEG) without clinical findings associated with GCSE.

This patient presents with: Tonic eye deviation that often occurs in

the setting of acute brain injury). Prolonged post-ictal state Complex partial seizures without

recovery of consciousness

Refractory Status Epilepticus (RSE)

Patients who continue to experience either clinical or electrographic seizures after receiving adequate doses of an initial benzodiazepine followed by a second acceptable antiepileptic drug (AED) will be considered refractory.

Refractory Status Epilepticus

Super-refractory SE: SE that continues 24 hours or more after the onset of anesthesia, including those cases in which the SE recurs on reduction or withdrawal of anesthesia.

Etiology of SE

Febrile convulsion is the most common cause in children 52%

Epilepsy: First presentation Difficult seizure control Subtheraputic anticonvulant levels

Cerebrovascular disease Metabolic abnormalities Idiopathic Hypoxia CNS infections Trauma Poisoning

Investigations

Basic lab investigations CBC Blood gases Blood: Magnesium, electrolytes,

calcium, glucose, and creatinine

Investigations

Advanced lab investigations Metabolic investigations: ammonia Toxicology screening Anti-convulsants level ESR, RF, antineutrophil cytoplasmic

antibodies ANCA, serum complement. Lumbar puncture

Investigations

Brain imaging CT MRI MR angiography to diagnose CNS

vascultitis EEG PET CT to diagnose epileptogenic zone

Cortical hyperperfusion frontal right during NCSE. Bottom left: EEG shows right frontal spike-and-wave activity.

Prognosis of SE

MortalityConvulsive status epilepticusMortality ranges from 3 to 11 %Mortality in the first year is 17.8% and

24% in the first six months.NCSE: mortality is higher in those

diagnosed after 24 hours reaching 75%RSE: the mortality rate is high in

symptomatic SE 20%

Morbidity Of SE

Convulsive SE Severe neurological or cognitive sequelae:

11–16 %.RSE A new deficit occurrs in 36 Motor and visual deficits may be seen at 1

year after SE. Morbidity and mortality is highest in those

with symptomatic SE or a progressive encephalopathy

Pathophysiology of SE

The exact mechanism is still unknown Theoretically it is either failure of

phyisologic neuronal inhibition or an excess of excitation.

Clinically demonstrated by poor response of prolonged seizures to the GABA agonists benzodiazepines and barbiturates, a phenomenon called time-dependant pharmaco-resistance

Complications of SE

Cardiovascular compromise and hypotension

Hyperpyrexia Rhabdomyolysis that may lead to renal

failure Raised intracranial pressure Risk of hospital acquired infection Cardiac arrhythmias are also common

with higher mortality in these patients

Complications of SE

Lactic acidosis Stress cardiomyopathy Neurogenic pulmonary edema Fractures

Guidelines for the treatment of SE

Initially Assessment Management of airway, breathing, and

circulation: Secure the airways Administer O2 Obtain IV access

ABC

Maintain air way Intubate if

necessary Supplement oxygen Continuous

cardiovascular monitoring

Find vascular access

Treatment of SE

All protocols take a staged approach to treatment.

Typically, in Stage 1 (early status Epilepticus),therapy is with benzodiazepines.

If seizures continue despite this therapy, the patient is said to be in Stage 2 (established status Epilepticus) and therapy is with intravenous anti-epileptic drugs such as phenytoin, Phenobarbital or valproate.

Treatment of SE

If seizures continue despite this treatment for up to 1 h, the patient is said to be in Stage 3 (refractory status Epilepticus) and GA is indicated .

If seizure continue despite GA for >24h the patient is said to be in Super Refractory SE.

support ABC and give oxygenestablish IV access

rapid glucose check and consider critical lab Na, Ca Mgproceed with AED

5 minute

s Diazepam or Midazolam

IV 0.3mg/kg intranasal: 0.2 mg/kg

pr 0.5MG/KG buccal : 0.5mg/kg

IV max < 5 years 5mg/dose Max 5mg/nostril

> 5 years 10 mg/dose buccal max 10 mg5

minutes

phenytoin IV 20mg/kg in NS only over 5-10 minutes

max 1000 mg

5 minutes

phenobarbitalIV 20 mg.kg in NS or D5W over 20 minutes

or IV push over 5- 10 minutesmax 1000 mg

10minutes

RSE intubate if needed

arrange for admission to ICU

Midazolam continuous infusion

IV 0.15 mg/kg bolus then 2mic/kg/min infusion

Increase as need 2 mic/kg/min q5 min

Bolus 0.15 mg/kg with each increase

Max infusion 25mic/kg/min

Midazolam continuous infusion

IV 0.15 mg/kg bolus then 2mic/kg/min infusion

Increase as need 2 mic/kg/min q5 min

Bolus 0.15 mg/kg with each increase

Max infusion 25mic/kg/min

If no seizures within for 48 hrs

Taper midazolam

Decrease by 1 mic/kg/min q 15 min

If seizures persist

Thiopental continuous infusion via CVL2- 4 mg/kg bolus then2-4 mg/kg/hr Increase as needed by 1 mg/kg/hr every 30 minutesBolus 2 mg/kg with each increase Max infusion 6 mg/kg/hr Discontinue midazolam and phenobarbitone

If seizure recur, reinstate midazolam for another 48 hr

If no seizures for 48 hrs

Taper thiopenatal

Decrease rate by 25% q3 hrs

Reinstitute phenobarbitone while tapering

if seizures persist If seizures recur

Individualized therapy

Management of SE

Treat the underlying condition; meningitis, or increased intracranial pressure

Start AED to abort the electrical activityBenzodiazepine is the first line therapy Routes of administration IV, IM, buccal,

nasal, and rectalLorazepam preferred IVMidazolam preferred IM, can be given buccal

and nasalDiazepam preferred rectal

Management of SE

Urgent control therapy is required after short acting benzodiazepine

For patients who respond to emergent initialtherapy and have complete resolution of SE,

the goal is rapid attainment of therapeutic levels of an AED and continued dosing for maintenance therapy.

For patients who fail emergent initial therapy, the goal of urgent control therapy is to stop SE.

2nd stage of management

The preferred top tier agents that are generally used for urgent control of SE are

IV fosphenytoin/phenytoin valproate sodium phenobarbital Levetiracetam continuous infusion midazolam

Treatment of RSE

Treatment of RSE is extremely difficult

Continuous infusion of benzodiazepines Propofol Phenobarbitone General anasthetics Surgical treatment

High dose benzodiazepines

Midazolam infusion 2-24Mic/kg/min Advantages Rapid onset of action Short half life Lower incidence of cardiovascular

depression Disadvantage Tachyphylaxis High dose diazepam is also an alternative

Propofol

Advantage Rapid onset of action Short half life Disadvantage Rapid tapering can precipitate seizures Respiratory depression Prolonged usage produce “propofol

infusion syndrome” (metabolic acidosis, rhabdomyolysis, bradyrhythmias, lipemia, death”

Valproic acid

Intravenous valproic acid Caution with hepatic patientsDisadvatage Hyperammonemia in predisposed child Thrombocytopenia Fatal hepatotoxicity

Ketamine and lidocaine

Ketamine is NMDA antagonist that has anticonvulsants and neuroprotective properties

Lidocaine is another option although not commonly uses

Pyridoxine

Pyridoxine (vitamin B6) is a cofactor for both glutamic acid decarboxylase and GABA transaminase

Pyridoxine dependency is a rare disorder it presents in neonatal or infantile period but may present as lates as age 2.5 years.