Status epilepticus in Children

Pediatric emergencies

Clinicals - History

Fact Explanation
Unresponsive Acute processes that cause status epilepticus include metabolic disturbances (e.g., electrolyte abnormalities, renal failure, and sepsis), central nervous system infection, stroke, head trauma, drug toxicity, and hypoxia. These can cause unresponsiveness. It can also occur due to the seizure
if left untreated, status epiliepticus is potentially fatal or can lead to irreversible brain damage
Unresponsive
Acute processes that cause status epilepticus include metabolic disturbances (e.g., electrolyte abnormalities, renal failure, and sepsis), central nervous system infection, stroke, head trauma, drug toxicity, and hypoxia. These can cause unresponsiveness. It can also occur due to the seizure
if left untreated, status epiliepticus is potentially fatal or can lead to irreversible brain damage
Seizures / Jerky movements of limbs The fundamental pathophysiology of status epilepticus involves a failure of mechanisms that normally abort an isolated seizure. This failure can arise from abnormally persistent, excessive excitation or ineffective recruitment of inhibition.Child can have tonic, clonic, or tonic–clonic movements of the extremities Seizures / Jerky movements of limbs
The fundamental pathophysiology of status epilepticus involves a failure of mechanisms that normally abort an isolated seizure. This failure can arise from abnormally persistent, excessive excitation or ineffective recruitment of inhibition.Child can have tonic, clonic, or tonic–clonic movements of the extremities
Twitching movements of the body Initially the child will have clinically obviou seizures.With time, however, the clinical manifestations often become subtle, and the diagnosis requires careful observation.Patients may have only small-amplitude twitching movements of the face, hands, or feet or nystagmoid jerking of the eyes Twitching movements of the body
Initially the child will have clinically obviou seizures.With time, however, the clinical manifestations often become subtle, and the diagnosis requires careful observation.Patients may have only small-amplitude twitching movements of the face, hands, or feet or nystagmoid jerking of the eyes
History of epilepsy Chronic processes that cause status epilepticus include preexisting epilepsy in which status epilepticus is due to breakthrough seizures or the discontinuation of antiepileptic drugs History of epilepsy
Chronic processes that cause status epilepticus include preexisting epilepsy in which status epilepticus is due to breakthrough seizures or the discontinuation of antiepileptic drugs
History of head trauma Acute processes that cause status epilepticus include head trauma History of head trauma
Acute processes that cause status epilepticus include head trauma
Fever Central nervous system infection can cause status epilepticus
Hyperpyrexia can also occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures
Fever
Central nervous system infection can cause status epilepticus
Hyperpyrexia can also occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures
Palpitations Occur due to arrhythmias.
Generalized convulsive status epilepticus is associated with serious systemic physiologic changes resulting from the metabolic demands of repetitive seizures. Many of these systemic changes result from the profound autonomic changes that occur during status epilepticus, including tachycardia, arrhythmias, hypertension, pupillary dilation, and hyperthermia because of the massive catecholamine discharge associated with continuous generalized seizures
Palpitations
Occur due to arrhythmias.
Generalized convulsive status epilepticus is associated with serious systemic physiologic changes resulting from the metabolic demands of repetitive seizures. Many of these systemic changes result from the profound autonomic changes that occur during status epilepticus, including tachycardia, arrhythmias, hypertension, pupillary dilation, and hyperthermia because of the massive catecholamine discharge associated with continuous generalized seizures
Vomiting Occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures Vomiting
Occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures
Incontinence Occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures Incontinence
Occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures

Clinicals - Examination

Fact Explanation
Unresponsive child Acute processes that cause status epilepticus include metabolic disturbances (e.g., electrolyte abnormalities, renal failure, and sepsis), central nervous system infection, stroke, head trauma, drug toxicity, and hypoxia. These can cause unresponsiveness. It can also occur due to the seizure Unresponsive child
Acute processes that cause status epilepticus include metabolic disturbances (e.g., electrolyte abnormalities, renal failure, and sepsis), central nervous system infection, stroke, head trauma, drug toxicity, and hypoxia. These can cause unresponsiveness. It can also occur due to the seizure
Seizures The fundamental pathophysiology of status epilepticus involves a failure of mechanisms that normally abort an isolated seizure. This failure can arise from abnormally persistent, excessive excitation or ineffective recruitment of inhibition.Child can have tonic, clonic, or tonic–clonic movements of the extremities Seizures
The fundamental pathophysiology of status epilepticus involves a failure of mechanisms that normally abort an isolated seizure. This failure can arise from abnormally persistent, excessive excitation or ineffective recruitment of inhibition.Child can have tonic, clonic, or tonic–clonic movements of the extremities
Twitching movements of the body Initially the child will have clinically obviou seizures.With time, however, the clinical manifestations often become subtle, and the diagnosis requires careful observation.Patients may have only small-amplitude twitching movements of the face, hands, or feet or nystagmoid jerking of the eyes Twitching movements of the body
Initially the child will have clinically obviou seizures.With time, however, the clinical manifestations often become subtle, and the diagnosis requires careful observation.Patients may have only small-amplitude twitching movements of the face, hands, or feet or nystagmoid jerking of the eyes
Febrile Central nervous system infection can cause status epilepticus
Hyperpyrexia can also occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures
Febrile
Central nervous system infection can cause status epilepticus
Hyperpyrexia can also occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures
Signs due to autonomic changes - tachycardia, arrhythmias, hypertension, pupillary dilation Generalized convulsive status epilepticus is associated with serious systemic physiologic changes resulting from the metabolic demands of repetitive seizures. Many of these systemic changes result from the profound autonomic changes that occur during status epilepticus, including tachycardia, arrhythmias, hypertension, pupillary dilation, and hyperthermia because of the massive catecholamine discharge associated with continuous generalized seizures Signs due to autonomic changes - tachycardia, arrhythmias, hypertension, pupillary dilation
Generalized convulsive status epilepticus is associated with serious systemic physiologic changes resulting from the metabolic demands of repetitive seizures. Many of these systemic changes result from the profound autonomic changes that occur during status epilepticus, including tachycardia, arrhythmias, hypertension, pupillary dilation, and hyperthermia because of the massive catecholamine discharge associated with continuous generalized seizures
Neurological examination May show focal neurological signs (eg : uni lateral limb paralysis ) Neurological examination
May show focal neurological signs (eg : uni lateral limb paralysis )

Investigations - Diagnosis

Fact Explanation
Electroencephalography (EEG) EEG may be considered in children presenting with new-onset status epilepticus because it can help to determine whether there are focal or generalized abnormalities, which can influence diagnosis and treatment Electroencephalography (EEG)
EEG may be considered in children presenting with new-onset status epilepticus because it can help to determine whether there are focal or generalized abnormalities, which can influence diagnosis and treatment
Blood cultures To identify sepsis.In six studies (n = 357) that reported on sepsis, a minimal diagnostic yield of a positive blood culture was found in 2.5 percent of children with status epilepticus.
The evidence is insufficient to support or refute whether blood cultures should be obtained routinely in children with no clinical suspicion of infection.
Blood cultures
To identify sepsis.In six studies (n = 357) that reported on sepsis, a minimal diagnostic yield of a positive blood culture was found in 2.5 percent of children with status epilepticus.
The evidence is insufficient to support or refute whether blood cultures should be obtained routinely in children with no clinical suspicion of infection.
Lumbar puncture Data from 18 studies (n = 1,859) showed that the diagnosed CNS infection rate was 12.8 percent. According to the results of one study involving 49 children with convulsive status epilepticus, 24 children (49 percent) had a fever, and 17 percent of those had bacterial meningitis; none of the children without fever had meningitis.
The evidence is insufficient to support or refute whether lumbar puncture should be performed routinely in children with no clinical suspicion of CNS infection
Lumbar puncture
Data from 18 studies (n = 1,859) showed that the diagnosed CNS infection rate was 12.8 percent. According to the results of one study involving 49 children with convulsive status epilepticus, 24 children (49 percent) had a fever, and 17 percent of those had bacterial meningitis; none of the children without fever had meningitis.
The evidence is insufficient to support or refute whether lumbar puncture should be performed routinely in children with no clinical suspicion of CNS infection
Antiepileptic drug levels Antiepileptic drug levels should be considered when a child with epilepsy on antiepileptic prophylaxis develops status epilepticus as discontinuation of the drugs can cause status epilepticus Antiepileptic drug levels
Antiepileptic drug levels should be considered when a child with epilepsy on antiepileptic prophylaxis develops status epilepticus as discontinuation of the drugs can cause status epilepticus
Neuroimaging - computed tomography / Magnetic resonance imaging brain Neuroimaging may be considered if clinically indicated or if the etiology of status epilepticus is unknown. It should be used only after the seizures are under control and the patient is stabilized. The data are insufficient to support or refute routine use of neuroimaging. Neuroimaging can identify structural causes of status epilepticus and eliminate the need for neurosurgical interventions in children with new-onset status epilepticus and no history of epilepsy, or in those in whom status epilepticus persists despite treatment Neuroimaging - computed tomography / Magnetic resonance imaging brain
Neuroimaging may be considered if clinically indicated or if the etiology of status epilepticus is unknown. It should be used only after the seizures are under control and the patient is stabilized. The data are insufficient to support or refute routine use of neuroimaging. Neuroimaging can identify structural causes of status epilepticus and eliminate the need for neurosurgical interventions in children with new-onset status epilepticus and no history of epilepsy, or in those in whom status epilepticus persists despite treatment

Investigations - Management

Fact Explanation
Neuroimaging - computed tomography / Magnetic resonance imaging brain Neuroimaging may be considered if clinically indicated or if the etiology of status epilepticus is unknown. It should be used only after the seizures are under control and the patient is stabilized. The data are insufficient to support or refute routine use of neuroimaging. Neuroimaging can identify structural causes of status epilepticus and eliminate the need for neurosurgical interventions in children with new-onset status epilepticus and no history of epilepsy, or in those in whom status epilepticus persists despite treatment Neuroimaging - computed tomography / Magnetic resonance imaging brain
Neuroimaging may be considered if clinically indicated or if the etiology of status epilepticus is unknown. It should be used only after the seizures are under control and the patient is stabilized. The data are insufficient to support or refute routine use of neuroimaging. Neuroimaging can identify structural causes of status epilepticus and eliminate the need for neurosurgical interventions in children with new-onset status epilepticus and no history of epilepsy, or in those in whom status epilepticus persists despite treatment
Hearing and vision assessment Status epilepticus is associated with neurological sequelae of impaired Hearing and vision Hearing and vision assessment
Status epilepticus is associated with neurological sequelae of impaired Hearing and vision
Serum electrolytes Important to assess as electrolyte imbalances can cause status epilepticus Serum electrolytes
Important to assess as electrolyte imbalances can cause status epilepticus
Full blood count Infections in the central nervous system can cause status epilepticus Full blood count
Infections in the central nervous system can cause status epilepticus
Plasma casual venous glucose levels Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected Plasma casual venous glucose levels
Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected
Electroencephalography (EEG) EEG may be considered in children presenting with new-onset status epilepticus because it can help to determine whether there are focal or generalized abnormalities, which can influence diagnosis and treatment Electroencephalography (EEG)
EEG may be considered in children presenting with new-onset status epilepticus because it can help to determine whether there are focal or generalized abnormalities, which can influence diagnosis and treatment
Blood cultures To identify sepsis.In six studies (n = 357) that reported on sepsis, a minimal diagnostic yield of a positive blood culture was found in 2.5 percent of children with status epilepticus.The evidence is insufficient to support or refute whether blood cultures should be obtained routinely in children with no clinical suspicion of infection. Blood cultures
To identify sepsis.In six studies (n = 357) that reported on sepsis, a minimal diagnostic yield of a positive blood culture was found in 2.5 percent of children with status epilepticus.The evidence is insufficient to support or refute whether blood cultures should be obtained routinely in children with no clinical suspicion of infection.
Lumbar puncture Data from 18 studies (n = 1,859) showed that the diagnosed CNS infection rate was 12.8 percent. According to the results of one study involving 49 children with convulsive status epilepticus, 24 children (49 percent) had a fever, and 17 percent of those had bacterial meningitis; none of the children without fever had meningitis.
The evidence is insufficient to support or refute whether lumbar puncture should be performed routinely in children with no clinical suspicion of CNS infection
Lumbar puncture
Data from 18 studies (n = 1,859) showed that the diagnosed CNS infection rate was 12.8 percent. According to the results of one study involving 49 children with convulsive status epilepticus, 24 children (49 percent) had a fever, and 17 percent of those had bacterial meningitis; none of the children without fever had meningitis.
The evidence is insufficient to support or refute whether lumbar puncture should be performed routinely in children with no clinical suspicion of CNS infection
Antiepileptic drug levels Antiepileptic drug levels should be considered when a child with epilepsy on antiepileptic prophylaxis develops status epilepticus as discontinuation of the drugs can cause status epilepticus Antiepileptic drug levels
Antiepileptic drug levels should be considered when a child with epilepsy on antiepileptic prophylaxis develops status epilepticus as discontinuation of the drugs can cause status epilepticus
Random blood sugar levels Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected Random blood sugar levels
Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected

Management - Supportive

Fact Explanation
Acute emergency management Proper assessment and control of the airway and of ventilation.patients should receive 100 percent oxygen by nasal cannula or a nonrebreathing mask, and airway patency should be maintained by an oral or nasopharyngeal device while the patient remains unresponsive. Nasal or orotracheal intubation or bag valve-mask ventilation should be undertaken if there is clinical or laboratory evidence of respiratory compromise.

Arterial-blood gas monitoring is especially useful. Many patients have a profound metabolic acidosis (e.g., arterial pH <7.0) that corrects itself once seizures are controlled; treatment with sodium bicarbonate should be reserved for the most extreme circumstances.

Hyperthermia occurs relatively frequently during status epilepticus (in 28 to 79 percent of patients), and in many cases it is primarily a manifestation of the seizures rather than evidence of an infection. Hyperthermia should be treated promptly with passive cooling.

Blood pressure and pulse should be checked.Intravenous fluid may be required to maintain circulation in hypotensive patients with sepsis.

A screening neurologic examination should be performed to check for signs of a focal intracranial lesion.

Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected
Acute emergency management
Proper assessment and control of the airway and of ventilation.patients should receive 100 percent oxygen by nasal cannula or a nonrebreathing mask, and airway patency should be maintained by an oral or nasopharyngeal device while the patient remains unresponsive. Nasal or orotracheal intubation or bag valve-mask ventilation should be undertaken if there is clinical or laboratory evidence of respiratory compromise.

Arterial-blood gas monitoring is especially useful. Many patients have a profound metabolic acidosis (e.g., arterial pH <7.0) that corrects itself once seizures are controlled; treatment with sodium bicarbonate should be reserved for the most extreme circumstances.

Hyperthermia occurs relatively frequently during status epilepticus (in 28 to 79 percent of patients), and in many cases it is primarily a manifestation of the seizures rather than evidence of an infection. Hyperthermia should be treated promptly with passive cooling.

Blood pressure and pulse should be checked.Intravenous fluid may be required to maintain circulation in hypotensive patients with sepsis.

A screening neurologic examination should be performed to check for signs of a focal intracranial lesion.

Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected

Management - Specific

Fact Explanation
Pharmacologic Management - benzodiazepines The benzodiazepines are some of the most effective drugs in the treatment of acute seizures and status epilepticus. The benzodiazepines most commonly used to treat status epilepticus are diazepam , lorazepam , and midazolam . All three compounds work by enhancing the inhibition of γ-aminobutyric acid (GABA) by binding to the benzodiazepine-GABA and barbiturate-receptor complex.
Diazepam is one of the drugs of choice for first-line management of status epilepticus. Because of its high lipid solubility the drug enters the brain rapidly, but after 15 to 20 minutes it redistributes to other areas of the body, reducing its clinical effect.
Lorazepam has emerged as the preferred benzodiazepine for acute management of status epilepticus.Lorazepam differs from diazepam in two important respects. It is less lipid-soluble than diazepam, with a distribution half-life of two to three hours versus 15 minutes for diazepam. Therefore, it should have a longer duration of clinical effect.
Pharmacologic Management - benzodiazepines
The benzodiazepines are some of the most effective drugs in the treatment of acute seizures and status epilepticus. The benzodiazepines most commonly used to treat status epilepticus are diazepam , lorazepam , and midazolam . All three compounds work by enhancing the inhibition of γ-aminobutyric acid (GABA) by binding to the benzodiazepine-GABA and barbiturate-receptor complex.
Diazepam is one of the drugs of choice for first-line management of status epilepticus. Because of its high lipid solubility the drug enters the brain rapidly, but after 15 to 20 minutes it redistributes to other areas of the body, reducing its clinical effect.
Lorazepam has emerged as the preferred benzodiazepine for acute management of status epilepticus.Lorazepam differs from diazepam in two important respects. It is less lipid-soluble than diazepam, with a distribution half-life of two to three hours versus 15 minutes for diazepam. Therefore, it should have a longer duration of clinical effect.
Pharmacologic Management - Phenytoin Phenytoin is one of the most effective drugs for treating acute seizures and status epilepticus. In addition, it is effective in the management of chronic epilepsy, particularly in patients with partial and secondarily generalized seizures.The main advantage of phenytoin is the lack of a sedating effect. However, a number of potentially serious adverse effects may occur. Arrhythmias and hypotension have been reported, particularly in patients older than 40 years Pharmacologic Management - Phenytoin
Phenytoin is one of the most effective drugs for treating acute seizures and status epilepticus. In addition, it is effective in the management of chronic epilepsy, particularly in patients with partial and secondarily generalized seizures.The main advantage of phenytoin is the lack of a sedating effect. However, a number of potentially serious adverse effects may occur. Arrhythmias and hypotension have been reported, particularly in patients older than 40 years
Pharmacologic Management - Fosphenytoin Fosphenytoin is a water-soluble pro-drug of phenytoin that completely converts to phenytoin following parenteral administration. Thus, the adverse events that are related to propylene glycol are avoided. Like phenytoin, fosphenytoin is useful in treating acute partial and generalized tonic-clonic seizures. Fosphenytoin is converted to phenytoin within eight to 15 minutes Pharmacologic Management - Fosphenytoin
Fosphenytoin is a water-soluble pro-drug of phenytoin that completely converts to phenytoin following parenteral administration. Thus, the adverse events that are related to propylene glycol are avoided. Like phenytoin, fosphenytoin is useful in treating acute partial and generalized tonic-clonic seizures. Fosphenytoin is converted to phenytoin within eight to 15 minutes
Pharmacologic Management - Phenobarbital Phenobarbital typically is used after a benzodiazepine or phenytoin has failed to control status epilepticus. The normal loading dose is 15 to 20 mg per kg. Because high-dose phenobarbital is sedating, airway protection is an important consideration, and aspiration is a major concern Pharmacologic Management - Phenobarbital
Phenobarbital typically is used after a benzodiazepine or phenytoin has failed to control status epilepticus. The normal loading dose is 15 to 20 mg per kg. Because high-dose phenobarbital is sedating, airway protection is an important consideration, and aspiration is a major concern
Pharmacologic Management - valproate Parenteral valproate is used primarily for rapid loading and when oral therapy is impossible. It has a broad spectrum of efficacy and may be useful in patients with absence or myoclonic status epilepticus. Adverse effects include local irritation, gastrointestinal distress, and lethargy.
However, further experience is needed before this therapy can be recommended
Pharmacologic Management - valproate
Parenteral valproate is used primarily for rapid loading and when oral therapy is impossible. It has a broad spectrum of efficacy and may be useful in patients with absence or myoclonic status epilepticus. Adverse effects include local irritation, gastrointestinal distress, and lethargy.
However, further experience is needed before this therapy can be recommended
Treatment of Refractory Status Epilepticus Benzodiazepine-refractory status epilepticus (established status epilepticus, ESE) is a relatively common emergency condition
Status epilepticus that does not respond to a benzodiazepine, phenytoin, or phenobarbital is considered refractory and requires more aggressive treatment. Continuous intravenous infusions with anesthetic doses of midazolam, propofol, or barbiturates are the most useful treatments.
The use of midazolam (0.2 mg per kilogram administered by slow intravenous bolus injection, followed by 0.75 to 10 μg per kilogram per minute) or propofol administered intravenously (1 to 2 mg per kilogram, followed by 2 to 10 mg per kilogram per hour) to induce anesthesia for the treatment of refractory status epilepticus has become very popular in recent years
Treatment of Refractory Status Epilepticus
Benzodiazepine-refractory status epilepticus (established status epilepticus, ESE) is a relatively common emergency condition
Status epilepticus that does not respond to a benzodiazepine, phenytoin, or phenobarbital is considered refractory and requires more aggressive treatment. Continuous intravenous infusions with anesthetic doses of midazolam, propofol, or barbiturates are the most useful treatments.
The use of midazolam (0.2 mg per kilogram administered by slow intravenous bolus injection, followed by 0.75 to 10 μg per kilogram per minute) or propofol administered intravenously (1 to 2 mg per kilogram, followed by 2 to 10 mg per kilogram per hour) to induce anesthesia for the treatment of refractory status epilepticus has become very popular in recent years

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