Pediatric Status Epilepticus
No Results
No Results
processing….
Status epilepticus (SE) is defined as a seizure that lasts more than 30 minutes. The annual incidence of convulsive SE among children in developed countries is about 20 per 100,000 population.
In a large international collaborative study of 356 patients with severe epilepsies and their parents, researchers identified 429 new synaptic transmission genes. These mutations were considered causative in 12% of the patients. DNM1, a gene that carries the code for the structural protein dynamin-1, which is involved in shuttling small vesicles between the body of the neuron and the synapse, was found to be mutated in five patients. De novo mutations in GABBR2, FASN, and RYR3 were found in two patients each. In all, 75% of the mutations detected were predicted to disrupt a protein involved in regulating synaptic transmission. [1]
Generalized tonic-clonic SE (GTCSE) has 3 phases, which have the following characteristics:
Phase 1: Discrete partial seizures or, less frequently, generalized seizures; blood pressure usually remains within the reference range
Phase 2: Discrete SE events fuse and partial seizures become secondarily generalized; the main outward manifestation of continuous seizure activity consists of a tonic phase (sustained muscle contraction) followed by clonic jerks (alternating contraction and relaxation of the 4 limbs); alteration of blood pressure may occur
Phase 3: Clinical seizures may become quite subtle, with brief rhythmic clonic or myoclonic movements often restricted to a single part of the body; rhythmic activity may be observed as myoclonus that affects only the feet, hands, facial muscles, or eyes (as nystagmus); hyperthermia, respiratory compromise, hypotension, and hypoglycemia may be observed
Nonconvulsive status epilepticus has the following characteristics:
Patients appear forgetful and sleepy, behaving as if deaf and blind (“like a zombie”) or drugged
In more severe cases, patients are described as unresponsive
Parents may describe frequent falls, poor motor control, or abnormal balance
Patients with absence SE present with the following:
Altered consciousness, with or without clonic movements of the eyelids or upper extremities
Automatisms involving the hands and face
A child may sometimes continue to perform a motor act that he or she was engaged in before onset of the absence seizure (eg, bouncing a basketball)
In some cases, the patient may answer simple questions, but detailed examination reveals slowed mentation and poor processing of complex information
Episodes of absence SE may last 12 hours or longer
When the patient’s situation stabilizes, look for lymphadenopathy, which suggests catscratch fever.
See Clinical Presentation for more detail.
Every patient who presents with SE requires an EEG; however, treatment should not be delayed to wait for EEG results. When a seizure persists longer than 30-60 minutes, making immediate arrangements for an EEG is advisable. Features of testing and procedures are as follows:
Laboratory testing should proceed concurrently with stabilization
Lumbar puncture with opening pressure measurement is performed for prolonged SE of unknown etiology and in immunocompromised patients
Obtain imaging studies based on likely etiologies; stabilize all children before performing CT scanning or other imaging studies
SE persisting beyond 24 hours needs further workup if routine blood work, brain MRI, and microbiological studies in serum and CSF do not provide clues to the etiology of the seizures. The following investigations are considered:
Urine organic acids, porphyrins (porphyria), and sulfites (sulfite oxidase deficiency and molybdenum co-factor deficiency)
Serum T4, T3, thyrotropin, and antiperoxidase antibody (autoimmune epilepsy)
Serological markers for collagen-vascular disorders
Serum and CSF NMDA-R antibody, ultrasound/CT/MRI of testes and abdomen to look for solid tumors (anti NMDA-R antibody encephalitis)
Other paraneoplastic antibodies (anti Hu, Yo, Ri)
See Workup for more detail.
Treatment of SE should be based on an institutional protocol, such as the following:
Attend to the ABCs before starting any pharmacologic intervention
Place patients in the lateral decubitus position to avoid aspiration of emesis and to prevent epiglottis closure over the glottis
Make further adjustments of the head and neck if necessary to improve airway patency
Immobilize the cervical spine if trauma is suspected
Administer 100% oxygen by facemask
Assist ventilation and use artificial airways (eg, endotracheal intubation) as needed
Suction secretions and decompress the stomach with a nasogastric tube.
Carefully monitor vital signs, including blood pressure
Carefully monitor the patient’s temperature, as hyperthermia may worsen brain damage
In the first 5 minutes of seizure activity, before starting any medications, try to establish IV access and to obtain samples for laboratory tests and for seizure medication
Infuse isotonic IV fluids plus glucose at a rate of 20 mL/kg/h (eg, 200 mL D5NS over 1 h for a 10-kg child)
In children younger than 6 years, use intraosseous (IO) infusion if IV access cannot be established within 5-10 minutes
If serum glucose is low or cannot be measured, give children 2 mL/kg of 25% glucose
If the seizure fails to stop within 4-5 minutes, prompt administration of anticonvulsants may be indicated
A treatment algorithm is shown in the image below.
Anticonvulsant selection can be based on seizure duration, as follows:
6-15 min: Lorazepam (0.05-0.1 mg/kg IV or IO slowly infused over 2-5 min); or diazepam per rectum at 0.5 mg/kg, not to exceed 10 mg
16-35 min: Phenytoin (Dilantin) or fosphenytoin, not to exceed infusion rate of 1 mg/kg/min; do not dilute in D5W; if unsuccessful, phenobarbital 10-20 mg/kg IV (not to exceed 700 mg IV); increase infusion rate by 100 mg/min; phenobarbital may be used in infants before phenytoin
45-60 min: Pentobarbital anesthesia (patient already intubated); or midazolam, loading dose 0.1-0.3 mg/kg IV followed by continuous IV infusion at a rate of 0.1-0.3 mg/kg/h
Pentobarbital anesthesia is administered as follows:
Loading dose: 5-7 mg/kg IV
May repeat 1-mg/kg to 5-mg/kg boluses until EEG exhibits burst suppression; closely monitor hemodynamics and support blood pressure as indicated
Maintenance dose: 0.5-3 mg/kg/h IV; monitor EEG to keep burst suppression pattern at 2-8 bursts/min
Other specific treatments may be indicated if the clinical evaluation identifies precipitants of the seizures. Selected agents and indications are as follows:
Naloxone – 0.1 mg/kg/dose, IV preferably (if needed may administer IM or SQ) for narcotic overdose
Pyridoxine – 50-100 mg IV/IM for possible dependency, deficiency, or isoniazid toxicity
Antibiotics – If meningitis is strongly suspected, initiate treatment with antibiotics prior to CSF analysis or CNS imaging
See Treatment and Medication for more detail.
Status epilepticus (SE) is defined as a seizure that lasts more than 30 minutes, constituting a neurological emergency. [2] The seizure may be continuous or may be intermittent without recovery of consciousness between seizures.
The rationale for equating intermittent seizures without recovery of consciousness with continuous seizures is twofold. First, in animal models, intermittent seizures were quite powerful agents in causing neuropathological changes. Second, in cases of prolonged status epilepticus, outward motor manifestations may become intermittent or less prominent over time without necessarily indicating decreasing intensity of electrical seizure activity in the brain.
In the past, the common definition of SE was seizure activity exceeding 60 minutes. This longer time limit (as opposed to the current definition of 30 minutes) may be one of the reasons for the higher incidence of sequelae in older studies. Other factors accounting for outcome differences include improvement in intensive medical care and the retrospective nature of these older studies, which tended to create a bias toward more severe cases.
For more information, see the Medscape Reference topic Status Epilepticus.
Most of the literature on SE deals with the generalized tonic-clonic SE (GTCSE), and the terms SE and GTCSE are often used synonymously. This article primarily addresses GTCSE; when appropriate, however, comments on other types of SE are included. Other types of SE include the following:
Simple partial SE
Complex partial SE
Absence SE
Nonconvulsive SE
Myoclonic SE
Simple partial status epilepticus
In simple partial SE, seizures may be quite sustained, especially when associated with focal brain lesions. Simple partial seizures may be tonic (sustained muscle contraction of part of the body) or clonic (alternating muscle contraction and relaxation). Prolonged simple partial seizures (often motor and clonic) are frequently termed epilepsia partialis continua.
Simple partial seizures do not cause major impairment of consciousness. However, they may be accompanied by recurrent subjective feelings, bodily sensations, or visual hallucinations.
Simple partial seizures are not necessarily associated with diffuse brain damage, unless they become complex partial SE or are associated with secondary generalization.
See also the Medscape Reference topic Partial Epilepsies.
Complex partial status epilepticus
Episodes of complex partial status epilepticus are characterized by major alteration in consciousness, lack of recollection for the event associated with stereotypic automatisms, staring, and, in some cases, vocalization or screaming. Most patients are described as confused (one third of cases) or unresponsive (one third of cases).
Complex partial SE episodes have been followed by cognitive deficits in some cases; recognizing the impairment is important.
See also the Medscape Reference topic Complex Partial Seizures.
Absence seizures
Typical absence seizures are prolonged episodes of alteration in responsiveness with poor or no recollection for events. They can last for hours or even days. Typical absence seizures that exceed 30 minutes in duration should be treated because of the risk of secondary generalization. However, prolonged absence SE has been described that was not associated with subsequent neurologic deterioration.
Alteration of consciousness may not be severe; automatic behavior sometimes occurs, with patients able to perform customary daily activities such as combing their hair, playing video games, and even driving. Preceding behavioral changes that cleared with antiepileptic drug therapy have been documented in some cases. In some cases, myoclonic jerking of the eyelids (eyelid myoclonia) provides the clue to absence SE.
Absence seizure status may occur in teenagers and adults who were thought to have outgrown the condition.
See also the Medscape Reference topic Absence Seizures.
Nonconvulsive status epilepticus
Many studies combine cases of complex partial and absence SE under the name nonconvulsive SE. This is because of the similarity in the seizure semiology, despite of the divergent EEG patterns. In children, about two thirds of nonconvulsive SE cases have generalized EEG changes suggestive of either typical or atypical absences with or without a myoclonic component.
Myoclonic seizures
Myoclonic seizures are characterized by quick, often repetitive, jerks that randomly involve the limbs. Seizures are often repetitive and, in some cases, may be unabated for lengthy periods.
Some patients with myoclonic epilepsies may sustain repetitive myoclonus that persists for days with or without altered consciousness. Myoclonic SE is a term sometimes used to describe these patients’ condition.
See also the Medscape Reference topics Myoclonic Epilepsy Beginning in Infancy or Early Childhood and Juvenile Myoclonic Epilepsy.
Most studies of SE epidemiology and outcome have used the following classification of episodes:
Acute symptomatic (26%) – Episodes caused by an acute infection, head trauma, hypoxemia, electrolyte disturbance, hypoglycemia, intoxication or drug withdrawal
Progressive encephalopathy (3%) – SE occurring with an underlying progressive CNS disorder, such as mitochondrial disorder, Rasmussen encephalitis, CNS lipid storage diseases, aminoacidopathies, or organic acidopathies
Remote symptomatic SE (33%) – Episodes secondary to static conditions (eg, remote cerebral insult in the perinatal period)
Remote symptomatic with an acute precipitant (1%) – SE in a patient with a chronic encephalopathy but precipitated by an acute event such as those in acute symptomatic SE
Febrile (22%) – SE for which the only provocation is a febrile illness, after excluding a direct CNS infection
Cryptogenic (15%) – SE without identifiable cause
Perform a rapid, directed history, physical examination, and neurologic examination during SE, followed by a detailed examination when the child is stabilized (see Clinical). Laboratory testing should proceed concurrently with stabilization, with the choice of laboratory studies based on age and likely etiologies (see Workup). The principles of treatment are to terminate the seizure while resuscitating the patient, treating complications, and preventing recurrence (see Treatment).[#IntroductionPatientEducation]
For patient education information, see the Brain and Nervous System Center, as well as Seizures Emergencies, Seizures in Children, and Epilepsy.
Seizures result from rapid abnormal electrical discharges from cerebral neurons. This presents clinically as involuntary alterations of consciousness or motor activity.
Consumption of oxygen, glucose, and energy substrates (eg, adenosine triphosphate [ATP], phosphocreatine) in cerebral tissue increases significantly during seizures. Optimal delivery of these metabolic substrates to cerebral tissue requires adequate cardiac output and intravascular fluid volume. See the Cardiac Output calculator.
SE occurs with the failure of the normal factors that serve to terminate a typical seizure. Sources of this failure include changes in gamma-aminobutyric acid (GABA) receptor composition, loss of benzodiazepine efficacy, excessive glutamate excitation, and activation of drug resistance genes.
GABA receptor–mediated inhibition may be responsible for the normal termination of a seizure. In addition, the activation of the N -methyl-D aspartate (NMDA) receptor by the excitatory neurotransmitter glutamate may be required for the propagation of seizure activity. In experimental models, resistance to benzodiazepines and barbiturates may develop during prolonged seizures that may alter the structure and function of GABAa receptors. [3]
Prolonged seizures are associated with cerebral hypoxia, hypoglycemia, and hypercarbia and with concurrent and progressive lactic and respiratory acidosis. When cerebral metabolic needs exceed available oxygen, glucose, and metabolic substrates (especially during status epilepticus), neuronal destruction can occur and may be irreversible.
Massive sympathetic discharge with SE may have the following consequences:
Hypoxia
Hypercarbia
Hyperthermia
Tachycardia
Hyperglycemia
Hyperkalemia
In adolescent baboons, brain damage can be observed after 90 minutes of sustained seizures, with the neocortex, thalamus, and hippocampus most affected. [4, 5] In the neocortex, small pyramidal cells in layers 3, 5, and 6 were most affected, and resultant lesions tended to be more prominent in the occipital lobe. In this animal model, in which seizures were induced by bicuculline or pentylenetetrazol (PTZ), intubation/ventilation and paralyzation did not improve these types of CNS lesions, suggesting that excessive neuronal discharge caused the damage.
These studies also established that hyperpyrexia may also contribute to CNS damage observed in prolonged seizures. This observation has been confirmed in studies of adult humans. Cerebellar damage can also be observed; however, because it is more prominent in the border zones of arterial blood supply, this type of damage probably relates to ischemia and/or hyperthermia.
Most definitions of SE do not distinguish between uninterrupted seizures and intermittent seizures without recovery of consciousness. This concept is supported by the finding that the pattern of brain damage in animals with repetitive seizures induced by allyl glycine (glutamic acid decarboxylase inhibitor) included hippocampal sclerosis (at times asymmetrical or unilateral), cortical gliosis, and ischemic cell-type damage. Lesions in the cortex sometimes were restricted to the occipital cortex or watershed zones, a pattern very similar to that observed in continuous prolonged seizures.
The etiology of SE tends to vary by the age of the child (ie, younger than versus older than 6 years). Causes of SE in early childhood (< 6 y) may include the following:
Birth injury
Febrile convulsions (3 mo to 6 y)
Infection
Metabolic disorders
Trauma
Neurocutaneous syndromes
Cerebral degenerative diseases
Tumors
Idiopathic
Causes in children and adolescents (>6 y) may include the following:
Birth injury
Trauma
Infection
Epilepsy with inadequate drug levels
Cerebral degenerative disease
Tumor
Toxins
Idiopathic
Toxins and medications that can cause SE include the following:
Topical anesthetics (eg, lidocaine)
Anticonvulsant overdose
Camphor
Hypoglycemic agents (eg, insulin, ethanol)
Carbon monoxide
Cyanide
Heavy metals (eg, lead)
Pesticides (eg, organophosphate)
Cocaine
Phencyclidine
Belladonna alkaloids
Nicotine
Sympathomimetics (eg, amphetamines, phenylpropanolamine [recalled from US market])
Tricyclic antidepressants
The etiologies of SE episodes can be classified as (1) acute symptomatic, (2) chronic-progressive neurologic disorders, and (3) remote symptomatic status epilepticus.
Acute symptomatic status epilepticus may be caused by an acute infection, head trauma, hypoxemia, hypoglycemia, or drug withdrawal. Acute symptomatic SE is the most common etiologic category in children, accounting for as many as 35% of cases. Idiopathic SE the second most common category, with a frequency of 30%; febrile SE constitutes 25% of cases.
Meningitis is a common cause of convulsive SE [6] ; fever is present in 17% of the cases in children. In patients with febrile convulsive SE, the classic signs of meningitis may not be present.
Chronic-progressive neurological disorders represent just 5% of cases. Remote symptomatic SE, referring to SE secondary to static conditions (eg, when a cerebral insult that occurred in the perinatal period causes SE later in childhood), constitutes 10-15% of cases.
The use of cephalosporin antibiotics (cefepime and ceftazidime) has been associated with the precipitation of SE. This association is especially important in patients with impaired renal function.
Some anticonvulsants may produce de novo nonconvulsive SE (both absence and complex partial types). Carbamazepine and tiagabine are commonly implicated. Patients with Lennox-Gastaut syndrome may develop SE due to excessive sedation (usually secondary to long-term benzodiazepine use).
Of the many acute precipitants described in children, infection and fever collectively constitute the most common (35.7%). Other common precipitants and their reported frequencies are as follows:
Medication changes – 20%
Metabolic precipitants – 8%
Congenital precipitants – 7%
Anoxia – 5%
CNS infection – 5%
Trauma – 3.5%
No precipitant is found in 8-10% of cases of generalized tonic-clonic SE. Generalized tonic-clonic SE may recur in 17-25% of children. Recurrent SE epilepticus primarily occurs in children with neurologic abnormalities. The risk of recurrence also varies among the etiologic groups. Idiopathic and remote symptomatic groups have the highest recurrence risk (28% in prospective studies). The febrile seizure group has a prospective recurrence risk of 3%.
Nonconvulsive SE is commonly associated with a prior diagnosis of one of the following epileptic syndromes:
Dravet syndrome
Myoclonic-astatic epilepsy
Childhood absence epilepsy
Localization-related epilepsy (partial seizures)
The annual incidence of convulsive SE among children in developed countries is about 20 per 100,000 population; however, the rate will vary depending on factors such as the socioeconomic and ethnic characteristics of the population. [7] The percentage of patients with epilepsy who develop status epilepticus varies from 1.3-16%. The first seizure lasts longer than 30 minutes in 12.6% of cases. Among patients with febrile seizures, duration exceeds 30 minutes in 5% of cases.
Almost half (48%) of adults who present with SE have no prior history of seizures. Among children diagnosed with SE, a history of prior unprovoked seizures was even less common (32%); pediatric patients who present with febrile SE rarely have a history of epilepsy.
Although the data are contradictory, SE incidence may have increased since the advent of modern antiepileptic drugs (AEDs). Data have showed that 43% of patients taking AEDs when SE occurred had low serum levels of the drugs. In 19% of cases, some levels were low and other levels were within the therapeutic range. In 38% of cases, all AED levels were in the therapeutic range.
Generalized tonic-clonic SE may be recurrent in 17-25% of children with SE. Risk of generalized tonic-clonic SE recurrence varies among etiologic groups. The idiopathic and remote symptomatic groups have the highest recurrence risk (ie, 28% in prospective studies). The febrile seizure group has a prospective recurrence risk of 3%.
Of children younger than 1 year who are subsequently diagnosed with epilepsy, 70% present with SE as the initial manifestation of their illness. In children with epilepsy, 20% have SE within 5 years of diagnosis. Of children with febrile seizures, 5% present with status epilepticus.
No sexual predilection or age variation is recognized. However, certain etiologies are more prevalent in selected age groups (see Etiology).
Several factors affect prognosis in patients with SE. These include seizure type (nonconvulsive versus generalized tonic-clonic), duration, and etiology and patient age.
After an episode of nonconvulsive SE, at least 60% of patients show some degree of cognitive deterioration. In contrast, one study of children with generalized tonic-clonic SE reported that sequelae occurred in 9% of cases. [8] Of these, approximately 58% were motor sequelae only, 29% were motor and cognitive, and 13% were cognitive only.
Patients with generalized tonic-clonic SE that lasts less than 1 hour have a better prognosis than do those with more prolonged SE. The relationship between seizure-mediated brain damage and duration of SE is not as clear with simple partial motor SE as it is with generalized tonic-clonic SE.
Seizure etiology has a strong effect on the frequency of SE sequelae. [8, 9] Maytal et al reported that the incidence of sequelae was low (1.4%) in patients classified as having idiopathic febrile seizures and remote symptomatic seizures, intermediate (12%) in those with acute symptomatic seizures, and highest (80%) in those with chronic progressive encephalopathy.
Sequelae rates for patients with generalized tonic-clonic SE declined with increasing age. Rates were highest among patients younger than 1 year (29%), declined to 11% for children aged 1-3 years, and fell further to 6% for children older than 3 years. [8] Although children younger than 1 year have greater incidence of acute symptomatic generalized tonic-clonic status epilepticus, no difference in the etiologic categories among the other age groups was observed.
Patients with refractory SE who require high-dose suppressive therapy (eg, barbiturate coma, midazolam infusion) often need prolonged therapy. The long-term outcome in previously healthy children who survive prolonged barbiturate coma or midazolam infusion for SE is not particularly favorable; these children may have long-term cognitive deficits and recurrent seizures. In one study performed at Boston Children’s Hospital, all patients developed intractable epilepsy, and none returned to baseline. [10]
De novo development of hippocampus sclerosis (ie, mesial temporal lobe sclerosis) is one of the possible complications of SE and possibly the reason that survivors may develop chronic recurrent and refractory complex partial seizures. [11]
Cognitive difficulties recognized after SE may represent pre-existent but unrecognized problems. Although learning disabilities and mental retardation are more common among children with epilepsy than in the general population, cognitive problems often remain undiagnosed until the patient’s first seizure and sometimes not until the first prolonged seizure. Occasionally, it is possible to obtain a history of abnormal language development and cognition prior to the seizures.
In pediatric patients, death after SE occurs almost exclusively among those in the acute symptomatic or progressive encephalopathy groups. Maytal et al found that the mortality rate for both classifications combined was 12%, whereas there were no deaths among patients in the remote symptomatic, idiopathic, and febrile status groups. [8]
Reporting on mortality within 8 years following an episode of convulsive status epilepticus, one study noted an overall fatality rate of 11% of the 226 patients studied. Seven children died within 30 days of their episode and 16 during follow-up; 25% of deaths during follow-up were associated with intractable seizures/convulsive status epilepticus, and the rest died as a complication of their underlying medical condition. The mortality rate was 46 times greater than expected and was associated with preexisting clinically significant neurological impairments; however, children without prior neurological impairment were not at a significantly increased risk of death during follow-up. No deaths were noted in children following prolonged febrile convulsions and idiopathic convulsive status epilepticus. These results suggest that while a high risk of death was realized within 8 years, most deaths were not seizure related; the main risk factor was the presence of preexisting neurologicalimpairments. [12]
Most modern pediatric series report that mortality directly related to SE occurs at a rate of 2%, whereas overall mortality rates range from 4-6%. This contrast with the much higher mortality rate in adults with SE, which ranges from 16-35%, with 1-5% of deaths directly related to status epilepticus. Early treatment of seizures with rectal medication (diazepam) is thought to be associated with a better outcome but further testing is required to confirm this statement.
De Novo Mutations in Synaptic Transmission Genes Including DNM1 Cause Epileptic Encephalopathies. Am J Hum Genet. 2014 Sep 24. [Medline].
Mitchell WG. Status epilepticus and acute serial seizures in children. J Child Neurol. 2002 Jan. 17 Suppl 1:S36-43. [Medline].
Tassinari CA, Daniele O, Michelucci R, Bureau M, Dravet C, Roger J. Benzodiazepines: efficacy in status epilepticus. Adv Neurol. 1983. 34:465-75. [Medline].
Meldrum BS, Horton RW, Brierley JB. Epileptic brain damage in adolescent baboons following seizures induced by allylgycine. Brain. 1974 Jun. 97(2):407-18. [Medline].
Meldrum BS, Vigouroux RA, Brierley JB. Systemic factors and epileptic brain damage. Prolonged seizures in paralyzed, artificially ventilated baboons. Arch Neurol. 1973 Aug. 29(2):82-7. [Medline].
Chin RF, Neville BG, Scott RC. Meningitis is a common cause of convulsive status epilepticus with fever. Arch Dis Child. 2005 Jan. 90(1):66-9. [Medline]. [Full Text].
Raspall-Chaure M, Chin RF, Neville BG, Bedford H, Scott RC. The epidemiology of convulsive status epilepticus in children: a critical review. Epilepsia. 2007 Sep. 48(9):1652-63. [Medline].
Maytal J, Shinnar S, Moshé SL, Alvarez LA. Low morbidity and mortality of status epilepticus in children. Pediatrics. 1989 Mar. 83(3):323-31. [Medline].
zz. zz.
Sahin M, Menache CC, Holmes GL, Riviello JJ Jr. Prolonged treatment for acute symptomatic refractory status epilepticus: outcome in children. Neurology. 2003 Aug 12. 61(3):398-401. [Medline].
Pohlmann-Eden B, Gass A, Peters CN, Wennberg R, Blumcke I. Evolution of MRI changes and development of bilateral hippocampal sclerosis during long lasting generalised status epilepticus. J Neurol Neurosurg Psychiatry. 2004 Jun. 75(6):898-900. [Medline]. [Full Text].
Pujar SS, Neville BG, Scott RC, Chin RF. Death within 8 years after childhood convulsive status epilepticus: a population-based study. Brain. 2011 Oct. 134:2819-27. [Medline]. [Full Text].
Haffey S, McKernan A, Pang K. Non-convulsive status epilepticus: a profile of patients diagnosed within a tertiary referral centre. J Neurol Neurosurg Psychiatry. 2004 Jul. 75(7):1043-4. [Medline]. [Full Text].
Riviello JJ Jr, Ashwal S, Hirtz D, Glauser T, Ballaban-Gil K, Kelley K, et al. Practice parameter: diagnostic assessment of the child with status epilepticus (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2006 Nov 14. 67(9):1542-50. [Medline].
Epilepsy Foundation of America’s Working Group on Status Epilepticus. Treatment of convulsive status epilepticus. Recommendations of the Epilepsy Foundation of America’s Working Group on Status Epilepticus. JAMA. 1993 Aug 18. 270(7):854-9. [Medline].
Chin RF, Verhulst L, Neville BG, Peters MJ, Scott RC. Inappropriate emergency management of status epilepticus in children contributes to need for intensive care. J Neurol Neurosurg Psychiatry. 2004 Nov. 75(11):1584-8. [Medline]. [Full Text].
Brevoord JC, Joosten KF, Arts WF, van Rooij RW, de Hoog M. Status epilepticus: clinical analysis of a treatment protocol based on midazolam and phenytoin. J Child Neurol. 2005 Jun. 20(6):476-81. [Medline].
[Guideline] Meierkord H, Boon P, Engelsen B, Göcke K, Shorvon S, Tinuper P, et al. EFNS guideline on the management of status epilepticus. Eur J Neurol. 2006 May. 13(5):445-50. [Medline].
Lang ES, Andruchow JE. Evidence-based emergency medicine. What is the preferred first-line therapy for status epilepticus?. Ann Emerg Med. 2006 Jul. 48(1):98-100. [Medline].
Prasad K, Al-Roomi K, Krishnan PR, Sequeira R. Anticonvulsant therapy for status epilepticus. Cochrane Database Syst Rev. 2005 Oct 19. CD003723. [Medline].
Choudhery V, Townend W. Best evidence topic reports. Lorazepam or diazepam in paediatric status epilepticus. Emerg Med J. 2006 Jun. 23(6):472-3. [Medline]. [Full Text].
Chamberlain JM, Capparelli EV, Brown KM, Vance CW, Lillis K, Mahajan P, et al. Pharmacokinetics of Intravenous Lorazepam in Pediatric Patients with and without Status Epilepticus. J Pediatr. 2011 Nov 1. [Medline].
Holsti M, Dudley N, Schunk J, Adelgais K, Greenberg R, Olsen C, et al. Intranasal midazolam vs rectal diazepam for the home treatment of acute seizures in pediatric patients with epilepsy. Arch Pediatr Adolesc Med. 2010 Aug. 164(8):747-53. [Medline].
Morrison G, Gibbons E, Whitehouse WP. High-dose midazolam therapy for refractory status epilepticus in children. Intensive Care Med. 2006 Dec. 32(12):2070-6. [Medline].
Tasker RC. Midazolam for refractory status epilepticus in children: higher dosing and more rapid and effective control. Intensive Care Med. 2006 Dec. 32(12):1935-6. [Medline].
Tarulli A, Drislane FW. The use of topiramate in refractory status epilepticus. Neurology. 2004 Mar 9. 62(5):837. [Medline].
Kahriman M, Minecan D, Kutluay E, Selwa L, Beydoun A. Efficacy of topiramate in children with refractory status epilepticus. Epilepsia. 2003 Oct. 44(10):1353-6. [Medline].
Perry MS, Holt PJ, Sladky JT. Topiramate loading for refractory status epilepticus in children. Epilepsia. 2006 Jun. 47(6):1070-1. [Medline].
Chez MG, Hammer MS, Loeffel M, Nowinski C, Bagan BT. Clinical experience of three pediatric and one adult case of spike-and-wave status epilepticus treated with injectable valproic acid. J Child Neurol. 1999 Apr. 14(4):239-42. [Medline].
Sheth RD, Gidal BE. Intravenous valproic acid for myoclonic status epilepticus. Neurology. 2000 Mar 14. 54(5):1201. [Medline].
Uberall MA, Trollmann R, Wunsiedler U, Wenzel D. Intravenous valproate in pediatric epilepsy patients with refractory status epilepticus. Neurology. 2000 Jun 13. 54(11):2188-9. [Medline].
Abend NS, Dlugos DJ. Treatment of refractory status epilepticus: literature review and a proposed protocol. Pediatr Neurol. 2008 Jun. 38(6):377-90. [Medline].
Patel NC, Landan IR, Levin J, Szaflarski J, Wilner AN. The use of levetiracetam in refractory status epilepticus. Seizure. 2006 Apr. 15(3):137-41. [Medline].
Gallentine WB, Hunnicutt AS, Husain AM. Levetiracetam in children with refractory status epilepticus. Epilepsy Behav. 2009 Jan. 14(1):215-8. [Medline].
Shorvon S. Emergency treatment of epilepsy. Handbook of Epilepsy Treatment. Oxford, UK: Blackwell Science; 2000. 173-93.
Parke TJ, Stevens JE, Rice AS, Greenaway CL, Bray RJ, Smith PJ, et al. Metabolic acidosis and fatal myocardial failure after propofol infusion in children: five case reports. BMJ. 1992 Sep 12. 305(6854):613-6. [Medline]. [Full Text].
Bray RJ. Propofol infusion syndrome in children. Paediatr Anaesth. 1998. 8(6):491-9. [Medline].
Coetzee JF, Coetzer M. Propofol in paediatric anaesthesia. Curr Opin Anaesthesiol. 2003 Jun. 16(3):285-90. [Medline].
Corbett SM, Montoya ID, Moore FA. Propofol-related infusion syndrome in intensive care patients. Pharmacotherapy. 2008 Feb. 28(2):250-8. [Medline].
Hill M, Peat W, Courtman S. A national survey of propofol infusion use by paediatric anaesthetists in Great Britain and Ireland. Paediatr Anaesth. 2008 Jun. 18(6):488-93. [Medline].
Fodale V, La Monaca E. Propofol infusion syndrome: an overview of a perplexing disease. Drug Saf. 2008. 31(4):293-303. [Medline].
Anderson P. Convulsive Status Epilepticus Has Prolonged Cognitive Effect. Medscape Medical News. April 10, 2013. Available at http://www.medscape.com/viewarticle/782293.. Accessed: April 22, 2013.
Baumeister FA, Oberhoffer R, Liebhaber GM, Kunkel J, Eberhardt J, Holthausen H, et al. Fatal propofol infusion syndrome in association with ketogenic diet. Neuropediatrics. 2004 Aug. 35(4):250-2. [Medline].
Brooks M. Lorazepam, diazepam similar in pediatric status epilepticus. Medscape Medical News. April 30, 2014. [Full Text].
Chamberlain JM, Capparelli EV, Brown KM, Vance CW, Lillis K, Mahajan P, et al. Pharmacokinetics of Intravenous Lorazepam in Pediatric Patients with and without Status Epilepticus. J Pediatr. 2011 Nov 1. [Medline].
Chamberlain JM, Okada P, Holsti M, et al. Lorazepam vs diazepam for pediatric status epilepticus: a randomized clinical trial. JAMA. 2014 Apr 23-30. 311(16):1652-60. [Medline].
Krishnamurthy KB, Drislane FW. Depth of EEG suppression and outcome in barbiturate anesthetic treatment for refractory status epilepticus. Epilepsia. 1999 Jun. 40(6):759-62. [Medline].
Martinos MM, Yoong M, Patil S, Chong WK, Mardari R, Chin RF, et al. Early developmental outcomes in children following convulsive status epilepticus: A longitudinal study. Epilepsia. 2013 Apr 8. [Medline].
Mathews HM, Carson IW, Lyons SM, Orr IA, Collier PS, Howard PJ, et al. A pharmacokinetic study of midazolam in paediatric patients undergoing cardiac surgery. Br J Anaesth. 1988 Sep. 61(3):302-7. [Medline].
Neville BG, Chin RF, Scott RC. Childhood convulsive status epilepticus: epidemiology, management and outcome. Acta Neurol Scand Suppl. 2007. 186:21-4. [Medline].
Step
Medication
Dose
Alternatives
Step 2 (6-15 min)
Diazepam (Valium)
5-20 mg IV slowly; not to exceed infusion rate of 2 mg/min; pediatric dose is 0.3 mg/kg
If IV line is unavailable, use rectally administered (PR) diazepam at 0.5 mg/kg (not to exceed 10 mg) or midazolam (Versed) at 0.2 mg/kg intramuscularly (IM)*, IV, or intranasally*
Lorazepam* (Ativan)
2-4 mg IV slowly*; not to exceed infusion rate of 2 mg/min or 0.05 mg/kg over 2-5 min; pediatric dose is 0.05-0.1 mg/kg
Step 3 (16-35 min)
Phenytoin (Dilantin) or fosphenytoin (Cerebyx)†
20 mg/kg IV over 20 min; not to exceed infusion rate of 1 mg/kg/min; do not dilute in 5% dextrose in water (D5W)
If seizures persist, administer 5 mg/kg for 2 doses (if blood pressure is within the reference range and no history of cardiac disease is present)
If unsuccessful, administer phenobarbital 10-20 mg/kg IV (not to exceed 700 mg IV); increase infusion rate by 100 mg/min; phenobarbital may be used in infants before phenytoin; be prepared to intubate patient; closely monitor hemodynamics and support blood pressure as indicated
Step 4 (45-60 min)‡
Pentobarbital anesthesia (patient already intubated)
Loading dose: 5-7 mg/kg IV; may repeat 1-mg/kg to 5-mg/kg boluses until EEG exhibits burst suppression; closely monitor hemodynamics and support blood pressure as indicated
Maintenance dose: 0.5-3 mg/kg/h IV; monitor EEG to keep burst suppression pattern at 2-8 bursts/min
Midazolam* infusion loading dose: 100-300 mcg/kg IV followed by IV infusion of 1-2 mcg/kg/min; increase by 1-2 mcg/kg/min every 15 min if seizures persist (effective range 1-24 mcg/kg/min); closely monitor hemodynamics and support blood pressure as indicated; when seizures stop, continue same dose for 48 h then wean by decrements of 1-2 mcg/kg/min every 15 min
Propofol* initial bolus: 2 mg/kg IV; repeat if seizures continue and follow by IV infusion of 5-10 mg/kg/h, if necessary, guided by EEG monitoring; taper dose 12 h after seizure activity stops; closely monitor hemodynamics and support blood pressure as indicated
With phenobarbital-induced anesthesia, repeated boluses of 10 mg/kg are administered until cessation of ictal activity or appearance of hypotension; closely monitor hemodynamics and support blood pressure as indicated
*Not approved by the FDA for the indicated use.
†Doses for fosphenytoin administered in phenytoin equivalents (PE).
‡An alternative third step preferred by some authors is midazolam
administered by continuous IV infusion with a loading dose 0.1-0.3 mg/kg followed by infusion at a rate of 0.1-0.3 mg/kg/h.
Rajesh Ramachandrannair, MBBS, MD, FRCPC Associate Professor, McMaster University School of Medicine; Staff Neurologist, McMaster Children’s Hospital, Canada
Disclosure: Nothing to disclose.
Marcio Sotero de Menezes, MD Clinical Associate Professor, Department of Neurology, Division of Pediatric Neurology, Seattle Children’s Hospital, University of Washington School of Medicine; Director, Pediatric Neuroscience Center and Genetic Epilepsy Clinic, Swedish Neuroscience Institute
Marcio Sotero de Menezes, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society
Disclosure: Received salary from Novartis for speaking and teaching; Received salary from Cyberonics for speaking and teaching; Received salary from Athena diagnostics for speaking and teaching.
Ednea Simon, MD Consulting Staff, Swedish Pediatric Neuroscience Center
Ednea Simon, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society
Disclosure: Nothing to disclose.
Timothy E Corden, MD Associate Professor of Pediatrics, Co-Director, Policy Core, Injury Research Center, Medical College of Wisconsin; Associate Director, PICU, Children’s Hospital of Wisconsin
Timothy E Corden, MD is a member of the following medical societies: American Academy of Pediatrics, Phi Beta Kappa, Society of Critical Care Medicine, Wisconsin Medical Society
Disclosure: Nothing to disclose.
G Patricia Cantwell, MD, FCCM Professor of Clinical Pediatrics, Chief, Division of Pediatric Critical Care Medicine, University of Miami, Leonard M Miller School of Medicine; Medical Director, Palliative Care Team, Director, Pediatric Critical Care Transport, Holtz Children’s Hospital, Jackson Memorial Medical Center; Medical Manager, FEMA, Urban Search and Rescue, South Florida, Task Force 2; Pediatric Medical Director, Tilli Kids – Pediatric Initiative, Division of Hospice Care Southeast Florida, Inc
G Patricia Cantwell, MD, FCCM is a member of the following medical societies: American Academy of Hospice and Palliative Medicine, American Academy of Pediatrics, American Heart Association, American Trauma Society, National Association of EMS Physicians, Society of Critical Care Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.
Barry J Evans, MD Assistant Professor of Pediatrics, Temple University Medical School; Director of Pediatric Critical Care and Pulmonology, Associate Chair for Pediatric Education, Temple University Children’s Medical Center
Barry J Evans, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.
Garry Wilkes MBBS, FACEM, Director of Emergency Medicine, Calvary Hospital, Canberra, ACT; Adjunct Associate Professor, Edith Cowan University; Clinical Associate Professor, Rural Clinical School, University of Western Australia
Disclosure: Nothing to disclose.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Wayne Wolfram, MD, MPH Associate Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center
Wayne Wolfram, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.
Grace M Young, MD Associate Professor, Department of Pediatrics, University of Maryland Medical Center
Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians
Disclosure: Nothing to disclose.
Pediatric Status Epilepticus
Research & References of Pediatric Status Epilepticus|A&C Accounting And Tax Services
Source
0 Comments