History

Fact Explanation
Unresponsive [1] 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 [1] if left untreated, status epiliepticus is potentially fatal or can lead to irreversible brain damage [4]
Seizures / Jerky movements of limbs [3] 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 [1]
Twitching movements of the body [1] 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 [1]
History of epilepsy [1] Chronic processes that cause status epilepticus include preexisting epilepsy in which status epilepticus is due to breakthrough seizures or the discontinuation of antiepileptic drugs [1]
History of head trauma [1] Acute processes that cause status epilepticus include head trauma [1]
Fever [1] Central nervous system infection can cause status epilepticus [1] 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 [2]
Palpitations [2] 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 [2]
Vomiting [2] Occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures [2]
Incontinence [2] Occur due to profound autonomic changes that occur during status epilepticus because of the massive catecholamine discharge associated with continuous generalized seizures [2]
References
  1. LOWENSTEIN DANIEL H., ALLDREDGE BRIAN K.. Status Epilepticus. N Engl J Med [online] 1998 April, 338(14):970-976 [viewed 30 July 2014] Available from: doi:10.1056/NEJM199804023381407
  2. SIRVEN JI, WATERHOUSE E. Management of status epilepticus. Am Fam Physician [online] 2003 Aug 1, 68(3):469-76 [viewed 30 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12924830
  3. KUMAR M, KUMARI R, NARAIN NP. Clinical Profile of Status epilepticus (SE) in Children in a Tertiary Care Hospital in Bihar. J Clin Diagn Res [online] 2014 Jul, 8(7):PC14-7 [viewed 27 September 2014] Available from: doi:10.7860/JCDR/2014/9288.4579
  4. TRINKA E, HöFLER J, ZERBS A, BRIGO F. Efficacy and safety of intravenous valproate for status epilepticus: a systematic review. CNS Drugs [online] 2014 Jul, 28(7):623-39 [viewed 27 September 2014] Available from: doi:10.1007/s40263-014-0167-1

Examination

Fact Explanation
Unresponsive child [1] 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 [1]
Seizures [1] 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 [1]
Twitching movements of the body [1] 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 [1]
Febrile [1] Central nervous system infection can cause status epilepticus [1] 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 [2]
Signs due to autonomic changes - tachycardia, arrhythmias, hypertension, pupillary dilation [2] 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 [2]
Neurological examination [1] May show focal neurological signs (eg : uni lateral limb paralysis ) [1]
References
  1. LOWENSTEIN DANIEL H., ALLDREDGE BRIAN K.. Status Epilepticus. N Engl J Med [online] 1998 April, 338(14):970-976 [viewed 30 July 2014] Available from: doi:10.1056/NEJM199804023381407
  2. SIRVEN JI, WATERHOUSE E. Management of status epilepticus. Am Fam Physician [online] 2003 Aug 1, 68(3):469-76 [viewed 30 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12924830

Differential Diagnoses

Fact Explanation
Infections [1] Always consider the possibility of infections in pediatric patients presenting with generalized tonic-clonic status epilepticus (GTCSE). Sources of infection often, but not always, are obvious (eg, otitis media, pneumonia). Treat these infections appropriately because they contribute to lowering the seizure threshold in predisposed patients [1]
Catscratch disease [2] Consider catscratch disease, particularly in a school-aged child with a cat or kitten at home who presents with a history of unexplained mental status changes, status epilepticus (SE) of unknown etiology, prolonged seizures, or persistent fatigue. Catscratch fever is an infection acquired from cats (often from kittens) infected with Bartonella henselae via the cat flea [2]
Psychogenic seizures [3] Patients with nonepileptic seizures can reproduce an outward clinical seizure pattern as a manifestation of an unresolved psychological conflict (psychogenic seizure), or the seizure may be a manifestation of malingering, providing the patient with a clear secondary gain. -No loss of consciousness in the presence of bilateral movements -Asynchronous, side-to-side, and out-of-phase movements -Pelvic thrusting -Inconsistency of movement patterns and waxing and waning patterns -Persistent eye closure -Crying during the seizure [3]
References
  1. LOWENSTEIN DANIEL H., ALLDREDGE BRIAN K.. Status Epilepticus. N Engl J Med [online] 1998 April, 338(14):970-976 [viewed 30 July 2014] Available from: doi:10.1056/NEJM199804023381407
  2. PULIGHEDDU M, GIAGHEDDU A, GENUGU F, GIAGHEDDU M, MARROSU F. Epilepsia partialis continua in cat scratch disease. Seizure [online] 2004 Apr, 13(3):191-5 [viewed 30 July 2014] Available from: doi:10.1016/S1059-1311(03)00159-6
  3. ALSAADI TM, MARQUEZ AV. Psychogenic nonepileptic seizures. Am Fam Physician [online] 2005 Sep 1, 72(5):849-56 [viewed 30 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/16156345

Investigations - for Diagnosis

Fact Explanation
Electroencephalography (EEG) [1] 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 [1]
Blood cultures [1] 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. [1]
Lumbar puncture [1] 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 [1]
Antiepileptic drug levels [1] 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 [1]
Neuroimaging - computed tomography / Magnetic resonance imaging brain [1] 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 [1]
References
  1. BROPHY GRETCHEN M., BELL RODNEY, CLAASSEN JAN, ALLDREDGE BRIAN, BLECK THOMAS P., GLAUSER TRACY, LAROCHE SUZETTE M., RIVIELLO JAMES J., SHUTTER LORI, SPERLING MICHAEL R., TREIMAN DAVID M., VESPA PAUL M.. Guidelines for the Evaluation and Management of Status Epilepticus. Neurocrit Care [online] December, 17(1):3-23 [viewed 30 July 2014] Available from: doi:10.1007/s12028-012-9695-z

Investigations - Fitness for Management

Fact Explanation
Serum electrolytes [1] Important to assess as electrolyte imbalances can cause status epilepticus [1]
Full blood count [1] Infections in the central nervous system can cause status epilepticus [1]
Plasma casual venous glucose levels [1] Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected [1]
References
  1. BROPHY GRETCHEN M., BELL RODNEY, CLAASSEN JAN, ALLDREDGE BRIAN, BLECK THOMAS P., GLAUSER TRACY, LAROCHE SUZETTE M., RIVIELLO JAMES J., SHUTTER LORI, SPERLING MICHAEL R., TREIMAN DAVID M., VESPA PAUL M.. Guidelines for the Evaluation and Management of Status Epilepticus. Neurocrit Care [online] December, 17(1):3-23 [viewed 30 July 2014] Available from: doi:10.1007/s12028-012-9695-z

Investigations - Followup

Fact Explanation
Neuroimaging - computed tomography / Magnetic resonance imaging brain [1] 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 [1]
Hearing and vision assessment [2] Status epilepticus is associated with neurological sequelae of impaired Hearing and vision [2]
References
  1. BROPHY GRETCHEN M., BELL RODNEY, CLAASSEN JAN, ALLDREDGE BRIAN, BLECK THOMAS P., GLAUSER TRACY, LAROCHE SUZETTE M., RIVIELLO JAMES J., SHUTTER LORI, SPERLING MICHAEL R., TREIMAN DAVID M., VESPA PAUL M.. Guidelines for the Evaluation and Management of Status Epilepticus. Neurocrit Care [online] December, 17(1):3-23 [viewed 30 July 2014] Available from: doi:10.1007/s12028-012-9695-z
  2. PRINS A, CHENGO E, MUNG'ALA ODERA V, SADARANGANI M, SEATON C, HOLDING P, FEGAN G, NEWTON CR. Long-term survival and outcome in children admitted to kilifi district hospital with convulsive status epilepticus. Epilepsy Res Treat [online] 2014:643747 [viewed 27 September 2014] Available from: doi:10.1155/2014/643747

Investigations - Screening/Staging

Fact Explanation
Electroencephalography (EEG) [1] 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 [1]
Blood cultures [1] 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. [1]
Lumbar puncture [1] 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 [1]
Antiepileptic drug levels [1] 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 [1]
Neuroimaging - computed tomography / Magnetic resonance imaging brain [1] 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 [1]
Serum electrolytes [1] Important to assess as electrolyte imbalances can cause status epilepticus [1]
Random blood sugar levels [1] Because hypoglycemia may precipitate status epilepticus and is quickly reversible, 50 mL of 50 percent glucose should be given immediately if hypoglycemia is suspected [1]
References
  1. BROPHY GRETCHEN M., BELL RODNEY, CLAASSEN JAN, ALLDREDGE BRIAN, BLECK THOMAS P., GLAUSER TRACY, LAROCHE SUZETTE M., RIVIELLO JAMES J., SHUTTER LORI, SPERLING MICHAEL R., TREIMAN DAVID M., VESPA PAUL M.. Guidelines for the Evaluation and Management of Status Epilepticus. Neurocrit Care [online] December, 17(1):3-23 [viewed 30 July 2014] Available from: doi:10.1007/s12028-012-9695-z

Management - General Measures

Fact Explanation
Acute emergency management [1] 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. [1] 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 [2]
References
  1. LOWENSTEIN DANIEL H., ALLDREDGE BRIAN K.. Status Epilepticus. N Engl J Med [online] 1998 April, 338(14):970-976 [viewed 30 July 2014] Available from: doi:10.1056/NEJM199804023381407
  2. SIRVEN JI, WATERHOUSE E. Management of status epilepticus. Am Fam Physician [online] 2003 Aug 1, 68(3):469-76 [viewed 30 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12924830

Management - Specific Treatments

Fact Explanation
Pharmacologic Management - benzodiazepines [1] 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. [2]
Pharmacologic Management - Phenytoin [1] 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 [2]
Pharmacologic Management - Fosphenytoin [1] 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 [2]
Pharmacologic Management - Phenobarbital [4] 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 [2]
Pharmacologic Management - valproate [1] 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 [1]
Treatment of Refractory Status Epilepticus [1] Benzodiazepine-refractory status epilepticus (established status epilepticus, ESE) is a relatively common emergency condition [3] 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 [1]
References
  1. LOWENSTEIN DANIEL H., ALLDREDGE BRIAN K.. Status Epilepticus. N Engl J Med [online] 1998 April, 338(14):970-976 [viewed 30 July 2014] Available from: doi:10.1056/NEJM199804023381407
  2. SIRVEN JI, WATERHOUSE E. Management of status epilepticus. Am Fam Physician [online] 2003 Aug 1, 68(3):469-76 [viewed 30 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12924830
  3. BLECK T, COCK H, CHAMBERLAIN J, CLOYD J, CONNOR J, ELM J, FOUNTAIN N, JONES E, LOWENSTEIN D, SHINNAR S, SILBERGLEIT R, TREIMAN D, TRINKA E, KAPUR J. The established status epilepticus trial 2013. Epilepsia [online] 2013 Sep:89-92 [viewed 27 September 2014] Available from: doi:10.1111/epi.12288
  4. SáNCHEZ FERNáNDEZ I, ABEND NS, AGADI S, AN S, ARYA R, CARPENTER JL, CHAPMAN KE, GAILLARD WD, GLAUSER TA, GOLDSTEIN DB, GOLDSTEIN JL, GOODKIN HP, HAHN CD, HEINZEN EL, MIKATI MA, PEARISO K, PESTIAN JP, REAM M, RIVIELLO JJ JR, TASKER RC, WILLIAMS K, LODDENKEMPER T, PEDIATRIC STATUS EPILEPTICUS RESEARCH GROUP (PSERG). Gaps and opportunities in refractory status epilepticus research in children: a multi-center approach by the Pediatric Status Epilepticus Research Group (pSERG). Seizure [online] 2014 Feb, 23(2):87-97 [viewed 27 September 2014] Available from: doi:10.1016/j.seizure.2013.10.004