History

Fact Explanation
Prolonged seizure attack Status epilepticus is defined as a seizure that lasts for more than 30 minutes or repeated seizures attacks without full recovery of consciousness in between attacks.[1] The presentation of the patient will be different according to the seizure semiology. Status epilepticus is a medical emergency which needs prompt treatment. The mortality rate of about 10-15% has being reported in certain settings.[2]
Generalized tonic-clonic status epilepticus The patient will experience a prolonged convulsion with phases of sustained muscle contraction (tonic) and rhythmic jerks of limbs (clonic).[3] Described as 3 phases, a partial seizure will be seen in phase 1 with gradual secondary generalization in phase 2. In phase 3 the seizure attack gradually subsides and rhythmic myoclonic movements may develop.
Systemic complications due to status epilepticus Seizures lasting for a prolonged duration may lead to widespread systemic derangements.[4] The patient may experience respiratory difficulty and pulmonary edema may develop. The laryngeal reflexes may diminish leading to aspiration. Cardiovascular changes such as hypotension and arrhythmias may be experienced. Prolonged muscular activity will lead to an increase in body temperature (hypothermia). Violent muscular contractions may cause trauma.
Symptoms related to the aetiological agent The patient may be a diagnosed patient with epilepsy previously. Abrupt drug regime changes and poor compliance to treatment may predispose to status epilepticus. Other aetiological agents for status epilepticus include central nervous system infection, head injury, cerebral damage at birth, exposure to toxins, systemic illness, neurocutaneous syndromes etc.[5] Symptoms related to both predisposing conditions and precipitating factors may be present.
References
  1. LOCKEY A S. Emergency department drug therapy for status epilepticus in adults. [online] 2002 March, 19(2):96-100 [viewed 16 June 2014] Available from: doi:10.1136/emj.19.2.96
  2. LACEY DJ. Status epilepticus in children and adults. J Clin Psychiatry1988;49 (suppl 12):33–6.
  3. SAGDUYU A, TARLACI S, SIRIN H. Generalized tonic-clonic status epilepticus: causes, treatment, complications and predictors of case fatality. J Neurol [online] 1998 Oct, 245(10):640-6 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9776462
  4. RASPALL-CHAURE M, CHIN RF, NEVILLE BG, SCOTT RC. Outcome of paediatric convulsive status epilepticus: a systematic review. Lancet Neurol [online] 2006 Sep, 5(9):769-79 [viewed 17 June 2014] Available from: doi:10.1016/S1474-4422(06)70546-4
  5. TRINKA E, HöFLER J, ZERBS A. Causes of status epilepticus. Epilepsia [online] 2012 Sep:127-38 [viewed 20 June 2014] Available from: doi:10.1111/j.1528-1167.2012.03622.x

Examination

Fact Explanation
In acute situation assess A- airway, B- breathing, C- circulation. Examine the nervous system briefly.[1] Status epilepticus is a medical emergency which requires resuscitation and prompt drug therapy. It is important note that initial history and examination must be brief and targeted. A detailed patient assessment can be conducted after stabilization of the patient has being achieved. Examination findings may help in determining an aetiological agent.
Features of head injury Look for external evidence of head injury. Head injury may lead to raised intracranial pressure which may present with pupillary reflex changes, reduced conscious level, abnormalities in neurological examination and cushing reflex (Bradycardia, tachypnea, and hypertension).[2]
Features of meningitis : Fever, Bulging fontanelle, neck stiffness, photophobia, positive Kernig’s sign, presence of a petechial rash. Central nervous system infection may lead to status epilepticus.[3] Inflammation of the meninges will present as neck stiffness and a positive Kernig’s sign (pain and discomfort on extending the flexed leg when the thigh is bent). Meningococcal infection leads to the development of a petechial rash on the body.
References
  1. HEAFIELD MT. Managing status epilepticus : New drug offers real advantages BMJ [online] 2000 Apr 8, 320(7240):953-954 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1117894
  2. CLEMENT CM, STIELL IG, SCHULL MJ, ROWE BH, BRISON R, LEE JS, PERRY JJ, WELLS GA, CCC STUDY GROUP. Clinical features of head injury patients presenting with a Glasgow Coma Scale score of 15 and who require neurosurgical intervention. Ann Emerg Med [online] 2006 Sep, 48(3):245-51 [viewed 20 June 2014] Available from: doi:10.1016/j.annemergmed.2006.04.008
  3. LOCKEY A S. Emergency department drug therapy for status epilepticus in adults. [online] 2002 March, 19(2):96-100 [viewed 16 June 2014] Available from: doi:10.1136/emj.19.2.96

Differential Diagnoses

Fact Explanation
Syncope Syncope is the result of generalized low cerebral perfusion. The patient experiences blackening of the environment accompanied with sweating and paleness. Loss of consciousness is limited to few seconds from which the patient rapidly recovers. Syncope is usually not associated with clonic limb movements & urine/faecal incontinence. There is no amnesia for the event. Syncope is often triggered by events the subject fears, emotional stress etc.[1] Differentiation from seizures depends on the clinical history.
Non epileptic pseudo-seizures. Patients may mimic seizures for primary or secondary gains. Clinical history is used to differentiate epileptic seizures from pseudo-seizures. Features that support a diagnosis of peudo-seizures include very prolonged seizures, very high frequency of seizures and occurrence of seizures of only in public. Features such as incontinence, injuries are usually absent.[2]
Toxin or drug induced status epilepticus Toxins such as pesticides (organophosphate), cyanide, heavy metals, amphetamine may precipitate status epilepticus. During patient assessment it is important to exclude accidental ingestion of these toxins. Medications such as oral hypoglycaemic agents, anticonvulsants overdose etc may also precipitate status epilepticus.[3]
Central nervous system infection Symptoms of CNS infection in children may be vague and vary according to the age of the child. Younger children present with vague, non-specific symptoms. Non specific symptoms such as drowsiness, excessive crying, intolerance of food, vomiting etc may be the only symptoms present in infants. Examination features include bulging fontanelle, neck stiffness and positive Kernig’s sign.[4] Diagnosis requires a high index of suspicion. The diagnosis can be confirmed by isolation of the causative organism in blood culture or cerebrospinal fluid assessment.
Metabolic derangement Hypernatraemia, hyponatraemia, hypoclacaemia, hyperkalaemia, hypokalaemia are common metabolic changes which may precipitate status epilepticus.[5] Both hyper/ hypoglycaemia may also precipitate an attack.
Other differential diagnoses Other differential diagnoses that should be considered are transient Ischemic attacks, migraine, encephalitis, somatoform disorders etc.[6]
References
  1. STRICKBERGER S. A.. AHA/ACCF Scientific Statement on the Evaluation of Syncope: From the American Heart Association Councils on Clinical Cardiology, Cardiovascular Nursing, Cardiovascular Disease in the Young, and Stroke, and the Quality of Care and Outcomes Research Interdisciplinary Working Group; and the American College of Cardiology Foundation: In Collaboration With the Heart Rhythm Society: Endorsed by the American Autonomic Society. Circulation [online] 2006 January, 113(2):316-327 [viewed 17 June 2014] Available from: doi:10.1161/​CIRCULATIONAHA.105.170274
  2. BOWMAN ES. Pseudoseizures. Psychiatr Clin North Am [online] 1998 Sep, 21(3):649-57, vii [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9774802
  3. AMINOFF MJ, SIMON RP. Status epilepticus. Causes, clinical features and consequences in 98 patients. Am J Med [online] 1980 Nov, 69(5):657-66 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/7435509
  4. HOFFMAN O, WEBER RJ. Pathophysiology and Treatment of Bacterial Meningitis Ther Adv Neurol Disord [online] 2009 Nov, 2(6):1-7 [viewed 17 June 2014] Available from: doi:10.1177/1756285609337975
  5. ZELANO JOHAN, HALAWA IMAD, CLAUSEN FREDRIK, KUMLIEN EVA. Hyponatremia augments kainic-acid induced status epilepticus in the mouse: A model for dysmetabolic status epilepticus. Epilepsy Research [online] 2013 September, 106(1-2):29-34 [viewed 19 June 2014] Available from: doi:10.1016/j.eplepsyres.2013.05.010
  6. MAURICIO EA, FREEMAN WD. Status epilepticus in the elderly: differential diagnosis and treatment. Neuropsychiatr Dis Treat [online] 2011:161-6 [viewed 19 June 2014] Available from: doi:10.2147/NDT.S10537

Investigations - for Diagnosis

Fact Explanation
Investigations aid in the diagnosis and help detect a causative factor The first priority in management is to stabilize the patient. Investigations should cause minimum delay to the initial management. The choice of investigations should be in accordance to the clinical information.[1][2]
Plasma glucose level Both hyper/ hypoglycaemia are known to precipitate status epilepticus. [3]
Full blood count/ Blood culture In the presence of fever rule out an infective aetiology.[2] Urinalysis may be required if urine tract infection is suspected.
Lumbar puncture Analysis of cerebrospinal fluid is required in the presence of symptoms or signs of CNS infection.[2]
Serum electrolytes & plasma calcium and magnesium level Abnormalities in the plasma concentration of potassium, sodium, magnesium, calcium may precipitate status epilepticus. Check the plasma calcium and magnesium level.[3]
Toxicological screen To identify or rule out toxin induced status epilepticus.[3]
Arterial blood gas (ABG) Prolonged muscular contractions lead to generation of excess lactic acid which in turn will reflect in ABG analysis as metabolic acidosis. ABG also allows assessment of adequacy of oxygenation and ventilation of tissues.[3]
CT scan/ MRI If head injury is suspected to be the cause.[4]
Electroencephalography (EEG) EEG can be used to confirm the diagnosis and exclude conditions that mimic status epilepticus. EEG can differentiate between generalized and partial seizures. The type of seizure attack may also be identified (e.g. - Absence seizures).[5]
References
  1. LOCKEY A S. Emergency department drug therapy for status epilepticus in adults. [online] 2002 March, 19(2):96-100 [viewed 17 June 2014] Available from: doi:10.1136/emj.19.2.96
  2. TRINKA E, HöFLER J, ZERBS A. Causes of status epilepticus. Epilepsia [online] 2012 Sep:127-38 [viewed 18 June 2014] Available from: doi:10.1111/j.1528-1167.2012.03622.x
  3. NANDHAGOPAL R. Generalised convulsive status epilepticus: an overview Postgrad Med J [online] 2006 Nov, 82(973):723-732 [viewed 20 June 2014] Available from: doi:10.1136/pgmj.2005.043182
  4. PEETS AD, BERTHIAUME LR, BAGSHAW SM, FEDERICO P, DOIG CJ, ZYGUN DA. Prolonged refractory status epilepticus following acute traumatic brain injury: a case report of excellent neurological recovery Crit Care [online] 2005, 9(6):R725-R728 [viewed 20 June 2014] Available from: doi:10.1186/cc3884
  5. BRENNER RP. EEG in convulsive and nonconvulsive status epilepticus. J Clin Neurophysiol [online] 2004 Sep-Oct, 21(5):319-31 [viewed 19 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/15592006

Investigations - Followup

Fact Explanation
Brian imaging: CT scan/ MRI scan These investigations are indicated if a structural brain lesion is suspected such as infarction, abscess, intracranial tumor.[1]
References
  1. MASCHIO M. Brain Tumor-Related Epilepsy Curr Neuropharmacol [online] 2012 Jun, 10(2):124-133 [viewed 20 June 2014] Available from: doi:10.2174/157015912800604470

Management - General Measures

Fact Explanation
Pre-hospital care Position the patient in the left lateral position. Commence resuscitation with ABCDE. Studies have shown that administration of Benzodiazepines in pre-hospital stage have found that to be effective. Lorazepam ideally adminstered IV is found to be effective than diazepam.[1]
Immediate resuscitation at the emergency care unit Use the A-airway, B-breathing, C-circulation, D-disability, E-exposure approach.[2] Place the patient in the left lateral position to minimize aspiration. Maintain airway patency where an oropharyngeal airway may be needed in some situations.Look, listen and feel for breathing. Administer oxygen via a face mask. Check the pulse rate, pulse volume and blood pressure to assess circulation. Try to achieve intravenous (IV) access. Management method differs depending on the availability of an IV access.[3]
Monitoring of the patient The vital parameters should be monitored during and after the seizure attack. Oxygenation and ventilation need careful monitoring.[4]
Parent education and counseling The parents should be provided with information on the aetiology, precipitating factors, risks to the child from status epilepticus. The future investigation and management plan should involve the parents. Parents should be provided with information on first aid measures when encountering a similar episode in the future.[4]
References
  1. KAPUR JAIDEEP. Prehospital Treatment of Status Epilepticus with Benzodiazepines Is Effective and Safe. Epilepsy Currents [online] 2002 July, 2(4):121-124 [viewed 19 June 2014] Available from: doi:10.1046/j.1535-7597.2002.00045.x
  2. LOCKEY A S. Emergency department drug therapy for status epilepticus in adults. [online] 2002 March, 19(2):96-100 [viewed 17 June 2014] Available from: doi:10.1136/emj.19.2.96
  3. The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. National institute for health and care excellence, 2012[Viewed on 18 June 2014]. Available from : http://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-and-cg137/appendix-f-protocols-for-treating-convulsive-status-epilepticus-in-adults-and-children-adults
  4. NANDHAGOPAL R. Generalised convulsive status epilepticus: an overview Postgrad Med J [online] 2006 Nov, 82(973):723-732 [viewed 16 September 2014] Available from: doi:10.1136/pgmj.2005.043182

Management - Specific Treatments

Fact Explanation
Initiate anticonvulsant therapy In patients whom an IV access is available lorazepam 0.1mg/kg is administered IV over 30-60 seconds. The patient is observed for improvement and if seizures continue at 10 minutes a repeat dose is administered. In patients who do not have an IV access, administer diazepam 0.5mg/kg per rectally. The patient is kept under observation while efforts are made to establish IV access. In failure of status epilepticus to respond paraldehyde 0.4mg/kg is administered per rectally. If IV access could be achieved IV larazepam could be administered.[1] [2]
Seizure continuing/ persisting at 10 minutes with minimum response Administer phenytoin 18mg/kg IV over 20 minutes or phenobarbitone 20mg/kg IV over 10 minutes. Paraldehyde could be administered at 0.4mg/kg per rectally in combination with olive oil. Inform the intensive care unit and the on-call anesthetist.[3]
Use of midazolam or diazepam infusion Patients who fail to respond following the previous step, are administered IV midazolam infusion 60-300 microgram/kg/h or IV diazepam infusion 100-400 microgram/kg/h.[4]
Use of rapid sequence induction of anesthesia. In this situation rapid sequence induction of anesthesia is carried out using thiopentone sodium 4mg/kg IV. Transfer patient to intensive care unit.[4]
Monitoring for drug complications These drugs are known to cause respiratory depression which should be monitored in the patient.[4]
Long term follow-up Once the aetiological agent which predisposed to status epilepticus is identified, long term management should be planned in accordance. Some patients should be started on anticonvulsant medication for prevention of future episodes.[5]
References
  1. LOCKEY A S. Emergency department drug therapy for status epilepticus in adults. [online] 2002 March, 19(2):96-100 [viewed 17 June 2014] Available from: doi:10.1136/emj.19.2.96
  2. The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. National institute for health and care excellence, 2012[Viewed on 18 June 2014]. Available from : http://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-and-cg137/appendix-f-protocols-for-treating-convulsive-status-epilepticus-in-adults-and-children-adults
  3. 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 2012, 17(1):3-23 [viewed 19 June 2014] Available from: doi:10.1007/s12028-012-9695-z
  4. YAMANOUCHI H. [Treatment of status epilepticus]. Nihon Rinsho [online] 2014 May, 72(5):895-901 [viewed 19 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24912292
  5. HEAFIELD MT. Managing status epilepticus : New drug offers real advantages BMJ [online] 2000 Apr 8, 320(7240):953-954 [viewed 19 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1117894