History

Fact Explanation
History of the patient is in a non convulsive continuous or distant stuporous state for 30 minutes or longer. Petit mal status epilepticus is a continuous or repetitive typical absence seizures lasting 30 minutes or longer without recovery of consciousness. The characteristic symptom is the altered state of consciousness while the patient is partly responsive. [1],[2],[3],[4],[5],[6]
History of jerky movements of muscles. In typical absence seizures periorbital, lid, perioral or limb myoclonic jerks occur.[1],[2],[4],[3],[5],[6],
History of previous similar episodes of staring spells spells If this is a known patient must take a descriptive past medical history from parents including onset, pattern, frequency of staring spells. The absence seizures do not show post ictal period and characterized by immediate resumption of what patient was doing before the seizure. The typical absence seizures usually start at 5-8 years of age. Typical absence attacks are never due to acquired lesions such as tumors. They are developmental abnormalities of neuronal control.[1],[2],[3],[4],[5],[6]
History of developmental delay. Typical absence seizures results due to developmental abnormalities of neuronal control. Therefore developmental history is very important to identify any developmental delay. [1],[2],[3],[4],[5],[6]
History of declining of school performance. Due to lapses of attention due to staring spells child gets difficulty in in school performance. [1],[2],[3],[4],[5],[6]
History of non compliance with treatment. If this is a known patient on anti epileptic therapy compliance on treatment is very important. Missing doses, inadequate dosing and discontinuation of anti epileptic treatments against physician's advise are the common treatment related precipitating factors. Take a drug history to identify if any other drugs has taken by patient which can interfere with metabolism of anti epileptics. [1],[2],[3],[4],[5],[6]
History of other precipitating factors. Sleep deprivation, stress, and flash lights and alcohol consumption in young adults are common precipitating factors. Single or combine effect of these factors provoke status epilepticus. [1],[2],[3],[4],[5],[6]
Family history of similar conditions. As petit mal epilepsy is related with genetic disorders, family history is important. [1],[2],[3],[4],[5],[6]
References
  1. TU TM, LOH NK, TAN NC. Clinical risk factors for non-convulsive status epilepticus during emergent electroencephalogram. Seizure [online] 2013 Nov, 22(9):794-7 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2013.05.019
  2. ALROUGHANI R, JAVIDAN M, QASEM A, ALOTAIBI N. Non-convulsive status epilepticus; the rate of occurrence in a general hospital. Seizure [online] 2009 Jan, 18(1):38-42 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2008.06.013
  3. GRECO F, COCUZZA MD, SMILARI P, SORGE G, PAVONE L. Nonconvulsive status epilepticus complicating epstein-barr virus encephalitis in a child. Case Rep Pediatr [online] 2014:547396 [viewed 18 June 2014] Available from: doi:10.1155/2014/547396
  4. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  5. LIBERALESSO PAULO BRENO NORONHA, GARZON ELIANA, YACUBIAN ELZA MARCIA T., SAKAMOTO AMéRICO C.. Refractory nonconvulsive status epilepticus in coma: analysis of the evolution of ictal patterns. Arq. Neuro-Psiquiatr. [online] 2012 July, 70(7):501-505 [viewed 16 June 2014] Available from: doi:10.1590/S0004-282X2012000700006
  6. IYER RAJESHSHANKAR, NISHA SR. Absence status epilepsy: Report of a rare electro-clinical syndrome. Neurol India [online] 2014 December [viewed 17 June 2014] Available from: doi:10.4103/0028-3886.132448

Examination

Fact Explanation
There is an altered state of consciousness while the patient is partly responsive. Memory and higher cognitive intellectual functions such as abstract thinking, computation, and personal awareness are the main areas of disturbance. The severity of impairment of consciousness varies from mild to severe state. [1],[2],[3],[4],[5]
Presence of myoclonic jerks Periorbital twitching, perioral jerks, eye lid flickering and rarely limb jerks can be noticed in typical absence seizures. The presence of myoclonic jerks predicts difficulty in controlling seizures. [1],[2],[3],[4].[5]
Absence seizures can be precipitated by hyperventilation. Both typical and atypical seizures can be precipitated by 3-5 minute hyperventilation. [1],[2],[3],[4],[5]
Developmental delay. Typical absence seizures results due to developmental abnormalities of neuronal control. A developmental delay may be noticed during developmental assessment. Neurological examination may show signs cerebral palsy. [1],[2],[3],[4],[5]
Signs of related genetic disorders. Their may be signs of related genetic disorders such as neurocutaneous disorder (eg, tuberous sclerosis) and metabolic disorders. [1],[2],[3],[4],[5]
References
  1. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  2. ALROUGHANI R, JAVIDAN M, QASEM A, ALOTAIBI N. Non-convulsive status epilepticus; the rate of occurrence in a general hospital. Seizure [online] 2009 Jan, 18(1):38-42 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2008.06.013
  3. TU TM, LOH NK, TAN NC. Clinical risk factors for non-convulsive status epilepticus during emergent electroencephalogram. Seizure [online] 2013 Nov, 22(9):794-7 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2013.05.019
  4. IYER RAJESHSHANKAR, NISHA SR. Absence status epilepsy: Report of a rare electro-clinical syndrome. Neurol India [online] 2014 December [viewed 17 June 2014] Available from: doi:10.4103/0028-3886.132448
  5. LIBERALESSO PAULO BRENO NORONHA, GARZON ELIANA, YACUBIAN ELZA MARCIA T., SAKAMOTO AMéRICO C.. Refractory nonconvulsive status epilepticus in coma: analysis of the evolution of ictal patterns. Arq. Neuro-Psiquiatr. [online] 2012 July, 70(7):501-505 [viewed 16 June 2014] Available from: doi:10.1590/S0004-282X2012000700006

Differential Diagnoses

Fact Explanation
Complex Partial Seizures To differentiate this from absence seizures, complex partial seizures show aura, automatisms and post ictal phase with gradual recovery. But in absence seizure has abrupt onset and abrupt cessation of seizure. [1],[2],[3],[4],[5]
Atypical absence status epilepticus. Atypical absence status epilepticus is clinically characterized by fluctuating impairment of consciousness, often with other ictal symptoms such as repeated serial tonic or atonic seizures and segmental or generalized jerks. [1],[2],[3],[4],[5]
Febrile seizures Atypical febrile seizures can present with prolonged or repetitive seizures. Elevated temperature is very important sign to suspect this. [1],[2],[3],[4],[5]
Attention deficit hyperactivity disorder It is a developmental condition characterized by inattention, impassivity and hyperactivity. [1],[2],[3],[4],[5]
Prolonged confusion condition Usually their are evidence from the history, physical examination or investigation that the impaired consciousness is caused by a direct physiological effect of a medical condition, intoxicating of substance, medication use, or more than one . [1],[2],[3],[4],[5]
Psychogenic Nonepileptic Seizures They are pseudoseizures. Pseudoseizures are paroxysmal episodes that resemble an epileptic seizure. Most of the time they are psychological in origin. [1],[2],[3],[4],[5]
Reflex Epilepsy Reflex epilepsy is a condition in which seizures can be provoked by an external stimulus. Common triggers for reflex seizures are visual stimuli, followed by sensory, auditory, somatosensory, olfactory, or proprioceptive stimuli. [1],[2],[3],[4],[5]
Breath-holding spells Common in some children after experiencing severe pain or severe anger. [1],[2],[3],[4],[5]
Migrain Sometimes migraine presents with atypical presentations other than headache. Common variant presentations are sensory, motor, or visual aura and confusion. [1],[2],[3],[4,[5]
References
  1. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  2. ALROUGHANI R, JAVIDAN M, QASEM A, ALOTAIBI N. Non-convulsive status epilepticus; the rate of occurrence in a general hospital. Seizure [online] 2009 Jan, 18(1):38-42 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2008.06.013
  3. GRECO F, COCUZZA MD, SMILARI P, SORGE G, PAVONE L. Nonconvulsive status epilepticus complicating epstein-barr virus encephalitis in a child. Case Rep Pediatr [online] 2014:547396 [viewed 18 June 2014] Available from: doi:10.1155/2014/547396
  4. LIBERALESSO PAULO BRENO NORONHA, GARZON ELIANA, YACUBIAN ELZA MARCIA T., SAKAMOTO AMéRICO C.. Refractory nonconvulsive status epilepticus in coma: analysis of the evolution of ictal patterns. Arq. Neuro-Psiquiatr. [online] 2012 July, 70(7):501-505 [viewed 16 June 2014] Available from: doi:10.1590/S0004-282X2012000700006
  5. IYER RAJESHSHANKAR, NISHA SR. Absence status epilepsy: Report of a rare electro-clinical syndrome. Neurol India [online] 2014 December [viewed 17 June 2014] Available from: doi:10.4103/0028-3886.132448

Investigations - for Diagnosis

Fact Explanation
Blood glucose level To identify hypoglycemia which is a metabolic condition provoke seizures. [1],[2],[3],[4]
Serum electrolyte level To identify electrolyte imbalance which is a metabolic condition provoke seizures. [1],[2],[3],[4]
Anticonvulsant level This is important in a diagnosed patient on anticonvulsant therapy. [1],[2],[3],[4]
Toxicology screen If drug overdose or drug abuse is suspected this is performed. [1],[2],[3],[4]
EEG Ictal EEG confirms the diagnosis with continuous, greater than 2.5 Hz generalized spike-and-slow wave complexes. Ictal EEG during the absence status may consist of repetitive discharges of multiple spikes and slow waves. Inter ictal EEG of idiopathic generalized epilepsy usually shows brief discharges with similar characteristics as those of the ictal EEG. [1],[2],[3],[4]
CT brain In idiopathic (typical) absence status epilepticus, all patients by definition are of normal physical and mental state and have normal brain imaging. [1],[2],[3],[4]
MRI brain MRI brain may be needed because of the possibility of a distinct epileptogenic focus amicable to surgery. [1],[2],[3],[4]
References
  1. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  2. ALROUGHANI R, JAVIDAN M, QASEM A, ALOTAIBI N. Non-convulsive status epilepticus; the rate of occurrence in a general hospital. Seizure [online] 2009 Jan, 18(1):38-42 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2008.06.013
  3. TU TM, LOH NK, TAN NC. Clinical risk factors for non-convulsive status epilepticus during emergent electroencephalogram. Seizure [online] 2013 Nov, 22(9):794-7 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2013.05.019
  4. LIBERALESSO PAULO BRENO NORONHA, GARZON ELIANA, YACUBIAN ELZA MARCIA T., SAKAMOTO AMéRICO C.. Refractory nonconvulsive status epilepticus in coma: analysis of the evolution of ictal patterns. Arq. Neuro-Psiquiatr. [online] 2012 July, 70(7):501-505 [viewed 16 June 2014] Available from: doi:10.1590/S0004-282X2012000700006

Investigations - Followup

Fact Explanation
Periodic monitoring of liver enzymes and full blood count Assess patient for side effects of antiepileptic medications and periodic monitoring of liver functions and full blood count because of the possibility of serious side effects such as hepatotoxicity and pancytopenia. [1],[2],[3].
References
  1. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  2. ALROUGHANI R, JAVIDAN M, QASEM A, ALOTAIBI N. Non-convulsive status epilepticus; the rate of occurrence in a general hospital. Seizure [online] 2009 Jan, 18(1):38-42 [viewed 18 June 2014] Available from: doi:10.1016/j.seizure.2008.06.013
  3. EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, INDIAN ACADEMY OF PEDIATRICS. Guidelines for diagnosis and management of childhood epilepsy. Indian Pediatr [online] 2009 Aug, 46(8):681-98 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19717860

Management - General Measures

Fact Explanation
Admit child to the HDU. This is a pediatric emergency with increased rate of morbidity and mortality. Any delay of management can cause hypoxic brain injury therefore emergency management with close observation is very important. [1],[2],[3]
Assess airway, breathing and circulation. Open the airway and give high flow oxygen via face mask. suck out the secretions. Assist ventilation and intubate if necessary. [1],[2],[3]
Insert IV cannulae Insert large bore IV cannulae and take blood for investigations. If found hypoglycemia, must treat it first. [1],[2],[3]
Monitor vital signs. Connect child to pulse oximeter and monitor pulse rate, blood pressure, saturation. [1],[2,[3]
Educate the patient. When patient is stable educate the patient about the condition with antiepileptic treatments and follow up he can lead a normal life. Advice to avoid precipitating factors such as sleep deprivation and alcohol in young adults. [1],[2],[3]
Educate the parents. Educate the parents and family members about the condition. Advise them how to identify a seizure episode. Advise them not to allow child to do unsafe activities such as climbing trees, bathing in rivers, standing near open fireplaces etc. Educate them about the importance of anti epileptic treatments, possible side effects and importance of follow up. [1],[2],[3]
References
  1. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  2. FERGUSON M, BIANCHI MT, SUTTER R, ROSENTHAL ES, CASH SS, KAPLAN PW, WESTOVER MB. Calculating the risk benefit equation for aggressive treatment of non-convulsive status epilepticus. Neurocrit Care [online] 2013 Apr, 18(2):216-27 [viewed 18 June 2014] Available from: doi:10.1007/s12028-012-9785-y
  3. EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, INDIAN ACADEMY OF PEDIATRICS. Guidelines for diagnosis and management of childhood epilepsy. Indian Pediatr [online] 2009 Aug, 46(8):681-98 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19717860

Management - Specific Treatments

Fact Explanation
Lorazepam IV Give IV lorazepam 0.005-0.1mg/kg. Because lorazepam not accumulate in lipid stores and has strong cerebral binding and long duration of action, it has distinct advantages over diazepam in early status epilepticus. [1],[2],[3],[4]
Diazepam PR If no IV access give diazepam 0.5mg/kg PR. Can repeat once again. [1],[2],[3],[4]
Buccal or IM midazolam Alternatively buccal midazolam 0.3mg/kg or IM 0.15mg/kg can be given. [1],[2],[3],[4]
Phenytoin IV or IO If seizure still continues give Phenytoin 18mg/kg IV or IO over 20 minutes. [1],[2],[3],[4]
Midazolam infusion or diazepam infusion If seizure still continues Midazolam 690-300 microgram/ kg/hr or Diazepam 100-400 microgram/ kg/hr IV infusion can be given. Use IO route if IV access is not available. [1],[2],[3],[4]
General anesthesia If seizure still continues do rapid sequence induction of anesthesia with thiopentone 4mg/kg/IV or IO. [1],[2],[3],[4]
Initiate long term maintenance therapy. If appropriate initiate long term maintenance therapy. Sodium Valporate, Ethosuximide and Clonazepam are commonly used. [1],[2],[3],[4]
References
  1. BILO LEONILDA, PAPPATà SABINA, DE SIMONE ROBERTO, MEO ROBERTA. The Syndrome of Absence Status Epilepsy: Review of the Literature. Epilepsy Research and Treatment [online] 2014 December, 2014:1-8 [viewed 17 June 2014] Available from: doi:10.1155/2014/624309
  2. PORTELA JL, GARCIA PC, PIVA JP, BARCELOS A, BRUNO F, BRANCO R, TASKER RC. Intramuscular midazolam versus intravenous diazepam for treatment of seizures in the pediatric emergency department: A randomized clinical trial. Med Intensiva [online] 2014 Jun 10 [viewed 18 June 2014] Available from: doi:10.1016/j.medin.2014.04.003
  3. FERGUSON M, BIANCHI MT, SUTTER R, ROSENTHAL ES, CASH SS, KAPLAN PW, WESTOVER MB. Calculating the risk benefit equation for aggressive treatment of non-convulsive status epilepticus. Neurocrit Care [online] 2013 Apr, 18(2):216-27 [viewed 18 June 2014] Available from: doi:10.1007/s12028-012-9785-y
  4. EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, INDIAN ACADEMY OF PEDIATRICS. Guidelines for diagnosis and management of childhood epilepsy. Indian Pediatr [online] 2009 Aug, 46(8):681-98 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19717860