Status epilepticus

lecture

1. Status epilepticus By NANCY MOHAMMED ALAA Assistant lecturer of pediatrics Assiut university

2. 1-definition 2-epidemiology 3-classification 4-pathophysiology 5-consequences 6-differential diagnosis 5-management A- prehospital B-at hospita l

3. status epileptics is defined as more than 30 minutes of either 1) continuous seizure activity clinically or subclinical (EEG) Or 2) two or more sequential seizures without full recovery of consciousness inbetween .

4. A new conceptual definition of status epilepticus with two operational dimensions ( t 1 and t 2 ) is proposed Time point t 1 indicates when treatment should be initiated, and time point t 2 indicates when long-term consequences may appear

5. The international League Against Epilepsy 2015 definition of status epilepticus indicates that emergency treatment of status epilepticus should be started at t 1 and long-term consequences may occur at t 2 . Time 2 (Consequences Expected) Time 1 (Treatment Started Status Epilepticus Type 30min 5min Tonic- clonic >60min 10min Focal with impaired consciousness unknown 15min Absence

6. EPIDEMIOLOGY 1-the annual incidence of SE in pediatrics is 10-73 episode/100,000 children and is highest in children < 2 years old 2-the higher incidence in vulenrable populations with acute or chronic neurological conditions 3 -75% of cases of SE may be the first seizure of life, and they will have 30% risk of later diagnosis of epilepsy

7. 4-The overall mortality of children is less than in adult, it has been reported to be 2-8% . 10-20% The morbidity is estimated to be

8. Classification According to A-TIME B-SEIZURE TYPE C-ETIOLOGY

9. A-TIME 1-The first five minutes of a seizure have been termed “ prodromal ” or “ incipient status epilepticus ” . 2-Continued seizure activity can be further subdivided into early status epilepticus (5–30 min), 3-established status epilepticus (>30 min), 4-refractory status epilepticus (ongoing status epilepticus clinical or electrographic) despite administration of 2–3 appropriately dosed anti-seizure medications).

10. 5- super-refractory status epilepticus is defined as recurrent seizures in spite of anticonvulsants and anaesthetic therapy beyond the 24-hour.

11. B-SEIZURE TYPE 1) convulsive status epileptics consisting of repeated A-generalized tonic– clonic (GTC) seizures B- myoclonus,tonic,clonic,atonic 2) Non convulsive :impairment of conscious level without convulsive movement but with EEG finding 3 ) repeated partial seizures partial seizures without impairment of consciousness or secondary generalisation , manifested as focal motor signs ( epilapsia partia;is continua ), focal sensory symptoms, or focal impairment of speech (aphasia)

12. focal generalized 1-epilapsia partia;is continua (motor) 1-GTC 2-tonic 3-myoclonic 4-atonic 5-clonic convulsive 1-aphasic 2-sensory 3-complex partial 1-abscence Non Convulsive (according to EEG)

13. C-ETIOLOGY

14. Pathophysiology 1-decrease inhibitory neurotransmitter At the transition from single seizures to status, GABA receptors move from the synaptic membrane to the cytoplasm, where they are functionally inactive. This reduces the number of GABA receptors available for binding GABA or GABAergic drugs, This explain the development of time-dependent pharmacoresistance to benzodiazepine s

15. 2-increase excitatory neurotransmitter . At the same time, 'spare' subunits of AMPA ( alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid ) and NMDA ( N-methyl-D-aspartic acid ) receptors move from subsynaptic sites to the synaptic membrane, causing further hyperexcitability and possibly explaining the preserved sensitivity to NMDA blockers( ketamine ) late in the course of SE.

16. Consequences

17. DIFFERENTIAL DIAGNOSIS

18. A-seizure and pseudoseizure . 2-pseudoseizure 1-seizure Often closed or fluttering Usually open, or with clonic blinking or nystagmus Eye no yes Rhythmic movement yes no Pelvic thrust non dilated Pupil Increase movement non restrain Decrease movement non reassurance no confused Direct after attack Crying/screaming occasiona Grunt with generalized convulsions; VOCALIZATIONS absent present cyanosis Waxes and wanes, some memory for event Lost suddenly, regained gradually; no memory RESPONSIVENESS Gradual, from waking or “ pseudosleep , Abrupt, from waking or sleep, ONSET

19. B- Generalized Convulsive Status Epileptics CHARACTERESTIC FEATURES NORMAL CONSCIOUS LEVEL rhythmic oscillation (back and forth movement) about a central point. INCREASE WITH STRESS DECREASE WITH SLEEP Tremor FIXED ABNORMAL POSITION Increase with voluntary action ,stress decrease at rest ,sleep BUT NORMAL CONSCIOUS LEVEL Dystonia FACIAL MUSCLE LOCK WITH OPISTHOTONUS POSITION Tetanus THE MOVEMENT IS EASILY ELICITED AND STOPPED WITH RAPID LIMB MOVEMENT Clonus

20. ( C -non convulsive status epileptics (NCSE Hallucinations Hypoglycemia Drug effects Psychosis POST ICTAL

21. EEG OF NCSE

22. D-Partial Convulsive (MOTOR ) CHARACTERESTIC FEATURES Usually at onset of sleep ,involve legs ,rarely repeat over long periods Hypnic Myoclonic jerks Rapid ,stereotyped involuntary movement usually involve face and neck. They disappear in sleep ,increase with stress Tic disorder Focal dystonia Focal dystonia involve eyes that increase with bright light and activity Blepharospasm

23. E-PARTIAL SENSORY 1-Ocular disorders 2-Hearing disorders 3-Migraine and its attendant neurologic phenomena ( eg , sensory, visual )

24. 3-Simple partial status epileptics Transient ischemia attack (TIA) and stroke Migraine and its attendant neurologic phenomena ( eg , sensory, visual) Transient postictal phenomena analogous to Todd paresis Ophthalmic disorders ( eg , retinal detachment), which may mimic visual simple partial (occipital) SE . ,.

25. Suggestion of partial status epileptics 1-when it arises in a patient with an established history of focal epilepsy 2-Evolution of SPSE from or to a clinically overt complex partial or secondarily generalized seizure also permits accurate diagnosis 3 -EEG validation can be especially important

26. Management A-pre hospital B-At hospital

27. A- prehospital

28. B-At hospital

29. 2-at hospital

30. 2-termination of seizures and prevention of recurrence disadvantage advantage Dose ,route Drugs hypotension, respiratory depression -If used within the first 20 min of seizure onset, termination rates of seizures can be as high as 70-85% . 1-LONGER DURATION 0.1mg/kg/IV UP TO 4 mg/dose repeat in 5 Min 0.15-0.2mg/kg IV up to 10mg/DOSE repeat in 5 min 0.2-0.5mg/kg PR up to 20mg/dose 1-benzodiazepine A- lorazepam B-diazepam

31. - disadvantage advantage Dose,route drug IM 5mg(13-40kg) 10mg( >40kg) IV 0.3mg/kg Buccal 0.5mg/kg Nasal 0.2mg/kg C- midazolam

32. 3-diagnosis and treatment of the life threatening causes of SE A- HISTORY Has the child ever had a seizure before? History of trauma? Fever? Ingestion? What medications (including nonprescription) does the child take? Any medical problems? Any neurologic/developmental problems? If child has known epilepsy Name and dosage of medications Has the child missed dosage of medication Be aware of paradoxical side effects of AEDs Phenytoin and carbamazepine toxicity may precipitate SE

33. B-EXAMINATION Head to toe examination aiming to determine underlying etiology General examination : vital signs: pulse, BP, RR,Temperature Anthropometric measures: HC( canavan ) Examination of the head: ant. fontanel ,skull shape and eyes (including fundus examination) Abnormal facies (metabolic disorders) Skin abnormality( neurocutaneous diseases) Search for evidence of trauma. Evidence of failure to thrive, particular body odour or hair pigmentation (in born error of metabolism) Cardiac examination : Congenital heart diseas

34. Chest examination : specially for accumulated secretions Abdominal examination : Hepatosplenomegaly Examination of the back Neurological examination: 1-Conscious level: AVPU system 2-Motor system. 3-Sensory system 4- tests of coordination 5- cranial nerves 6- Superficial reflexes 7- Gait 8- Meningeal signs

35. C-INVESTIGATIONS A- Lab investigations : 1.CBC: in patients who looks like ill, suspect sepsis 2.Serum electrolytes (Na, Ca ,Mg): Because 6% of pts with convulsions showing electrolyte and blood glucose disturbance 3.Serum level of anti epileptic drugs (AEDs): to detect compliance or toxcicity 4. Toxicology screen: if clinically suspected 5. Metabolic screen: depends upon history and examination 6-liver function test

36. B . lumbar puncture : it is indicated in: a-Any convulsions with fever below age of one year b-Convulsions with fever+ manifestations of CNS affection c-Atypical febrile convulsions after age of one year d-diagnosis of demyelinating disease or inflammatory (MS, GBS )

37. CONTRAINDICATIONS A-ABSOLUTE 1-signs of ICP ( papilledema , 2-local skin infections 3-Imaging show (midline shift, posterior fossa mass, 4-clinically unstable B-RELATIVE 1-bleeding tendency (DIC,PLT < 50,000)

38. C-indication for CT : 1- Any convulsions below age of one year except febrile convulsions 2-Focal convulsions except simple focal convulsions 3-Covulsions with focal neurological deficit, 4- disturbed conscious level or increased intracranial pressure" 5-Intractable convulsions 6-Status epileptics with unknown cause. 7-Abnormal fundus examination 8-History of trauma 9-EEG with focal source

39. D-MRI It is indicated if: - Auspect neurodegenrative disease - Areas not well visualized by CT -Uncertain lesion in CT

40. E - EEG 1-continuous EEG monitoring if the patient is not waking up after clinical seizures cease ( (15–60minutes 2-critically ill encephalopathic child 3-Patients who ultimately require continuous infusion with a barbiturate or benzodiazepine should undergo EEG monitoring It is helpful in defining the limits of therapy by the occurrence of burst suppression. .

41. 4-MANAGEMENT OF REFRACTORY STATUS EPILPTICS If the convulsive activity does not stop : 1.Fosphenytoin and phenytoin:the2 nd line medication : * Fosphenytoin is a prodrug of phenytoin has advantages of being water soluble, less irritating after IV injection and well absorbed after IM injection. * The loading dose :15 -20mg/kg IV at the rate of 1 mg/kg/min added to normal saline given up to three loading doses. * The maintenance dose of 3-6 mg/kg divided into two equal doses daily is begun 12-24 hr later * Side effects: 1-the undiluted drug can cause pain, irritation, and phlebitis of the vein 2- Arrhythmias and bradycardia 3- Systemic hypotension

42. 2.Phenobarbital is the 3rd line medication: is initiated before phenytoin in some centers and in neonates. Loading dose : 20 mg/kg in neonates IV during 10-30 min. :: 5-10mg/kg Infant and children Side effects: Respiratory depression It is given up to three doses , the maintenance dose is 3-5 mg/kg/24 hr divided into two equal doses

43. 4-valproic acid Dose : 20-40 mg/kg/IV May give additional 20 mg/kg Side effects : 1-hyperammonemia 2-thrombocytopenia 3-hepatotoxicity 3-levitracetam Dose :20-60 mg/kg/IV Precaution 1-dose adjustment in renal failure

44. 5. Induction of pharmacological coma :

45. 1-monitoring A-mechanical ventilation for airway protection ventilation B-central line (control BP, frequent sampling) C-EEG monitor D-temperature management 2-goal A-termination of seizure B-EEG; burst suppression 3-time of withdrawal : 24-48 hrs of seizure, EEG control