السلام عليكم ورحمة الله وبركاته

1. CONVULSIONS السلام عليكم ورحمة الله وبركاته By: Dr. Mohamed Abunada Head Pediatric Neurology Department El Rantisi specialized Pediatric Hospital - Gaza

2. INTRODUCTION • The word convulsion (or seizure) describes an involuntary violent spasm, or a series of jerking of the face, trunk, or extremities with or without loss of consciousness, sensory, autonomic or behavioral disturbances. • The word epilepsy describes a syndrome of recurrent unprovoked, seizure unrelated to fever or to acute cerebral insult.

3. INTRODUCTION • Epilepsy is a symptom complex arising from disordered brain function that it self may be secondary to a variety of pathologic processes. • Status epilepticus is a severe form of seizure activity lasting more than 30 minutes or recurrent seizures with failure to recover consciousness between repeated attacks.

4. Common causes • Head injury • CNS infection • Toxins • Metabolic disorder • Systemic disorders (endocrine, renal, hepatic) • Degenerative brain disorder • Cerebrovascular disease • Pyridoxine defeciency • Hereditary disorder • Specific epilepsy syndromes

5. EEG AND NEUROIMAGING A routine interictal (between seizures) EEG will show an epileptiform abnormality in only 60% of patients with epilepsy. Procedures that may activate a convulsion during the EEG include eye closure, hyperventilation and sleep deprivation. Angiography for excluding cerebrovascular accident. CT & MRI to exclude structural brain disorder Other investigations include metabolic, toxic and septic screen.

6. PATHOPHYSIOLOGY OF CONVULSIONS During a convulsive fit: -Cerebral O2 consumption increases to 300% Cerebral blood flow increases to 900%. The previous changes lead to: -Hypoxic ischemic brain injury. - Metabolic brain injury. - Structural brain injury.

7. PATHOPHYSIOLOGY OF CONVULSIONS Therefore systemic pathophysiological changes may include: • CNS: • Cerebral ischemia. • Brain edema. • Cerebral hemorrhage. • Brain damage.

8. PATHOPHYSIOLOGYOF CONVULSIONS Respiratory: Airway obstruction. Apnea. Pulmonary edema. Aspiration pneumonia. CVS: Shock. Heart failure. Hypertension. Cardiac arrest.

9. PATHOPHYSIOLOGYOF CONVULSIONS • Metabolic: • Metabolic acidosis. • Hyperpyrexia. • Hypoglycemia, hyponatremia. • Short repetitive fits are more serious than prolonged fits as long fits induce cerebral vascular compensatory changes better than short repetitive fits.

10. Classification of convulsions • Partial seizures: • Simple partial seizures. • Complex partial seizures. • Partial seizures with secondary generalization. • Generalized seizures: • Generalized typical absence seizure (petit mal). • Generalized atypical absence seizure (atypical petit mal). • Generalized myoclonic seizure. • Generalized tonic, clonic,or tonic-clonic seizure (grand mal) • Infantile spasms (with hypsarrhythmic EEG)

11. Simple partial seizures Consciousness retained Asynchronous. Clonic or tonic motor movement (such as eye twitches). Rarely persisting longer than 10-20 seconds. The EEG characteristically shows unilateral spikes or sharp waves in the anterior temporal region.

12. Complex partial seizures • consciousness impaired • The average duration is 1-2 minutes. • The aura signals the onset of convulsion in 30% of children. • In the aura, the child complains of epigastric discomfort, fear, or unpleasant feeling.

13. Complex partial seizures • Automatisms are repetitive stereotyped behaviors that occur in 50-70% of children with complex partial seizures. • Automatism follows loss of consciousness and may include lip smacking, chewing, repetitive swallowing, excessive salivation, picking and pulling at clothing. • The EEG is characterized by sharp waves or spike discharges in the anterior temporal or frontal lobe, or by multifocal spikes.

14. Partial seizures with secondary generalization During the partial Seizure the epileptiform discharge may spread from the temporal lobe throughout the cortex causing a generalized tonic-clonic convulsion.

15. Generalized typical absence seizure(Petit mal) Onset at age more than 3 years. Brief (5-20 sec.) lapses in consciousness, speech or motor activity. Not accompanied by an aura. Hyperventilation for 3-4 minutes frequently induces a seizure. The EEG is characterized by 3/sec spike and generalized wave discharges.

16. Generalized myoclonic seizures Brief repetitive symmetric muscle contractions with loss of body tone. Cause the child to fall because of a sudden loss of postural tone.

17. Generalized tonic, clonic,or tonic-clonic seizures (grand mal) • Sudden loss of consciousness. • Loss of bladder control. • Perioral cyanosis. • Followed by 30-60 minute peroid of deep sleep and postalictal headache.

18. Infantile spasms(with hypsarrhythmic EEG) • Begins in the first year of life. • Characterized by large myoclonic (salaam) spasms. • There are at least 3 types of spasms: flexor, extensor and mixed. • High incidence of subsequent retardation.

19. FEBRILE CONVULSION Definition: convulsion associated with fever between 6 months and 5 years of age without evidence of intracranial infection or other CNS pathology.. The most common convulsive disorder of childhood. The most common age of onset is 14-18 months. The convulsion is usually generalized tonic- clonic of few seconds duration. The problem always resolves without sequelae.

20. FEBRILE CONVULSION • Exceedingly long febrile convulsions may carry some risk of brain damage. • A history of febrile convulsion in a close relative is a risk factor for the development of febrile convulsion. • Recurrence of convulsion after the first febrile convulsion is common (>33%). • Recurrence is more common in young infants. • Fewer than 5% of children who have febrile convulsion develop epilepsy.

21. Risk factors for developing epilepsy • Family history of afebrile convulsion. • Complex first febrile convulsion. • Initial febrile seizure at age < 9 months. • Prior neurologic or developmental abnormalities existed.

22. Types of febrileconvulsion • Simple febrile convulsions • Complex febrile convulsions

23. Simple febrile convulsions • Brief (< 15 minutes). • Occurs as a solitary event (one attack/24 hours). • Typically generalized tonic-clonic convulsions. • Followed by a brief period of postictal drowsiness.

24. Complex febrile convulsions • Long (> 15 minutes). • Repeated convulsions for several hours or days. • May be focal, or generalized tonic-clonic convulsions. • Followed by a long period of postictal drowsiness.

25. EEG is indicated for • The patient with atypical seizure. • The child at risk for developing epilepsy.

26. Lumbar Puncture • LP is indicated for all infants aged < one year with febrile convulsions. • LP should be considered for any patient aged 12-18 months if a primary focus is not found. • For age > 18 months meningeal signs are typically present. LP is deferred if such signs are not present.

27. Prophylacticanti-epileptic treatment Following a febrile seizure, treatment with prophylactic anti-epileptics may be considered in: The very young child if febrile seizures recur. Children with pre existing neurologic abnormalities. Children with complex febrile seizures.

28. Treatment of febrile convulsion • Active measures to control fever. • Treatment of the cause. • Reassurance of the parents. • Oral diazepam 0.3 mg/kg/every 8 hours (1 mg/kg/day) reduces the risk of febrile seizures by nearly %50. It is administered at the onset of each febrile illness for 2-3 days.

29. NON FEBRILE CONVULSIONS: Indications for anticonvulsant therapy Typical or atypical petit mal, Myoclonic seizures, and Infantile spasms. Two or more unprovoked seizures occur within 6-12 months. A single afebrile tonic-clonic seizure has a high probability of not recurring, therefore anticonvulsant is not advised following the initial tonic-clonic seizure. Prolonged convulsions may require large and repeated doses of anticonvulsant, and consequently mechanical ventilation.

30. ANTICONVULSANT CHOICE BY SEIZURE TYPE:

31. COMMON ANTICONVULSANT DRUGS:

32. THERAPEUTIC DRUG LEVEL MONITORING At the initiation of therapy to ensure achievement of therapeutic range. During times of accelerated growth. If the seizures are out of control.

33. STATUS EPILEPTICUS: SE is defined as continuous seizure activity for at least 30 minutes, or recurrent seizures without a return to base line level of consciousness between seizures. Generalized convulsive SE is the most common type in pediatric population. · 

34. The etiologies of SE The etiologies of SE can be organized into 4 broad categories: • Atypical febrile seizures. • Acute CNS disorders (trauma, infection, metabolic) • Idiopathic symptomatic epilepsy. • Chronic, or progressive neurological disorders.

35. STATUS EPILEPTICUS • Morbidity is more likely in individuals with severe CNS pathology. • Seizures of prolonged duration may be associated with increased morbidity. • Mortality is often related to the underlying etiology, and, therefore highest mortality is associated with tumors.

36. The pathophysiological changes : The pathophysiological changes can be organized into 3 broad categories: At the cellular level: the unique events that cause continued seizures may lead to cell injury and death. The brain adaptive mechanisms, which attempt to limit injury, and, with continued seizures, may contribute to its pathology. Profound systemic effects that may affect morbidity and mortality. -

37. Goals of treatment Goals of treatment can be organized into 4 broad categories: • Initial stabilization. • Terminate seizure activity. • Prevent seizure recurrence. • Establish a diagnosis and initiate therapy for treatable causes.

38. TREATMENT STRATEGY • Initial stabilization. • First line ( benzodiazepines). • Second line (phenytoin & barbiturates). • Third line (Refractory status epilepticus). • Unique therapeutic modalities

39. Initial stabilization Establish airway, apply oxygen and ventilation, establish IV access and take samples for initial studies.

40. First line ( benzodiazepines) • Diazepam: 0.5 mg/Kg (maximum 10 mg) slow IV (not > 1mg/min) or per rectum (undiluted). • Lorazepam: 0.05-0.1 mg/Kg intravenous, per rectum or sublingual. • Midazolam: 0.1-0.2 mg/Kg IV or IM • Benzodiazepines dose may be repeated every 10 minutes up to 4 doses. Monitor respiration.

41. Second line (phenytoin & barbiturates) • Phenytoin: 20 mg/Kg slow IV (no faster than 1 mg/Kg/min with a maximum of 50 mg/min). An additional 5 mg/kg may be given prior to initiation of barbiturates. Monitor heart rate and blood pressure. • Phenobarbitone: 15-20 mg/Kg slow IV (no faster than 1 mg/Kg/min). Monitor blood pressure and respiration.

42. Third line(Refractory status epilepticus) RSE: Ongoing seizure activity that fails to respond to initial doses of benzodiazepines and loading doses of phyenytoin and phenobarbitone. Phenobarbitone: use repeated bolus doses of 5-20 mg/Kg, spaced by enough time to allow penetration of the drug to the CNS (approximately 30-60 min). Maximum doses administered in 24 h ranged from 30-120 mg/Kg (median 60 mg/Kg) and the maximum blood levels achieved were 80-350 μ/ml.

43. Third line(Refractory status epilepticus) • Midazolam: use a bolus of 0.2 mg/Kg followed by infusion of 0.2/Kg/h, and increase the dose until seizures are terminated. Monitor heart rate, blood pressure and ECG. • Pentobarbital (pentothal): use repeated doses of 3-5 mg/Kg slow IV (no faster than 50 mg/min) followed by infusion of 2-10 mg/Kg/hour. Monitor heart rate, blood pressure and respiration.

44. Unique therapeutic modalities : Lidocaine: use a bolus dose of 1-2 mg/Kg slow iv followed by constant infusion of 2 mg/Kg/h. Monitor the heart for heart block and blood pressure. Paraldehyde 150 mg/kg IM, or 300 mg/Kg per rectum. Moderatehypothermia 34˚ C

45. Unique therapeutic modalities • General anaesthesia probably acts by reversing cerebral anoxia and the concomitant metabolic abnormalities allowing the previously anticonvulsants to exert their effect. General anaesthesia needs to be administered in an operating room for long periods with anaesthesia equipment.

46. First line (Reception room) • Diazepam 0.5 mg/kg • Slow iv (max. dose 10 mg) • Undiluted per rectum • Or, lorazepam 0.05-1 mg/kg • Slow iv • Per rectum • sublingal • Or, midazolam 0.2 mg/kg • Slow iv • intramuscular

47. Second line (General ward) • Phenytoin 15-20 mg/kg • Slow iv • No faster than 1 mg/kg/min. • Maximum 50 mg/min • If seizures persist • Phenobarbitone 15-20 mg/kg • Slow iv • No faster than 1 mg/kg/min

48. Third line (Intensive care unit) • Midazolam 0.2 mg/kg • Slowly iv bolus dose • Followed by 0.2 mg/kg/h • By iv infusion titrated to effect • If seizures persist • Phenobarbitone 5-20 mg/kg • Slow iv repeated boluses every hour • Up to 30-120 mg/kg/day If seizures persist pentobarbitone (pentothal) 3-5 mg/kg • Slow iv repeated boluses every 30-60 min • Followed by 2-10 mg/kg/h iv infusion titrated to effect

49. Fourth line (Unique therapeutic modalities) • Lidocaine 1-2 mg/kg • Slow iv bolus • Followed by 2 mg/kg/h iv infusion • Paraledhyde 150 mg/kg intramuscular • Or, 300 mg/kg per rectum Moderate hypothermia • General anasthesia • Halothane, or isoflurane

50. Neonatal seizures • Subtle seizures • Deviation of the eyes • Eyelids are flickering • Swimming or pedaling movements • Apnoeic spells • Tonic • Clonic • Myoclonic • Seldom tonic clonic seizures