2. Seizures (convulsion): Seizures (convulsion): Define as is a transient occurrence of signs and/or symptoms resulting from abnormal excessive neuronal activity in the brain. Epilepsy: Epilepsy: It is a disorder of the brain characterized by an enduring predisposition to generate seizures, it is considered to be present when two or more unprovoked seizures occur at an interval greater than 24 hr apart.

5. Epileptic syndrome An epileptic syndrome is represent clinical entities in which age, pattern of clinical events, EEG features, natural history, and prognosis are distinctive.  Benign childhood epilepsy with centrotemporal spikes (rolandic epilepsy)  Benign neonatal convulsions  Benign myoclonic epilepsy in infancy  West syndrome (infantile spasms)

6. Laboratory Evaluation of Seizures CBC Blood chemistry: glucose, Ca, Na, K, Cl, HCO3, Mg, Ph Urea & creatinine Blood & urine for toxicology screen. In neonates blood for ammonia & inborn error of metabolism. CSF analysis. EEG & brain imaging. Viral study specially for enterovirus.

7. Principles of seizure therapy

8. Approach to the child with a suspected convulsive disorder

9. Principles of seizure therapy The 1 st step in the management of epilepsy is to exclude a condition that mimics epilepsy. A negative result on a neurologic examination and EEG usually supports the approach of watchful waiting rather than administration of an anticonvulsant. The 2nd step is when to start antiepileptic:  No need for antiepileptics for a previously healthy child with the 1st afebrile convulsion if there is a negative family history, normal results of a physical examination and EEG.  Approximately 75% of those patients with two or three unprovoked seizures have additional seizures. A recurrent seizure, particularly if it occurs in close proximity to the 1st seizure, is an indication to begin an anticonvulsant.  No need for long term treatment of benign febrile convulsion.  Therapy indicated for absence, atypical absence seizures & infantile spasm. The 3rd step involves choosing an anticonvulsant:  The drug of choice depends on the classification of the seizure, determined by the history and EEG findings.  Single, cheap, less toxic drug is the goal that lead to better compliance. Because most serious adverse anticonvulsant drug reactions develop during the initial 2–3 mo of therapy, monthly blood screening for the 1st 3 mo is recommended. Subsequently, routine blood tests are ordered only when clinically indicated.

10. Duration of therapy :  If complete seizure control is accomplished by an anticonvulsant, a minimum of two seizure-free years is an adequate and safe period of treatment for a patient with no risk factors.  When the decision is made to discontinue the drug, the weaning process should occur over 3–6 mo, because abrupt withdrawal may cause status epilepticus. generalized tonic clonic,tonic, clonic, absence & focal seizures not > than 2-4 yrs. Myoclonic seizures, atypical absence, lennox-Gastaut treatment for life. Risk for recurrence of seizures after discontinuation of therapy Those children who are neurologically abnormal. Those having seizures that were initially difficult to control. Those having persistent epileptiform EEGs. Best prognosis after anticonvulsant withdrawal : benign epilepsy with rolandic spikes. idiopathic generalized seizures.

11. Generalized tonic, clonic & tonic clonic seizures:  Most common childhood type.  May follow a focal seizure (second generalization) or occur de novo.  They may be associated with an aura, suggesting a focal origin of the epileptiform discharge.  Loss of posture & consciousness →tonic stiffening & upward deviation of eyes →pooling of secretion, pupillary dilation, diaphoresis → clonic jerk → briefly tonic again followed by flaccidity & urinary incontinence. During the seizure, children may bite their tongue.  Postictally, children are initially semicomatose and typically remain in a deep sleep from 30 min to 2 hr, weakness, often associated with vomiting and an intense bifrontal headache.  EEG  EEG during an attack demonestrate repetitive synchronus burst of spike activity followed by periodic paroxysmal discharges.  Brief seizures of any type not lead to brain damage but GTCS lasting > 30 min. is defined as status epilepticus & may lead to brain damage.Treatment:  A B C  Medication : LTG(lamotrigine), TPM(topiramate), VPA(valproic acid), carbamazepine, phenobarbital, phenytoin & LEV(levetiracetam).

13. Absence ( petit mal ) seizures :  Age 4 – 6 yrs  > in female  The clinical hallmark is a brief loss of enviromental awareness accompanied by eye fluttering or simple automatisms, such as head bobbing and lip smacking.  Patients do not lose body tone, but their head may fall forward slightly.  Provoked by hyperventilation or flashing light stimulation.  Never associated with an aura.  No LOC.  Children may have dozens of absence seizures per day.  Rarely persist longer than 30 sec.  Not associated with a postictal state.  Neurologic examination and brain imaging are normal  Characteristic EEG patterns (synchronous 3-Hz spike-and-wave activity /sec).  Treatment :Ethosuximide or Valproate + Clonazepam

14. Infantile spasms ( west syndrome )  Begin between the age of 3-8 mo.  Consists of a triad of infantile epileptic spasms, developmental regression, and a typical EEG picture (hypsarrhythmia).  Characterized by brief symmetrical contractions of the neck, trunk and extremities, followed by phase of sustained muscle contraction lasting 2 sec.  Of 3 types: 1-flexor spasm 2- extensor spasm 3- mixed.  Mixed spasm is the most common type.  A cry may precede or follow the spasm, (that confuse with colic).  The clusters of seizures may persist for minutes.  Spasm occur before sleeping or immediately after awakening.  Infantile spasms are typically classified into two groups: 1.An infant with cryptogenic infantile spasms: 10–20%, which have good prognosis. 2.Symptomatic infantile spasms are related directly to several prenatal, perinatal, and postnatal factors (metabolic like PKU,congenital malformation of the brain, tuberous sclerosis, congnital infection, HIE…etc.) those with the symptomatic type have an 80–90% risk of mental retardation. Treatment: ACTH, corticosteroid, benzodiazepine, valproic acid, vegabatrine

15. Febrile seizures : Definition : Genetic predisposition to seizures in infancy that precipitate by rapid rise in body temperature. Age 6 months-7 years (50% occur between 1-2 yrs). Febrile convulsion either simple or complex: Simple: * Generalized * < 15 min. * once/24hr Complex: * Focal * prolonged * multiple

16. Prognosis:  Excellent  Normal IQ  Further febrile seizure occur

17.  Epilepsy after seizure rare. Risk for epilepsy:  As general population 1%  risk increase in:  Complex febrile convulsion.  F.Hx. of epilepsy  Fever <1 hr before febrile seizure.  Neurodevelopmental abnormalities.

18. Management :  ABC  most important responsibility is to determine the cause of the fever and to rule out meningitis or encephalitis ; CSF if indicated.  laboratory testing: such as glucose, serum electrolytes and toxicology screening should be ordered based on individual clinical circumstances such as evidence of dehydration.  EEG: is not warranted after a simple febrile seizure but may be useful for evaluating patients with complex or atypical features or with other risk factors for later epilepsy.  Neuroimaging: is also not useful for children with simple febrile convulsions, but may be considered for children with atypical features.

19. Treatment (cont.) :  Reassurance and education of the parents.  Antipyretics: can decrease the discomfort of the child  If the seizure lasts for longer than 5 min, acute treatment with iv or rectal diazepam, lorazepam, or intranasal midazolam is needed.  IV benzodiazepines, phenobarbital, phenytoin, or valproate may be needed in the case of febrile status epilepticus.  Prolonged anticonvulsant prophylaxis for preventing recurrent febrile convulsions is controversial and no longer recommended for most children.  If parental anxiety is very high, oral diazepam may be used as an effective and safe method of reducing the risk of recurrence of febrile seizures. At the onset of each febrile illness, oral diazepam(1 mg/kg/24 hr  q8h), is administered for the duration of the illness (usually 2–3 days).  Iron deficiency is associated with an increased risk of febrile seizures, and thus screening for that problem and treating it appears appropriate.

20. Status epilepticus is a medical emergency of continuous seizure lasting longer than 20- 30 min or the occurrence of serial seizure activity between which there is no return of consciousness.

21. Etiology : 1.Prolonged febrile seizure. 2.Idiopathic status epilepticus. 3.Symptomatic status epilepticus due to underlying neurologic or metabolic abnormalities as meningitis & encephalitis, congenital malformation, electrolyte abnormalities…

22.  If appropriate therapy is delayed, SE can cause permanent neurologic sequelae or death …” thus  “ … any child who presents actively convulsing should be assumed to have SE.”

23. The longer status epilepticus persists,  the lower is the likelihood of spontaneous cessation  the harder is it to control  the higher is the risk of morbidity and mortality Treatment for most seizures needs to be instituted after > 5 minutes of seizure activity

24. Management: 1. STABILIZATION:  ABCs (airway, breathing, circulation)  Cardiac monitoring  O2 and pulse oximetry  Intravenous access  Immediate laboratory tests Glucose Basic metabolic panel-sodium, calcium, magnesium Anticonvulsant drug levels Toxicology studies as appropriate CBC, platelets, & differential

25. 1. PHARMACOLOGIC MANAGEMENT  Initial treatment: Benzodiazepine:  Lorazepam 0.05-0.1 mg/kg  Diazepam 0.2-0.5 mg/kg  Midazolam 0.1-0.2 mg/kg  Second line treatment: if the sz not resolved after 2 doses of benzodiazepine.  Fosphenytoin 10-20 mg/kg (phenytoin equivalents)  Phenobarbital 10-20 mg/kg  Valproic acid 20 mg/kg  3rd line treatment: continous infusions of  Pentobarbital or midazolam  + continous EEG monitoring  4 th line treatment:  General anesthesia

26. NOTE: The use of anticonvulsant therapy after status epilepticus is controversial. There is little question that a long-term antiepileptic should be maintained in children with a progressive neurologic disorder or with a history of recurrent seizures before the onset of status epilepticus. It is unlikely that a lengthy period of anticonvulsant treatment is necessary after an initial attack of idiopathic status epilepticus, particularly when a prolonged febrile seizure was the cause. Anticonvulsant therapy is maintained for 3 mo in this case and is discontinued if the child remains asymptomatic.