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SEIZURE DISORDERS

IN CHILDREN
Marietta M. Diaz,
MD,FPPS,FCNSP

Definition of Terms
 Seizure – transient occurrence of signs and symptoms
due to an abnormal excessive or synchronous neuronal
activity in the brain

 Epilepsy/Seizure Disorder – a disease of the brain
defined by any of the ff: 1. at least 2 unprovoked(or reflex
sz) occurring>24 hrs apart 2. 1 unprovoked(or reflex sz)
and a probability of further seizure similar to gen
recurrence risk after 2 unprovoked sz occurring within the
next 10 yrs 3. diagnosis of epilepsy syndrome
 Recurrrence Reflex seizure- represents provoked sz
defined as epilepsy;tendency to respond to such stimuli
with sz

Definition of Terms
 Epileptic Syndrome- disorder consisting of cluster of signs and
symptoms customarily occurring together plus its EEG findings.
The syndrome includes type of seizure, etiology, anatomy,
precipitating factors, age of onset, severity, prognosis and
genetic implication

 Non-epileptic Paroxysmal Disorders- paroxysmal events that
may mimic seizures

Resolved epilepsy- a person no longer has epilepsy but still
may recur; it is considered when 1. pt who had age dependent
epilepsy syndrome but now past the applicable age 2. pt.
remains sz free for 10 yrs or off AED for 5 yrs

Mechanism of Seizure Generation
Seizure
Cellular level generation Synaptic level

Cations neurotransmitters

Ca** channels
Na* channels K* channels
(T type Channel)

GABA
Inc. influx of Na Inc. influx of calcium
glutamate
Inc. intracellularly Decrease
Na & water Cell hyperexcitability Increase
concentration
concentration
Neuronal swelling Firing of thalamic and
Hyperexcitable
cortical neurons
state
Increase tissue
excitability

SEIZURE

Causes of Seizures .

2006-attempted  2013-on line feedbacks from updates clinicians and epileptologists- ILAE stand . therapeutics. meaningful revision to the  1981-1st seizure 1981 and 1989 classification classification published  Based on inputs from  1989 syndrome genetics. neuroimaging. classification.Classification: Epilepsy/Seizures Then Now  1960-1st suggested  2010-Developed methodically classification and conceptually sound. adult and pediatric conceptual epileptology  2001. research design.

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3 partial seizures evolving to secondarily generalized seizures 3.1 Absence 1. Generalized seizures-involvement of both hemispheres 1.4 Tonic 1. Unclassified seizures .2 complex 2.5 Tonic –clonic 1.6 Atonic 2.International Classification of Epilepsy 1981 1.1 simple 2. Partial seizures 2.3 Clonic 1.2 Myoclonic 1.

Generalized 2.1 idiopathic with age related onset 1.2 symptomatic 2.3 symptomatic 3.4 chronic progressive epilepsia partialis continua .1 situation related seizures 4. Special syndromes 4.3 epilepsies characterized by specific modes of seizure precipitation 4. Epilepsies and syndromes undetermined as to whether they are focal or generalized 3.1 with both gen and focal seizures 3.2 without unequivocal or focal features 4 . Localization related (focal local partial) 1.2 isolated apparently unprovoked seizures 4.2 idiopathic /symptomatic in order of age appearance 2.International Classification of Epilepsies and Epileptic Syndromes 1989 1.1 idiopathic with age related onset 2.

Classification as to Etiology Then Now  Idiopathic-presumed  Genetic. cortical malformation  Unknown-cause is unknown and might be genetic.genetic defect genetic directly contributes to the  Symptomatic-secondary to epilepsy and seizures are the core of the disorder- a known or presumed channelopathies disorder of the brain  Structural-metabolic-caused  Cryptogenic-presumed by a structural or metabolic symptomatic disorder of the brain- Tuberous sclerosis. structural or metabolic .

 Generalized Sz.initiate  Focal Sz-originate from activation of seizures within networks limited to limited to one part of one hemisphere.originates involves both at some point. cortical and subcortical  Focal Sz. may involve contralateral notion hemisphere  Separate manifestations from underlying cause . may be the hemisphere discretely localized or more  Discrete anatomical widely distributed.Classification as to Mode of Presentation Then Now  Generalized Sz. bilaterally hemisphere distributed.

Electroclinical Syndromes and Other Epilepsies Grouped by Specificity of Diagnosis As to Age of Onset Distinctive constellations/surgical syndromes Nonsyndromic epilepsies .

Electroclinical Syndromes As to Age of Onset  Neonatal period Benign familial neonatal seizures (BFNS) Early myoclonic encephalopathy (EME) Ohtahara syndrome  Infancy Migrating partial seizures of infancy West syndrome Myoclonic epilepsy in infancy (MEI) Benign infantile seizures Benign familial infantile seizures Dravet syndrome Myoclonic encephalopathy in nonprogressive disorders .

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Electroclinical Syndromes As to Age of Onset Childhood Febrile seizures plus (FS+) (can start in infancy) Early onset benign childhood occipital epilepsy (Panayiotopoulos type) Epilepsy with myoclonic atonic (previously astatic) seizures Benign epilepsy with centrotemporal spikes (BECTS) Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE) Late onset childhood occipital epilepsy (Gastaut type) Epilepsy with myoclonic absences Lennox-Gastaut syndrome Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) including: Landau-Kleffner syndrome (LKS) Childhood absence epilepsy (CAE) .

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Adult Juvenile absence epilepsy (JAE) Juvenile myoclonic epilepsy (JME) Epilepsy with generalized tonic-clonic seizures alone Progressive myoclonus epilepsies (PME) Autosomal dominant partial epilepsy with auditory features (ADPEAF) Other familial temporal lobe epilepsies .Electroclinical Syndromes As to Age of Onset Adolescence .

Electroclinical Syndromes and other epilepsies  Less Specific Age Relationship Familial focal epilepsy with variable foci (childhood to adult) Reflex epilepsies  Distinctive Constellations Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE with HS) Rasmussen syndrome Gelastic seizures with hypothalamic hamartoma .

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Sturge-Weber. hetertopias etc) Neurocutaneous syndromes (Tuberous sclerosis complex. Electroclinical Syndromes and other epilepsies(Nonsyndromic) Epilepsies attributed to and organized by structural- metabolic causes Malformations of Cortical development (hemimeganencephaly. etc) Tumor Infection Trauma Angioma Peri-natal insults Stroke Etc. .

Generalized seizures-involvement of both hemispheres 1.1 simple 2. Unclassified seizures .1 Absence 1.6 Atonic 2.5 Tonic –clonic 1. Partial seizures 2.4 Tonic 1.International Classification of Epilepsy 1981 1.3 Clonic 1.3 partial seizures evolving to secondarily generalized seizures 3.2 complex 2.2 Myoclonic 1.

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Febrile Seizures  Definition: seizures with fever in a child 3 months to 5 years of age without evidence of CNS infection  Types: simple and complex  Treatment intermittent continuous .

children with febrile seizures> 6 yrs with or without associated afebrile generalized tonic-clonic seizures . Commission. 1989)  Febrile Seizure Plus.< 6 yrs and associated with low overall risk of later epilepsy( Nelson and Ellenberg 1976.Febrile Seizures and Generalized Epilepsy  Febrile Convulsion(Seizure) Syndrome- confined to early childhood.

FS+ and atonic seizures and Myoclonic Astatic Epilepsy .FS+. GEFS+(Generalized Epilepsy with Febrile Seizures Plus)  Genetic syndrome-autosomal dominant gene  Epilepsy prototype  Phenotypic expression comprises a spectrum of clinical epilepsy prototype.febrile convulsion syndrome. FS+ and absences.FS+ and myoclonic seizures .

Lorazepam c. Proper positioning c. adequate airway b. Midazolam .Status Epilepticus  Continuous seizure for 30 minutes  Repeated seizures for 30 minutes without recovery of consciousness  Supportive measures- a. Diazepam b. Oxygenation  Specific medications a. Phenytoin e. Phenobarbital d.

Neurodiagnostic Evaluation in Patients with Seizures 1. Chromosomal analysis 7. Skull x-ray 6. Electrolyte determination *There are no routine examinations. Video EEG monitoring 5. There are only those that are indicated by the specific diagnostic problems presented by the patient . CSF analysis 4. Neuroimaging 3. Biochemical studies 8. EEG 2.

Electroencephalogram .

MRI 4. MRA and Angiography 5. Cranial Ct scan 3. Cranial ultrasound 2.Neuroimaging 1. SPECT .

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If the first AED does not control the seizure. When is an antiepileptic drug (AED) started? 2. What are the options if seizures are not controlled by AED? . What is the best AED for a patient? 3.Drug Therapy for Epilepsy  Objective: prevent seizure recurrence by prescribing appropriate anticonvulsant  Guideline questions 1. How long is the AED given? 5. what will be the next step? 4.

acute illness. EEG with definite pattern  Possibly. alcohol withdrawal.drug abuse. certain epilepsy syndromes .history of epilepsy in siblings. without structural lesion. with structural lesions.unprovoked seizure  Probably not.brain tumor.When is an AED started?  If a single seizure is followed rapidly by a second seizure  Definitely a.AVM b.

Carbamazepine Pregabalin 2004  1970.Ethosuximide Oxcarbazepine 2000 Levetiracetam 2000  1970.Valproic Acid Zonisamide 2005 Rufinamide 2007  1970.Trimethadione Felbamate 1993 Topiramate 1995  1950.Primidone Tiagabine 1998  1960. What is the best AED? Then Now  1857-K Bromide Clobazam 1982  1908.Phenobarbital Gabapentin 1993  1940.Clonazepam Stiripentol 2007 Lacosamide 2008 Eslicarbazepine 2009 Retigabine 2011 Perampanel 2012 .Phenytoin Vigabatrin 1989 Lamotrigine 1991  1912.

dyspepsia. dizziness. dysarthria. aplastic anemia.Table 1. hyperkinesia. skin rashes. partial and generalized seizures Sedation. I Phenytoin Partial seizures. agranulocytosis. behavioral disturbances Valproic acid Partial and generalized sz Tremor.thrombocytopenia . weight gain. Ethosuximide Absence seizures GI changes. Drugs Clinical uses Adverse effects Phenobarbital Partial and generalized Sedation. headache. distractability. lethargy. Clinical uses in common seizure types and syndromes and adverse effects of antiepileptic drugs. generalized Diplopia. hairloss. agranulocytosis Carbamazepine Partial seizures. generalized Ataxia. hirsutism. Benzodiazepines Status epilepticus. diplopia. agranulocytosis. drowsiness. mood changes. weight gain. nystagmus. lethargy.

visual field df Oxcarbazepine partial/gen sz dizziness.LGS dizziness.hyperactivity Pregabalin adjunct partial sz weight gain.sedation.Table 1. Clinical uses in common seizure types and syndromes and adverse effects of antiepileptic drugs.insomnia.refractory sz dizziness.weight loss Tiagabine adjunct partial/gen sz dizziness.ataxia Gabapentin adjunct partial sz drowsiness.ataxia. rashes Topiramate adjunct partial/gen sz cognitive problems.drowsiness Lamotrigine adjunct partial sz.edema. emotional changes Vigabatrin infantile spasms. Drugs Clinical uses Adverse effects Felbamate severe/refractory epilepsies LGS anorexia.ataxia.skin rash .diplopia.

diplopia.GIstress. blurred vision Rufinamide Lennox Gastaut Somnolence.skin rash. pyrexia Stripentol Dravet’s synd anorexia.ataxia. Clinical uses in common seizure types and syndromes and adverse effects of antiepileptic drugs.leucopenia Lacosamide Adjunct partial sz nausea. SJS Zonisamide partial/gen sz sedation.rash.Table 1. Drugs Clinical uses Adverse effects Oxcarbazepine partial/gen sz dizziness. vomiting.diplopia.fatigue.drowsiness. GI prob .agranulocytosis Levetiracetam adjunct for par/gen sedation.

Antiepileptic Drugs Then Now  Same efficacy  Same efficacy  Complex  simpler pharmacokinetics pharmacokinetics 1. Enzyme inducing properties properties  Better tolerability  Less tolerability  Less teratogenic  Teratogenic potential potential  Cheaper  More expensive . Bound to plasma to plasma protein protein 3. longer half life 2. Minimally or not bound 2. No enzyme inducing 3. Short half life 1.

what next?  Dosage should be increased slowly until seizures are controlled or maximum dose is attained or when side effects ensue  If first AED is not effective. Slowly taper first drug once adequate levels of 2nd drug is attained .If the first AED does not control the seizures. start a 2nd AED.

How long is the AED given?  Treatment may be stopped after 2 seizure free years  Factors related to successful withdrawal single type of seizure normal NE normal IQ normal EEG following treatment .

How is an epileptic patient monitored?  Compliance  Monitoring body weight  Monitoring toxicity  Serum levels of AED  Use individual drugs to its full potential  Treatment failure on initial drug .

Non-Pharmacologic Treatment:What are the options if seizures do no respond to AED?  Dietary  Vagus Nerve Stimulation  Epilepsy Surgery .