Epilepsy in children Amira Masri Prof of child neurology

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Presentation transcript:

Epilepsy in children Amira Masri Prof of child neurology Faculty of medicine –The university of Jordan

Objectives History Epidemiology Definitions : seizure , epilepsy , febrile convulsion , status epilepticus ,epileptic encephalopathy Classification Aetiology Treatment Outcome

History of epilepsy Epilepsy : comes from the latin word epilepsia which means : to be ceased , to be attacked by surprise Hippocrate 400 B.C.: the sacred disease Ibn Sina +Al Tabari :1000 years ago =Al Saraa, disease of the brain

Epidemiology incidence rates of epilepsy in childhood :0.5 to 8 per 1,000 0.5 - 1%: experience at least one afebrile seizure by age adolescence. Of all children, 3 - 5% will have a single febrile seizure 30 %: will have additional febrile seizures 3 – 6% of those with febrile seizures will develop afebrile seizures or epilepsy 3.6% risk of experiencing at least one seizure in an 80-year lifespan focal seizures (with or without impairment of awareness) are the most common seizure type in all age groups and account for more than 50 percent of all seizures in children Focal seizures with alterations of awareness are the most common subtype Uptodate 2019

Basic mechanisms The basic mechanism of neuronal excitability is the action potential Action potentials occur due to depolarization of the neuronal membrane Membrane potential thus varies with 1-activation of ligand- gated channels :conductance is affected by binding to neurotransmitter 2- activation of voltage-gated channels: conductance is affected by changes in transmembrane potential; or with changes in intracellular ion compartmentalization.

various glutamate receptor subtypes and locations GABA receptors GABA site Barbiturate site Benzodiazepine site

Definitions Seizure Epilepsy Febrile convulsions Epileptic encephalopathy Status epilepticus

Seizure Disorder of cerebral function characterized by sudden brief attacks of: -loss of consciousness -motor activity -sensory phenomena - inappropriate behavior Caused by excessive discharge of cerebral neurons.

Epilepsy

At least two unprovoked (or reflex) seizures occurring > 24 h apart Epilepsy is a disease of the brain defined by any of the following: At least two unprovoked (or reflex) seizures occurring > 24 h apart 2.One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years 3.Diagnosis of an epilepsy syndrome Epilepsy is considered to be resolved for the following individuals: Those who had an age-dependent epilepsy syndrome but are now past the applicable age Those who have remained seizure-free for the last 10 years, with no antiseizure medicines for the last 5 years

Febrile convulsion A seizure in association with febrile illness in the absence of CNS or acute electrolyte imbalance in children >1 month of age without prior afebrile seizures Age :6m-6years (peak 18 m ) 2-5% of children Most common form of seizures in children Simple and complex

Epileptic encephalopathy A condition in which the epileptiform abnormalities themselves are believed to contribute to the progressive disturbance of cerebral dysfunction.

Developmental and/or Epileptic encephalopathies Epileptic activity itself contributes to severe cognitive and behavioral impairment above and beyond that expected from the underlying pathology and that these can worsen over time Berg et al 2010

Status epilepticus Emergency room definition : 5 minutes shortening the required seizure duration from 30 min to 5 min Why ?????? The longer SE persists: 1-the lower is the likelihood of spontaneous cessation 2-the harder it is to control 3-the higher is the risk of morbidity and mortality Seizures that do not cease in 5-10min are less likely to terminate without intervention

Classification of epilepsy 1- According to the clinical picture : generalized ,focal 2 - According to the aetiology: genetic , structural , metabolic infections, immune, unknown. 3-According to the epilepsy syndrome: age of onset , type of seizures and EEG findings

International league against epilepsy website

Purpose of the new classification 2017: for clinical diagnosis Transparent language: use words that mean what they say

Co-morbidities Seizure types Etiology Epilepsy types Unknown Immune Infectious Structural Etiology Metabolic Genetic Co-morbidities Seizure types Focal onset Generalized onset Unknown onset Epilepsy types Focal Generalized Combined & Focal Unknown Epilepsy Syndromes

Generalized seizures Originate at some Originate at some point within and rapidly engage bilaterally distributed networks Can include cortical and subcortical structures but not necessarily the entire cortex Here is a diagram that shows a conceptual network for generalized seizures involving the corticothalamic circuitry. Theoretically a generalized seizure could start at different points in the network and engage bilaterally distributed networks. Thus a seizure could start frontally or even parietally.The key point is that a generalized seizure can start from a focal point. 20

Focal seizures Originate within net Originate within networks limited to one hemisphere May be discretely localized or more widely distributed.… 21

Free text can be added to characterise sz Note When a seizure type begins with ”focal, generalized or absence” then the word “onset” can be presumed

Pedalling grouped in hyperkinetic rather than automatisms (arbitrary) Cognitive seizures impaired language other cognitive domains positive features eg déjà vu, hallucinations, perceptual distortions Emotional seizures: anxiety, fear, joy, etc

Aetiology Structural :example porencephalic cyst Genetic :example : absence epilepsy Metabolic : example :penylketonurea Infection :example : meningitis Immune :example :Rasmussen Unknown

Tuberous Sclerosis GLUT1 deficiency Seizure types Etiology Generalized onset Unknown onset Focal onset Structural Genetic Tuberous Sclerosis Infectious Metabolic Immune GLUT1 deficiency Unknown

Tuberous sclerosis : 2 genes ( TSC1 and TSC2 )

Terms no longer used Complex partial Simple partial Partial Psychic Dyscognitive Secondarily generalized tonic- clonic

Genetic versus idiopathic ‘Idiopathic’ = presumed hereditary predisposition Genetic ≠ inherited Importance of de novo mutations in both mild and severe epilepsies Critical problem of stigma in some parts of the world

Benign Self-limited Pharmacoresponsive Many epilepsies not benign Childhood hood absence epilepsy : psychosocial impact Benign epilepsy with centrotemporal spikes : learning concerns Replaced by terms: Self-limited Pharmacoresponsive No longer use Malignant Catastrophic

Changing Lexicon and Concepts for the Epilepsies New Terminology Old Terminology Person with epilepsy Epileptic Epilepsy seizures Epileptic seizures Antiseizure medications Antiepileptic drugs Focal onset Partial Focal dyscognitive Complex partial Epileptic spasms Infantile spasms Genetic Idiopathic Structural-metabolic Symptomatic Unknown Cryptogenic

Epilepsy syndromes

Epilepsy syndromes by age NEONATAL Benign familial neonatal epilepsy Early myoclonic encephalopathy Ohtahara syndrome INFANCY Epilepsy of infancy with migrating focal seizures West syndrome (infantile spasms, hypsarrhythmia; to be distinguished from benign myoclonus of early infancy, a nonepilepsy) Myoclonic epilepsy in infancy (benign Dravet variant) Benign infantile epilepsy Benign familial infantile epilepsy Severe myoclonic epilepsy of infancy (classic Dravet syndrome) Myoclonic encephalopathy in nonprogressive disorders Epilepsy syndromes by age

Epilepsy syndromes by age CHILDHOOD Genetic epilepsy with febrile seizures plus (GEFS +; can begin in infancy) Panayiotopoulos syndrome Epilepsy with myoclonic atonic (previously astatic) seizures (Doose syndrome) Benign epilepsy with centrotemporal spikes (BECTS, or benign rolandic epilepsy) Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE) Late-onset childhood occipital epilepsy (Gastaut syndrome) Epilepsy with myoclonic absences (Tassinari syndrome) Lennox–Gastaut syndrome Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) Landau–Kleffner syndrome (LKS) Childhood absence epilepsy (pyknolepsy) Generalized epilepsy with eyelid myoclonia (Jeavons syndrome)* ADOLESCENCE–ADULT Juvenile absence epilepsy (JAE) Juvenile myoclonic epilepsy (JME) Epilepsy with generalized tonic-clonic seizures alone Progressive myoclonus epilepsies (PME) Autosomal-dominant epilepsy with auditory features Other familial temporal lobe epilepsies LESS SPECIFIC AGE RELATIONSHIP Familial focal epilepsy with variable foci (childhood to adult) Reflex epilepsies (e.g., photosensitive, audiogenic, or reading-induced seizures; may or may not coexist with spontaneous seizures) Epilepsy syndromes by age

https://www.epilepsydiagnosis.org

How to confirm the diagnosis History + examination : most important = 30% of subjects diagnosed with epilepsy had been misdiagnosed and do not have epilepsy Rule out disorders that mimic seizures Role of neuroimaging : Brain MRI superior to CT scan EEG:standard=positivity rate 60% Sleep =positivity rate 90%

Misdiagnosis of epilepsy is common :Rule out epilepsy imitators False tendency : think of epilepsy as a single disorder Diagnosis : history , usually no confirmatory test: There is a large differential diagnosis Many clinicians charged with diagnosing paroxysmal disorders do not have sufficient knowledge of the clinical features of epileptic and non-epileptic seizure disorders Abuse of EEG =overreading Most clinicians with responsibility for diagnosing epilepsy do not have easy access to the full range of appropriate investigations. There is a false perception that to miss a diagnosis of epilepsy carries grave risks

Differential diagnosis: epilepsy imitators Huge list >36 disorders Breath holding spells GE reflux cardiogenic syncope Pseudoseizures Benign infantile myoclonus Migraine Night terrors Paroxysmal dyskinesisa Self gratification Somnambulism hyperekplexia

When should we start ttt First seizure : general rule :do not ttt (risk of recurrence 30% ) First prolong seizure (status ) : risk of recurrence same ( however higher risk of recurrence of prolonged seizures Multiple seizures within 24 hours : considered first seizure : recurrence risk same

Recurrence of unprovoked seizures First seizure : 40% will have recurrence Second seizure : 80% will have recurrence Recurrence usually within the first 6 m (first 2 years ) Recurrence : rare after 2 years

Evaluating a first non febrile seizure in children: Laboratory tests :If suggestive historic or clinical findings such as vomiting, diarrhea, dehydration, or failure to return to baseline alertness. (Option) Toxicology screening :if there is any question of drug exposure or substance abuse (option) LP :if possible meningitis or encephalitis (option) EEG : recommended for all :Establish Dx + recurrence risk If a neuroimaging study is obtained, MRI is the preferred modality

Non urgent neuroimaging Urgent neuroimaging in any age who exhibits a postictal focal deficit (Todd’s paresis) not quickly resolving or who has not returned to baseline within several hours after the seizure in any child with a significant cognitive or motor impairment of unknown etiology unexplained abnormalities on neurologic examination a seizure of partial (focal) onset with or without secondary generalization. an EEG that does not represent a benign partial epilepsy of childhood or primary generalized epilepsy children under 1 year of age.

Principles and goals of treatment 60-70% of children : seizure-free with no treatment or a low-to- moderate dose of monotherapy 30–40% : likely to continue to have seizures despite multiple AED Seizure control with one AED and without adverse events for some no treatment is appropriate equilibrium between maximum seizure control with minimum adverse effects, to ensure the best quality of life

Which drug to use type of seizure + epileptic syndrome Patient age Presence of other associated diseases (eg :patients with kidney stones avoid topiramate + zoisamide, patients with psychiatric disorders avoid leveteracitam ) Most likely to be effective Least likely to cause side effects Evidence based approach

Which is better the old or new In terms of efficacy : both same If patient fails to respond to one drug due to lack of efficacy : the success with 2nd or 3rd drug is only 11% Refractory epilepsy : 1/3 of patients

Refractory epilepsy : what to do ? Other non pharmacological modalities Ketogenic diet Epilepsy surgery Brain stem stimulation

Ketogenic diet Available since 1920 High fat (80%), low carbohydrate , adequate protein (20%)=results in ketosis Supplemented with vitamins + calcium Needs admission for few days to hospital : to induce ketosis Common SE: non compliance , acidosis, constipation, failure to gain weight, increase cholesterol Successful control of seizures :equal or surpasses that of AED Children who become seizure free will continue to be seizure free even when diet is discontinued

Epilepsy surgery Lesionectomy Lobectomy Corpus callosotomy Hemispherectomy Multilobar resection Multiple subpial transection

Vagal nerve stimulation First implantation in humans :1988 FDA approved : 1997 Tech. : pacemaker behind left carotid ( electrode for regulation of stimulation)=transmit intermittent electrical stimulation to an electrode wrapped around the left vagus nerve

Mech.: stimulation with high frequency : desynchronisation of EEG ( anticonvulsant effect ), increase GABA , decrease excitatory a.a Indication : pharmacoresistant epilepsy that is not candidate for surgery Rarely causes seizures to remit completely Decrease seizure frequency : 25-30% Adjunctive ttt to AED

Deep brain stem stimulation

Prognosis of epilepsy in children When to stop treatment 50% will outgrow their epilepsy and stop treatment Syndromic approach to discontinuation of ttt. Depends on epileptic syndrome In general : 2 years seizure free

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