ILAE 2017 Classification of Seizure Types Expanded Version Peto foment tors srr proves peers ees ord eon epileptic spasms firme? tonie Crt ern) Shen pes: eee pen oer Reser Fome ie ee pm SC) coo Cd Ne oes Pe er eases femerand fag See ery Pony pa De een ee eee i Eereeses orsiecers rec: teat Spe) Soo a Se ae eee ere eco en]Electroclinical syndromes arranged by age at onset Neonatal period Benign familial neonatal epilepsy (BFNE) Early myoclonic encephalopathy (EME) Ohtahara syndrome Infancy Epilepsy of infancy with migrating focal seizures West syndrome Myoclonic epilepsy in infancy (MEI) Benign infantile epilepsy Benign familial infantile epilepsy Dravet syndrome Myoclonic encephalopathy in non-progressive disorders io.Benign infantile epilepsy and benign familial infantile epilepsy Onset 3-20 months. Clusters of brief partial seizures. Normal development before onset. Responsive to antiepileptic therapies. Favorable outcome. Familial form based on a family history of infantile convulsions without later development of other forms of epilepsy Inherited as autosomal dominant. Peak age of seizure onset in familial form is 4-7 monthsEpilepsy of infancy with migrating focal seizures Malignant migrating partial epilepsy in infancy Onset in first 6 months. Clusters of severe, polymorphous focal seizures, frequently evolving into generalized. Progressive decline in psychomotor development Within weeks to months, patients enter a “stormy phase” with frequent polymorphous focal seizures that become virtually continuous. EEG shows multifocal discharges, typically rhythmic theta activity, that progressively expand to adjacent cortical areas Ictal and interictal EEG sbecome indistinguishable Prognosis is poor> Seizures are markedly drug resistant and outcome is generally severe. > Some patients may respond favourably to > Stiripentol associated with clonazepam, > Levetiracetam. > Vagus nerve stimulation and a ketogenic diet have been tried also but with poor or inconclusive results. Dr. Arijit Chattopadhyay's screenElectroclinical syndromes arranged by age at onset Childhood Febrile seizures plus (FS+) (can start in infancy) Panayiotopoulos syndrome 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)* Landau-Kleffner syndrome (LKS) Childhood absence epilepsy (CAE)Electroclinical syndromes arranged by age at onset 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 (ADEAF) Other familial temporal lobe epilepsies Less specific age relationship Familial focal epilepsy with variable foci (childhood to adult) Reflex epilepsies* Early motor involvement of the contralateral hand. * Late age of onset * No early risk factors * No hippocampal atrophy on MRI * Minimal automatisms * Auditory aura or seeing faces Dr. Arijit Chattopadhyay's screen* Stereotyped pattern * Frequent seizures, often in clusters ¢ Brief seizures, under 1 minute * Bizarre attacks that appear hysterical * Prominent motor automatisms, usually complex * Aggressive sexual automatisms * Vocalizations, with variable complexity * Short postictal period, rapid clearing * Complex partial status epilepticus common Wiliarnson J. ef al. Annals of Neurolog}Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) Childhood/adolescent onset (mean 11 years). Autosomal dominant inheritance Channelopathy. Focal sensory-motor seizures occurring in NREM sleep. Variable manifestations including prominent motor features such as jerking, dystonia, and automatisms, as well as vocalizations, and non- specific auras Ictal EEGwith frontally dominant slow discharges. Prognosis is typically favorable.foc} > eter ert Pee eae e tn Pee ant io Dae Metenelesios) BSCS iter eetereer eget eed oboe> If typical history, normal neuro exam, and characteristic EEG findings, an MRI is not necessary > No treatment is necessary in patients with infrequent, nocturnal, partial seizures > If seizures are frequent (20%)and disturbing to patient and family, treatment with CBZ or OXC is usually successful ia |» Early-onset benign childhood epilepsy with occipital paroxysms » Average age of 3-6 years » Normal development » Characterized by nocturnal seizures in two-thirds, with tonic eye deviation, vomiting (autonomic) and impaired consciousness » Hemiconvulsions and GIC seizures are commonInterictal EEG shows normal background with high- amplitude occipital spike-wave complexes on eye closure Seizures are infrequent and one third of patients will only have a single seizure Paradoxically, seizures are frequently prolonged (status epilepticus) Excellent prognosis -seizures usually cease within 2 years onset Treatment with medication may be necessaryeI Mae ened oar) > Seizures respond well to carbamazepine/ OXC Mea Raa Alot)Landau-Kleffner syndrome (LKS) Onset between 3 and 8 years (peak 5-7 years). Acquired aphasia (verbal auditory agnosia). Continuous spike and wave discharges on EEG, activated in sleep. Resolution of EEGabnormalities in adolescence. Deterioration or significant fluctuation in language are indications to evaluate for LKS. Generalized or focal seizures occur in up to 80% of children and may precede or follow the onset of aphasia Seizures commonly resolve before age 15 years Neuropsychological deficits tend to persist. many epileptologists consider CSWSand LKS on a common syndromic spectrum and consider LKSa specific presentation of epilepsy withRasmussen syndrome Onset 1-14 yrs of age Focal onset motor seizures: simple partial or evolve into complex partial or secondary generalisation Seizures start in same hemisphere Progressive hemiatrophy with lesser atrophy on other side More pronounced in perisylvian region Treatment- IVIg, corticosteroids Hemispherectomy for resistant cases.>» Recommended drugs are Oxcarbazepine, Carbamazepine, Phenobarbitone, Phenytoin, Topiramate, Valproate, Vigabatrin, Lele Nercole Me ole rotsTe a aka i lao seats acon ILAE recommendations for childhood focal onset seizures a Pee oa > Level C: CBZ, PB, PHT, TPM, VPA, VGB >» Level D: CLB, CZP, LTG, ZNS Penton Peau} EPILEPSY Established 1909> Anti-epileptic drugs to offer based on epilepsy syndrome — » Offer carbamazepine or lamotrigine as first-line treatment to children and young people with BECTS, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) > If carbamazepine or lamotrigine are unsuitable or not tolerated for benign epilepsy — > Offer levetiracetam or oxcarbazepine to women and girls of childbearing potential> Be aware that carbamazepine and oxcarbazepine may exacerbate or unmask continuous spike and wave during slow sleep, which may occur in some children with benign epilepsy with centrotemporal spikes Offer levetiracetam, oxcarbazepine or sodium valproate to boys, men and women who are not of childbearing potential. If the first AED tried is ineffective, offer an alternative from these AEDs.® Seizure free for 2-3 years ® Single seizure type ® Normal EEG on AED's ® Normal IQ ® Normal neurologic examination