Mayo School of Professional Development Epilepsy and EEG in Clinical Practice November 10-12, 2016 Hard Rock Hotel at Universal Orlando Orlando, FL Course Directors Jeffrey Britton, MD and William Tatum, DO 2016 MFMER slide-1
Mayo School of Professional Development Common Pediatric Epilepsy November 10-12, 2016 Hard Rock Hotel at Universal Orlando Orlando, FL Raj D. Sheth, MD 2016 MFMER slide-2
Disclosures Relevant financial relationship(s) with industry None References to off-label usage(s) of pharmaceuticals or instruments None 2016 MFMER slide-3
Seizure either Focal Generalized FP1-F7 F7-T3 T3-T5 T5-O1 FP1-F3 F3-C3 C3-P3 P3-O1 FP2-F4 F4-C4 C4-P4 P4-O2 FP2-F8 F8-T4 T4-T6 T6-O2 50 µv 1 sec FP1-F7 F7-T3 T3-T5 T5-O1 FP1-F3 F3-C3 C3-P3 P3-O1 FP2-F4 F4-C4 C4-P4 P4-O2 FP2-F8 F8-T4 T4-T6 T6-O2 50 µv 1 sec 2016 MFMER slide-4
100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% <1m 1m-2y 2-10y 10-18y 18-60y >60y Generalized Seizures 2016 MFMER slide-5
Electroclinical Syndromes EEG Clinical Imaging Syndrome 3 Hz SW 4yo Nl CAE Hypsarrhythmia spasms +/- West Spikes O1 visual Tram-track Sturge-Weber 2016 MFMER slide-6
EEG helps in Epilepsy Syndrome Diagnosis Development Exam Imaging EEG background Idiopathic Normal Normal Normal Normal Symptomatic /cryptogenic MR Deficits + Lesion+ Slow + Epileptiform discharges Focal or Gen Focal or Gen 2016 MFMER slide-7
Epilepsy Syndrome = Seizure type + EEG Background + Epileptiform discharge + Neuro Exam + Imaging + Cognitive development Why bother? AED selection Prognosis Seizure freedom?out grow epilepsy genetics 2016 MFMER slide-8
Focal Epilepsy Syndromes Idiopathic BRE BPEOP Symptomatic Rasmussen s Temporal epilepsy 1m 1 y 2y 5y 10y 15y 18y Adult 2016 MFMER slide-9
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Centro-Temporal Spikes 2016 MFMER slide-11
17 yo with epigastric rising & Staring 2016 MFMER slide-12
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Seizure either Focal Generalized Idiopathic Symptomatic Idiopathic Symptomatic BRE TLE eg= Absence LGS 2016 MFMER slide-15
Generalized Epilepsy JME Idiopathic Absence Symptomatic West LGS 1m 1 y 2y 5y 10y 15y 18y Adult 2016 MFMER slide-16
Childhood Absence Epilepsy 2.5-5 Hz bisynchronous and symmetrical spike and wave Frontal central voltage predominance Precipitated by hyperventilation May be associated with OIRDA Discharges fragmented during sleep 2016 MFMER slide-17
GSW distribution before AED 2016 MFMER slide-18
GSW distribution after initiation of AED 2016 MFMER slide-19
GSW distribution with maintenance AED 2016 MFMER slide-20
Poly-Spike & wave - JME 3.5-6 Hz generalized spike and wave /polyspike and wave Prominent in the frontal central regions Precipitated by photic stimulation and hyperventilation Less prominent during sleep, but abundant upon awakening 2016 MFMER slide-21
EEG in Catastrophic Epilepsies of childhood Common: West - Infantile Spasms Lennox Gastaut Syndrome Sturge Weber Syndrome 2016 MFMER slide-22
Infantile Spasms West syndrome Triad: Infantile Spasms EEG Hypsarrhythmia MR Onset: 3-18 months Most <12 mo (clustering between 4 and 7 months) 2016 MFMER slide-23
Infantile Spasms Frequency: Clusters, brief, consisting of as many as 150 seizures At onset of sleep or after awakening. Crying or irritability during or after a flurry of spasms is commonly observed. Dramatic correlation between irritability/regression and onset of spasms and improvement following successful Rx! 2016 MFMER slide-24
IS: EEG patterns Hypsarrhythmia High-voltage, slow record dominated by delta activity with frequent and multifocal spikes, sharp waves, and spike and slow wave complexes Chaotic! 2016 MFMER slide-25
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Lennox-Gastaut Syndrome Slow spike & wave (2 Hz) Seizures Tonic Atonic Atypical Absence MR 2016 MFMER slide-27
Lennox-Gastaut Syndrome 2016 MFMER slide-28
3 year old Perinatal L MCA stroke Wyllie E et al. Neurology 2007;69:389-397 2013 American Academy of Neurology 2016 MFMER slide-29
Sturge-Weber 8 yo 2013 American Academy of Neurology Wyllie E et al. Neurology 2007;69:389-397 2016 MFMER slide-30
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Less common: Ohtahara Infantile epileptic encephalopathy with suppression burst neonatal myoclonic encephalopathy and early epileptic encephalopathy in the neonatal period Dravet s Severe myoclonic epilepsy of infancy Doose Astatic-myoclonic epilepsy Rasmussen encephalitis Continuous spike waves in slow sleep and Hemimegalencephaly Other migrational disorders Progressive myoclonus epilepsy 2016 MFMER slide-33
Ohtahara syndrome or Early Infantile Epileptic Encephalopathy Onset age:<3 months (often <10 days) Tonic seizures Can have partial seizures Rarely myoclonic seizures. Often unknown Metabolic disorders non-ketotic hyperglycinemia Hemispeheric underdevelopment EEG Burst suppression 2016 MFMER slide-34
EEG in PME 2010 P Satishchandra, S Sinha 2016 MFMER slide-35