Introduction To Epilepsy M. Scott Perry, M.D. Emory University April 18, 2007
Introduction To Epilepsy Semiology diagnosis Treatment QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 Objectives • Recognize different types of seizures. • Discuss workup for new onset seizures • Learn classification of epilepsy types based on history, seizure type, MRI, and EEG findings • Review common treatments used in epilepsy • Learn prognosis based on epilepsy type • Briefly review some frequently asked questions Spells Seizure Equivalents GERD Breath Holding Infantile Masturbation Syncope Benign Sleep Myoclonus Recurrent (Epilepsy) Seizure Symptomatic Electrolytes Trauma Ingestion Case 1 QuickTime™ and a Sorenson Video 3 decompressor are needed to see this picture. QuickTime™ and a Sorenson Video 3 decompressor are needed to see this picture. Seizure Imitators • Benign Neonatal Sleep Myoclonus • Myoclonic jerks are focal, multifocal, unilateral or bilateral • 1-5 hz, distal>proximal • Begins in first weeks, diminishes by 2nd month, generally gone by 6 months • Episodes may be exacerbated by benzos Seizure Imitators Breath Holding Spells • Incidence: 4.6% (population study, N=4980) • Onset: 6-18 months • 90% resolve by age 6y • cyanotic and pallid CYANOTIC BREATHHOLDING SPELLS • 60 % are cyanotic • stimulus triggered (anger, frustration) • short cry • breathing interrupted in expiration • cyanotic, limp, LOC • +/- sleep COMPLICATED BREATH-HOLDING SPELLS • Breath-holding spells + seizure-like activity • usually more prolonged • 15% have complicated features • clonic activity follows LOC • stiffening Seizures: What information is useful? • What was the patient doing when it started? Unresponsive?...are you sure? Asleep or awake? • Tell us exactly what you saw: • E.R.B.S.A.O? • Does it make anatomical sense? Same side, both sides, just arms, etc. • How long did it last? Clinical Characteristics of Seizures in Neonates (Scher, et al 1989) •No accepted classification for neonatal seizures •80 neonates with suspicious movements. Only 8 had electrographic seizures. •Focal/multifocal clonic: 44% epileptic •“Subtle seizures”-roving eye movements, arrest of behavior, lip smacking, autonomic-30% •Tonic(focal or generalized) 8% •Myoclonic 7% Clinical Characteristics of Seizures in Neonates (Scher, et al. 1993) •92 neonates with electrographic seizures (345 EEG recordings) •48% had electroclinical event •Subtle 71%, clonic 41%, myoclonic 20%, tonic 9% •34% with only electrographic events •17/90 (19%) of paralyzed neonates had electrographic events Clinical Seizures in Neonates •Gen. Tonic Clonic seizures don’t happen in neonates. •69 infants, 101 seizures, only 4 resembled GTCS, none truly were. (Nordli, et al.) •Duration- average duration 2.25 minutes. Usually shorter, rarely longer. Intertictal recovery 8 minutes (Clancy and Legido, 1987) •Status Epilepticus- clinical SE is rare, electrographic may not be •487 seizures, only 2 SE (Clancy, et al) •33% FT infants, 9% PT (Scher, et al) Seizure Semiology of Neonates Focal/Multifocal Tremors Subtle Tonic Clonic QuickTime™ and a QuickTime™ Sorenson Video 3 decompressor Sorenson picture. are needed needed to see this picture. are Seizure Types Partial Simple Partial Complex Partial Partial Secondarily Generalized Generalized Simple partial QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. •Preserved consciousness •Isolated motor/sensory •Complex partial involves loss of consciousness Partial Secondarily Generalized QuickTime™ and a YUV420 codec decompressor are needed to see this picture. •Starts partial, rapidly spreads •You have to ask the questions to get the answers Partial Seizure Clues • Contralateral • Head Deviation, Eye Deviation, Dystonic Posturing, Unilateral Clonic Activity, Postictal Paralysis • Ipsilateral • Automatisms, Eye Blinking, Nose Wiping Head and Eye Deviation Differentiating Seizure Types - Semiology Partial Seizures QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. What do you see? 1. Head Deviation 2. Automatism 3. Eye Deviation 4. Unilateral Dystonic/Clonic Activity QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. Seizure Types Partial Generalized Simple Partial Complex Partial Partial Secondarily Generalized Generalized Tonic Clonic Tonic Clonic Atonic Myoclonic Absence Generalized Seizure Myoclonic • Characterized by quick, arrhythmic, and symmetric/asymmetric movements •Often not reported by patients. •Ask about sudden falls or dropping objects QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. See the Difference? QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. Myoclonic-fast, jerking motion QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. Clonic-rhythmic Semiology Typical Absence Seizures •Characterized by brief, abrupt impairment of consciousness associated with EEG demonstrating 3 Hz spike and slow wave complexes with normal interictal background •May also demonstrate: •mild clonic, tonic, or atonic components Panayiotopoulos “The Epilepsies: •automatisms Seizures, Syndromes, and Manangement •autonomic components Generalized Seizure Semiology Infantile Spasms QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. Review So Far • Common seizure imitators in pediatrics • Seizures come in two basic types. You have to ask the right questions to distinguish them • Now...how do you diagnose epilepsy (i.e. when is EEG/MRI necessary) and why do we care? Epilepsy Types Partial Generalized Cryptogenic Idiopathic Benign Rolandic Epilepsy Benign Occipital Epilepsy Cryptogenic Symptomatic Idiopathic Symptomatic Childhood Absence Juvenile Absence Juvenile Myoclonic Grand Mal Upon Awakening West Syndrome Lennox-Gastaut •Primary = Idiopathic = presumed genetic •Secondary = Symptomatic=underlying cerebral cause (i.e. injury, dyplasia, etc.) Idiopathic Partial Epilepsy Benign Rolandic Epilepsy (Benign Childhood Epilepsy with Centro-Temporal Spikes) •Onset 1-14 years, 75% between 7-10 years of age •Prevalence is 15% of children with seizures •Characterized by infrequent, often single, focal seizures consisting of unilateral facial sensorimotor symptoms, oropharyngolaryngeal manifestations, speech arrest, or hypersalivation lasting 1-2 minutes •1/3 -2/3 will have secondarily generalized seizures •75% are nocturnal •MRI normal •Typical EEG Benign Rolandic Epilepsy Prognosis/Treatment •2-3% school age children have CT spikes with <10% having BRE •Remission usually within 2-4 years from onset and before the age of 16 years •Less than 2% will develop infrequent generalized seizures in adulthood •Treat or not to treat Benign Occipital Epilepsy Gastaut Type •Onset 3-15 years •Manifest as visual hallucinations, blindness, or both-lasting seconds to <3 minutes •Rarely terminate with hemiconvulsions or generalized convulsion •50% have postictal headache •Similar manifestation to seizures from occipital lesions - MRI needed •Typical EEG Benign Occipital Epilepsy Fixation-Off EEG Benign Occipital Epilepsy Prognosis/Treatment •Remission occurs 2-4 years from onset for 50-60% of patients •Dramatic response to carbamazepine in >90% •15% association with celiac disease Symptomatic Partial Epilepsy • Abnormal MRI (stroke, dyplasia, etc.) or abnormal EEG without classic pattern • History not consistent with primary partial epilepsy • Prognosis varies Secondary Partial Epilepsy MRI Heterotopia Mesial Temporal Sclerosis Idiopathic Generalized Epilepsy Childhood Absence Epilepsy •Onset 2-10 years, peak 5-6 years •2/3 are females •Abrupt cessation of activity or speech last 4-20 seconds followed by return to baseline •Normal MRI •Typical EEG with 3Hz SW often provoked with HV Idiopathic Generalized Epilepsy Childhood Absence Epilepsy Idiopathic Generalized Epilepsy Childhood Absence Epilepsy Prognosis/Treatment •Remission often occurs before 12 years of age •Less than 10% develop infrequent generalized tonic clonic seizures in adolescence or adult life •Rarely will patients continue to have absence seizures as adults •Treatment with valproic acid, ethosuximide, or lamotrigine will control absences in >80% •Possible role for topiramate and levetiracetam Idiopathic Generalized Epilepsy Juvenile Absence Epilepsy •Age of onset 9-13 years •80% suffer from GTCS and 15-25% have Myoclonic seizures with onset 1-10 years after absences •Frequent/severe absences •Absence status in 20% •Prognosis: 70-80% will be controlled, though this is a lifelong disorder •20% may have intractable absences and GTCS Idiopathic Generalized Epilepsy Juvenile Myoclonic Epilepsy •Characterized by myoclonic jerks upon awakening starting in adolescence •GTCS (>90%) may begin a few months later, occasionally earlier •Absence seizures (33%), if present, begin between 5-16 years •M:F equal •Seizure precipitants: Sleep deprivation, alcohol, stress, video games •EEG: Idiopathic Generalized Epilepsy Juvenile Myoclonic Epilepsy irregular generalized 3-6hz spike/polyspike-slow wave discharges and generalized fragments. 33% have photoparoxysmal responses Epilepsy Juvenile Myoclonic Epilepsy Treatment/Prognosis •Valproic Acid, levetiracetam most commonly used monotherapy treatment •Lamotrigine, clonazepam •Prognosis: Seizures well controlled in up to 90% of patients. Treatment is lifelong, as 80% relapse after drug withdrawal •Carbamazepine, oxcarbazepine, phenytoin, gabapentin, tiagabine, and vigabatrin are contraindicated •Lifestyle management with regards to alcohol use, sleep deprivation, etc. Symptomatic Generalized Epilepsy Infantile Spasms West Syndrome “...these bobbings...they come on whether sitting or lying; just before they come on he is all alive and in motion...and then all of a sudden down goes his head and upwards his knees; he then appears frightened and screams out. --W.J. West (1841) Symptomatic Generalized Epilepsy Infantile Spasms West Syndrome •Onset between 3-12 months, peak at 5 months •Incidence: 3-5/10,000 •Spasms are flexor, extensor, or combined •Clusters with 20-150 seizures per day, occurring most often on awakening or prior to sleep •Developmental delay preceeds spasms in 2/3 •Classified as symptomatic, probably symptomatic, and cryptogenic Symptomatic Generalized Epilepsy Infantile Spasms West Syndrome •80% symptomatic with most caused by pre-, peri-, or post-natal insults (i.e. HIE, ICH, dysplasias, trauma) •50% of patients with TS have spasms •3% of patients with Trisomy 21 •Aicardi’s syndrome (spasms, agenesis of the corpus callosum, and retinal lacunes •EEG demonstrates hypsarrhythmia •High voltage, chaotic, arrhythmic and asynchronous which becomes more synchronous in NREM sleep •Multifocal independent spike wave discharges •Periods of electrodecrement Symptomatic Generalized Epilepsy Infantile Spasms West Syndrome Symptomatic Generalized Epilepsy Infantile Spasms Prognosis •Spasms typically will remit, even without treatment, by 18 months of age •60% of patients develop other seizure types, CPS and Lennox-Gastaut syndrome are most common •90% of patients have developmental delay, 66% are severely cognitively impaired Symptomatic Generalized Epilepsy Infantile Spasms •ACTH - 50% remission, all or none. No Treatment proven dosing regimen, no clear reason why it works •Topiramate - similar efficacy usually in high doses (25-30mg/kg/d) •Vigabatrin - especially useful in TS (90%), beware of irreversible visual field defects •Pyridoxine, valproate, zonisamide, levetiracetam, lamotrigine, felbatol, keto diet •Surgery Symptomatic Generalized Epilepsy Lennox-Gastaut Syndrome •Three criteria •Multiple intractable seizures including tonic (80-100%), atypical absence (66%), and atonic (50%) •cognitive and behavioral abnormalities •Slow (<2.5 Hz) generalized spike wave •Onset 1-7 years, peak 3-5 •10-30% develop from West syndrome or other epileptic encephalopathies Symptomatic Generalized Epilepsy Lennox-Gastaut Syndrome Symptomatic Generalized Epilepsy Lennox-Gastaut Syndrome Prognosis/Treatment •5% die, 80-90% have seizures as adults, and approximately 90% have severely impaired cognition and behavior •Treatment includes almost every AED with polypharmacy common. •Ketogenic diet, VNS, corpus callosotomy Choosing an AED • Type of epilepsy Treatment of Epilepsy: AEDs Partial Phenytoin Phenobarbital Valproic Acid Carbamazepine Oxcarbazepine Gabatril Gabapentin Topiramate Lamotrigine Zonisamide Levetiracetam Generalized Valproic Acid Topiramate Zonisamide Lamotrigine Levetiracetam Ethosuximide Felbamate Choosing an AED • Type of epilepsy • Type of formulation (IV, capsule, sprinkle, etc.) Choosing an AED Formulation • IV: Benzos, phenytoin, phenobarbital, valproic acid, levetiracetam • Sprinkles: valproate, topiramate • Liquids: carbazepine, oxcarb, levetiracetam, valproate, dilantin. zonegran,lamictal,topiramate will dissolve in H20 • Extended release: valproate, carbamazepine Choosing an AED • Type of epilepsy • Type of formulation (IV, capsule, sprinkle, etc.) • Time to onset Choosing an AED Time To Onset • Rapid onset: Any IV form • Onset in 24 hours: Levetiracetam • Onset in Days: carbamazepine, oxcarb, dilantin, valproate, zarontin. • Slow titration: Topiramate, zonisamide • Really slow: Lamictal Choosing an AED • Type of epilepsy • Type of formulation (IV, capsule, sprinkle, etc.) • Time to onset • Side Effects Choosing An AED Side Effects Somnolence •All Rash Renal Stones •Topiramate •Zonisamide Hyponatremia •Carbamazepine •Oxcarbazepine Cognitive •Phenobarb •Topiramate Parasthesia •Topiramate •Zonisamide Behavior •Levetiracetam Labs draws •Carbamazepine •Valproic Acid •Phenytoin Levels •All •Lamictal •Phenytoin •Phenobarb Choosing an AED • Type of epilepsy • Type of formulation (IV, capsule, sprinkle, etc.) • Time to onset • Side Effects • Dosing Schedule Choosing An AED Dosing Schedule • QD: Depakote ER, Zonisamide • TID: Depakene, Neurontin, Tegretol, Phenytoin (neonates) • BID: Everything else Febrile Seizure • 3 types (simple, complex, status) • NIH consensus: Febrile seizure is an event in infancy or childhood, usually 3m-5 years, associated with fever but without evidence of intracranial infection or defined cause. Seizures with fever in children who have suffered a previous nonfebrile seizure are excluded. • incidence- 4%: absolute risk increased with family hx (1 relative 10%, 2-32%), daycare (7%), dev delay (10%) • Risk of recurrence: 1 in 24 • risk of future epilepsy: 2-10% • workup - MRI/EEG does not predict recurrence • treatment Practice Parameter Febrile Seizures • Current Recommendations AAP [Pediatrics 97(5), May 1996, 769-71.] • Age 6-12 months with febrile seizure should strongly consider LP • Age 12-18 months should consider • >18 months may use physical exam, associated symptoms to drive need • Based recommendations on 4 studies reporting 13-15% of children will present with seizures as the initial manifestation of seizures with 30-35% having no meningeal signs. • More recent reviews have suggested the presence of meningitis in the absence of associated signs is rare (1/200), with a large percentage of such patients with normal CSF at presentation. The introduction of the H.Flu vaccine has significantly altered the epidemiology of infantile bacterial meningitis making present treatment different from that 30 years ago (which the AAP based their recommendations). • Most physicians would agree that LP in children outside the range of febrile convulsions is necessary, as well as children within the range with sign or symptoms of CNS infection, such as nuchal rigidity, altered mental status, Practice Parameters First Unprovoked Seizure • Laboratory investigations (CBC, CMP, tox screens) should be considered based on historic and clinical findings • LP is of limited value in first unprovoked afebrile seizure • EEG is recommended to dx epilepsy syndromes and provide for prognosis • MRI is preferred modality and should be considered in children with cognitive/motor impairment that is unexplained, focal onset seizures, or in children < 1y. • Emergenat imaging should be performed in children with prolonged todd’s, or prolonged (several hours) postictal state. • Treatment: 46% have recurrence in 10years, 19% > 4 seizures, and 10%>10 seizures FAQ (the ED) • I have a 14 month old with a febrile seizure and a “raging otitis,” do I need to do a LP? • (3a.m.) Hey, how are you? I have a kid here with known epilepsy that had a breakthrough seizure (like he does once every 3 months or so), do you want to increase his medicine? • Do I need to CT this kid? • I have a patient of Dr. Flamini’s here, how do you want to treat him? FAQ (the parents) • Why does my child have seizures? • Will my child be stupid? • How long does my child need treatment? • What do I do when my child has a seizure? Case • 16 year old female with first unprovoked seizure, described as generalized tonic clonic •Focal signs at onset? •Myoclonic or absence type episodes? •Time of day? •Previous workup? •Treatment choices: Depakote, Keppra, Trileptal •How long will she need medicine? Case • 8 y/o with frequent episodes of staring, at times associated with lip smacking •Focal signs at onset? Can they be stopped? •Myoclonic or GTC episodes? •Duration? •Time of day? •Previous workup? •Treatment choices: Depakote, Keppra, Trileptal
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