#CHAIR2016. September 15 17, 2016 The Biltmore Hotel Miami, FL. Sponsored by

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1 #CHAIR2016 September 15 17, 2016 The Biltmore Hotel Miami, FL Sponsored by

2 #CHAIR2016 Seizures and Epilepsies Enrique Serrano, MD University of Miami Miller School of Medicine Miami, FL

3 #CHAIR2016 Learning Objective Identify the seizure type and epilepsy syndrome, and establish a possible etiology when applicable

4 Enrique Serrano, MD Disclosures Dr. Serrano has no disclosures to report.

5 Definitions The definition of epilepsy has been traditionally defined as two unprovoked seizures A patient with brain insults (stroke, CNS infection, trauma) has a risk of a second unprovoked seizure that is comparable to the risk for further seizures after two unprovoked seizures Fisher R, et al. Epilepsia 2014;55(4):

6 Definitions Clinical (practical) definition of epilepsy At least two unprovoked seizures occurring >24 hours apart One unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next ten years Diagnosis of an epileptic syndrome Fisher R, et al. Epilepsia 2014;55(4):

7 Definitions Provoked seizure: caused by temporary and/or reversible factors. Synonymous with acute symptomatic seizure. Examples A seizure after a concussion Seizures associated with fevers Alcohol-withdrawal seizures Metabolic disturbances (hypoglycemia, hyponatremia) Unprovoked seizure: caused by irreversible factors. Etiology should not be confused with provoked Some etiologies will produce an enduring tendency to have seizures (irreversible) making them unprovoked. (eg: brain tumor, stroke)

8 Why Classifying? Organization to the knowledge Improve communication among researches/clinicians Improve patient care Facilitate research Teaching Identify unique diagnostic entities with etiologic, therapeutic, and prognostic implications, so that when a syndromic diagnosis could not be made, then the therapy and prognosis would be based on seizure type Grouping of reasonably pure cultures of patients for discovery of etiologies, including genetic factors, research into fundamental mechanisms, and even clinical trial

9 Seizure Classification Generalized seizures: Originate within (or rapidly engaging) bilaterally distributed networks. Involvement of both hemispheres. Not necessarily including the entire cortex. Focal seizures: Originate within networks limited to one hemisphere. May be discretely localized or more widely distributed. Unknown onset

10 Seizure Classification (2010) Focal Seizure Types: Without impairment of consciousness or awareness (previous classification: simple) With impairment of consciousness or awareness, also called dyscognitive (previous classification: complex) Focal evolving to a bilateral convulsive seizure (previous classification: secondarily generalized)

11 Seizure Classification (2010) Generalized Seizure Types (based on clinical manifestations): Tonic-Clonic Tonic Clonic Atonic Myoclonic Myoclonic-atonic Absence

12 ILAE Seizure Classification 2016 Basic Scheme Focal Generalized Unknown Onset Motor Non-Motor Motor Absence Nonmotor Absence Aware Impaired Awareness Unknown Awareness Aware Impaired Awareness Unknown Awareness To Bilateral Tonic-Clonic Unclassified Fisher RS, et al.

13 ILAE Seizure Classification 2016 Expanded Scheme Focal Generalized Motor Atonic Myoclonic Clonic Epileptic spasms Hypermotor Non-Motor Sensory Cognitive Emotional Autonomic Aware Impaired Awareness Unknown Awareness To Bilateral Tonic-Clonic Motor Tonic-clonic Tonic Atonic Myoclonic Myoclonic-atonic Clonic Clonic-tonic-clonic Epileptic spasms Absence Typical Atypical Myoclonic Eyelid myoclonia Unknown Onset Motor Tonic-clonic Tonic Atonic Epileptic spasms Non-motor Aware Impaired Awareness Fisher RS, et al. Unknown Awareness Unclassified

14 Descriptors of Behaviors During Focal Seizures Cognitive Acalculia, aphasia, attention impairment, déjà vu, dysphasia, hallucinations, illusions, jamais vu, memory impairment, neglect, forced thinking, responsiveness impairment Emotional or affective Agitation, anger, anxiety, crying (dacrystic), fear, laughing (gelastic), paranoia, pleasure Autonomic Asystole, bradycardia, cold, erection, flushing, gastrointestinal, heat, hyperventilation, hypoventilation, nausea or vomiting, pallor, palpitations, piloerection Automatisms Aggression, manual, oral facial, perseveration, sexual, vocalization, walking/running

15 Descriptors of Behaviors During Focal Seizures Sensory Auditory, gustatory, olfactory, somatosensory, vestibular, visual Motor Arrest, dysarthria, dystonic, fencer s posture, figure-4, hypomotor, hypermotor, incoordination, Jacksonian, paralysis, paresis, pedaling, pelvic thrusting, versive Fisher RS, et al. Operational Classification of Seizure Types by the International League Against Epilepsy.

16 Seizure Classification Glossary Clonic: regularly repetitive contraction which involves the same muscle groups, at a frequency of 2-3 cycles/s and is prolonged Tonic: a sustained increase in muscle contraction lasting a few seconds to minutes Tonic-clonic: combination of above. Atonic: sudden loss or diminution of muscle tone Myoclonic: sudden, brief, involuntary, single or multiple, irregular contraction of muscles or group of muscles of variable topography, eg: sudden jerks Myoclonic-atonic: combination of above Absence: brief sudden loss of consciousness Epileptic spasm: a sudden flexion, extension, or mixed extension flexion of predominantly proximal and truncal muscles that is usually more sustained than a myoclonic movement but not as sustained as a tonic seizure

17 Epilepsy Classification Two primary axes: Etiology Genetic (previous classification: idiopathic) Structural/metabolic (previous classification: symptomatic) Unknown (previous classification: cryptogenic) Mode of presentation Generalized Focal

18 Epilepsy Classification Genetic (previous classification: idiopathic) Direct result of a known or presumed genetic defect Structural/metabolic (previous classification: symptomatic) Distinct structural or metabolic condition or disease demonstrated to be associated with a substantial increase risk of developing epilepsy Unknown (previous classification: cryptogenic) Many of the former cryptogenic epilepsies have been shown to have genetic basis

19 Mode of Presentation Generalized: epileptic disorders with generalized seizures Focal: epileptic disorders in which seizure semiology and findings disclose a focal origin

20 Age of Onset Neonatal Benign familial neonatal epilepsy Early myoclonic encephalopathy Ohtahara syndrome Hyperekplexia Infancy Epilepsy of infancy with migrating focal seizures West Syndrome Myoclonic epilepsy of infancy Benign infantile epilepsy Dravet Syndrome Childhood Epilepsy Epilepsy in females with mental retardation Febrile seizures plus Panayiotopoulos Syndrome Epilepsy with myoclonic-atonic seizures Benign epilepsy with centro-temporal spikes (Rolandic) Lennox-Gastaut Syndrome Landau-Kleffner Syndrome Childhood Absence Epilepsy Adolescent Epilepsy Juvenile absent epilepsy Juvenile myoclonic epilepsy Progressive myoclonic epilepsies

21 Generalized Epilepsies Idiopathic: Childhood absence epilepsy Juvenile absence epilepsy Juvenile myoclonic epilepsy Epilepsy with GM seizures on awakening Cryptogenic West syndrome Lennox-Gastaut syndrome

22 Focal Epilepsies (Etiology Axis) Idiopathic Benign childhood epilepsy with centrotemporal spikes (Rolandic) Childhood epilepsy with occipital paroxysms Primary reading epilepsy Symptomatic Chronic progressive epilepsy partialis continua of childhood (Kojewnikow s syndrome)

23 Epilepsy Classification (Anatomical Axis) Focal epilepsies Temporal lobe epilepsies Frontal lobe epilepsies Parietal lobe epilepsies Occipital lobe epilepsies

24 Temporal Lobe Epilepsies Autonomic or psychic phenomena à motor arrest à oroalimentary automatisms à other automatisms à postictal confusion amnesia Duration >1 min History of febrile seizures and family history common EEG shows spike discharges over the temporal lobes Mesiotemporal Marked autonomic signs: epigastric rising sensation,nausea, pallor, flushing, fear, panic, olfactory/gustatory hallucinations Latero-temporal Auditory/illusions/dreamy states, visual misperceptions

25 Frontal Lobe Epilepsies Seizures frequently occur during sleep Generally short seizures with minimal post-ictal confusion and rapid secondary generalization Status epilepticus common Complex gestural automatisms Prominent motor manifestations (often mistaken for psychogenic spells)(hypermotor) Todd s paralysis (post-ictally) is frequent EEG with frontal spikes/sharps, often normal EEG

26 Parietal Lobe Seizures Most remain simple Predominantly sensory (positive and negative) Hands,arm, face more often involved (larger cortical representation) Intra-abdominal sinking sensation Metamorphosia (distortions, foreshortenings, elongations

27 Occipital Lobe Epilepsies Visual manifestations Negative (scotoma, hemianopsia, amaurosis) Positive (sparks, flashes) Perceptive illusions (changes in size, distance, or inclination)

28 Case A woman awakens to find her husband having a seizure in bed. The onset is not witnessed, but she is able to describe bilateral stiffening followed by bilateral shaking. EEG and MRI are normal. How do you classify seizure?

29 Bilateral Tonic-clonic, Onset Unknown

30 Case 2 A woman awakens to find her husband having a seizure in bed. The onset is not witnessed, but she is able to describe bilateral stiffening followed by bilateral shaking. EEG shows a clear right parietal slow wave focus. The MRI shows a right parietal region of cortical. How do you classify seizure?

31 Focal to Bilateral Tonic-clonic

32 Case 3 A 22 year-old man has seizures during which he remains fully aware, with the hair on my arms standing on edge and a feeling of being flushed. How do you classify seizure?

33 Focal aware non-motor autonomic, or focal aware autonomic. - The old classification would have called them simple partial autonomic seizures. Focal aware non-motor autonomic, or focal aware autonomic. - The old classification would have called them simple partial autonomic seizures.

34 Call to Action Appropriate identification of the seizure type can help us select the best treatment to optimize outcomes

35 Bibliography Commission on classification and terminology of the ILAE. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia. 1989; 30(4): Engel, J. Report of the ILAE Classification Core Group. Epilepsia. 2006;47(9): Berg A et al. Revised terminology and concepts for the organization of seizures and epilepsies: Report of the ILAE Comission on Classification and Terminology, Epilepsia. 2010;51(4): ). Berg A, Millichamp J. The 2010 Revised Classification of seizures and Epilepsy. Continuum. Neurology. 2013;19(3): Fisher R, et al. ILAE Official Report: A practical clinical definition of epilepsy. Epilepsia. 2014;55(4):

36 #CHAIR2016 Questions Answers & The End