Overview of Seizures and Epilepsy Lucyna Zawadzki, MD Director of Pediatric Epilepsy Program UWHC Madison

Learning Objectives 1. After completing this activity, the learner will be able to define current prevalence and trends related to epilepsy and seizures. 2. After completing this activity, the learner will be able to describe current treatments-including alternative care and new methods of drug delivery for children and adolescents with epilepsy. 3. After completing this activity, the learner will be able to propose a plan of care for a student with epilepsy, including activity restrictions. 4. After completing this activity, the learner will be able to describe and implement effective strategies for communicating and collaborating with health care providers around care for students with epilepsy.

Definitions Epileptic seizure- a transient occurrence of signs and/or symptoms due to abnormal excessive pattern of excitability and synchrony among neurons in select brain areas. Epilepsy- persistent epileptogenic abnormality of the brain that is able to spontaneously generate paroxysmal activity Recurrent seizures (two or more)which are not provoked by systemic or acute neurologic insults Epilepsy syndrome- complex of signs and symptoms that define a unique epileptic condition

Epilepsy Facts 1 in 26 people will develop epilepsy during their lifetime More common then autism, cerebral palsy, MS and Parkinson’s disease combined Epilepsy accounts for 1% of the global burden of disease ( WHO), equal to burden of lung ca in men and breast ca in women

Risk of Epilepsy Highest at extremes of life ( 1st year of life and in elderly) Risk of having at least 1 seizure in lifetime is 10%, and 1/3 will develop epilepsy 2.2 mln of Americans 65 mln people worldwide

Incidence and Prevalence in Pediatric Population 5 per 1000 in school-aged children 1.5 per 1000 in preschool-aged children Affects more then 300,000 children under age 15 >90,000 not adequately treated

Seizure Classification International League Against Epilepsy (ILAE) 2010 revised classification Based on known causes of epilepsy: Genetic Structural/metabolic Unknown cause

Generalized Seizures Tonic–clonic (in any combination) Absence Typical Atypical Absence with special features Myoclonic absence Eyelid myoclonia Myoclonic Myoclonic atonic Myoclonic tonic Clonic Tonic Atonic

Focal Seizures Without impairment of consciousness or awareness With observable motor or autonomic components. This roughly corresponds to the concept of “simple partial seizure. Involving subjective sensory or psychic phenomena only. This corresponds to the concept of an aura. With impairment of consciousness or awareness (dyscognitive). This roughly corresponds to the concept of complex partial seizure. Evolving to a bilateral, convulsive seizure (involving tonic, clonic, or tonic and clonic components). This expression replaces the term “secondarily generalized seizure.”

Epileptic Syndromes Defined by: Clinical features Seizure type(s) Age of onset Family history EEG findings Diagnostic Investigations Treatment Prognosis

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 nonprogressive disorders

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)

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

Distinctive constellations Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE with HS) Rasmussen syndrome Gelastic seizures with hypothalamic hamartoma Hemiconvulsion–hemiplegia–epilepsy Epilepsies that do not fit into any of these diagnostic categories can be distinguished first on the basis of the presence or absence of a known structural or metabolic condition (presumed cause) and then on the basis of the primary mode of seizure onset (generalized vs. focal) Epilepsies attributed to and organized by structural-metabolic causes Malformations of cortical development (hemimegalencephaly, heterotopias, etc.) Neurocutaneous syndromes (tuberous sclerosis complex, Sturge-Weber, etc.) Tumor Infection Trauma Angioma Perinatal insults Stroke Etc. Epilepsies of unknown cause Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy per se Benign neonatal seizures (BNS) Febrile seizures (FS)

Not everything that looks like a seizure is a seizure Migraine headache Syncope Psychogenic seizure Behavioral outbursts Breath holding spells Tics Sleep disorders

Diagnosing Epilepsy Detailed history and physical examination EEG, video EEG Imaging ( CT, MRI, PET)

History Precipitating factors Aura Area of body first involved Progression of activity Specific activity observed (head or eye deviation, type of movement or posturing) Level of consciousness Incontinence Apnea or cyanosis Duration of seizure Postictal symptoms ( confusion, hemiplegia, aphasia)

Seizure Triggers Missed dose of medication Sleep deprivation Illness Stress, anxiety, overstimulation Hormonal changes Alcohol and drugs of abuse Hyperventilation Flashing lights Temperature extremes dehydration

Spectrum of Severity

Psychosocial effects of epilepsy Stigma of epilepsy Compromised quality of life Lower self-esteem “Vulnerable child” High incidence of depression, anxiety, ADHD and learning problems Adverse effects of medications Parental stress