Pediatric Epilepsies Ali Alwadei, MD R4-Peds Neurology

Outline Definitions Classifications General Approach to Epilepsies Etiologies / DDx (Sz Imitators) Common Pediatric Epilepsy Syndromes

Definitions Seizure Convulsion Epilepsy Epilepsy Syndrome

Definitions Seizure/Convulsion Seizure: Abnormal Electrical Activity in the brain, its manifestations depend on where the abnormal electrical activity takes place in the brain (i.e. localization: Motor/convulsive, sensory/visual, autonomic). abbreviated sz. Convulsion: motor seizure Epilepsy 2 or more (RECURRENT) unprovoked seizures.

Definitions Epilepsy Syndrome refer to ‘‘a complex of symptoms and signs that define a unique epileptic condition.’’ denotes specific constellations of clinical seizure type(s) EEG findings other characteristic clinical features, such as age at onset course of epilepsy associated neurologic and neuropsychological findings underlying pathophysiologic or genetic mechanisms.

Clinical Classification Partial Simple (without impairment of consciousness) Complex (with impairment of consciousness) Gelastic Reflex Generalized Absence/Atypical Absence Myoclonic/spasms/-ve myoclonus GTC Tonic/Atonic Clonic with or without tonic features Partial with 2ry Generalization

Precipitating stimuli for reflex seizures Visual stimuli Flickering light -color to be specified when possible Patterns Other visual stimuli Thinking Music Eating Praxis Somatosensory Proprioceptive Reading Hot water Startle ILAE, Benjamin G. Zifkin and Frederick Andermann

Clinical Classification Status Epilepticus Generalized status epilepticus Absence status epilepticus Myoclonic status epilepticus GTC status epilepticus Tonic status epilepticus Clonic status epilepticus Focal status epilepticus Epilepsia Partialis Continua of Kojenikov (Kojewnikow syndrome) Hemiconvulsive status with Hemiparesis Aura Continua Limbic status epilepticus (psychomotor status)

Etiological Classification Idiopathic presumed genentic Symptomatic Cryptogenic probably symptomatic

Classification Etiology Dev MRI Unknown ? genetic NL Known Idiopathic presumed genentic Unknown ? genetic NL Symptomatic Known Usually structural AbN Cryptogenic probably symptomatic ?abN-3T

General Approach to Epilepsies Aim to classify the patient under specific: Clinical class ( SPC, CPS, Gen…etc) Etiologic class (idio, symp, crypto), more difficult Syndrome if possible. Much more difficult Hx Ex EEG MRI

General Approach to Epilepsies Identification of a specific syndrome is important to define the best treatment and accurately prognosticate long-term outcome. Most syndromes recognized in epilepsy are genetic and developmental disorders that begin in the pediatric years.

Hx Age and sex FHx: if +ve ? genetic Perinatal Hx: e.g. HIE Developmental: helpful to differentiate idiopathic vs Sympto /Cryptogenic Vaccination and relation to sz onset PHx Meds (current and previous) Allergies

Hx HPI: Age at 1st sz ( onset) if known epileptic Aura Sz types (semiology) Partial vs Generalized. Sz Frequency Sz Duration Post-Ictal Urinary/Fecal incontinence Tongue Biting R/O Sz imitators ( see DDx) Previous work up

Exam Full neuro exam including: May help localize Head C. Neuro-Cutaneous Stigmata May help localize Usually not helpful in epilepsy

W/up Consider: EEG ( sensitivity, SD, prolonged) blood and urine: CSF SMA10 S. glucose LFTs Metabolic, Ammonia CGH micro-array Toxic screening CSF MRI, 3-Tesla magnet.

MRI Performing an MRI is important particularly when partial seizures are suspected. MRI has been shown to be superior to CT as the overall resolution for potential epileptogenic lesion detection is superior. many patients with epilepsy do not have identifiable lesions on MRI

Common examples include: mesial temporal sclerosis (MTS) primary brain neoplasms remote trauma stroke developmental abnormalities

Common Pediatric Epilepsy Syndromes IDIOPATHIC GENERALIZED EPILEPSY (IGE) SYNDROMES Infantile Onset: Benign Neonatal Convulsions (BNC/BNS) Benign Familial Neonatal Sz (BFNS) Benign Ideopathic Neonatal Sz (BINS) Benign Myoclonic Epilepsy of Infancy (BMEI) Childhood Onset: Generalized Epilepsy With Febrile Seizures Plus (GEFS+) Myoclonic Astatic Epilepsy of Doose (MAE) Childhood Absence Epilepsy (CAE)

Common Pediatric Epilepsy Syndromes IDIOPATHIC GENERALIZED EPILEPSY (IGE) SYNDROMES Adolescence Onset: Juvenile Absence Epilepsy (JAE) Juvenile Myoclonic Epilepsy (JME) Generalized Tonic-Clonic Sz (GTCS) upon awakening

Common Pediatric Epilepsy Syndromes IDIOPATHIC PARTIAL EPILEPSY SYNDROMES Benign Epilepsy of Childhood With Rolandic Spikes (BECRS) Benign Occipital Epilepsy Early and Late (BOE) Autosomal Dominant frontal lobe epilepsy ( ADNFLE) Familial Temporal lobe epilepsy ( FTLE)

Common Pediatric Epilepsy Syndromes SYMPTOMATIC EPILEPSY SYNDROMES Infantile Onset: Early Myoclonic Encephalopathy (EME) Early Infantile Epileptic Encephalopathy (EIEE) or Ohtahara Syndrome (OS) West Syndrome (WS) Dravet Syndrome (SMEI) Childhood Onset: Lennox-Gastaut Sydrome (LGS) Landau-Kleffner Syndrome (LKS) Continuous Spike-Wave in sleep (CSW-SWS) Rasmussen Encephalitis Varying Age: Progressive Myoclonic Epilepsy (PMEs)

Benign Familial Neonatal Sz (BFNS)

Benign Ideopathic Neonatal Sz (BINS)

Benign Myoclonic Epilepsy of Infancy (BMEI)

Generalized Epilepsy With Febrile Seizures Plus (GEFS+)

Myoclonic Astatic Epilepsy of Doose (MAE)

Childhood Absence Epilepsy (CAE)

Juvenile Absence Epilepsy (JAE)

Juvenile Myoclonic Epilepsy (JME)

Generalized Tonic-Clonic Sz (GTCS) upon awakening

Benign Epilepsy of Childhood With Rolandic Spikes (BECRS)

Benign Occipital Epilepsy Early and Late (BOE)

Benign Occipital Epilepsy Early and Late (BOE)

Autosomal Dominant frontal lobe epilepsy ( ADNFLE)

Familial Temporal lobe epilepsy ( FTLE)

Early Myoclonic Encephalopathy (EME)

Early Infantile Epileptic Encephalopathy (EIEE) or Ohtahara Syndrome (OS)

West Syndrome (WS)

Dravet Syndrome (SMEI)

Lennox-Gastaut Sydrome (LGS)

Landau-Kleffner Syndrome (LKS)

Continuous Spike-Wave in sleep (CSW-SWS)

Rasmussen Encephalitis

Progressive Myoclonic Epilepsy (PMEs)

Progressive Myoclonic Epilepsy (PMEs)

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