Pediatric Epilepsies

Ali Alwadei, MD

R4-Peds Neurology

Outline

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

Definitions

1. Seizure

2. Convulsion

3. Epilepsy

4. 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 1. clinical seizure type(s)2. EEG findings 3. other characteristic clinical features, such as

• age at onset • course of epilepsy• associated neurologic and neuropsychological findings• underlying pathophysiologic or genetic mechanisms.

Clinical Classification1. Partial

1. Simple (without impairment of consciousness)2. Complex (with impairment of consciousness)3. Gelastic4. Reflex

1. Generalized1. Absence/Atypical Absence2. Myoclonic/spasms/-ve myoclonus3. GTC

1. Tonic/Atonic2. Clonic with or without tonic features

2. 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 ClassificationStatus Epilepticus

• Generalized status epilepticus 1. Absence status epilepticus 2. Myoclonic status epilepticus 3. GTC status epilepticus

1. Tonic status epilepticus 2. Clonic status epilepticus

• Focal status epilepticus 1. Epilepsia Partialis Continua of Kojenikov (Kojewnikow syndrome)2. Hemiconvulsive status with Hemiparesis3. Aura Continua4. Limbic status epilepticus (psychomotor status)

Etiological Classification

1. Idiopathic presumed genentic

2. Symptomatic

3. Cryptogenic probably symptomatic

Classification

Etiology Dev MRI

Idiopathic presumed genentic

Unknown ? genetic

NL NL

Symptomatic KnownUsually

structural

AbN AbN

Cryptogenic probably symptomatic

Unknown ? genetic

AbN NL ?abN-3T

General Approach to Epilepsies

• Aim to classify the patient under specific: 1. Clinical class ( SPC, CPS, Gen…etc)

2. Etiologic class (idio, symp, crypto), more difficult

3. Syndrome if possible. Much more difficult

1. Hx

2. Ex

3. EEG

4. 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:– Head C.– Neuro-Cutaneous Stigmata

• May help localize

• Usually not helpful in epilepsy

W/up

• Consider:– EEG ( sensitivity, SD, prolonged) – blood and urine:

• 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:1. mesial temporal sclerosis (MTS)

2. primary brain neoplasms

3. remote trauma

4. stroke

5. developmental abnormalities

Common Pediatric Epilepsy Syndromes

• IDIOPATHIC GENERALIZED EPILEPSY (IGE) SYNDROMES– Infantile Onset:

1)Benign Neonatal Convulsions (BNC/BNS)

A)Benign Familial Neonatal Sz (BFNS)

B)Benign Ideopathic Neonatal Sz (BINS)

1)Benign Myoclonic Epilepsy of Infancy (BMEI)

– Childhood Onset:

1)Generalized Epilepsy With Febrile Seizures Plus (GEFS+)

2)Myoclonic Astatic Epilepsy of Doose (MAE)

3)Childhood Absence Epilepsy (CAE)

Common Pediatric Epilepsy Syndromes

• IDIOPATHIC GENERALIZED EPILEPSY (IGE) SYNDROMES– Adolescence Onset:

1)Juvenile Absence Epilepsy (JAE)

2)Juvenile Myoclonic Epilepsy (JME)

3)Generalized Tonic-Clonic Sz (GTCS) upon awakening

Common Pediatric Epilepsy Syndromes

• IDIOPATHIC PARTIAL EPILEPSY SYNDROMES

1)Benign Epilepsy of Childhood With Rolandic Spikes (BECRS)

2)Benign Occipital Epilepsy Early and Late (BOE)

3)Autosomal Dominant frontal lobe epilepsy ( ADNFLE)

4)Familial Temporal lobe epilepsy ( FTLE)

Common Pediatric Epilepsy Syndromes

• SYMPTOMATIC EPILEPSY SYNDROMES– Infantile Onset:

1)Early Myoclonic Encephalopathy (EME)

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

3)West Syndrome (WS)

4)Dravet Syndrome (SMEI)– Childhood Onset:

1)Lennox-Gastaut Sydrome (LGS)

2)Landau-Kleffner Syndrome (LKS)

3)Continuous Spike-Wave in sleep (CSW-SWS)

4)Rasmussen Encephalitis – Varying Age:

1)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)

Thank You