Pediatric Epilepsies
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Transcript of Pediatric Epilepsies
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Pediatric Epilepsies
Ali Alwadei, MD
R4-Peds Neurology
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Outline
• Definitions • Classifications• General Approach to Epilepsies • Etiologies / DDx (Sz Imitators) • Common Pediatric Epilepsy Syndromes
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Definitions
1. Seizure
2. Convulsion
3. Epilepsy
4. Epilepsy Syndrome
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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.
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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.
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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
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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
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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)
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Etiological Classification
1. Idiopathic presumed genentic
2. Symptomatic
3. Cryptogenic probably symptomatic
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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
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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
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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.
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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
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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
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Exam
• Full neuro exam including:– Head C.– Neuro-Cutaneous Stigmata
• May help localize
• Usually not helpful in epilepsy
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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.
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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
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• Common examples include:1. mesial temporal sclerosis (MTS)
2. primary brain neoplasms
3. remote trauma
4. stroke
5. developmental abnormalities
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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)
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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
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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)
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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)
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Benign Familial Neonatal Sz (BFNS)
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Benign Ideopathic Neonatal Sz (BINS)
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Benign Myoclonic Epilepsy of Infancy (BMEI)
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Generalized Epilepsy With Febrile Seizures Plus (GEFS+)
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Myoclonic Astatic Epilepsy of Doose (MAE)
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Childhood Absence Epilepsy (CAE)
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Juvenile Absence Epilepsy (JAE)
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Juvenile Myoclonic Epilepsy (JME)
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Generalized Tonic-Clonic Sz (GTCS) upon awakening
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Benign Epilepsy of Childhood With Rolandic Spikes (BECRS)
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Benign Occipital Epilepsy Early and Late (BOE)
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Benign Occipital Epilepsy Early and Late (BOE)
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Autosomal Dominant frontal lobe epilepsy ( ADNFLE)
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Familial Temporal lobe epilepsy ( FTLE)
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Early Myoclonic Encephalopathy (EME)
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Early Infantile Epileptic Encephalopathy (EIEE) or
Ohtahara Syndrome (OS)
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West Syndrome (WS)
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Dravet Syndrome (SMEI)
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Lennox-Gastaut Sydrome (LGS)
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Landau-Kleffner Syndrome (LKS)
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Continuous Spike-Wave in sleep
(CSW-SWS)
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Rasmussen Encephalitis
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Progressive Myoclonic Epilepsy (PMEs)
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Progressive Myoclonic Epilepsy (PMEs)
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