Transcript Slide 1

Pediatric Epilepsies
Ali Alwadei, MD
R4-Peds Neurology
Outline
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Definitions
Classifications
General Approach to Epilepsies
Etiologies / DDx (Sz Imitators)
Common Pediatric Epilepsy Syndromes
Definitions
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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
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Epilepsy Syndrome
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refer to ‘‘a complex of symptoms and signs that
define a unique epileptic condition.’’
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denotes specific constellations of
1. clinical seizure type(s)
2. EEG findings
3. other characteristic clinical features, such as
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age at onset
course of epilepsy
associated neurologic and neuropsychological findings
underlying pathophysiologic or genetic mechanisms.
Clinical Classification
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Partial
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2.
Simple (without impairment of consciousness)
Complex
(with impairment of consciousness)
Gelastic
Reflex
Generalized
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Absence/Atypical Absence
Myoclonic/spasms/-ve myoclonus
GTC
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2.
Tonic/Atonic
Clonic with or without tonic features
Partial with 2ry Generalization
Precipitating stimuli for
reflex seizures
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Visual stimuli
– Flickering light -color to be specified when possible
– Patterns
– Other visual stimuli
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Thinking
Music
Eating
Praxis
Somatosensory
Proprioceptive
Reading
Hot water
Startle
ILAE,
Benjamin G. Zifkin and Frederick Andermann
Clinical Classification
Status Epilepticus
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Generalized status epilepticus
1. Absence status epilepticus
2. Myoclonic status epilepticus
3. GTC status epilepticus
1. Tonic status epilepticus
2. Clonic status epilepticus
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Focal status epilepticus
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Epilepsia Partialis Continua of Kojenikov (Kojewnikow syndrome)
Hemiconvulsive status with Hemiparesis
Aura Continua
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
Known
Usually
structural
AbN
AbN
Cryptogenic
probably
symptomatic
Unknown
? genetic
AbN
NL
?abN-3T
General Approach to Epilepsies
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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
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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
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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:
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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:
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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
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Common examples include:
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mesial temporal sclerosis (MTS)
primary brain neoplasms
remote trauma
stroke
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:
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)
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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