management of pediatric epilepsy - st. louis children's · pdf file ·...
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10/7/2013
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Robin Ryther, MD, PhD
Pediatric Epilepsy
Clinical Pediatric Update
October 11, 2013
Management of Pediatric Epilepsy 2013 Update
Overview
• Definitions
• Acute Seizure Treatment
– Seizure
– Seizure Precautions
– When to treat with anti‐seizure medication
• Treatment options for epilepsy
– Medication
– Dietary Therapy
– Epilepsy Surgery
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Seizure
Single electrographic event characterized by abnormal neuronal discharges resulting in disturbance of sensorium, motor activity, sensation, autonomic fx or combos thereof
Netter
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1. Is consciousness impaired?
– Simple
– Complex
2. Does the SZ involve one part of the brain or the entire brain?
– Focal
– Generalized
– Secondary Generalization
Seizure Terminology
2010 Seizure Terminology
• Simple Without impairment of consciousness
• Complex with impairment of consciousness (dyscognitive)
• Partial seizures Focal seizures
• Generalized Seizures (entire cortex) originating in and rapidly spreading to bilateral networks (not necessarily entire cortex)
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2010 Seizure Terminology
• Idiopathic (no known cause presumed genetic) Genetic
• Symptomatic Structural or Metabolic
• Cryptogenic (presumed symptomatic but cause unknown) Unknown etiology
Epilepsy
Greater than two unprovoked seizures
Electroclinical Syndrome
Specific types of epilepsy which share characteristic clinical features, age of onset, seizure types and EEG patterns
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Epilepsy in children
• 5% of children have a seizure by age 20
• 25% with single seizure develop recurrent unprovoked seizures = “epilepsy”
• Cumulative incidence of epilepsy in childhood is ~ 1%
Overview
• Definitions
• Acute Seizure Treatment
– Seizure
– Seizure Precautions
– When to treat with anti‐seizure medication
• Treatment options for epilepsy
– Medication
– Dietary Therapy
– Epilepsy Surgery
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Seizure Recovery Position
Acute Seizure Treatment
• Diastat
• Klonopin ODT
– 0.1 mg/kg/dose
– Seizure clusters
– Short seizures
• Lorazepam (Ativan®) via IV, IM
• Midazolam via buccal or intranasal route
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Seizure Precautions
Water
Wheels
Heights
Epilepsy: when to treat
Risk of additional seizures vs risk of medication
• Second seizure
• Syndrome
• EEG
• Family History
• Family, Religious, and Cultural Beliefs
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Overview
• Definitions
• Acute Seizure Treatment
– Seizure
– Seizure Precautions
– When to treat with anti‐seizure medication
• Treatment options for epilepsy
– Medication
– Dietary Therapy
– Epilepsy Surgery
Therapeutic Approach to Pediatric Epilepsy
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• Type of Seizures
• Epilepsy Syndrome
• Side effects
• Cost
• Efficacy
First Medication Options
Initial AED
Other Medical
Conditions
Potential Side Effects
Epilepsy Syndrome
Cost??
Courtesy J. Weisenberg
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Generalized Medications
Levetiracetam
(Keppra)
• Pills, Liquid, IV
• Renally Cleared
• Behavioral SE
• Vitamin B6
Zonisamide
(Zonegran)
• Capsules
• Weight Loss
• Behavioral SE
• Sedation
• Heat Intolerance
• Kidney Stones
• Acidosis
Lamotrigine
(Lamictal)
• Pills, Chewable
• Slow Start
• Rash
Focal Medications
Oxcarbazepine (Trileptal)
• Tablets, Liquid
• Sleepiness
• Hyponatremia
• Behavioral problems
Lacosamide (Vimpat)
• Tablets, Liquid, IV
• Well tolerated at low doses
• Diplopia, Dizziness, Fatigue
• PR prolongation
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Absence Epilepsy
• Ethosuximide
– Liquid, Capsules
– Abdominal pain
– Mood changes
• Depakote
• Lamictal
• Zonegran
• Methsuximide (Celontin)
– Capsules only
– Cost
– Abdominal pain
Special Medications
Clobazam
(Onfi)
• Tablets
• Inc Appetite
• Behavioral SE
• Dec Tone
Valproic Acid
(Depakote)
• Liquid, Sprinkles, Tablets, ER, DR
• TID
• Weight Gain
• Behavioral SE
• LFT, CBC
• Mitochondrial
Felbamate
(Felbatol)
• Liquid, Tablets
• Wt loss
• Sleep Disruption
• LFTs
• CBC/diff
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Epilepsy Syndromes and Bad Meds
• Dilantin
– Dravet Syndrome
– Juvenile Myoclonic Epilepsy
• Depakote
– Mitochondrial Disease
• Focal Medications in Generalized Epilepsies
• Sodium Channel Blockers
Is stopping seizures enough?
• Behavior
• School Performance
• Side Effects
• EEG Activity
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Maximizing Treatment
• Medication Compliance
• Avoid Triggers
• Avoid Sleep Deprivation
• Illness / Immunizations
• Alcohol, Substance Abuse
• Pregnancy
Goal = No seizures, No side effects
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Response to anti‐seizure medication*
1st AED
60‐70%Seizure‐Free
2ndAED
20‐30%Seizure‐Free
3rdAED
5‐10%Seizure‐Free
*New‐Onset Seizure Clinic St. Louis Children’s Hospital (n=2000)
• Dietary Therapy
• Epilepsy Surgery
• Focal Resection
• Neurostimulation (VNS, Neuropace)
• Corpus Callosotomy
Courtesy J. Weisenberg
Ketogenic Diet
Standard American Diet
Ketogenic Diet
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Ketogenic Diet
• Ratios = Dose
• 50% with a >50% seizure reduction
• 30% with >90% reduction
• Side Effects
– Constipation
– Acidosis
– Kidney Stones
– Bone Health
– Growth
– Cholesterol
• Zonegran / Topamax
Modified Atkins
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Low Glycemic Index Treatment
Epilepsy Surgery
• Eliminate SZ
– Focal Resection
– Hemispherotomy
• Palliative Options
– Corpus Callosotomy
– Neurostimulation
• VNS
• Neuropace
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Focal Resections
• Lesional vs nonlesional
– MRI epilepsy protocol
– PET scan
– MEG
• Eloquent Cortex
• Invasive Monitoring
– Mapping
– Ictal Onset Zone
Hemispherotomy
• SZ from one hemisphere
• Multifocal interictal discharges ok
• Lateralized defects on imaging
• 80% seizure free post‐op
• SE
– Hand weakness
– Visual defect
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Corpus Callosotomy
• Complete vs Anterior 2/3
• Palliative
• 1/2 ‐ 2/3 imp in seizure frequency and severity
• Drop Seizures
• Disconnection Syndrome
Vagal Nerve Stimulator
• 18 months for benefit
• Seizure Reduction
• Improved Mood
• Hoarseness, Throat Sensation
• Settings
• Battery Replacement
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Phase I Evaluation for Surgery
1. Clinic Visit
– History and Exam
– Seizure history: partial, CP, drop attacks, findings to localize or lateralize
– Skin lesions, atrophy, paresis
– Routine EEG
2. Video‐EEG admission
• Neuropsychological Evaluation
– language / learning / handedness / memory
– developmental progress
• Neuroimaging
• High‐resolution epilepsy protocol MRI
• PET
• +/‐ fMRI
3. Epilepsy Management Conf
• Surgery Recommended
• Further medical management
• Other Tests
• Ictal SPECT
• MEG
• WADA
• DTI
• Invasive Monitoring
Invasive Monitoring
• Indications:
– “Non‐lesional” epilepsy or poorly defined regions of seizure onset
– Incongruent results from non‐invasive investigations
– Eloquent cortex involved in/near epileptogenic region
• Advantages:– Precise localization of ictal onset
– Cortical stimulation mapping outside of OR
Courtesy D. Limbrick
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A diagnosis of epilepsy is not an excuse to accept recurring seizures
Future
• Immune Modulation
– Steroids
– IV IG
– Rituximab
• Neuropace
• DBS
• Anti‐seizure vs Anti‐epileptogenic
• Identification of ictal onset zone
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Neuropace
When do you stop treatment?
• Seizure Control
– Time
– Etiology
• EEG activity
• Activity Limitations
– Driving
– Seizure Precautions
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The Epilepsy Team
Urgent Epilepsy Access
• New Onset Seizure Clinic 314‐454‐4355
– NP, Seen within 1 week, coordinated visits with EEG
– 6 mo – 18 years
– Developmentally Normal
• Neurology Urgent Clinic 314‐454‐6120 ext 1
– Wed AM, opens for scheduling 1 week before
– Target Population
• 0 to 18 years
• New SZ or spells, developmentally abn or other medical complications
• Developmental regression
• Urgent 2nd opinion in epilepsy
• PCP concern
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Epilepsy Access314‐454‐6120 ext 1
• Ketogenic Diet Clinic
– Liu Lin Thio, MD, PhD
• Epilepsy Center Clinics
– Liu Lin Thio, John Zempel, Mary Bertrand, Judy Weisenberg, Chris Gurnett, Mike Wong, Robin Ryther
– Fellows Clinics: Adam Ostendorf, Rohini Coorg
• Tuberous Sclerosis Clinics
– Mike Wong, MD, PhD
• Rett Syndrome and Early Epileptic Encephalopathies Clinic (coming soon)
– Robin Ryther (MECP2)
– Judy Weisenberg (CDKL5)
Questions?
Thank You
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