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Medical Management of Epilepsy
Anna Larson, MD
Child Neurology Resident
MassGeneral Hospital for Children
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Clinical overview
DefinitionsClinical evaluationSeizure etiologiesSeizure types
Treatment of epilepsy
MedicationsDietary therapy Vagus nerve stimulatorEpilepsy surgery
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Epilepsy affects 1-2% of individuals worldwide
It is the most common neurologic problem in
childhood
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Seizure
Manifestation of abnormal and excessive
synchronized discharge of a set of cerebral
neurons. Clinical manifestations are sudden and
transient. Symptoms depend on part of brain
involved in discharge
Epilepsy
Two or more seizure episodes
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Status epilepticus
Condition in which seizure activity
continues or is repeated without regaining
consciousness for a period of 15 minutes or more
Medical emergency
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Epileptic encephalopathy
Cognitive and behavioral impairments in
individuals with epilepsy that result from
epileptiform activity
Add to baseline impairments
May worsen over time
? May improve with treatment
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Clinical evaluation
History, general and neurological examination
PhysiologyEEG, ictal video MEG, PET, SPECT
AnatomyMRI
GeneticsDravet’s, GLUT1, ARX, CDKL5, TSC
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Clinical evaluation
History of the seizure from the patient situation in which arose, trigger factors, pattern, prodromal symptoms, onset of seizure including aura or focal motor features
Seizure itselfDuration of loss of consciousnessPostictal symptomsHistory of seizure from witness
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Differential diagnosis for seizure
SyncopeVasovagalCardiacPostural Breath-holding spells
Daydreaming
Migraine
Transient ischemic events
Parasomnias
Normal physiological movements in sleep
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Differential diagnosis for seizure
Acute rise in intracerebral pressure
Gastro-esophageal reflux
Vestibular disorders
Hyperekplexia
Involuntary movement disorders
Psychotic hallucinations and delusions
Panic attacks
Psychogenic non-epileptic seizures
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Etiology of epilepsy
Inherited genetic
Congenital (inherited or acquired)Cortical dysplasia/dysgenesisTumorVascular malformationPrenatal injury
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Etiology of epilepsy
Trauma
Neurosurgery
Infection
Vascular disease
Hippocampal sclerosis
Neurodegenerative disorders
Metabolic disorders
Toxic exposure
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Clinical seizure types
Focal
Starts in a specific part of the brain
Generalized
Starts in a large area the brain in both
hemispheres
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Focal seizure types
Focal seizure with preserved awareness
simple partial
Focal seizure with impaired awareness
complex partial seizure
Focal seizure with secondary generalization
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Focal seizure with impaired awareness
Aura
Altered consciousness
Automatisms
Oro-alimentary – chewing, lip smacking, swallowing, drooling
Mimicry – laughter, fear, anger or excitement
Gestural – fiddling movements of hands, tapping, patting or
rubbing, ordering and tidying movements
Ambulatory – walking, circling or running
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Focal seizure with impaired awareness
Aura
Altered consciousness
Automatisms
Verbal – meaningless sounds, humming, whistling, grunting,
repeating words
Responsive – quasi-purposeful behavior, seemingly
responsive to environmental stimuli
Violent behavior
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Generalized seizure
Consciousness impaired from onset due to
extensive cortical and subcortical involvement
Motor changes bilateral, symmetric
EEG patterns bilateral, grossly synchronous and
symmetrical
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Generalized seizure
Typical absence (petit mal)
Atypical absence
Myoclonic
Clonic
Tonic
Tonic-clonic
Atonic
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Typical absence seizures
Abrupt onset with loss of consciousness
Abrupt end with resumption of activity
No postictal period, child often unaware of seizure
Automatisms can occur
EEG
ictal: 3 Hz spike and wave
interictal: normal
Induced by hyperventilation, occasionally photic stimulation
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Tonic clonic seizure
Prodromal period
Aura: focal seizure with secondary generalization
Loss of consciousness, occasional vocalization
Tonic phase, cyanosis common (10-30 sec)
Clonic phase (30-60 sec)
Postictal phase (2-30 min)
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Epilepsy treatment
Goal is for seizure control without significant side effects
Appropriate therapy requires accurate diagnosis
Seizure classification
Epilepsy classification
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1857 Bromides
1912 Phenobarbital
1920’s (Ketogenic Diet)
1938 Phenytoin
1950’s ACTH
1970’s Valproate, carbamazepine
1993 Felbamate, Gabapentin
1994 Lamotrigine
1997 (Vagal Nerve Stimulator), Topiramate
1998 Tiagabine
2000 Levetiracetam, Oxcarbazepine, Zonisamide
2005 Pregabalin
2009 Rufinamide, lacosamide, vigabatrin
2010 ACTH
2011 Clobazam
2017 Breviteracetam
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Primary generalized
Absence Myoclonic
Atonic
Tonic
Tonic-Clonic Focal
w/ impaired
awareness
Secondary
Generalized
Tonic-Clonic
Focal onset
Ethosuximide Benzodiazepines Carbamazepine, Phenytoin, Phenobarbital, Primidone,
Gabapentin, Tiagabine, Pregabalin, Oxcarbazepine,
Vigabatrin, Lacosamide
Valproate, Felbamate, Lamotrigine, Topiramate, Levetiracetam, Zonisamide, Rufinamide
?Lacosamide, Clobazam
Focal
w/ preserved
awareness
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Intractable seizures
Seizures that are not controlled by medications,
or are controlled only by medications that have
significant side effects.
1/3 of children with epilepsy will develop
intractable epilepsy
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Dietary therapy of epilepsy
Ketogenic Diet
Low Glycemic Index Treatment
Modified Atkin’s diet
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Ketogenic Diet
High fat diet designed to mimic the biological
effects of starvation
Developed in the 1920’s
Probably the best single anticonvulsant treatment
But not easy for the child or family
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Vagus Nerve Stimulator
Pacemaker generator
Nerve stimulation
electrode
Intermittent stimulation
Magnet on demand
therapy
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Epilepsy surgery
Should be considered after failure of 2
appropriately chosen medications
“Ideal” surgical candidates
Single seizure phenotype
Focal seizures
Focal EEG
MRI shows a single well differentiated lesion
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Pediatric epilepsy
Age-related seizures
Etiology of seizures
Benign syndromes
Developmental delay
CNS plasticity
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Thank you
Elizabeth Thiele
Ron Thibert
Patricia Bruno
Leigh Horne-Mebel
Amy Morgan
Grace Shanks
Robyn Bluestein
T’Asia Zayas
Patients and families