What to do IF What to do IF Medications Medications

Fail?Fail?Dr Linda HuhDr Linda Huh

Pediatric NeurologistPediatric NeurologistBCCHBCCH

Outline and ObjectivesOutline and Objectives

What is intractable epilepsy?What is intractable epilepsy?

Discuss why medications may failDiscuss why medications may fail

Learn more about alternate treatments to Learn more about alternate treatments to medicationsmedications Epilepsy SurgeryEpilepsy Surgery Vagal Nerve StimulatorVagal Nerve Stimulator Ketogenic DietKetogenic Diet

Discuss future potential therapies Discuss future potential therapies

Intractable EpilepsyIntractable Epilepsy

47% seizure free on first medication47% seizure free on first medication

13% seizure free on second medication13% seizure free on second medication

<5% seizure free on third medication<5% seizure free on third medication

30% of patients have difficult to control 30% of patients have difficult to control epilepsyepilepsy

Intractable EpilepsyIntractable Epilepsy

Why may Why may anticonvulsants fail?anticonvulsants fail?

Incorrect diagnosisIncorrect diagnosis

Incorrect anticonvulsantIncorrect anticonvulsant

Intolerable side effects or noncomplianceIntolerable side effects or noncompliance

Drug-resistant epilepsyDrug-resistant epilepsy

Goals of Epilepsy Goals of Epilepsy SurgerySurgery

Seizure-freedomSeizure-freedom

Improvement of quality of lifeImprovement of quality of life

Do no harm (minimize deficits)Do no harm (minimize deficits)

Decrease anticonvulsantsDecrease anticonvulsants

Aims of Epilepsy Surgery Aims of Epilepsy Surgery WorkupWorkup

Find where seizures are coming fromFind where seizures are coming from

To spare important brain functionsTo spare important brain functions

Evaluation for Epilepsy Evaluation for Epilepsy SurgerySurgery

History and Physical ExaminationHistory and Physical Examination

Evaluation for Epilepsy Evaluation for Epilepsy SurgerySurgery

History and Physical ExaminationHistory and Physical Examination

Video EEG monitoringVideo EEG monitoring

Evaluation for Epilepsy Evaluation for Epilepsy SurgerySurgery

History and Physical ExaminationHistory and Physical Examination

Video EEG monitoringVideo EEG monitoring

Evaluation for Epilepsy Evaluation for Epilepsy SurgerySurgery

History and Physical ExaminationHistory and Physical Examination

Video EEG monitoringVideo EEG monitoring

Neuropsychological assessmentNeuropsychological assessment

MRI – 1.5 or 3TMRI – 1.5 or 3T

Evaulation for Epilepsy Evaulation for Epilepsy SurgerySurgery

Ictal SPECT, PETIctal SPECT, PET

MEGMEG

fMRI fMRI

Wada testWada test

EcoG (electrocorticography)EcoG (electrocorticography)

Subdural/Depth electrodes +/- mappingSubdural/Depth electrodes +/- mapping

Ictal SPECTIctal SPECT

Increased blood flowIncreased blood flow

PETPET

Decreased Decreased metabolismetabolismm

MagnetoencephalograMagnetoencephalogramm

Finger Tapping- Right vs. Left

q= 0.01Right Finger Tapping

Left Finger Tapping

Finger Tapping- Right vs. Left

q= 0.01Right Finger Tapping

Left Finger Tapping

PRE-RESECTION - POSITION 1

832

529

What to do? What to do?

Resective surgeryResective surgery Focal neocortical resectionFocal neocortical resection Anteromedial temporal lobectomyAnteromedial temporal lobectomy HemispherectomyHemispherectomy

Palliative surgeryPalliative surgery Corpus callosotomyCorpus callosotomy Multiple subpial transectionsMultiple subpial transections Vagal nerve stimulationVagal nerve stimulation

JulieJulie

Prolonged febrile seizuresProlonged febrile seizures

Developed partial seizures age 9Developed partial seizures age 9 Tried 5 medicationsTried 5 medications

Video EEG showed left temporalVideo EEG showed left temporal

MRIMRI

Anterior Medial Temporal Anterior Medial Temporal LobectomyLobectomy

HemispherectomyHemispherectomy

Corpus CallosotomyCorpus Callosotomy

Anterior 2/3Anterior 2/3 FullFull

Multiple Subpial Multiple Subpial TransectionsTransections

OutcomeOutcome

Seizure Freedom or reductionSeizure Freedom or reduction

Decrease in medicationsDecrease in medications

Improvement in Cognition and behaviourImprovement in Cognition and behaviour

Improvement in Quality of LifeImprovement in Quality of Life

Vagus Nerve Vagus Nerve StimulationStimulation

Vagal Nerve StimulatorVagal Nerve Stimulator

1997: approved by FDA (US) as 1997: approved by FDA (US) as adjunctive treatment of medically adjunctive treatment of medically refractory epilepsy in adolescents and refractory epilepsy in adolescents and adultsadults

1998: approved by HPB (Canada)1998: approved by HPB (Canada)

> 40,000 patients world-wide have > 40,000 patients world-wide have been implanted been implanted

IndicationsIndications

No correlation with type of epilepsyNo correlation with type of epilepsy

Intractable epilepsy with no better surgical Intractable epilepsy with no better surgical optionoption

To shorten a seizureTo shorten a seizure

To stop a seizureTo stop a seizure

To decrease severityTo decrease severity

To shorten post-ictal To shorten post-ictal

phasephase

Magnet UseMagnet Use

Vagal Nerve StimulatorVagal Nerve Stimulator

VNS for Intractable EpilepsyVNS for Intractable Epilepsy

50% of patients have >50% 50% of patients have >50% reduction in seizuresreduction in seizures

May allow reduction in dosage May allow reduction in dosage of antiepileptic medications of antiepileptic medications

Majority have improved quality Majority have improved quality of lifeof life

Magnet use can abort seizures Magnet use can abort seizures

Ketogenic DietKetogenic Diet

Ketogenic DietKetogenic Diet

15-35% seizure-free15-35% seizure-free

30-50% with >90% reduction in seizures30-50% with >90% reduction in seizures

50-75% with >50% reduction in seizures50-75% with >50% reduction in seizures

Improved alertness and developmentImproved alertness and development

Ketogenic DietKetogenic Diet

Modified Atkins DietModified Atkins Diet

Low Glycemic DietLow Glycemic Diet

Ketogenic DietKetogenic Diet

Prescribed therapy by Ketogenic Diet TeamPrescribed therapy by Ketogenic Diet Team

Side effects Side effects

Ketogenic DietKetogenic Diet

Ketogenic DietKetogenic Diet

Hundreds of seizures/dayHundreds of seizures/day

Plateauing of developmentPlateauing of development

> 6 medications in 6 months> 6 medications in 6 months

Seizure free on the Ketogenic Diet with Seizure free on the Ketogenic Diet with improvement of developmentimprovement of development

Future therapies to Future therapies to look to…look to…

New AnticonvulsantsNew Anticonvulsants

Seizure predictionSeizure prediction

NeurostimulationNeurostimulation Neuropace (Responsive Neuronal Stimulation)Neuropace (Responsive Neuronal Stimulation) Deep brain stimulationDeep brain stimulation

Magnetic Stimulation and CoolingMagnetic Stimulation and Cooling

Gene and biological agent therapyGene and biological agent therapy

What can WE do when What can WE do when medications fail?medications fail?

Optimize quality of lifeOptimize quality of life

Look for comorbidities and treat as neededLook for comorbidities and treat as needed

Maintain good relationships – family and Maintain good relationships – family and friendsfriends

Optimize school and employmentOptimize school and employment

Maintain a healthy lifestyleMaintain a healthy lifestyle ExerciseExercise DietDiet Bone healthBone health

Thank you for your Thank you for your attentionattention