Transcript Pediatric Epilepsy Update - FACES (Finding a Cure for Epilepsy and

PEDIATRIC EPILEPSY
UPDATE
O R R I N D E V I N S K Y, M D
SEIZURES AND EPILEPSY
• A seizure is a brief, excessive discharge of brain electrical activity that changes
how a person feels, senses, thinks, or behaves.
• Epilepsy is a disorder in which a person has two or more seizures without a
clear cause.
• More than 2 million Americans have epilepsy; 9 million will have epilepsy at
some time in their life.
• One in 11 people will have at least one seizure in their lifetime.
• Most individuals with epilepsy have normal intelligence, behavior and are
seizure free on medications
• 70% of children with Epilepsy will outgrow it
COMMON QUESTIONS
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Will my child be alright?
Can he/she ever lead a normal life?
Can seizures ever be controlled?
Can I ever leave he/she alone?
Will he/she have to take medicine for the rest of their life?
What will my friends think?
Will I ever be able to ride my bike again? Play on my school
team? Go to college? Drive a car?
ROUTINE MEDICAL CARE AND EPILEPSY
• My child suffers from allergies? Is it safe to administer
allergy medicine?
• My child has a cold, are there any medications I should
avoid?
• Can my child receive immunizations?
• My child was prescribed antibiotics, is it safe to
administer with her seizure medication?
EDUCATION/SCHOOL PLACEMENT
• Most children with epilepsy attend regular classes,
although in some cases they need special aides to work
with them.
• Special education programs- instruction in regular
classrooms or separate facilities for all or part of the
day.
• If child is not doing well in mainstreamed classroom,
parents should meet with teachers to identify the
problem
• Comprehensive Evaluation
CAN I LET MY CHILD GO OUT AND PLAY?
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YES!!!
Can he ride a bike?
Can she swim?
Can he play football?
Can she go to sleep away camp?
Should we tell the coach he has epilepsy?
• Common sense goes a long way. Each decision should be on an
individualized basis. The goal should be safety and a lifestyle as
normal as possible.
VIDEO GAMES AND SEIZURES
• Video games do not cause epilepsy.
• Children who are photosensitive, and in whom
flashing lights or flickering images can trigger
seizures or epilepsy waves on EEG may have
seizures playing video games. This occurs in
approximately 3 % of people with epilepsy, so
almost all children who have epilepsy should be
able to play video games without seizures.
VIDEO GAMES AND SEIZURES
• Play in well lighted room
• Maintain distance between the screen
• Avoid playing for long periods of time
• Take regular breaks
• Stop the game if strange/unusual feeling
develops
COPING WITH EPILEPSY
• Be truthful and simple.
• Reassurance
• You can’t catch a seizure as if it were a cold.
• Fear, Grief, Anger are all normal responses.
Acceptance takes time. Acceptance means that you
consider your child a normal child who happens to
have seizures.
COPING WITH EPILEPSY
• How do you feel about having epilepsy?
• How do you think other kids react to you
because you have epilepsy?
• Do you understand what the doctor said to
you?
BIASES IN EPILEPSY CARE: LESSONS OF
BEHAVIORAL ECONOMICS
• Diagnostic Bias
• Law of Small Numbers
• Status Quo Bias
• Availability Heuristic
BIASES IN MEDICINE: KAHNEMAN &
TVERSKY’S LESSONS
• Loss aversion
• Anchoring
• Framing
• What You See is All There Is
WHAT DO NBA COACHES, MOTHERS
AND DOCTORS HAVE IN COMMON?
• The Diagnostic Bias
• 1st round v. 2nd round choice
• Diagnosis to doctor = child to mother
• Reliance on prior diagnosis
• Failure to consider other disorders
Convulsive syncope
Nonepileptic psychogenic seizures
• Failure to consider diagnostic changes
LAW OF SMALL NUMBERS
• Hasty generalizations from a few
examples
• Initial set of data is usually biased
• Scientists understand power and
statistics in their discipline, but often
forget it when they think outside their
discipline
HUMANS ARE ANECDOTE DRIVEN
• We evolved to understand individual instances
very well, not statistics
• A moving story about a castaway dog or sick
children v. a genocide of ~800k
• Would you give more for a dog or 100 sick kids?
• Rwanda v. OJ Simpson – media coverage
• Vaccines cause autism (NO!)
HUMANS ARE ANECDOTE DRIVEN
• Sabril (vigabatrin) can cause blindness
• Felbatol (felbamate) can be deadly
• People can become vegetables after spinal taps
• You only need to hear about one bad case…and
it doesn’t have to be true
• Need to examine the evidence
FAILURE TO UNDERSTAND NUMBERS
• The medical literature is very confusing, even
for scientists and doctors
• Few doctors and fewer patients have formal
statistical training
• The Monte Hall problem
• AED/blood count/liver tests and Cancer
Screening – America makes political not wise
choices
STATUS QUO BIAS
• Doctors and patients fall victim
• Doctors accept previous diagnoses
• Doctors advocate treatments that are ‘accepted’
but not ‘proven’
• Patients accept poorly controlled seizures and/or
side effects
• Patients accept ‘communal experience’ although
unproven/anecdotal
WE GET USED TO WHAT
WE GET USED TO
• What do these all have in common?
• Lottery winners
• Quadriplegics
• Farmers whose roosters rape chickens
• People who eat mediocre blueberries
• Parents of kids with Lennox-Gastaut Syndrome
ERRORS IN ASSESSING RISK
• Surgery is too dangerous
• Living with chronic epilepsy can be dangerous
• Changing medications is too risky
• Change can be risky; No change can be risky
• The grass is browner on the other side
• Breakthrough seizure
• Living with chronic side effects has risks
• We accept the negatives we think we know but fear
the change to make them better
• Do no harm, but judiciously assess risk
WHAT YOU SEE IS ALL THERE IS
(WYSIATS)
People make decisions based on limited data by
using available information and ignoring
information that is not available
In Epilepsy: assume we understand causes of
seizures when we may only have 10-20% of
the data
CARE OF THE CHILD WITH EPILEPSY:
UNDERSTANDING THE MECHANISMS
Treatment
• Anti-epileptic Drugs
• Metabolism-based
Treatment (KGD)
• Neuromodulation
• Epilepsy Surgery
Quality of Life
• Co-Morbidities
• Cognitive
• Behavioral
CO-MORBIDITY
DEFINITIONS AND CONCEPTS
• Co-morbidity refers to the co-occurrence
of two supposedly separate conditions that
occur together more than chance.
• Depression occurs more frequently in
patients with epilepsy than in the normal
population, thus epilepsy and depression
are co-morbidities.
CO-MORBIDITY
DEFINITIONS AND CONCEPTS
• Co-morbidities may be related to the epilepsy or
treatment, e.g.:
• Frequent absence seizures may result in attention deficit
• Headaches can occur following seizures
• Cognitive impairment may be related to epileptiform
discharges/seizures
• AED-induced agitation, ADHD, suicidality
CO-MORBIDITY
DEFINITIONS AND CONCEPTS
• Co-morbidities are not necessarily causal.
• Both conditions may have a common biological
substrate
• An independent variable triggers one of the comorbidities
• Phenobarbital triggers depression
• Gabapentin triggers agitation
• Co-morbidities often precede onset of the
epilepsy
BEHAVIOR PROBLEMS IN CHILDREN
WITH EPILEPSY: AUSTIN 2002
• Major risk factors for behavior problems in pediatric epilepsy:
• neurological dysfunction
• seizure variables
• family environment
• side effects of AEDs
• Children with new-onset seizures (rank order of total behavior
problems):
• recurrent seizures > single seizure > sibling
• Raises possibilities that both are caused by an
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underlying neurological disorder.
BEHAVIORAL PROBLEMS IN NEW-ONSET
EPILEPSY: AUSTIN 2002
• Evaluated 224 children with new-onset seizures (aged 4-14 years)
and 159 siblings (4-18 years).
• During the 2-year evaluation period, 163 (73%) children had at
least one additional seizure, and 61 (27%) had none.
• Children had more behavioral problems when experiencing
recurrent seizures than when not experiencing recurrent seizures.
• Siblings had significantly lower behavioral problems than both
children experiencing and not experiencing recurrent seizures.
• Recurrent seizures significantly predicted behavior problems very
early in the course of a seizure condition.
PRACTICE TOOLS FOR COGNITIVE AND
BEHAVIORAL EFFECTS OF EPILEPSY (AES)
• Cognitive (neuropsychological) well being: Children with epilepsy
are at an increased risk for cognitive and behavioral impairment.
Consider referral for neuropsychological evaluation for
children/adolescents with epilepsy who are experiencing difficulty at
home or in school. In particular, children are at risk of
neuropsychological deficits who present with two or more of the
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epileptiform activity on EEG
regression in academic abilities or motor function
abnormality on MRI (or symptomatic epilepsy syndrome)
absence seizures
use of antiepileptic medications
undercontrolled (pharmacoresistent) seizures
EPILEPSY AND COMMON COMORBIDITIES:
IMPROVING THE OUTPATIENT ENCOUNTER
• Screen for adverse AED effects
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Assess physical fitness, activity
Review sleep hygiene
Screen for mood disorders (depression, anxiety)
Screen for educational functioning
Screen for behavior problems
Assess other family, patient concerns
RELATIONSHIP BETWEEN
SEIZURES & COGNITIVE DYSFUNCTION
Brain Injury
Seizures and
EEG anormalities
Cognitive
Impairment
RESCUE MEDICATIONS OR
NO IV ACCESS
• Midazolam: nasal, buccal, IM
• Diazepam: rectal, buccal
• Clonazepam: oral, dissolvable
METABOLISM-BASED TREATMENTS
(KETOGENIC DIET): FOUR VERSIONS
Four Versions
1) Classic ketogenic diet
2) MCT ketogenic diet
can’t do 3/1 ratio, usually
g-tube fed
3) Low glycemic index
treatment
4) Modified Atkins/ modified
ketogenic diet
Certain Syndromes
• Myoclonic-Atonic Epilepsy
(Doose Syndrome)
• Severe Myoclonic Epilepsy of
Infancy (Dravet Syndrome)
• Infantile Spasms
• Tuberous Sclerosis
• Rett Syndrome
• Pyruvate dehydrogenase
Deficiency
• Glucose Transporter Defect
WHEN TO REFER FOR
EPILEPSY SURGERY
• Medically Refractory Epilepsy
• Children
• Uncontrolled by medical therapy
• Failure two or three AEDs
• Disabling seizures, side effects
• MRI reveals surgical lesion (epileptogenic lesion)
• Criteria and Referral for evaluation of pediatric epilepsy surgery;
ILAE Epilepsia 2006;47:952-959.
NEUROMODULATION
• Vagus Nerve Stimulation
• Deep Brain Stimulation (anterior thalamic
nucleus)
• Responsive Neurostimulation (NeuroPace)
• Transcranial Magnetic Stimulation
THE RNS™ SYSTEM
• Cranially implanted
battery powered
responsive
neurostimulator
• Connected to 2 leads
(depth and/or
subdural) with 4
electrode contacts each
Figure 3 The NeuroPace Responsive Neurostimulator (RNS®)
System (Mountain View, CA)
Stacey WC and Litt B (2008) Technology Insight: neuroengineering and epilepsy—designing
devices for seizure control Nat Clin Pract Neurol 10.1038/ncpneuro0750
THE RNS® SYSTEM: RESPONSIVE
STIMULATION
Neurostimulator
and Leads
Remote Monitor
Programmer
Patient Data
Management System
(PDMS)
RNS® SYSTEM: RESPONSIVE
STIMULATION
• Physician identifies
electrocortico-graphic
activity to be detected
• Detection and
stimulation settings
programmed, then
adjusted as needed
• Varied seizure patterns
require individualized
settings
THE NEUROPACE RESPONSIVE NEUROSTIMULATOR
(RNS®) SYSTEM TRIAL RESULTS
191 subjects implanted and randomized 1 month after
implant to active v. sham
38% Seizure Reduction in Active Treatment
17% Seizure Reduction in Sham Treatment
Open Label followup seizure reduction
44% 1 year
53% 2 years
Heck et al, Epilepsia, 2014