Mistakes in Epilepsy Mistakes in Epilepsy CareCare

Orrin Devinsky, M.D.NYU Epilepsy Center

What do NBA coaches, What do NBA coaches, mothers and doctors have mothers and doctors have in common?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 syncopeNonepileptic psychogenic

seizures Failure to consider diagnostic changes

Missing The Big Missing The Big Picture Picture

Focus on person, not diagnosis Listen, beyond the words to feelings See their world: situations influence health

Look patient in the eyes Speak with family and friends

Therapies are limited by medical box Therapists - cognitive, psychological, etc Pragmatic approaches (sometimes key!)

Compliance Sleep hygiene Memory lists

Missing Mood Missing Mood Disorders Disorders

All epilepsy patients at increased risk

Patients must tell; doctors must ask – both often fail

Refractory epilepsy Greater contributor to impaired Quality of Life than seizures

Depression in up to 50% Suicidal ideation - 20% in past 6 mos

Majority are untreated

Not So Benign Not So Benign EpilepsyEpilepsy

“Benign Epilepsies” Yes or No?

Absence Epilepsy Childhood (5-9 yo) vs. Adolescent (10-14 yo)

Benign Occipital Epilepsy Benign Rolandic Epilepsy

Absence Epilespy: A Absence Epilespy: A Wolf in Sheep’s Wolf in Sheep’s

Clothing Clothing 56 Absence Epilepsy v. 61 Juvenile Rheumatoid Arthritis patients Wirrell et al, Arch Pediatr Adolesc Med 1997;151:152-158

Remission 57% of absence epilepsy and 28% of JRA patients.

Absence epilepsy - increased academic, personal, and behavioral disorders (p<.001)

Ongoing seizures - poor prognosis Instead of saying ‘everything is fine’, we need to find ways to improve outcome

Circular Reasoning: Circular Reasoning: Benign Rolandic Benign Rolandic

Epilepsy is BenignEpilepsy is Benign The “Party Line”

99% outgrown No cognitive or behavioral problems Seizures easily controlled

Reality Are thousands of spikes each night really benign?

Increased attention and language disorders

Some are not so easy to control: clusters, aggressive course, poor AED response

Sarah Sarah

Mild epilepsy; considered ‘benign’ Onset at age 4; off meds - age 9 Attention deficit disorder - age 6 Reading comprehension problems at age 8

Problems persisted after meds were stopped

Was there a missed opportunity??

Juvenile Myoclonic Juvenile Myoclonic Epilepsy (JME) is Epilepsy (JME) is Lifelong: ?Wrong!Lifelong: ?Wrong!

JME: an idiopathic (genetic) epilepsy with myoclonic as predominant seizure type; +/- absence, tonic-clonic

A lifelong disorder requiring AEDs - standard teaching in current texts

Except that it is wrong - 25 year followup: one-third are seizure free off meds with no seizures or only myoclonus (Camfield & Camfield)

The Dangers of The Dangers of Expert ConsensusExpert Consensus

MRI offers no real advantage over CT in epilepsy diagnosis - 1986

Ketogenic diet is not effective - 1990 Felbatol (felbamate) is extremely safe – 1993

Experts convince themselves, other doctors and patients

Demand evidence or humility

We get used to what We get used to what we get used towe 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

Failure to ReassessFailure to Reassess Disorders change and evolve New situational factors arise Need to keep a fresh perspective Need to cast a broad differential diagnosis and consider a broad therapeutic strategy

What was is an excellent but sometimes dead-wrong indicator of what is

Errors in Assessing Errors in Assessing RiskRisk

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

Fear of Failure: Fear of Failure: Loss AversionLoss Aversion

People are loss averse ~ 2:1 ratio, irrationally avoid loss - neuroeconomics

People value what they have more than what they don’t have – Duke tickets Medications? Seizure control? The devil you know…

Doctors like to add medicines more than they like to take them away The gabapentin story

Failure to Understand Failure to Understand FramingFraming

“Surgery is 99.95% safe” is very different than “Someone died from surgery” or “1 in 1500 die”. Substitute benign brain tumor for epilepsy surgery

Mentally invert presentations to better understand pros and cons

Patients must trust their doctors, but they must also assess their doctor’s bias

The neurosurgeon, the radiation oncologist & the neuro-oncologist

Doctors and Patients Move Doctors and Patients Move in Packsin Packs

Doctors are influenced by peers, thought leaders, marketing – they are as susceptible to status quo, texts (eg, JME, absence) framing as are patients

Doctors in different medical centers, cities, and regions have different practices

Patients strongly influence each other – support groups, internet, etc

Humans are Anecdote Humans are Anecdote DrivenDriven

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 Humans are Anecdote DrivenDriven

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 Failure to Understand NumbersUnderstand 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 often makes the politically correct choice, not the best patient care choice

Failure to be HumbleFailure to be Humble

Most people don’t enjoy admitting that they don’t know something

Doctors are expected to have answers, to have therapies, and if they are honest, people go to other doctors or alternative therapists – catch 22

Tell a white lie or admit ignorance?

Common Errors in Common Errors in TherapyTherapy

Wrong diagnosis Wrong medication selection Failure to use medications systematically Start low, go slow Consider time of doses v. seizure

Benign Rolandic Epielspy Consider strategies to reduce side effects

For dizziness – oxcarbazepine (Trileptal) after solid breakfast, not empty stomach

Failure to document changes carefully Nonadherence (noncompliance)

Fatigue: Diagnosis Fatigue: Diagnosis and Causation and Causation

Premature exhaustion in mental or physical activities, weariness, lack of energy

Common in epilepsy patients AEDs Other drugs (eg, psychiatric drugs) Seizures

Epilepsy wave activity Depression Sleep disorders

Two Great Lies in Two Great Lies in EpilepsyEpilepsy

Seizures don’t hurt the brainThey cause structural and functional impairment that can progress over time

Seizures are never fatalSUDEP

0 25 50 75 100 125 150 175 200

Annual incidence per 10,000 population

Sudden Unexplained Sudden Unexplained Death Death

in Epilepsy (SUDEP)in Epilepsy (SUDEP)General population (2–3)

Epilepsy incidence population (5)

Epilepsy prevalence population (7)

Patients in clinical trials (30–50)

Patients undergoing vagus nerve stimulation (41)

Patients referred to epilepsy centers (50–60)

Surgical candidates (90)

Surgical failures (150)

QOL: QOL: A Different ViewA Different View

QOL - Defined by patient not MD Should patient’s perspective be filtered through “objective medical lens”? - NO

QOL is about listening, changing perspective, and using the patients’ view as ultimate measure of outcome

QOL: Clinical QOL: Clinical RelevanceRelevance

QOL issues most relevant to chronic disorders, problems beyond disease symptoms

Hypertenstion – -blockers v. ACE inhibitors (Experts wrong!)

Epilepsy is a paradigm of a QOL disorders: seizures are infrequent, AED effects, comorbid disorders (depression, migraine) & psychosocial problems are often chronic

Stay Focused Stay Focused PositivelyPositively

Learn to reduce Learn to reduce stressstress

Use your mindUse your mindExerciseExercise