A. LeBron Paige, M.D.Clinical Associate

ProfessorDepartment of Neurology

A. LeBron Paige, M.D.Clinical Associate

ProfessorDepartment of Neurology

Iowa EFA Seizure Smart Conference – 3/2/2013

Epilepsy is a medical condition that produces seizures affecting a variety of mental and physical functions. When a person has two or more unprovoked seizures, they are considered to have epilepsy.

A seizure is the result of a strong sustained surge of electrical activity (discharges) that affects part or all of the brain.

Seizures can be brief or prolonged, typically less that 3 minutes, and can cause unusual movements, sensations, thinking, or can disturb many other body functions.

Seizures can be a subtle unresponsive staring (partial), a frighteningly dramatic convulsion (generalized), or in between.

Seizures can be caused by anything that can affect the brain, such as tumors, strokes, bleeding, infection, trauma, or unknown factors.

Treatments for epilepsy include medications, surgery, diet, electrical stimulators, and other emerging options.

Epilepsy Around the World

Epilepsy is one of the world’s oldest diseases.

Worldwide, 50-65 million people are living with epilepsy

80% of all people with epilepsy live in the developing regions.

75% of affected people in these developing countries do not get the treatment they need (9 out of 10 in Africa).

The World Health Organization (WHO) estimates that 6-10 people per 1000 have active epilepsy in the developing world. (conservative)

Epilepsy accounts for 0.5% of the global burden of disease

The social stigma surrounding epilepsy worldwide is often more difficult to overcome than the seizures themselves

Epilepsy - US Incidence

200,000 new epilepsy cases diagnosed annually in the USo 50,000 new cases of Parkinson’s Disease in the US annually

67 new cases of epilepsy per 100,000 population each yearo 62.6 individuals with lung cancer per 100,000 population per year

100 individuals with first convulsion per 100,000 population each yearo 124 women with breast cancer per 100,000 population

50% of new onset epilepsy cases present w/ Generalized Sz

70% of these new cases have no apparent cause

Incidence is greater in Black and socially disadvantaged populations

Highest epilepsy incidence is in > 65 y/o & is increasing in recent years

Partial seizures predominate in patients >10 y/o

Childhood Epilepsy – US Incidence

120,000 children per year will experience their first seizureo 75,000 – 100,000 of these are < 5 y/o with febrile seizures

45,000 children will receive a diagnosis of epilepsy per year

Highest incidence found in < 2 y/o, with adult levels in those > 5y/o

Childhood epilepsy incidence has been trending down in recent years

Generalized seizures are the most common in patients < 10 y/o

Most children with seizures will not develop epilepsy

Most children with epilepsy will outgrow the condition

Most children with epilepsy are normal in every other respect

300

250

200

150

100

50

00 20 40 60 80 100

AlcoholNeonatalOther provokedEpilepsySingle Total

Age

Inci

denc

e P

er 1

00,0

00 P

atie

nt Y

ears

l 4):S6-S14.

Complex 23%

Other partial

7%

Myoclonic 7%

Unknown/multiple

9%

Simple partial

11%

Other generalized

11%

Absence 13%

Complex partial

39%

Myoclonic 2%

Other generalized

4%

Other partial

9%

Simple partial

21%Tonic-clonic

25% Tonic-clonic

19%

Children <15 Years Adults 35-64 Years

Idiopathic 65.5%

Vascular 10.9%

Congenital 8.0%

Trauma 5.5%

Neoplastic 4.1%

Degenerative 3.5%

Infectious 2.5 %

Epilepsy in the Elderly

Stefan, H. Epilepsy in the Elderly, Acta Neurol Scand., 124: 223-237: 2011.

ELDERLY (>65 y/o) 18 y/o < ADULTS < 65 y/o

Epilepsy Prevalence - US

2.7 - 3 million active epilepsy patients in the US (~1% of the population)

30,000 Iowans carry a diagnosis of epilepsy

1% of Americans aged birth to 20 y/o have a diagnosis of epilepsy

3% of Americans aged birth to 75 y/o have a diagnosis of epilepsy

10% of Americans aged birth to 80 y/o have had at least one seizure

There are more Americans living Epilepsy than MS, Cerebral Palsy, Muscular Dystrophy, Parkinson’s Disease, ALS, and Tourette’s syndrome combined.

Of all neurological Dz, only Migraine & Stroke is more prevalent than Epi

70% of people with epilepsy are controlled (> 5yr) on medications

Overall almost 10 % of new patients fail to gain control of seizures despite optimal medical management.

• 25.8% of children with mental retardation

• 13% of children with cerebral palsy

• 50% of children with both disabilities

• 10% of Alzheimer patients

• 22% of stroke patients

• 8.7% of children of mothers with epilepsy

• 2.4% of children of fathers with epilepsy

• 33% of people who have had a single, unprovoked seizure

The basic underlying risk of developing epilepsy is about 1%, but individuals in certain populations are at higher risk.

Seizure Freedom by Seizure Type

One year seizure free rates in patients treated with medications alone.

Epilepsy Mortality

Cause of death among 3340 Epilepsy patients over 30 years

Cause of Death Proportion of all Deaths

Non-CNS Tumors 23%

CNS Tumors 5%

Heart Disease 26%

Stroke 15%

Epilepsy 7%

Trauma 9%

Respiratory Disease 3%

Pneumonia 1%

Other 11%

Epilepsy Mortality - SUDEP

Sudden, Unexpected, Death in Epilepsy1. Decedent has diagnosis of epilepsy2. Death is Sudden3. Death is Unexpected4. Death is not the result of trauma5. Death is not the result of drowning6. No evidence that death was associated with status epilepticus7. No structural or toxicological cause on post-mortem examination

Factors that increase the risk of SUDEP1. Young adulthood (children at low risk)2. Early epilepsy onset & long duration3. Poorly controlled seizures4. Poor medication compliance5. Unsupervised sleep environment (prone position)6. Generalized convulsion > Complex partial seizures >> myoclonic or absence7. Epilepsy Surgical Candidates

16

SUDEP Incidence by Group

Information Sources

The Institute of Medicine (NAS), Epilepsy Across the Spectrum, 2012

The Epilepsy Foundation of America [http://www.epilepsyfoundation.org/]

The World Health Organization (WHO) [http://www.who.int/research/en/]

National Cancer Institute [http://seer.cancer.gov/statfacts/html/all.html]

Neurological disorders: public health challenges, World Health Organization, 2006

Hauser WA, Annegers JF, Kurland LT. Incidence of epilepsy and unprovoked seizures in Rochester, Minnesota: 1935-1984. Epilepsia. 1993;34:453-468.

D. Hirtz, D. J. Thurman, K. Gwinn-Hardy, et al., How common are the ''common'' neurologic disorders? Neurology 2007;68;326.

Ficker, DM, Sudden Unexplained Death and Injury in Epilepsy, Epilepsia, (Supp1.2):S7-S12, 2000

Thomson, T. , Sudden unexpected death in epilepsy: current knowledge & future directions. The Lancet Neurology, Sept. 2008

Stefan, H. Epilepsy in the Elderly, Acta Neurol Scand., 124: 223-237: 2011.

Trinka, E, et al., Cause-specific mortality among patients with epilepsy: Results from a 30-year cohort study, Epilepsia, ** (*):1–7, 2012

Epilepsy Discrimination

The Code of Hammurabi (1780 B.C.) established that a person with epilepsy could not marry, testify in court, and voided slave purchases

In both China and India, epilepsy is commonly viewed as a reason for prohibiting or annulling marriages.

In the United Kingdom, a law forbidding people with epilepsy to marry was repealed only in 1970.

In the United States, until 1956 it was illegal in 17 states for people with epilepsy to marry, and into the 1970s it was legal to deny them access to restaurants, theatres, rec centers and other public buildings.

1973 brought the first limited US laws to prohibit discrimination, but not until 1990 with the Americans with Disabilities Act was there uniform protection against discrimination.

PMD

General Neurologist(s)

Level-4Epilepsy Center

Spells are Likely Seizures

Seizures are Refractory to two Medications

Epilepsy Care Pathway

Patient has “Spells”

Level-4 Epilepsy Center•Comprehensive Diagnostic Capability

•New & Experimental Medications

•Epilepsy Surgery Experience

•Implantable Stimulators

•Radiation treatment vs. Surgery

•Access to National Epilepsy Expertise

National Association of Epilepsy Centers

22Englot, DJ, et al., Epilepsy Surgery Trends in the United States 1990-2008, Neurology, 78;1200 (2012)