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North American Commission Symposium

Epilepsy Classification:
Hot Controversies in 2012
Symposium Co-Chairs:
Ingrid E Scheffer, MBBS, PhD, FRACP
The Florey Institute
University of Melbourne
Australia
and
Sheryl Haut, M.D.
Montefiore Medical Center
Bronx, NY
Tuesday, December 4, 2012
Convention Center Ballroom 6A, Upper Level
8:30 am 10:00 am

OVERVIEW
Classification of the epilepsies is a dynamic concept that continues to undergo reevaluation, especially
in light of advances in structural and functional neuro-imaging, genetics and neuro-immunology. This
symposium, sponsored by the International League Against Epilepsy, will focus on the newly updated
organization of the epilepsies, exploring the emerging concept of diagnostic specificity and how this
relates to clinical practice. Controversies that have arisen regarding the specific aspects of
classification, namely structural, genetic and immune, will be presented.
LEARNER OUTCOMES
x Utilize the greater diagnostic specificity provided by the revised classification in managing
patients and in doing research
x Utilize newly described genetic and immunologic testing in order to provide greater
specificity in diagnosing epilepsy and in managing patients
x Utilize the revised classification to improve diagnostic specificity and coding accuracy for
clinical epilepsy practice.
TARGET AUDIENCE
Basic: Those new to epilepsy treatment or whose background is limited, e.g., students, residents,
general physicians, general neurologists and neurosurgeons, other professionals in epilepsy care,
administrators.
Intermediate: Epilepsy fellows, epileptologists, epilepsy neurosurgeons mid-level providers with
experience in epilepsy care (e.g., advanced practice nurses, nurses, physician assistants),
neuropsychologists, psychiatrists, basic and translational researchers.
Advanced: Symposium will address highly technical or complex topics (e.g., neurophysiology,
advanced imaging techniques, advanced treatment modalities, including surgery)
AGENDA

8:30 8:35 am

Introduction and Overview


Sheryl Haut, M.D.

8:35 8:45 am

Update on the new Organization: Where Have the Modifications Taken


Us?
Ingrid E. Scheffer, Ph.D.

8:45 9:00 am

Diagnostic Specificity: Applying This Concept to Every Patient


J. Helen Cross, M.D., Ph.D.

9:00 9:10 am

Controversies: Genetic: How Do I Tell the Patient?


Sameer Zuberi, MD

9:10 9:25 am

Controversies: Genetic: Structural: Genetic or Acquired?


James Barkovich

9:25 9:40am

Immune: Which Patients Should Be Tested?


Christian Bien, M.D.

9:40 9:55 am

Coding: Will This Make a Difference to My Practice?


Donna C. Bergen, M.D.

9:55 10:00 am

Conclusions
Ingrid E. Scheffer, Ph.D.

ACCREDITATION
The American Epilepsy Society is accredited by the Accreditation Council for Continuing Medical
Education (ACCME) to provide continuing medical education for physicians.
CREDIT DESIGNATION
The American Epilepsy Society designates this educational activity for a maximum of 1.5 AMA PRA
Category 1 CreditsTM. Physicians should only claim credit commensurate with the extent of their
participation in the activity.
NURSING CREDIT
EDUPRO Resources LLC is an approved provider of continuing nursing education by Pennsylvania
State Nurses Association, an accredited approver by the American Nurses Credentialing Centers
Commission on Accreditation, Provider number P208-8/08-11.
EDUPRO is also an approved provider by the California Board of Registered Nursing, Provider number
CEP-14387.
Disclaimer: Accreditation refers to educational content only and does not imply endorsement of
products by PSNA, ANCC, CBRN, or EDUPRO Resources LLC.
Nurses may claim up to 1.5 contact hours for this session.
PHARMACY ACCREDITATION INFORMATION
Projects In Knowledge is accredited by the Accreditation Council for Pharmacy Education as
a provider of continuing pharmacy education.
This knowledge-based activity provides up to 1.5 contact hours. Following attendance, completion of
the activity evaluation and verification of attendance, participants will be provided an electronic
statement of credit.
ACPE Universal Activity Number (UAN) is 0052-9999-12-2326-L04-P and provides 1.5 contact hours.
International Credits: The American Medical Association has determined that non-U.S. licensed
physicians who participate in this CME activity are eligible for AMA PRA Category 1 CreditTM.
ABPN Core Competencies
The American Board of Psychiatry and Neurology has reviewed the North American Commission and
has approved this program as part of a comprehensive lifelong learning program, which is mandated
by the ABMS as a necessary component of maintenance of certification.
Core Competencies: Medical Knowledge, System-Based Practice, and Practice-Based Learning and
Improvement
FACULTY/PLANNER DISCLOSURES

It is the policy of the AES to make disclosures of financial relationships of faculty, planners and staff
involved in the development of educational content transparent to learners. All faculty participating in
continuing medical education activities are expected to disclose to the program audience (1) any real
or apparent conflict(s) of interest related to the content of their presentation and (2) discussions of
unlabeled or unapproved uses of drugs or medical devices. AES carefully reviews reported conflicts of
interest (COI) and resolves those conflicts by having an independent reviewer from the Council on
Education validate the content of all presentations for fair balance, scientific objectivity, and the
absence of commercial bias. The American Epilepsy Society adheres to the ACCMEs Essential Areas
and Elements regarding industry support of continuing medical education; disclosure by faculty of
commercial relationships, if any, and discussions of unlabeled or unapproved uses will be made.
FACULTY / PLANNER BIO AND DISCLOSURES
Ingrid Scheffer, M.B.B.S., Ph.D., FRACP (Co-Chair)
Ingrid Scheffer is a paediatric epileptologist and Senior Principal Research Fellow at the Florey
Institute. She holds a Chair at the University of Melbourne, Austin Health and Royal Children's
Hospital, Melbourne, Australia. Her major interests are epilepsy syndrome classification and the
genetics of the epilepsies. In recent years, she has focused on the epileptic encephalopathies and all
genetic forms of epilepsy. She is currently the chair of the ILAE Commission for Classification and
Terminology.
Ingrid Scheffer, M.B.B.S., Ph.D., has nothing to disclose.
Sheryl Haut, M.D. (Co-Chair)
Dr. Sheryl Haut is Director of the Montefiore-Einstein Adult Epilepsy Program and Director of
Neurology Residency Training at Albert Einstein College of Medicine. She is also the Chair of the North
American Commission of the International League Against Epilepsy. Her research interests include: the
temporal distribution of seizures, with emphasis on seizure clustering; seizure prediction and preemption; and alternative therapies for epilepsy. Dr. Haut has a Masters in Clinical Research Methods,
and completed a K23 career development award from the NIH. She maintains an active adult epilepsy
practice at Montefiore Medical Center, Bronx
NY.
Sheryl Haut, M.D. discloses receiving support as Consulting/Advisory Board Activity from MAP
Pharmaceuticals; Vivus; Neuronex; Upsher Smith.
James Barkovich, M.D.
Dr. Barkovich is Chief of Pediatric Neuroradiology at UC San Francisco. He is a neuroradiologist
whose clinical specialties are pediatric neuroradiology and epilepsy imaging. Dr. Barkovich's
research interests include normal brain development and disruptions of normal developmenta l
processes including genetic causes, disruptions with resultant malformations of the brain, and
perinatal/neonatal neonatal brain injury. In addition, he helps to develop and use novel imaging
techniques to detect developmental and acquired causes of epilepsy and abnormal
neurodevelopment.
James Barkovich, M.D. has nothing to disclose.
Donna Bergen, M.D.
I am Professor of Neurological Sciences at Rush University, working mainly in clinical epileptology. I
have a special interest in issues of provision of care, and in drug development. For the past several
years I have served on the World Health Organisation's Task Force Advisory Group in Neurology, for
the revision of the ICD-10 codes.

Donna Bergen, M.D. discloses receiving support as Research Funding from For Profit Commercial
Sources/Principle Investigator from Medtronic, therapeutic trial.
Christian Bien, M.D.
Dr. Christian G. Bien is (since Januar 2011) Clinical Director of Krankenhaus Mara, Epilepsy Center
Bethel, Bielefeld/Germany. He obtained his Medical Degree from the Free University of
Berlin/Germany and completed his neurology training at the Medical Center of the University of
Bonn/Germany. His main fields of clinical and research activities are presurgical assessment of
pharmacoresistant epilepsy patients chronic immune-mediated brain disorders as a cause of seizure
disorders. He runs a lab for determination of antineural antibodies at his hospital.
Christian Bien, M.D. discloses receiving support as Speakers Bureau Member (supported by for-profit
entities) from Eisai, UCB, GlaxoSmithKline and Desitin (all Germany).; as Consulting/Advisory Board
Activity from Eisai and UCB, both Germany
J. Helen Cross, M.D., ChB, Ph.D.
Professor Helen Cross is The Prince of Waless Chair of Childhood Epilepsy and Honorary Consultant
in Paediatric Neurology at UCL-Institute of Child Health, Great Ormond Street Hospital for Children
NHS Trust, London and Young Epilepsy, Lingfield, UK. She currently sits on the ILAE Commission for
European Affairs., and has recently been elected as ILAE Secretary General (from 2013). In 2007 she
was awarded an Ambassador for Epilepsy Award by the ILAE. Her research interests include aetiology
of the epilepsies, as well as the role of early surgical intervention and other new treatments in
childhood epilepsy.
J. Helen Cross, M.D., ChB, Ph.D. discloses receiving support as Consulting/Advisory Board Activity
from Advisory Board for Eisai and Viropharma, honoraria paid to department; as Honoraria from
Commercial Sources from Viropharma; paid to department.
Sameer Zuberi, M.D.
Sameer M Zuberi is Consultant Paediatric Neurologist at the Royal Hospital for Sick Children,
Glasgow, UK. He is Clinical Lead of the Glasgow Epilepsy Genetics Service and Paediatric
Neurosciences Research Group. He is Education Officer of the ILAE Commission for Classification and
sits on the Board of the European Paediatric Neurology Society. His clinical and research interests
include epilepsy genetics (particularly impact on clinical care), differential diagnosis and the
channelopathies.
Sameer Zuberi, M.D. has nothing to disclose.
Paul Levisohn (Medical Content Specialist)
Dr. Levisohn is Associate Professor of Pediatrics and Neurology at the University of Colorado School
of Medicine and Childrens Hospital Colorado. He is former medical director of the Epilepsy Monitoring
Unit at The Children's Hospital. He has served as chair of the AES Practice Committee, is co-chair of
the advisory committee for the National Center for Project Access at the Epilepsy Foundation and is a
member of the EF Professional Advisory Board. He currently serves as consultant to AES on medical
content of AES continuing medical education activities.
Paul Levisohn, M.D. has nothing to disclose.
Ajay Gupta, MD (Liaison Reviewer)
Ajay Gupta, M.D., is Professional Staff in the Epilepsy Center/Neurological Institute at The Cleveland
Clinic Foundation. He is Associate Professor at The Cleveland Clinic Lerner College of Medicine, Case
Western Reserve University. He is also the founder director of multidisciplinary Tuberous Sclerosis

Program at the Cleveland Clinic. Dr. Gupta is an expert in the field of childhood and adolescent
epilepsy with emphasis on epilepsy surgery evaluation and intra-operative monitoring. He has authored
more than 75 original research articles and review papers in the peer-reviewed international journals.
Ajay Gupta, M.D. discloses receiving support as Salary from Commercial Sources generating W-2 from
Cleveland Clinic; as Speakers Bureau Member (supported by for-profit entities) from Lundbeck Inc.
DISCLAIMER
Opinions expressed with regard to unapproved uses of products are solely those of the faculty and are
not endorsed by the American Epilepsy Society or any manufacturers of pharmaceuticals.
MEDICAL EDUCATION EVALUATOR AND CERTIFICATES
The Medical Education Evaluator is an online system allows any attendee to self-manage the process
of completing course evaluations, tracking credits and printing out the appropriate certificate for either
AMA PRA Category 1 CreditsTM, CE or ACPE pharmacy statement of credits.
Log on to the Evaluator via the AES website at www.aesnet.org. Once you are on the Evaluator, you
will be asked to enter your MyAES ID # and password. The certificate(s) are saved to your personal
account page which is cumulative. You may print the certificate(s) in PDF format at any time.
To help support this process, AES members who want CME will be asked to pay $35 before January
18 and $50 between January 19 and February 28. Non-AES attendees who want CME will be asked to
pay $50 before January 18 and $75 between January 19 and February 28.
The online Evaluator will be left open through February 28, 2013. You must complete the
evaluations and credit tracking by that date.
By completing this information online, attendees greatly assist the Council on Education and Annual
Meeting Committee with important needs assessment data whereby the AES can further plan and
address educational gaps to meet the needs of our learners.
A meeting attendance certificate will be available for international meeting attendees at the registration
desk.
SYLLABUS
Syllabi for the educational symposia are available to print in the AES Virtual Tote Bag. Paper handouts
will not be provided on site.

11/13/2012

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Genetic/Structural/Metabolic
Causes
Genetic

LM'.2:8JF(7.2.(;'260;6(0=42N0;2K(;K2.?O(.K0'0
4='C6?'C0<,

Sodium/Postassium/NMDA receptor mutations


Many metabolic disorders
Many malformations

Structural
Genetic malformations (hemimegalencephaly, cobblestone
cortex, heterotopia, some PMG, some FCDs, etc)
Acquired malformations (infection, trauma, ischemia, seizures)

Metabolic
Can cause primary (PMG in peroxisomal disorders) or
secondary (e.g., from hyperammonemia) structural abnormalities
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Age of baby at time of scan (FCD difficult to detect 518 months of age)
Scanner (magnetic field and gradient) strength
Sequences obtained
Section thickness (thin with reformats is best)
Signal to noise
Expertise of interpreter (You see what you look for,
you look for what you know)

Inadequate MRIs must be repeated (often


with anesthesia)!
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10 yo with
ith
refractory
Epilepsy
G-W
Blurring

G-W blurring
due to incomplete
myelination.
Try IR or wait until
24-30 months.

Dx: HME

22 year old woman


Mature PMG

Focal Polymicrogyria: 3 mo with seizures

5 yo girl
IQ ~50
Normal neuro exam
Epilepsy (gen) x 1 yr
Reelin pathway
defect: Pachygyria

High resolution to distinguish PMG from Pachygyria.


Currently MRI cannot ddx genetic PMG from acquired

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Typical

Mild

5 month old with seizures 2 birth


injury

Band Heterotopia due to DCX mutations

16 mo with
Refractory Rt
Frontal Seizures
Absent subcortical
Myelin reduced
Myelin,
FDG uptake.
Dx: FCDI

12 yo with refractory sz from left frontal


FCD I

9 yo with
refractory
epilepsy
localized to right
parietal lobe
Dx: FCDIIa
Blurring of GWJ

FCD IIb
Transmantle Sign
Specific MRI sign

!!"!#"$%!$

Frontal Lobe: FCD2a


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Magnetic Source Imaging (MSI)

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MEG interictal spike foci

coregistered with axial SPGR images

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10 year old boy with partial complex epilepsy


FCD IIIa (FCD and MTS)

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=8JF
R24;4:8J@C'(;240(4C<U=42/';.V4?6((.K0'0X

2 year old girl with seizures, dev delay

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