Treatment Algorithms in the Diagnosis and Treatment of Epilepsy

Discussing SUDEP November 30, 2012

Jeffrey Buchhalter MD, PhD, FAAN

Professor of Pediatrics & Clinical Neurosciences

University of Calgary Faculty of Medicine

Alberta Children’s Hospital

American Epilepsy Society | Annual Meeting

Disclosure

Name of Commercial Interest

American Epilepsy Society | Annual Meeting 2012

Type of Financial Relationship

None relevant to this presentation

Learning Objectives

• Be prepared to discuss SUDEP with

patients/families utilizing knowledge of indications

for discussion

• Frame the discussion of SUDEP based upon your

knowledge of patient/family risks and desire to

know

American Epilepsy Society | Annual Meeting 2012

Impact on Clinical Care and Practice

• Physicians will be more aware of which groups are at greatest risk of dying due to SUDEP

• Families will be able to discuss this greatest of all fears with their physician

Intended Audience

Epilepsy specialists

Those involved with clinical care of people with epilepsy

Doctors, nurses, counselors, social workers…

First seizure clinics

AED oriented practices

Epilepsy monitoring unit practices

What are the goals?

Knowledge of risk

Compliance with medication

More aggressive treatment of refractory seizures

Reassurance that risk is low

Barriers to Discussion

Doctors’ perception of what patients want

The time available for discussion

The other resources available

Cultural issues

When to Discuss*

When asked directly by the patient/family

When concerned about compliance

When “sense” an un-addressed fear

As part of the general education of all people living with epilepsy

* NOT “IF”

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Creating an Algorithm

Potential factors in the algorithm

New onset Intractable

Child Adult

Seizure type Seizure frequency

Low risk High risk

Diurnal seizures Diurnal seizures

DEFINITIONS

What is SUDEP?

SUDEP Definition

Annegers

1. Patient suffered from epilepsy

2. Death occurred suddenly

3. Patient died unexpectedly, while in reasonable state of health

4. Death occurred during normal activities

5. No determinable cause of death

6. Death was not directly caused by a seizure or status epilepticus

Annegers. Epilepsia 1997 (Suppl 11): S9-12

SUDEP Definition Annegers

Definite SUDEP

Meet all 6 criteria after autopsy

Probable SUDEP

Meet all 6 criteria but no autopsy

Possible SUDEP

Cases where SUDEP cannot be ruled out, but with insufficient evidence regarding circumstances and no autopsy

Annegers. Epilepsia 1997 (Suppl 11): S9-12

SUDEP Definition Nashef

“… sudden unexpected, non-traumatic and

non-drowning death in an individual with

epilepsy with or without evidence for a

seizure and excluding documented status

epilepticus where post-mortem examination

does not reveal a cause for death”

Epilepsia 1997;38(S11):56-8

SUDEP Definition

The role of autopsy confirmation is the most

problematic issue for research studies of

SUDEP

“Unifying the definitions of SUDEP”

Key Features

Specify with or without witnessed seizure

Specify with or without autopsy

Include “suffocation”

Include “dry drowning”

Specify known competing causes

1 hr arbitrarily selected as time from terminal event

Nashef, So, Ryvlin, and Tomson. Epilepsia, 53(2):227–233, 2012

Patient-years

• Allows looking at a population, rather than an

individual

• PY = total of all yrs that all patients were followed /

number of events of interest

– e.g. 10 patients followed for 10 yrs each = 100 pt yrs

– 1 case of cancer = 1 case per 100 pt years

Community-based studies of SUDEP

0.9 – 2.3 per 1000 person-years

Tomson T, et al. Lancet 2008

Tomson T, et al. Lancet 2008

“Long-Term Mortality in Childhood-Onset Epilepsy”

Prospective

Population-based

40 year follow-up (since 1964)

245 individuals

Sillanpaa & Shinnar. N Engl J Med 2010;363:2522-9

“Long-Term Mortality in Childhood-Onset Epilepsy”

Sillanpaa & Shinnar. N Engl J Med 2010;363:2522-9

“Long-Term Mortality in Childhood-Onset Epilepsy”

Sillanpaa & Shinnar. N Engl J Med 2010;363:2522-9

“Long-Term Mortality in Childhood-Onset Epilepsy”

Summary

Rate of death 3x greater than gen population

7% life-time risk of SUDEP

48% of all deaths not in remission

Idiopathic : Symptomatic = 12% vs 37%

33 of 60 (55%) related to epilepsy

SUDEP 30%

Seizure 15%

Drowning 10%

Sillanpaa & Shinnar. N Engl J Med 2010;363:2522-9

Two pediatric studies

Donner et al, Ontario, Neurology, 2001

27 cases, all with autopsies

Symptomatic 52%, cryptogenic 18%, idiopathic,

30% idiopathic. All with GTCs

No relationship to number or level of AEDs

Camfields, Nova Scotia, Sem Ped Neurol, 2005

629 children with epilepsy

Neuro normal- no increased risk of death, 1/4 SUDEP

Neuro abnormal- 22x higher than gen pop, 1/22

SUDEP- risk among children

Victoria, Australia 0.36 per 1000

Ontario, Canada 0.2

Switzerland 0.3

Nova Scotia, Canada 0.11

Tellez-Zento Epilepsy Res 2005;65:101-115

SUDEP- Associated Seizure Types

History of GTCs (88-100% in case series)

> 3 increased risk by 8 fold

Complex partial seizures (reported, rare)

Absence & myoclonic only (no reports)

Langan et al. Neurology 2005:64;1131-33

Walzack et al. Neurology 2001:56;519-25

*Hitiris et al. Epilepsy & Behavior 2007:10:138-41

What providers do &

What families want

Association of British Neurologists Survey

National Institute for Clinical Excellence guidelines,

2004

“...should be given information on SUDEP”

387 of 738 questionnaires returned

Open-ended questions

Morton, Richardson & Duncan. (2005). Sudden unexpected death in epilepsy: don’t

ask, don’t tell? J Neurol Neurosurg Psychiatry 77, 199-202. Modified from Tess Sierzant

N

%

Discuss SUDEP with all

patients

18 4.7

Discuss with majority of

patients

99 25.6

Discuss with very few of

my patients

237 61.2

Discuss with none of my

patients

29 7.5

Total number of

respondents

383 100

Morton (2006) Table 1: Analysis of responses

from medical personnel

Informing patients about sudden unexpected death in epilepsy: A survey of specialist nurses

250 postal questionnaires to Epilepsy Nurse

Association, 58% returned

Lewis, Higgins. Brit J Neurosci Nursing 2008; 4:30-34

Discussed with all

6%

Discussed with majority 50%

Discussed with few

37%

Epilepsia 1-6, 2010

Two questionnaires

- Parents/guardians of children at pediatric epilepsy

clinic

100 given- 67 (1st), 47 (2nd)

- Physicians, UK based pediatric neurologists

71 mailed, 45 (56%) returned

Results

“The majority (74%) of pediatric neurologists provided SUDEP information only to a select group of children with epilepsy and were uncertain about the effect such information would have upon the parent and child. Conversely, 91% of parents expected the pediatric neurologist to provide SUDEP risk information. The provision of this information did not have a significant immediate and longer-term negative impact.” 67% of parents wanted the information at the time of dx

Gayatri et al, 2010

Impact of SUDEP disclosure

Gayatri et al, 2010

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Algorithm for Low Risk Group

Going to discuss as part of general education

Specific intent: reassurance

Timing: at the first office visit if time allows

Repetition: none unless requested

Algorithm for High Risk Group

Going to discuss as part of general education

Specific intent: compliance (medication, surgery,

device, diet)

Timing: at the first office visit

Repetition: PRN compliance & seizure control

Thank-you for your attention

Epilepsy Specialist Symposium Treatment Algorithms in the Diagnosis

and Treatment of Epilepsy

Conclusions Fred Lado, MD, Chair

Montefiore Medical Center

Albert Einstein College of Medicine

Bronx, NY

American Epilepsy Society | Annual Meeting

Questions?

American Epilepsy Society | Annual Meeting 2012

Thank you!

American Epilepsy Society | Annual Meeting 2012