treatment algorithms in the diagnosis and treatment of
TRANSCRIPT
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