Harden C, Tomson T, Gloss D, Buchhalter J, Cross JH, Donner
E, French JA, Gil-Nagel A, Hesdorffer DC, Smithson WH, Spitz MC, Walczak
TS, Sander JW, Ryvlin P. Practice guideline summary: Sudden unexpected death in
epilepsy incidence rates and risk factors: Report of the Guideline Development,
Dissemination, and Implementation Subcommittee of the American Academy of
Neurology and the American Epilepsy Society. Neurology. 2017 Apr 25;88(17):1674-1680.
Abstract
OBJECTIVE:
To determine the incidence rates of sudden unexpected death
in epilepsy (SUDEP) in different epilepsy populations and address the question
of whether risk factors for SUDEP have been identified.
METHODS:
Systematic review of evidence; modified Grading
Recommendations Assessment, Development, and Evaluation process for developing
conclusions; recommendations developed by consensus.
RESULTS:
Findings for incidence rates based on 12 Class I studies
include the following: SUDEP risk in children with epilepsy (aged 0-17 years)
is 0.22/1,000 patient-years (95% confidence interval [CI] 0.16-0.31) (moderate
confidence in evidence). SUDEP risk increases in adults to 1.2/1,000
patient-years (95% CI 0.64-2.32) (low confidence in evidence). The major risk
factor for SUDEP is the occurrence of generalized tonic-clonic seizures (GTCS);
the SUDEP risk increases in association with increasing frequency of GTCS
occurrence (high confidence in evidence).
RECOMMENDATIONS:
Level B: Clinicians caring for young children with epilepsy
should inform parents/guardians that in 1 year, SUDEP typically affects 1 in
4,500 children; therefore, 4,499 of 4,500 children will not be affected.
Clinicians should inform adult patients with epilepsy that SUDEP typically
affects 1 in 1,000 adults with epilepsy per year; therefore, annually 999 of
1,000 adults will not be affected. For persons with epilepsy who continue to
experience GTCS, clinicians should continue to actively manage epilepsy
therapies to reduce seizures and SUDEP risk while incorporating patient
preferences and weighing the risks and benefits of any new approach. Clinicians
should inform persons with epilepsy that seizure freedom, particularly freedom
from GTCS, is strongly associated with decreased SUDEP risk.
A new practice guideline on sudden unexpected death in
epilepsy (SUDEP) has been released by the American Academy of Neurology (AAN)
and the American Epilepsy Society (AES).
For the document, the writing committee sought to establish incidence
rates of SUDEP and identify any risk factors.
"We found that SUDEP is relatively rare in children,
affecting 1 in 4500 children with epilepsy per year; that's according to
moderate evidence," said coauthor Elizabeth Donner, MD, Division of Neurology,
Department of Pediatrics, The Hospital for Sick Children, University of
Toronto, Ontario, Canada, and chair of the AES SUDEP Task Force.
SUDEP was more common in adults than in children, typically
affecting 1 in 1000 adults living with epilepsy per year, Dr Donner said.
The major risk factor was generalized tonic-clonic seizures.
"People with three or more of this type of seizure…were 15 times more
likely to die suddenly than people who did not have this seizure type, so
that's a very significant finding," Dr Donner said. "In total, this
translates to up to 18 in 1000 deaths per year for people with epilepsy with
frequent generalized tonic-clonic seizures."
"Our guideline brings clarity to the discussion, giving
healthcare providers information they can use to help people with epilepsy
reduce their risk," said lead author, Cynthia Harden, MD, Department of
Neurology, Mount Sinai Health System, New York, New York, during a press
conference here.
The guideline was co-developed by AAN and AES, and is
endorsed by the International Child Neurology Association. It is published
online April 24 in Neurology and was presented during a press conference here
at the American Academy of Neurology 2017 Annual Meeting (AAN)…
A previous study showed that patients and their families
prefer to be informed of individual risk for an event like SUDEP, they note,
but after being informed of an adverse event, they tend to overestimate the
risk for that adverse event affecting them.
"Overestimation can be lessened by presenting the risk
as a probability of both having and not having the event, and by using numbers
in addition to words and frequencies rather than percentages to convey the
risk," the authors write.
Accordingly, they suggest some language physicians may use
to inform patients in a balanced way.
"Clinicians caring for young children with epilepsy
should inform parents/guardians that in 1 year, SUDEP typically affects 1 in
4,500 children with epilepsy; therefore, annually 4,499 of 4,500 children will
not be affected," they write (Level B).
Similarly, "Clinicians should inform adult patients
with epilepsy that SUDEP typically affects 1 in 1,000 adults with epilepsy per
year; therefore, annually 999 of 1,000 adults will not be affected (Level
B)."…
The major risk factors they found were the presence and
frequency of generalized tonic-clonic seizure (GTCS). People with three or more
of these seizures per year had a 15-fold increased SUDEP risk.
"The large SUDEP risk increase from GTCS, coupled with
epilepsy monitoring unit evidence demonstrating that a GTCS was always the
precipitating event of SUDEP, strongly suggests that GTCS are not just
associated with SUDEP but, rather, are in the causal path to SUDEP," the
authors note. "From this, it seems reasonable to infer that improved
control of an individual's GTCS will result in a reduced risk of SUDEP. Thus, a
reduction in SUDEP risk is an additional benefit to the many benefits resulting
from improved seizure control."…
In terms of recommendations, then, they suggest that for
patients who continue to have GTCS, "clinicians should continue to
actively manage epilepsy therapies to reduce seizures and SUDEP risk while
incorporating patient preferences and weighing the risks and benefits of the
new approach."
They also suggest that clinicians inform their patients that
seizure freedom, "particularly freedom from GTCS (which is more likely to
occur with medication adherence), is strongly associated with a decreased SUDEP
risk."…
Children with Dravet syndrome have an unusually high risk
for SUDEP, they add. "Mutations in sodium channel gene SCN1A are the most
common cause of Dravet syndrome and this gene is expressed in brain and heart.
It is possible that these mutations render patients more susceptible to cardiac
arrhythmias in the setting of a seizure.
http://www.medscape.com/viewarticle/879029?
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