Verducci C, Hussain F, Donner E, Moseley BD, Buchhalter J,
Hesdorffer D, Friedman D, Devinsky O. SUDEP in the North American SUDEP
Registry: The full spectrum of epilepsies. Neurology. 2019 Jun 19. pii:10.1212/WNL.0000000000007778. doi:
10.1212/WNL.0000000000007778. [Epub ahead of print]
Abstract
OBJECTIVE:
To obtain medical records, family interviews, and
death-related reports of sudden unexpected death in epilepsy (SUDEP) cases to
better understand SUDEP.
METHODS:
All cases referred to the North American SUDEP Registry
(NASR) between October 2011 and June 2018 were reviewed; cause of death was
determined by consensus review. Available medical records, death scene
investigation reports, autopsy reports, and next-of-kin interviews were
reviewed for all cases of SUDEP. Seizure type, EEG, MRI, and SUDEP
classification were adjudicated by 2 epileptologists.
RESULTS:
There were 237 definite and probable cases of SUDEP among
530 NASR participants. SUDEP decedents had a median age of 26 (range 1-70)
years at death, and 38% were female. In 143 with sufficient information, 40%
had generalized and 60% had focal epilepsy. SUDEP affected the full spectrum of
epilepsies, from benign epilepsy with centrotemporal spikes (n = 3, 1%) to
intractable epileptic encephalopathies (n = 27, 11%). Most (93%) SUDEPs were
unwitnessed; 70% occurred during apparent sleep; and 69% of patients were
prone. Only 37% of cases of SUDEP took their last dose of antiseizure
medications (ASMs). Reported lifetime generalized tonic-clonic seizures (GTCS)
were <10 in 33% and 0 in 4%.
CONCLUSIONS:
NASR participants commonly have clinical features that have
been previously been associated with SUDEP risk such as young adult age, ASM
nonadherence, and frequent GTCS. However,
a sizeable minority of SUDEP occurred in patients thought to be treatment
responsive or to have benign epilepsies. These results emphasize the
importance of SUDEP education across the spectrum of epilepsy severities. We
aim to make NASR data and biospecimens available for researchers to advance
SUDEP understanding and prevention.
___________________________________________________________________
SUDEP can affect men, women, and children. It can affect
"children diagnosed with benign epilepsies who are told they don't need to
be on medication, that they will outgrow the epilepsy. Unfortunately, [some]
die before they have a chance to outgrow it," Devinsky said. The severity
of epilepsy cases in which SUDEP occurs varies widely, from cases characterized
by centrotemporal spikes to epileptic encephalopathies and failed resective
surgeries...
SUDEP is not the only epilepsy-related cause of death —
drowning, car accidents, falling down a flight of stairs, and severe burns also
occur. Several risk factors are modifiable, so it is possible to lower the risk
for SUDEP.
"Medication adherence is probably the most important
one. Getting adequate sleep is probably a close second or tied, more or less,
and in adult populations, it's also excess alcohol," Devinsky said.
Combining one or more of these risk factors, such as
drinking more alcohol than usual while sleep deprived, has a synergistic impact
on risk.
Many previous population-based studies, medical examiner
case-control series, and clinical case-control studies have focused on
subgroups of epilepsy patients or patients whose condition was refractory to
medication or surgery.
In contrast, Devinsky and colleagues evaluated a more
general epilepsy population enrolled in a large international registry...
"The other finding from our paper, which has been
identified before...is 84% of the family members we spoke to had never heard of
SUDEP, and they believed their loved one had never been told about SUDEP,"
Devinsky said.
Clinicians should discuss SUDEP with all patients with
epilepsy for two reasons.
"First, some reassurance can be given to relieve
anxiety for those at low risk (eg, focal aware or absence seizures only).
Second and most important, patients and families must understand the critical —
and potentially lifesaving — importance of seizure control," he said.
"Someone might think: 'If I have an occasional seizure
because I missed a med, no big deal, I will be a little tired for an hour, and
then I will be on my way.' But if they understand there is a chance they could
die from that seizure, one would hope they would be better about being fully
adherent with their medication," Devinsky added...
A conversation with a patient whose seizures are well
controlled could start, for example, as follows: "Listen, I just want you
to know you're doing fantastic. There is a problem called SUDEP, or sudden
death in epilepsy. Your chances of suffering it are extremely low; however,
they do exist. If you can take your medications religiously and you can get
good sleep and avoid excess alcohol, you will dramatically reduce your chances
of getting this from very low to close to zero. But if you miss a single dose
of medication, unfortunately, you could have a big seizure, and that big
seizure could be deadly,' " said Devinsky.
Going forward, the investigators will evaluate individuals
who experienced seizures that were recorded in an epilepsy unit. They plan to
assess those who subsequently died in order to identify distinguishing factors
in their clinical histories or diagnostic test findings in comparison with age-
and sex-matched control persons who had a similar seizure but did not die from
SUDEP.
"We hope studies like this will allow us to identify
markers that more accurately identify patients at high risk for SUDEP and
inform us about the mechanisms for SUDEP so we can more effectively prevent
it," he said.
"There are many unknowns about SUDEP, and we do not
talk about it with our patients, likely because of the lack of knowledge about
the mechanism causing this condition, or the risk factors associated with
it," Jorge G. Burneo, MD, MSPH, FAAN, writes in an accompanying editorial.
The current study "represents a great effort, as it
collected a substantial amount of data from different referral sources in the
United States and Canada, and is the largest cohort of SUDEP cases so
far," writes Burneo, the Jack Cowin Chair in Epilepsy Research at Western
University and coleader of EpLink, the epilepsy research program of the Ontario
Brain Institute.
"The important take-home message from this study is
that from now on, clinicians should start conversations about SUDEP with their
patients with epilepsy earlier rather than later," he notes.
https://www.medscape.com/viewarticle/914617
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