Sudden Unexpected

Death in Epilepsy
神經內科 洪國華
 Background
• Patients with epilepsy have a mortality rate
2-3 times that of the general population
• Epilepsy-related causes of death in this
population account for 40% of the deaths
 Epilepsy-related
causes of death
• Death due to underlying disease in symptomatic
epilepsy
• Sudden unexpected death in epilepsy (SUDEP)
– Account for 8-17% of deaths in this population
• Accidents during epileptic attack (i.e. trauma,
drowning, burning, choking)
• Status epilepticus
• Suicide
• Treatment-related death
 Criteria for SUDEP

1. The patient has epilepsy, which is defined as recurrent

unprovoked seizures
2. The patient died unexpectedly while in a reasonable state
of health
3. The death occurred suddenly (i.e. within minutes)
4. The death occurred during normal and benign
circumstances
5. An obvious medical cause of death could not be
determined at autopsy
6. The death was not the direct result of a seizure or status
epilepticus
Developed by FDA and Burroughs-Wellcome in 1993
 Categories
• Definite SUDEP: Cases meet all criteria and have a
postmortem report
• Probable SUDEP: Cases meet all criteria but lack
postmortem data
• Possible SUDEP: SUDEP cannot be ruled out but evidence
is insufficient regarding the circumstances of death and no
postmortem report is available
• Not SUDEP: Other causes of death are established clearly
or the circumstances make the diagnosis of SUDEP highly
improbable
 Cause of Death

• Possible SUDEP (Sudden

unexpected death in Epilepsy)
• DDx: Sudden cardiac death
 Caveats
• Arrhythmia can present with seizures
• Cardiovascular disease responsible for 75%
of all sudden deaths
• Subarachnoid hemorrhage
was not excluded
 Autopsy Reports
• Brain
– Edema without mass effect; hypoxic in some instances
• Lungs
– Mild to moderate pulmonary edema with protein-rich
fluid and alveolar hemorrhage in 62~84%
• Heart
– Nonfatal condition, including fibrosis of conductive
system, in 33%
• Liver
– Venous congestion
 In witnessed cases
• Seizures stopped before death (in many
cases patients regained consciousness)
• The immediate event before death was
respiratory arrest (obstructive and central)
in a few witnessed cases (most have
difficulty breathing before death)
• Attempts at CPR were unsuccessful
 Proposed
Mechanisms
• Cardiac: Ventricular arrhythmia; asystole
• Autonomic dysfunction
• Apnea during seizure
• Neurogenic pulmonary edema
• Brain death
• Anti-epileptic drugs
 Possible Risk Factors

1. Patient-related
2. Seizure-related
3. Treatment-related
 Patient-related
Risk Factors
• Young (20~40 y/o)
• Male
• Developmentally delayed (IQ < 70)
• Excessive alcohol consumption
 Seizure-related
Risk Factors
• Symptomatic epilepsy (1/100 Vs 1/1000 for
idiopathic epilepsy annually)
• Younger age of seizure onset
• Duration of seizure disorder > 10 yr
• Higher number of seizures
• The high risk group = medically intractable
epilepsy
 Treatment-related
Risk Factors
• Subtherapeutic serum level of antiepileptic
medication
• Higher number of antiepileptic medications
• Recent change of regimen
• Seizure persists post-epileptic surgery
• Use of carbamazepine?
 To Decrease
Risk of SUDEP
• Optimal seizure management with effective
monotherapy
• Compliance with medication
• Avoiding alcohol, drugs, seizure-provoking
situations, and high-risk situations (e.g.
driving, swimming)
 For Caregivers
• SUDEP was far more common in an
outpatient setting than in a group home
setting
• Proper positioning after the attack
• Respiration needs to be monitored during
the postictal period
• Stimulating the patients postictally also is
believed to reduce the chances of apnea
 Patient Education

• Should we tell every epilepsy

patient they are at risk for SUDEP?
– Certainly if they ask
 Is there any reason not
to discuss this topic?
YES!
• Pediatricians are reluctant to tell
parents that their child could have
SUDEP for fear they will upset the
parents and make them overly
protective
 Is there any reason not
to discuss this topic?

YES!
• Risk of SUDEP seems too low
• It would upset the patient
• It could start a conversation which
could consume considerable time
 Is there any reason not
to discuss this topic?

YES!
• If we tell, it will make it happen.
 Death foretold: timor
mortis conturbat me?
Three people with epilepsy, of sound
mind, spontaneously declared their belief
that they would shortly die in a seizure, and
did so within 6 months of the declaration.
All 3 had read about SUDEP and had
discussed it with their partner. Although
expressing regret, all 3 seemed resigned to
their fate and told the clinician that he
would not be blamed.
T. Betts Seizure 9:370, 2000
 Recommendations

• Tell every new patient about SUDEP

• Tell every patient considering epilepsy
surgery
• Document in the notes about the
disclosure