Sudden Death

What the Patient and Doctor should Know

DEFINITION

SUD in people with epilepsy involves a nontraumatic death occurring within a short time following a seizure, or presumed seizure. Status epilepticus is excluded, as is asphyxiation from physical causes (e.g. pillow over face). An autopsy should show no anatomical cause of death. Most cases are unwitnessed and without autopsy, so history and inference of events predominate the literature.

CAVEAT 1

The literature is replete with cases of seizures and nonfatal cardiac arrhythmias, including tachyarrhythmias, prolonged bradycardia and asystole. This is certainly the basis for a cardiac/autonomic etiology, but doesn't explain why some people have it and do not die, and some do.

CAVEAT 2

Misdiagnosis.
Children with loss of consciousness may have a cardiovascular cause. The Long Q-T syndromes (Romano-Ward syndrome; Jervell and Lange-Nielson syndrome) can present with seizures. Such patients can experience SCD misdiagnosed as SUDEP.

Fictious epilepsy.
Rarely, seizures in young children are really anoxic episodes caused by the parent. In some cases these lead to brain damage or death. In the latter it may be confused with sudden unexplained death of infancy (cot death).

CARDIOLOGY

Sudden death can occur in non-epileptic patients, with cardiovascular disease responsible for 75% of all sudden deaths. The autopsy should show heart disease in this group. Sudden death accounts for 25% of all deaths in the United States.

EPIDEMIOLOGY


SCD autopsy positve
SCD autopsy negative
SUD total U.S. pop.
SUD general epilepsy pop.
SUD intractable epilepsy
 per 1,000 per year
1
0.001
0.004-0.01
1-2
9

SEIZURE TYPES ASSOCIATED WITH SUD
Convulsive
Complex Partial
Simple Partial
Myoclonic
Absence
Pseudoseizure (in people who also have epilepsy)

EPILEPSY TYPES ASSOCIATED WITH SUD
Complex Partial
Primary generalized comvulsive
Myoclonic
Juvenile Myoclonic Epilepsy

CIRCUMSTANCES
Witnessed seizure - unresuscitatable
Found dead in bed or home

AGE:SEX
mean 20-40 y.o., range 1 - 73
males > females

PROPOSED MECHANISMS
Cardiac: ventricular arrhythmia; asystole

    Seizure causes a heart attack.
    neural invasion?
    exertion of seizure?
    stress - hormone release?
Autonomic dysfunction
    Seizure causes sympathetic and parasympathetic discharge with cardiovascular compromise.
Apnea during seizure
    Tonic seizures typically paralyze the diaphragm for a minute.
    This is more malignant.
Pulmonary edema
    Seizure causes neurogenic pulmonary edema.
    Person drowns.
Brain death
    Seizure causes intense inhibition of brain function and shutdown.
Low AED blood level
    Seizures associated with low AED blood level is some how more malignant.
Street drugs
    Interaction between drug and seizure makes the seizure more malignant.

WISCONSIN EXPERIENCE - FRANCIS M. FORSTER EPILEPSY CENTER

Dasheiff RM, Dickinson LJ: Sudden unexpected death following a seizure in an epileptic patient: A case report. Arch. Neurol. 43:194-196, 1986.

Population: VA Epilepsy Center

Incidence: 1.4/1,000 per year

Case: 48 y.o. male had witnessed complex partial seizure and seemed to recover. Then seemed to have a second seizure but in fact was in ventricular fibrillation by EKG. Was coded immediately but could not be resuscitated. Autopsy negative.

PITTSBURGH EXPERIENCE - UPEC

Dasheiff RM: Sudden unexpected death in epilepsy and its relationship to sudden cardiac death. J. Clin. Neurophysiol. 8:216-22, 1991.

Population: University Epilepsy Surgery Program

Incidence: 9/1,000 per year [based on seven deaths]

Case 4: Patient in MICU s/p temporal lobectomy for epilepsy. Well, sitting in bed without monitors, waiting for wheelchair to transfer her to regular hospital room. Suddenly lying in bed dead. Coded immediately but could not be resuscitated. Autopsy negative for cause of death.

UNIVERSITY OF CALGARY

Lee, MA: EEG Video recording of sudden unexpected death in epilepsy (SUDEP). Epilepsia 39 (suppl 6):123-4, 1998.

Population: University Epilepsy Center

Case: 41 y.o. woman, uncontrolled complex partial epilepsy undergoing CCTV/EEG. Had 70 sec convulsion followed by bradycardia of 30 bpm which slowly returned to 70 bpm over 5 min. However the EEG went flat 1 minute after the seizure, never recovered and the patient was dead 18 min. after the seizure. Autopsy negative.

UNIVERSITY OF BRITISH COLUMBIA

Purves, SJ, Wilson-Young, M, Sweeney, VP: Sudden death in epilepsy: Single case report with video-EEG documentation. Epilepsia 33 (suppl 3):123, 1992.

Population: University Epilepsy Center

Case: 27 y.o. woman, uncontrolled complex partial epilepsy undergoing CCTV/EEG. Had 4 complex partial seizures followed by a convulsion, then rolled herself into the prone position and was found cyanotic 24 min. later. Resuscitation was unsuccessful. Cause attributed to deep postictal depression leading to asphyxia.

ISSUES
PREVENTION

    Requires identification of risk factors and a proposed treatment.
    So far the only high risk group are medically intractable patients.
    This risk does not seem to be reduced following VNS or epilepsy brain surgery.
Variables which are not a significant risk factor
    age
    sex
    AED medicine
    (although carbamazepine has been suggested in some reports)
    AED blood level
    (frequently low in SUD but low in many patients without SUD)
    pre-existing heart disease
    seizure type
CO-MORBIDITY
Assignment of co-morbidity to patients for purposes of:
    insurance rates
    medical resource allocation
    disability compensation
    physician malpractice
CLINICAL TRIALS
Aid design and interpretation of clinical trials
Increased importance to identify non-treatment causes of death in study populations of intractable epileptics.
Saved the VNS trial.

DISCLOSURE

Is this a medicolegal, moral, or ethical issue?

Does the patient have a right to know?

Do doctors tell every patient with heart disease they are at risk for SCD?

Should we tell every epilepsy patient they are at risk for SUD? Certainly if they ask.

Should it be part of the general information we disclose along with the driving laws, birth control, pregnancy, AED side-effects, genetics of their epilepsy, prognosis of seizure control, treatment options (no treatment, AEDs, Ketogenic diet, VNS, brain surgery)?

Is there any reason not to discuss this topic? YES

Pediatricians are reluctant to tell parents that their child could have SUD for fear they will upset the parents and make them overly proctective. Especially so if the patient has only a few seizures and a good prognosis.

Is there any reason not to discuss this topic? YES

Even in adults, the risk of SUD seems too low to justify a disclosure. It would upset the patient. It could start a conversation which could consume considerable time (that may not be available in a busy clinic).

Is there any reason not to discuss this topic? YES

If we tell, it will make it happen.
T. Betts Seizure 9:370, 2000 Of death foretold: timor mortis conturbat me? People with epilepsy who foretold their own demise from SUDEP. 

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 
		‘you have done your best’

As a result of the eventual outcome, it is difficult to
contemplate the next declaration encountered
with a quiet mind.

Presently, community standard care does not include disclosure on this issue.

What compelling issues would mandate a change?
Lawsuits?
New information about SUD?
A way to prevent it?

Remember when the spouse requested
the physician NOT tell the patient they
had cancer, and the doctor complied !

RECOMMENDATION

Tell every new patient about SUD, young or old.
Why?
Because many patients, and their relatives, seek medical care because they feared death as a consequence of that first seizure. This is one of the reasons we treat epilepsy.

Tell every patient considering epilepsy surgery.
Why?
It gives a balanced view of the risks of continuing to have epilepsy versus the the risks of treatment. Granted, the studies show SUD is still a risk after surgery, but at least they could be seizure free while waiting for SUD.

Physicians should document in the notes about the disclosure.
Many patients and relatives later deny they were told.

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