Epilepsy related death in children
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EPILEPSY RELATED DEATH IN CHILDREN. Chris Rittey - Sheffield Children's Hospital. Definitions. Definite SUDEP Patient suffered from epilepsy Patient died unexpectedly in reasonable health Death occurred suddenly (where known) An obvious medical cause of death was not found

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EPILEPSY RELATED DEATH IN CHILDREN

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Epilepsy related death in children

EPILEPSY RELATED DEATH IN CHILDREN

  • Chris Rittey - Sheffield Children's Hospital


Definitions

Definitions

  • Definite SUDEP

    • Patient suffered from epilepsy

    • Patient died unexpectedly in reasonable health

    • Death occurred suddenly (where known)

    • An obvious medical cause of death was not found

    • Death was not the direct result of seizure or SE

  • Probable SUDEP

    • As above but no PM

  • Possible SUDEP

    • SUDEP cannot be ruled out but insufficient information

Nashef et al, 1997


Epilepsy related death in children

  • Epidemiology

  • Risk factors

  • Mechanisms

  • Management strategies

  • Providing information

  • Checklist


Epidemiology

EPIDEMIOLOGY

  • Incidence much lower in reported series in children

  • Rates fairly consistent across studies in UK, Europe and USA

  • Main issue remains ascertainment


Incidence

Incidence


Camfield camfield

Population based cohort study

All children who developed epilepsy in Nova Scotia (1977-1985) – population 850000

692 children – 26 deaths

Only 4 unexpected deaths – 2 epilepsy related, 1 SUDEP (21 year old woman)

Camfield & Camfield

Lancet 2002, 359: 1891-95


Callenbach et al

Dutch epilepsy study group

1988-1992 – all children with epilepsy (< 16 years) – 494 children (22 excluded)

5 year follow-up or to death

9 deaths – no SUDEP

Callenbach et al

Pediatrics 2001; 107: 1259-63


Nesbitt et al

Retrospective UK review from tertiary paediatric neurology service (97 deaths)/CEMACH (168 deaths)

Neurology cohort - 66% deaths unrelated to seizure disorder, 7 unexplained deaths (7.3%)

CEMACH cohort – 79% unrelated to seizure disorder, 25 unexplained (9.7%)

Nesbitt et al

Dev Med Child Neurol 2012; 54: 612-17


Risk factors for sudep

Majority of cases of SUDEP in adults had childhood onset epilepsy

Most deaths occur in sleep

Risks for childhood SUDEP include

Male sex

Symptomatic epilepsy

GTCS

Prone sleep posture (?)

Risks not identified

Low AED levels, polypharmacy, specific AEDs

Risk factors for SUDEP


Sudep in adults

Risks identified:

GTCS

Polypharmacy

Duration of epilepsy

Young age at onset of epilepsy

Male gender

Symptomatic epilepsy

Lamotrigine therapy

Lack of terminal remission

SUDEP in adults

Hesdorfer et al, Epilepsia 2011; 52: 1150-59


Idiopathic epilepsy

Extremely low risk of epilepsy related death and SUDEP in children with idiopathic epilepsy

Very rare reports of epilepsy related deaths in children with idiopathic epilepsy

Nesbitt et al suggest risk of 65/100000 (cf diabetes 45/100000)

Idiopathic epilepsy


Causes of death

In childhood majority of deaths unrelated to seizure disorder (i.e. due to underlying condition or co-morbidities)

Causes of death


Sillanpaa shinnar

Long term follow-up of childhood onset epilepsy - 245 children, 40 years, 60 deaths

33/60 epilepsy related deaths

23/60 SUDEP (8 < 19 years)

4/60 status epilepticus (2 < 19 years)

6/60 drowning (3 < 19 years)

Sillanpaa & Shinnar

Epilepsy & Behaviour 2013; 28: 249-55


Mechanisms of sudep

SUDEP is likely to be the consequence of a variety of processes

Likely mechanisms

respiratory

cardiac

‘electrocerebral shutdown’

Mechanisms of SUDEP


Respiratory mechanisms

Most witnessed cases occur with GTCS

Most reported cases had difficulty with breathing

Apnoea a frequent finding in VT recorded seizures

Likely that central and obstructive apnoea plays a role

Respiratory mechanisms


Respiratory mechanisms1

Several postulated mechanisms

Respiratory arrest

Neurogenic pulmonary oedema

Asphyxiation

Recent interest in 5HT defects in SUDEP (Richerson and Buchanan, Epilepsia 2011)

mouse models

5HT role in control of breathing

common pathway with SIDS

Respiratory mechanisms


Cardiac mechanisms

Most important mechanism likely to be cardiac dysrhythmia caused by seizure

bradyarrhythmia

ventricular tachyarrhythmias

role of long QT syndromes

Right hemispheric control of sympathetic cardiac control

cardiovascular dysregulation common in children with right temporal lobe seizures

Cardiac mechanisms


Cardiac mechanisms1

Possible role of stress induced release of catecholamines

Potential role of environmental stress  possible therapeutic interventions

SUDEP reported in people with VNS but evidence suggests slight reduction of SUDEP risk in this population

Cardiac mechanisms


Management i

Almost all witnessed cases of SUDEP are associated with a seizure

Reduced SUDEP rate in people undergoing successful epilepsy surgery

Phase II trials in adults suggest increased mortality and SUDEP in those randomised to placebo

Suggests causal relationship between seizure and SUDEP

Management I


Management ii

Aim for seizure freedom

Suggestion that where this cannot be achieved aggressive attempts at seizure control can reduce but not eliminate risk of SUDEP

Careful attention to basic safety precautions (bathing, swimming)

Pet ownership (?) – see Terra et al, Seizure 2012; 21: 649-51

Management II


What do we tell our patients

SUDEP is a risk for patients with epilepsy not in remission

In neurologically normal children risk is not significant until adolescence/adulthood

? Need to discuss at all with families of children with idiopathic epilepsy likely to remit in childhood (e.g. BCECTS)

What do we tell our patients?


Gayatri et al

Questionnaires to parents attending regional paediatric neurology service and to 71 UK paediatric neurologists

Parental questionnaire – repeated after 3 months

100 children (57 focal/epileptic encephalopathy)

1/3 had heard of SUDEP before the study

91% wanted to be told about SUDEP (74% at diagnosis, 16% when seizures poorly controlled)

Gayatri et al


Gayatri et al1

Majority of parents reported no adverse effects of being given SUDEP information

Approx 50% said they would alter care for their child following information

Neurologist questionnaire – 46 responses

43/46 (93%) provided SUDEP information

20% - to all patients

63% - to patients with intractable seizure

46% - to parents and children (> 12 years)

Gayatri et al


Sudep checklist

Several risk factors for SUDEP are potentially modifiable

Shankar et al suggest use of evidence based checklist may allow clinicians and patients to identify and act on these

Potential benefit in improving discussion about epilepsy related death

SUDEP checklist

Seizure 2013 – in press


Conclusions

Children with epilepsy have a 3-4 x increase risk of death than the general population

Most deaths in children with epilepsy are not related to the seizure disorder

Death may occur as a result of SE, accident and SUDEP

Conclusions


Conclusions1

SUDEP risks are extremely low in children but finite risk in those with poorly controlled symptomatic epilepsies

Strategies directed towards optimal seizure control likely to be most useful in reducing SUDEP rate

Parents want to be told about SUDEP

Checklist may be of value but will probably need modification in childhood

Conclusions


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