SUDEP Remembrance wall.jpg

Remembrance Wall at the National Walk for Epilepsy honors those who have been lost to epilepsy and seizures.

S. Shmuely, S.M. Sisodiya, W. Boudewijn Gunning, J. W. Sander, R. D. Thijs. In Epilepsy & Behavior 2016 ;64(Part 1):69-74.

Purpose

This review article was done to better understand causes of premature death in people with Dravet syndrome (DS), with an emphasis on sudden unexpected death in epilepsy (SUDEP).

Description of Study

  • A search of the medical literature was done to find articles relating to this topic. The search terms used included:
    • Dravet syndrome
    • SMEI (which stands for severe myoclonic epilepsy in infancy and is another name for Dravet syndrome)
    • Severe myoclonic epilepsy
    • Mortality
    • Survivors
    • Prognosis
    • Death
  • The authors looked at 30 reports from 762 studies published between 1989 and 2015. The articles included 177 unique cases of Dravet syndrome (93% tested positive for SCN1A mutation). SCN1A is a genetic mutation related to Dravet syndrome.

A summary of Study Findings

  • SUDEP was a likely cause of death in nearly half of the people (87 out of 177 or 49% of deaths). Other causes of death included:
    • Status epilepticus (32% of deaths)
    • Drowning or accidents (8% of deaths)
    • Infection (5% of deaths)
    • Other or unknown cause (6% of deaths)
  • Death occurred before the age of 10 years in 3 out of 4 people (73%).

What does this mean?

  • This literature review confirmed that death occurs at a young age in children with Dravet syndrome.
  • The two most frequent causes of premature death are SUDEP and status epilepticus.
  • It is unknown if having an SCN1A mutation leads to a genetic susceptibility to SUDEP beyond the severity of epilepsy or seizures in children with Dravet syndrome.

Article published in Epilepsy & Behavior, November 2016.

Authored By: 
Sookyong Koh MD, PhD
Authored Date: 
02/2017
Reviewed By: 
Christianne N. Heck MD, MMM
on: 
Tuesday, February 14, 2017