Baby being monitored for seizures

Defining Impact of Early Life Epilepsy on Patient Outcomes

 It is well known that early-life epilepsies (ELEs) are associated with poor outcomes including intractable or drug-resistant seizures, developmental and cognitive disabilities, poor regulation of other functions controlled by the nervous system, as well as increased risk for mortality. Early-life epilepsies are defined by a diagnosis of epilepsy greater than 28 days of life and less than 3 years of age and are often divided into syndromic (identifiable features and/or characteristics such as genetic diseases, structural abnormalities, or metabolic diseases) versus nonsyndromic types.

Over the last several years, Cynthia Keator M.D. has led the efforts of Cook Children’s Epilepsy Program and has been an active contributor to the Pediatric Epilepsy Research Consortium (PERC). As a participant in the multi-center early life epilepsy group, Cook Children’s enrolled patients with new onset epilepsy (ages 28 days to 36 months); to identify variables which serve as predictors of outcome in ELEs. Their most recent article is a review of outcomes in ELE. Given that modern day medicine has allowed easier and better access to high quality imaging studies (MRI), more comprehensive genetic testings and newer medications, one might hypothesize that outcomes of ELE are improving. A total of 775 children were recruited from 17 centers and a total of 680 children met criteria for review in this study.

Many variables were identified including age at seizure onset, underlying etiology, seizure types and epilepsy type/syndrome, initial developmental status at the time of diagnosis/seizure onset, and mortality. The outcomes of these variables were then reviewed at follow up. Children included in the study had to have follow-up information for at least 6 months after the initial diagnosis of epilepsy. Some of the primary findings are summarized below.

Thirty-five percent of the patients developed drug resistance. Statistically significant factors that were identified to predict drug resistance were:

  • Development of epilepsy at < 1 year of age
  • Definitive developmental delay at the initial evaluation

The patients at lowest risk to develop intractability were those who:

  • Had normal development at diagnosis
  • Onset of epilepsy > 1 year of age

Factors predicting evolution from non-syndromic epilepsy to infantile spasms (15% of the patients):

  • Developmental delay at initial diagnosis
  • Development of epilepsy very early (i.e. 0-2 months)

Factors predicting development of new seizure types after initial presentation with infantile spasms (23% of the patients):

  • Developmental delay at initial diagnosis

Factors associated with developmental delay after initial diagnosis (23% of the patients)

  • Onset of epilepsy in infancy
  • Identified etiology
  • Initial epilepsy type
  • Development of drug resistance in infancy (but not > 1 year)

Death occurred in 22 patients (2.9%) of the cohort. No deaths occurred in patients with unknown etiology and who were developing normally at the time of initial evaluation. Specific causes of death were not available.

This current data suggests that even with advances in medicine and technology there is continued poor outcome for ELEs. This article also demonstrates that development of epilepsy within the first year of life (regardless of etiology) should always be viewed with utmost urgency and importance until proven otherwise. Most importantly this data emphasizes the ongoing need to develop evidenced based standard care for ELEs with the goal to optimize early outcomes.

This article is available for free download from Epilepsy and Behavior until 7/27/2019 by clicking here.

Contributing author:

Cynthia Keator, MD

Cynthia Keator, MD
Medical Director, Epilepsy Monitoring Unit
Jane and John Justin Neuroscience Center

Meet Dr. Keator

Cook Children’s Neurology team

Great outcomes begin with great input. Having a medical system where every department, doctor, and care team member works together means that your child can have quick access to testing, diagnosis and treatment, and that means better outcomes now and in the future.

Contact the Jane and John Justin Neuroscience Center at Cook Children’s with your questions at 682-885-2500.

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