Jeavons Syndrome: Clinical Features and Response to Treatment

Kelsey M. Smith, Paul E. Youssef, Elaine C Wirrell, Katherine C Nickels, Eric T. Payne, Jeffrey W. Britton, Cheolsu Shin, Gregory D Cascino, Marc C. Patterson, Lily Wong-Kisiel

Research output: Contribution to journalArticle

2 Scopus citations

Abstract

Background: Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized. Methods: We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016. Criteria for Jeavons syndrome included all of the following: (1) eyelid myoclonia with or without absences, (2) eye–closure-induced seizures or electroencephalography paroxysms, and (3) seizure onset after 12 months of age. We reviewed and described the epilepsy history, antiepileptic drug trials, and response to treatments. Results: Mean age at seizure onset was 7.3 years, and 80% were female. Absence seizures (63%) and generalized tonic-clonic seizures (23%) were most common at onset. Diagnosis was delayed by an average of 9.6 years. After a median follow-up of two years, 80% of patients had drug resistant epilepsy and 70% experienced generalized tonic-clonic seizures. Generalized tonic-clonic seizures and seizure types other than absence seizures increased the risk of drug-resistant epilepsy (P values 0.049 and 0.03, respectively). Valproic acid, lamotrigine, ethosuximide, and levetiracetam were the most effective in reducing seizures by more than 50%. Conclusions: The diagnosis of Jeavons syndrome is often delayed. Generalized tonic-clonic seizures and seizure types other than absence seizures may be predictors of drug-resistant epilepsy among patients with Jeavons syndrome.

Original languageEnglish (US)
JournalPediatric Neurology
DOIs
StateAccepted/In press - Jan 1 2018

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Keywords

  • drug-resistant epilepsy
  • epilepsy
  • eyelid myoclonia with or without absence
  • generalized epilepsy
  • Jeavons syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology

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