Abstracts

Coexistence of Epilepsy with Eyelid Myoclonia (Jeavon Syndrome) in a Patient with Childhood Epilepsy with Centrotemporal Spikes

Abstract number : 3.193
Submission category : 4. Clinical Epilepsy / 4A. Classification and Syndromes
Year : 2019
Submission ID : 2422091
Source : www.aesnet.org
Presentation date : 12/9/2019 1:55:12 PM
Published date : Nov 25, 2019, 12:14 PM

Authors :
Gamaleldin Osman, Henry Ford Hospital; Jules Constantinou, Henry Ford Hospital

Rationale: Childhood Epilepsy with Centrotemporal Spikes (CECTS; previously known as Benign epilepsy of childhood with centrotemporal spikes (BECTS)) is the most frequent pediatric focal epilepsy syndrome, accounting for around 10-25% of new onset pediatric epilepsies. Epilepsy with Eyelid Myoclonia (EEM; also known as Jeavon syndrome) is a rare generalized epilepsy syndrome characterized by daily eyelid myoclonias with or without absence seizures induced by eye closure and photosensitivity. While generalized discharges are occasionally seen in children with CECTS leading some experts to propose a continuum between this condition and genetic generalized epilepsy, the actual coexistence of both conditions is rare. To our knowledge , the coexistence of EEM and CECTS has not been previously reported. Methods: Case report Results: An 11-year-old boy with normal developmental history and no family history of epilepsy presented to our clinic with two episodes of choking sensation followed by drooling and left facial twitching lasting about 3 minutes with preserved consciousness. In addition, the patient's mother reported history of frequent episodes of eyelid flickering associated with eyes rolling , often induced by eye closure which were not associated with any lapses of interaction. Patient underwent VEEG recording which demonstrated frequent sleep-activated right centro-temporal and centroparietal spikes with tangential dipole orientation characteristic for CECTS. Also noted were occasional fragmentary generalized spike-wave discharges occurring at a rate of 3 to 4 Hz and occasional runs of generalized polyspikes. 43 episodes of eyelid myoclonias were captured.  Electrographic patterns consisted of eye closure artifact followed by bi-posterior squeaky beta at 12 to 13 Hz without significant anterior propagation which lasted between 2 to 4 seconds. Patient was started on levetiracetam 500 mg bid with marked improvement of eyelid myoclonias and no further focal seizures. Conclusions: To our knowledge , this is the first case report of the coexistence of epilepsy with eyelid myoclonias and childhood epilepsy with centrotemporal spikes. Care must be taken not to miss co-existent primary (genetic) generalized epilepsy in children with childhood epilepsy with centrotemporal spikes. Funding: No funding
Clinical Epilepsy