Abstracts

Eyelid Myoclonia in a Patient With Juvenile Myoclonic Epilepsy

Abstract number : 2.434
Submission category : 18. Case Studies
Year : 2018
Submission ID : 501561
Source : www.aesnet.org
Presentation date : 12/2/2018 4:04:48 PM
Published date : Nov 5, 2018, 18:00 PM

Authors :
Salman Zahoor, Henry Ford Hospital and Jules E. Constantinou, Henry Ford Hospital

Rationale: Eyelid myoclonia with absences (EMA), or Jeavons syndrome, and JME constitute distinct seizure types included in the new operational classification of seizure types by ILAE in 2017. JME, first described by Janz and Christian, is characterized by different seizure types, including myoclonic seizures, generalized tonic-clonic seizures (GTCS), mostly in the awakening phase, and absences. EMA or Jeavons syndrome is a generalized epileptic condition clinically characterized by a distinctive seizure type consisting of eyelid myoclonia (EM) with or without absences, eye closure-induced electroencephalography (EEG) paroxysms, and photosensitivity  Methods: We describe the case of a 14-year-old female, a competitive swimmer, presenting to clinic with myoclonic jerks affecting the arms, occurring in series, in the early morning. These were precipitated by sleep deprivation Results: Her presentation was suggestive of JME. There were concordant EEG findings of generalized spike and slow wave discharges at 3-4 Hz, occurring in bursts of up to 3 seconds in duration.  Extended video EEG was carried out with the patient being on levetiracetam 500 mg BID. Interictal record showed brief bursts of generalized spike and slow wave activity at 5-6 Hz with bi-occipital predominance and incomplete generalization anteriorly. Seizures of an unexpected type consistent with EM were captured. Electrographically, there was a classic ictal paroxysm with eye blink followed by delayed eyelid opening with an increase in lid muscle artifact and an evolution of bi-occipital 5-6 Hz spike wave patterns and bi-anterior propagation lasting 3.5 to 4.5 seconds. Clinical correlates consisted of a delayed eye blink with eye opening and subtle eyelid flutter  Conclusions: This case underscores the not-well recognized observation that EM seizures are not necessarily diagnostic of Jeavons syndrome. EM seizures may occur as overlap phenomenon in other idiopathic generalized epilepsies such as JME Funding: None