IS JEAVONS SYNDROME (EYELID MYOCLONIA WITH ABSENCES) GENERALIZED OR LOCALIZATION RELATED EPILEPSY?
Abstract number :
2.111
Submission category :
4. Clinical Epilepsy
Year :
2009
Submission ID :
9828
Source :
www.aesnet.org
Presentation date :
12/4/2009 12:00:00 AM
Published date :
Aug 26, 2009, 08:12 AM
Authors :
Cristina Go, S. Viravan, A. Ochi, O. Snead, III and H. Otsubo
Rationale: Jeavons syndrome is one of the underreported epileptic syndromes characterized by eyelid myoclonia, eye closure-induced seizures/EEG paroxysms, and photosensitivity. Jeavons syndrome has been proposed as idiopathic generalized epilepsy because of normal posterior dominant background activity and paroxysmal generalized ictal EEG discharges. However, we noticed subtle preceding focal EEG change before eyelid myoclonia using digital video EEG. We studied clinical and EEG findings in Jeavons syndrome to determine whether this is generalized or localization related epilepsy. Methods: We identified 12 patients who met the diagnostic criteria of Jeavons syndrome from January 2004 to April 2009 at the Hospital for Sick Children, Toronto, Canada. All patients had eyelid myoclonia captured by video-EEG or routine EEG monitoring. We reviewed and described ictal EEG pattern, interictal abnormalities, demographic, clinical data, and neuroimaging finding. Results: All patients but one are female (92%). Age at seizure onset ranged from 1.5 to 9 years, with a mean age of 5 years. Six of them (50%) were previously diagnosed as absence epilepsy and 10 of them were on antiepileptic medication before EEG study. Seizures or EEG paroxysms were induced by photic stimulation (75%) and hyperventilation (58%). All of them had normal posterior dominant alpha rhythm, reactive to eye opening and closure. Ten patients (83%) had focal ictal EEG findings either posterior leading in 200ms to 1s before generalized spike/polyspike-wave complexes or posterior spike-wave complexes induced by prolonged eye closure. Interictally, there were focal epileptiform discharges mainly over the left and/or right posterior head region in 8 out of 12 patients (67%). Conclusions: This evidence of focal ictal and interictal EEG findings from posterior head region in Jeavons syndrome patients along with EEG paroxysms associated with seizures induced by eye closure and photic stimulation raises the possibility of idiopathic occipital lobe epilepsy.
Clinical Epilepsy