Jeavons syndrome existing as occipital cortex initiating generalized epilepsy

Sorawit Viravan, Cristina Go, Ayako Ochi, Tomoyuki Akiyama, O. Carter Snead, Hiroshi Otsubo

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

Purpose: Jeavons syndrome (JS) is one of the underreported epileptic syndromes characterized by eyelid myoclonia (EM), eye closure-induced seizures/electroencephalography (EEG) paroxysms, and photosensitivity. JS has been proposed as idiopathic generalized epilepsy (IGE) because of normal posterior dominant background activity and paroxysmal generalized ictal epileptiform discharges (EDs). However, we noticed subtle occipital EDs preceding EM and interictal posterior EDs using digital video-EEG. We studied clinical and EEG findings in JS to determine the specific occipital lobe relation to this "eye closure-induced" reflex IGE. Methods: We identified 12 children who met the diagnostic criteria of JS from January 2004 to April 2009 at the Hospital for Sick Children, Toronto, Canada. All patients had EM captured by video-EEG. We reviewed and described ictal EEG patterns, interictal abnormalities, and demographics, clinical, and neuroimaging findings. Key Findings: All patients but one were female (92%). Age at seizure onset ranged from 1.5 to 9 years, with a mean age of 4.9 years. Six patients (50%) were previously diagnosed as having absence epilepsy and 10 patients were on antiepileptic medications. All 12 patients had normal posterior dominant alpha rhythm, reactive to eye opening and closure. Spiky posterior alpha activity was noted with sustained eye closure in six patients (50%). Interictally, there were generalized EDs found in 10 patients (83%); four of them also had focal interictal EDs over the posterior head region. Eleven patients (92%) had evidence of focal posterior ictal EDs. EM and/or paroxysmal EDs were induced by photic stimulation in 9 (75%) and hyperventilation in 7 (58%). Significance: We observed two neurophysiologic findings in JS: (1) focal interictal EDs from posterior head region; and (2) predominant focal posterior ictal EDs preceding generalized EDs. Further clinical observations of seizures induced by eye closure, photic stimulation, and hyperventilation along with EEG paroxysms would raise the possibility of the occipital cortex initiating generalized epilepsy network involving the brainstem, and thalamocortical and transcortical pathways in JS.

Original languageEnglish
Pages (from-to)1273-1279
Number of pages7
JournalEpilepsia
Volume52
Issue number7
DOIs
Publication statusPublished - Jul 2011
Externally publishedYes

Fingerprint

Generalized Epilepsy
Occipital Lobe
Electroencephalography
Eyelids
Stroke
Photic Stimulation
Seizures
Hyperventilation
Head
Alpha Rhythm
Absence Epilepsy
Age of Onset
Neuroimaging
Anticonvulsants
Brain Stem
Canada
Reflex
Demography

Keywords

  • Children
  • Eye closure-induced seizure
  • Eyelid myoclonia with and without absences
  • Jeavons syndrome
  • Occipital spikes
  • Spiky posterior alpha activity

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Jeavons syndrome existing as occipital cortex initiating generalized epilepsy. / Viravan, Sorawit; Go, Cristina; Ochi, Ayako; Akiyama, Tomoyuki; Carter Snead, O.; Otsubo, Hiroshi.

In: Epilepsia, Vol. 52, No. 7, 07.2011, p. 1273-1279.

Research output: Contribution to journalArticle

Viravan, S, Go, C, Ochi, A, Akiyama, T, Carter Snead, O & Otsubo, H 2011, 'Jeavons syndrome existing as occipital cortex initiating generalized epilepsy', Epilepsia, vol. 52, no. 7, pp. 1273-1279. https://doi.org/10.1111/j.1528-1167.2011.03068.x
Viravan, Sorawit ; Go, Cristina ; Ochi, Ayako ; Akiyama, Tomoyuki ; Carter Snead, O. ; Otsubo, Hiroshi. / Jeavons syndrome existing as occipital cortex initiating generalized epilepsy. In: Epilepsia. 2011 ; Vol. 52, No. 7. pp. 1273-1279.
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AU - Otsubo, Hiroshi

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N2 - Purpose: Jeavons syndrome (JS) is one of the underreported epileptic syndromes characterized by eyelid myoclonia (EM), eye closure-induced seizures/electroencephalography (EEG) paroxysms, and photosensitivity. JS has been proposed as idiopathic generalized epilepsy (IGE) because of normal posterior dominant background activity and paroxysmal generalized ictal epileptiform discharges (EDs). However, we noticed subtle occipital EDs preceding EM and interictal posterior EDs using digital video-EEG. We studied clinical and EEG findings in JS to determine the specific occipital lobe relation to this "eye closure-induced" reflex IGE. Methods: We identified 12 children who met the diagnostic criteria of JS from January 2004 to April 2009 at the Hospital for Sick Children, Toronto, Canada. All patients had EM captured by video-EEG. We reviewed and described ictal EEG patterns, interictal abnormalities, and demographics, clinical, and neuroimaging findings. Key Findings: All patients but one were female (92%). Age at seizure onset ranged from 1.5 to 9 years, with a mean age of 4.9 years. Six patients (50%) were previously diagnosed as having absence epilepsy and 10 patients were on antiepileptic medications. All 12 patients had normal posterior dominant alpha rhythm, reactive to eye opening and closure. Spiky posterior alpha activity was noted with sustained eye closure in six patients (50%). Interictally, there were generalized EDs found in 10 patients (83%); four of them also had focal interictal EDs over the posterior head region. Eleven patients (92%) had evidence of focal posterior ictal EDs. EM and/or paroxysmal EDs were induced by photic stimulation in 9 (75%) and hyperventilation in 7 (58%). Significance: We observed two neurophysiologic findings in JS: (1) focal interictal EDs from posterior head region; and (2) predominant focal posterior ictal EDs preceding generalized EDs. Further clinical observations of seizures induced by eye closure, photic stimulation, and hyperventilation along with EEG paroxysms would raise the possibility of the occipital cortex initiating generalized epilepsy network involving the brainstem, and thalamocortical and transcortical pathways in JS.

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