Jeavons Syndrome: 12 Cases

Abstract

Objectives:Jeavons Syndrome (JS), also known as eyelid myoclonia with absences epilepsy, is a type of idiopathic reflex epilepsy that has primary seizure type eyelid myoclonuses and is characterized by blinking due to seizures or generalized paroxysms at electroencephalography (EEG) and photosensitivity. This study aimed to review JS by following up with the patients with this diagnosis.Methods:The records of the patients who were followed up with JS diagnosis in the pediatric neurology clinic of Cerrahpaşa Medical Faculty Hospital were reviewed. These patients were interviewed, and the data obtained from their file records were evaluated.Results:Eight female and four male patients aged between 7 and 22 years were included in the evaluation. The age at onset of seizure varied from 18 months to 14 years, often between 6 and 12 years of age. The majority of the patients visited the clinic because of generalized tonicclonic (GTC) seizures. The results of video EEG monitorization performed for all patients revealed that eyelid myoclonuses were found in all patients and absence seizures were also seen in some of them. All of the patients had generalized spike-and-wave discharges, some of which showed asymmetric features and some of which were accompanied by eyelid myoclonus and absence seizures. Moreover, focal findings were detected in the EEG analyses of eight patients. The most preferred drug was valproic acid for treatment.Conclusion:Eyelid myoclonuses or even absence seizures are usually not distinguished or mixed with some other cases such as tic disorder or daydream. The reason that brings a patient to the doctor is often the GTC seizures. In JS, focal/asymmetric findings in EEG are not uncommon. The focal findings seen in EEG may be misinterpreted and may lead to errors in diagnosis and drug selection.

Keywords

• eyelid myoclonic absence epilepsy
• eyelid myoclonus
• jeavons syndrome