Does Jeavons syndrome exist? A report of a series of 10 cases

作者：Perez Errazquin Francisco^*; Isabel Chamorro Munoz M; Garcia Martin Guillermina; Romero Acebal Manuel

来源：Revista de Neurologia, 2010, 50(10): 584-590.

• Eyelid closure sensitivity
• Eyelid myoclonia
• Idiopathic generalised epilepsy
• Jeavons syndrome
• Photoparoxysmal response
• Photosensitivity
• Reflex epilepsy

Introduction. Jeavons syndrome (JS) or eyelid myoclonia (EM) with or without absences is an epileptic syndrome characterised by EM on closing the eyes, in bright environments, which coincides on the electroencephalogram with brief generalised polyspike and polyspike-wave discharges at 3-6 Hz. Photoparoxysmal response is associated in photostimulation. The ILAE recognises EM as a special type of myoclonic seizures, yet it still does not include JS as a separate condition in its classification of epileptic syndromes
Aim. The aim of this article is to report on a series of 10 cases, with special attention given to their clinical-electroencephalo-graphic description We believe it is a condition that is not infrequent, but one which is underdiagnosed Moreover, we also want to highlight the pros and cons determining the fact that today there is still controversy about whether or not it should be recognised as an independent epileptic syndrome
Patients and methods. We conducted a retrospective study of 10 cases collected over the period between 2002 and 2009 in our adult Epilepsy Unit
Results. All our patients fulfilled JS criteria. There is a predominance of female S (n = 8), with the existence of cases of idiopathic generalised epilepsy in the family (n = 5), which are associated with clonic-tonic generalised seizures in nearly all of them (n = 9) and are well controlled with treatment in monotherapy, although the EM persist in three cases The results of a neurological exploration and neuroimaging are normal in all cases
Conclusions. JS is a photosensitive epilepsy that is not easily confused with other conditions, nevertheless, there can be an overlap with other idiopathic generalised epilepsy syndromes that lead to their being underdiagnosed Genetics and functional neuroimaging will determine whether it is an independent condition or not

• 出版日期2010-5-16