Revista de neurologia · 2010
[Does Jeavons syndrome exist? A report of a series of 10 cases].
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Pérez-Errazquin Francisco, Chamorro-Muñoz M Isabel, García-Martín Guillermina, Romero-Acebal Manuel
Аннотация
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-electro-encephalo-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 females (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.
AIM: The aim of this article is to report on a series of 10 cases, with special attention given to their clinical-electro-encephalo-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 females (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.