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.
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Pediatr Neurol
December 2024
Department of Internal Medicine, Seth GS Medical College and KEM Hospital, Mumbai, India. Electronic address:
Jeavons syndrome (JS), also known as epilepsy with eyelid myoclonia (EEM), is an idiopathic epileptic syndrome that primarily affects children. JS constitutes a significant portion of idiopathic generalized epilepsies and overall epileptic conditions and is characterized by frequent eyelid myoclonia. JS is often triggered by factors such as eyelid closure and exposure to light, leading to absence seizures with photoparoxysmal responses.
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December 2024
Department of Pediatric Epileptology, Functional Neurology and Sleep Disorders, Hôpital Femme Mère Enfant, University Hospitals of Lyon (HCL), Member of ERN EpiCARE, Lyon, France.
Child Neurol Open
August 2024
[This corrects the article DOI: 10.1177/2329048X231183524.].
View Article and Find Full Text PDFNeurophysiol Clin
May 2024
Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France; Research Unit (URCMA: Unité de Recherche sur les Comportements et Mouvements Anormaux), INSERM, U661 Montpellier, France.
Epilepsy with eyelid myoclonia (EM) or Jeavons syndrome (JS) is an epileptic syndrome related to the spectrum of genetic generalized epilepsies (GGE). We report two untreated children on which EEGs were performed several hours after a generalized tonic-clonic seizure (GTCS). These showed a unilateral, nearly continuous posterior slowing.
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May 2024
LSU Department of Otolaryngology, Louisiana State University School of Medicine-New Orleans, USA.
Background: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl.
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