A Case of Jeavons Syndrome: Eyelid Myoclonus with Myoclonus Status
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Copyright (c) 2022 Zeba Khan, Nayab Anjum, M Reyazuddin
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Jeavons initially explained Jeavons syndrome or eyelid myoclonus with or without absence in 1977.1 It has not yet been acknowledged as a categorical epileptic syndrome.2 The electroclinical trinity of photosensitivity, eye closure-induced seizures, and electroencephalography (EEG) paroxysms serve this disease’s hallmarks.3 The normal onset occurs in childhood, peaks between the ages of 2 and 14, and is more prevalent in women (F:M= 2-3:1).4Abstract
Repetitive episodes of eyelid jerking, along with eye upward deviation and head retropulsion, constitute the symptoms.5 A limited minority of patients may have eyelid myoclonus status; seizures are frequent and brief, occurring hundreds of times each day. It is known as eyelid myoclonus with absence (EMA) when it occurs in conjunction with a loss of consciousness.
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Jeavons PM. Nosological problems of myoclonic epilepsies in childhood and adolescence. Dev Med Child Neurol 1977;19:3–8. https://doi.org/10.1111/j.1469- 8749.1977.tb08014.x. ] Koutroumanidis M, Arzimanoglou A, Caraballo R, Goyal S, Kaminska A, Laoprasert P, et al. The role of EEG in the diagnosis and classification of the epilepsy syndromes: a tool for clinical practice by the ILAE Neurophysiology Task Force (Part 1). Epileptic Disord 2017;19:233–98. https://doi.org/10.1684/ epd.2017.0935. ] Covanis A. Jeavons syndrome – updated review. J Epileptol 2015;23(2):113–23. https://doi.org/10.1515/joepi-2015-0033. ] Striano S, Striano P, Nocerino C, Boccella P, Bilo L, Meo R, et al. Eyelid myoclonia with absences: an overlooked epileptic syndrome? Neurophysiol Clin 2002;32: 287–96. https://doi.org/10.1016/s0987-7053(02)00343-x. ] Panayiotopoulos CP. Jeavons syndrome. In: Panayiotopoulos CP, editor. A clinical guide to epileptic syndromes and their treatment. Based on the ILAE classifications and pratice parameters guilines. 2nd. Springer; 2010. p. 513–7 Panayiotopoulos CP. Syndromes of idiopathic generalized epilepsies not recognized by the international league against epilepsy. Epilepsia. (2005) 46(Suppl. 9):57–66. doi: 10.1111/j.1528-1167.2005.00314.x Viravan S, Go C, Ochi A, Akiyama T, Carter Snead 3rd O, Otsubo H. Jeavons syndrome existing as occipital cortex initiating generalized epilepsy. Epilepsia. (2011) 52:1273–9. doi: 10.1111/j.1528-1167.2011.03068.x
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