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Jeavons-Syndrom

Jeavons syndrome

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Zusammenfassung

Das Syndrom der Augenlidmyoklonien mit Absencen (Jeavons-Syndrom) ist durch augenschlussinduzierte Lidmyoklonien mit oder ohne Absencen, begleitende generalisierte EEG-Paroxysmen und Fotosensibilität charakterisiert. Schwierigkeiten in der Diagnosefindung bereiten die Abgrenzung einfacher Lidmyoklonien als Anfallsform bei idiopathisch generalisierten Epilepsien und eine veränderte Fotosensitivität unter antikonvulsiver Medikation. Es werden anhand zweier klinischer Beispiele die typischen Befunde des Jeavons-Syndroms dargestellt und differenzialdiagnostische Schwierigkeiten aufgezeigt.

Abstract

Eyelid myoclonia with absences (Jeavons syndrome) is characterized by eye closure-induced eyelid myoclonias with or without absences, generalized EEG paroxysms, and photosensitivity. Simple eyelid myoclonias as seizure semiology in other idiopathic generalized epilepsies and the loss of photosensitivity under antiepileptic medication complicate the diagnosis. The history and clinical features of two patients with Jeavons syndrome are described and difficulties with the differential diagnosis are discussed.

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Literatur

  1. Hall A (1945) The origin and purpose of blinking. Br J Ophthalmol 29(9):445–467

    Article  CAS  PubMed  Google Scholar 

  2. Duncan JS, Panayiotopoulos C (1996) Eyelid myoclonia with absences: the symptoms. In: Duncan JS, Panayiotopoulos C (Hrsg) Eyelid myoclonia with absences. Libbey, London, S 17–26

  3. Engel J (2001) A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia 42(6):796–803

    Article  PubMed  Google Scholar 

  4. Covanis A (2004) Eyelid myoclonia and absences. In: Delgado-Escueta AV, Guerrini R, Bureau M (Hrsg) Myoclonic epilepsies. Advances in neurology. Lippincott Williams & Wilkins, Philadelphia, S 185–196

  5. Panayiotopoulos C (2005) Jeavons syndrome (eyelid myoclonia with absences). In: Panayiotopoulos CP (Hrsg) The epilepsies: seizures, syndromes and management. Bladon, Oxfordshire, S 475–480

  6. Panayiotopoulos C, Giannakodimos S, Agathonikou A, Koutroumanidis M (1996) Eyelid myoclonia is not a manoeuvre for self-induced seizures in eyelid myoclonia with absences. In: Duncan JS, Panayiotopoulos C (Hrsg) Eyelid myoclonia with absences. Libbey, London, S 93–106

  7. Yang T, Liu Y, Liu L et al (2008) Absence status epilepticus in monozygotic twins with Jeavons syndrome. Epileptic Disord 10(3):227–230

    PubMed  Google Scholar 

  8. Scuderi C, Musumeci SA, Ferri R et al (2000) Eyelid myoclonia with absences in three subjects with mental retardation. Neurol Sci 21(4):247–250

    Article  CAS  PubMed  Google Scholar 

  9. Sevgi Demirci EB, Saygi S (2006) Unusual features in eyelid myoclonia with absences: a patient with mild mental retardation and background slowing on electroencephalography. Epilepsy Behav 8(2):442–445

    Article  Google Scholar 

  10. Ogura K, Maegaki Y, Koeda T (2005) EEG evaluation of fixation-off sensitivity in eyelid myoclonia with absences. Pediatr Neurol 33(2):142–145

    Article  PubMed  Google Scholar 

  11. Caraballo RH, Fontana E, Darra F et al (2009) A study of 63 cases with eyelid myoclonia with or without absences: type of seizure or an epileptic syndrome? Seizure 18(6):440–445

    Article  PubMed  Google Scholar 

  12. Capovilla G, Striano P, Gambardella A et al (2009) Eyelid fluttering, typical EEG pattern, and impaired intellectual function: a homogeneous epileptic condition among the patients presenting with eyelid myoclonia. Epilepsia 50(6):1536–1541

    Article  PubMed  Google Scholar 

  13. Giannakodimos S, Panayiotopoulos CP (1996) Eyelid myoclonia with absences in adults: a clinical and video-EEG study. Epilepsia 37(1):36–44

    Article  CAS  PubMed  Google Scholar 

  14. Panayiotopoulos CP (2001) Treatment of typical absence seizures and related epileptic syndromes. Paediatr Drugs 3(5):379–403

    Article  CAS  PubMed  Google Scholar 

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Danksagung

Ein besonderer Dank gilt unseren Kolleginnen und Kollegen aus dem Epilepsiezentrum Bethel, insbesondere Frau Stutterheim, Herrn Specht, Herrn Schoendienst und Herrn Woermann.

Interessenkonflikt

Die Autoren geben an, dass kein Interessenkonflikt besteht.

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Authors and Affiliations

  1. Epilepsiezentrum Bethel/Klinik Mara, Maraweg 21, 33617, Bielefeld, Deutschland

    T. Cloppenborg, G. Classen, E. Korn-Merker, T. Polster & A. Ebner

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  1. T. Cloppenborg
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  2. G. Classen
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  3. E. Korn-Merker
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  4. T. Polster
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  5. A. Ebner
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Corresponding author

Correspondence to T. Cloppenborg.

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Cloppenborg, T., Classen, G., Korn-Merker, E. et al. Jeavons-Syndrom. Z. Epileptol. 23, 244–248 (2010). https://doi.org/10.1007/s10309-010-0124-z

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  • DOI: https://doi.org/10.1007/s10309-010-0124-z

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