Skip to main content

Photosensitivity in Epilepsy Syndromes: Age Differences?

  • Chapter
  • First Online:
The Importance of Photosensitivity for Epilepsy

Abstract

The presence of paroxysmal abnormalities of brain electrical activity provoked by intermittent photic stimulation, is an age-dependent EEG pattern usually first seen in childhood and adolescence. Indirect evidence suggests that this maps onto the time-course of the maturation of excitatory and inhibitory brain systems. Recent studies have confirmed the role of abnormal gating mechanisms in the abnormal susceptibility of the posterior cortex to visual stimuli in young patients with photosensitive epilepsy. Similarly, the later maturation of the parvocellular systems which has been associated with the photoparoxysmal EEG response might account for the low prevalence of photosensitivity in the very young patients. From the clinical perspective, while photosensitivity has a preferential onset in pediatric age, it tends to persist in adulthood, with two-thirds of the patients continuing to show the EEG trait even though 85% of them will no longer experience overt clinical seizures. The data presented in this chapter give an account of the role of maturational progression in the manifestations of photosensitivity at mesoscopic level and their integration with genetic data might provide a clearer account of the variability in genotype/phenotype association seen in photosensitive patients.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Bickford RG, Sem-Jacobsen CW, White PT, Daly D. Some observations on the mechanism of photic and photo-metrazol activation. Electroencephalogr Clin Neurophysiol. 1952;4:275–82.

    Article  CAS  PubMed  Google Scholar 

  2. Wilkins AJ, Darby CE, Binnie CD. Neurophysiological aspects of pattern-sensitive epilepsy. Brain. 1979;102:1–25.

    Article  CAS  PubMed  Google Scholar 

  3. Kasteleijn-Nolst Trenité DG. Provoked and reflex seizures: surprising or common? Epilepsia. 2012;53:105–13.

    Article  PubMed  Google Scholar 

  4. Guerrini R, Genton P. Epileptic syndromes and visually induced seizures. Epilepsia. 2004;45(Suppl 1):14–8.

    Article  PubMed  Google Scholar 

  5. Waltz S, Christen HJ, Doose H. The different patterns of the photoparoxysmal response--a genetic study. Electroencephalogr Clin Neurophysiol. 1992;83:138–45.

    Article  CAS  PubMed  Google Scholar 

  6. Kasteleijn-Nolst Trenité DG, Guerrini R, Binnie CD, Genton P. Visual sensitivity and epilepsy: a proposed terminology and classification for clinical and EEG phenomenology. Epilepsia. 2001;42:692–701.

    Article  PubMed  Google Scholar 

  7. Harding GFA, Jeavons PM. Photosensitive epilepsy. London: Mac Keith Press; 1994.

    Google Scholar 

  8. Wolf P, Goosses R. Relation of photosensitivity to epileptic syndromes. J Neurol Neurosurg Psychiatry. 1986;49:1386–91.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Verrotti A, Basciani F, Trotta D, Cutarella R, Salladini C, Morgese G, Chiarelli F. Photoparoxysmal responses in non-epileptic children in long-term follow-up. Acta Neurol Scand. 2002;105:400–2.

    Article  CAS  PubMed  Google Scholar 

  10. Harding GFA, Edson A, Jeavons PM. Persistence of photosensitivity. Epilepsia. 1997;38:663–9.

    Article  CAS  PubMed  Google Scholar 

  11. Koutroumanidis M, Tsirka V, Panayiotopoulos C. Adult-onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy. Epileptic Disord. 2015;17:275–86.

    Article  PubMed  Google Scholar 

  12. Scott DF, Elian M. The effects on the EEG of intermittent photic stimulation in patients over 30 years of age. Clin Electroencephalogr. 1981;12:21–3.

    Article  CAS  PubMed  Google Scholar 

  13. Siniatchkin M, Groppa S, Jerosch B, Muhle H, Kurth C, Shepherd AJ, Siebner H, Stephani U. Spreading photoparoxysmal EEG response is associated with an abnormal cortical excitability pattern. Brain. 2007;130:78–87.

    Article  PubMed  Google Scholar 

  14. Porciatti V, Bonanni P, Fiorentini A, Guerrini R. Lack of cortical contrast gain control in human photosensitive epilepsy. Nat Neurosci. 2000;3:259.

    Article  CAS  PubMed  Google Scholar 

  15. Brazzo D, Di Lorenzo G, Bill P, Fasce M, Papalia G, Veggiotti P, Seri S. Abnormal visual habituation in pediatric photosensitive epilepsy. Clin Neurophysiol. 2011;122:16–20.

    Article  CAS  PubMed  Google Scholar 

  16. Harding GFA, Fylan F. Two visual mechanisms of photosensitivity. Epilepsia. 2005;40:1446–51.

    Article  Google Scholar 

  17. Kovács I. Human development of perceptual organization. Vis Res. 2000;40:1301–10.

    Article  PubMed  Google Scholar 

  18. Lu Y, Waltz S, Stenzel K, Muhle H, Stephani U. Photosensitivity in epileptic syndromes of childhood and adolescence. Epileptic Disord. 2008;10:136–43.

    PubMed  Google Scholar 

  19. Obeid T, Panayiotopoulos CP. Juvenile myoclonic epilepsy: a study in Saudi Arabia. Epilepsia. 1988;29:280–2.

    Article  CAS  PubMed  Google Scholar 

  20. Appleton R, Beirne M, Acomb B. Photosensitivity in juvenile myoclonic epilepsy. Seizure. 2000;9:108–11.

    Article  CAS  PubMed  Google Scholar 

  21. Shiraishi H, Fujiwara T, Inoue Y, Yagi K. Photosensitivity in relation to epileptic syndromes: a survey from an epilepsy center in Japan. Epilepsia. 2001;42:393–7.

    Article  CAS  PubMed  Google Scholar 

  22. Dravet C, Bureau M. The benign myoclonic epilepsy of infancy (author’s transl). Rev d'Electroencephalogr Neurophysiol Clin. 1981;11:438–44.

    Article  CAS  Google Scholar 

  23. Auvin S, Pandit F, De Bellecize J, Badinand N, Isnard H, Motte J, Villeneuve N, Lamblin MD, Vallee L. Benign myoclonic epilepsy in infants: electroclinical features and long-term follow-up of 34 patients. Epilepsia. 2006;47:387–93.

    Article  PubMed  Google Scholar 

  24. Turco EC, Pavlidis E, Facini C, Spagnoli C, Andreolli A, Geraci R, Pisani F. Reflex myoclonic epilepsy of infancy: seizures induced by tactile stimulation. J Pediatr. 2016;173:250–253.e4.

    Article  PubMed  Google Scholar 

  25. Capovilla G, Beccaria F, Gambardella A, Montagnini A, Avantaggiato P, Seri S. Photosensitive benign myoclonic epilepsy in infancy. Epilepsia. 2007;48:96–100.

    Article  PubMed  Google Scholar 

  26. Ricci S, Cusmai R, Fusco L, Vigevano F. Reflex myoclonic epilepsy in infancy: a new age-dependent idiopathic epileptic syndrome related to startle reaction. Epilepsia. 1995;36:342–8.

    Article  CAS  PubMed  Google Scholar 

  27. Panayiotopoulos CP. Reflex seizures and related epileptic syndromes. London: Springer; 2012.

    Book  Google Scholar 

  28. Doose H. Myoclonic-astatic epilepsy. Epilep Res Suppl. 1992;6:163–8.

    CAS  Google Scholar 

  29. Oguni H, Fukuyama Y, Tanaka T, Hayashi K, Funatsuka M, Sakauchi M, Shirakawa S, Osawa M. Myoclonic-astatic epilepsy of early childhood—clinical and EEG analysis of myoclonic-astatic seizures, and discussions on the nosology of the syndrome. Brain Dev. 2001;23:757–64.

    Article  CAS  PubMed  Google Scholar 

  30. Kelley SA, Kossoff EH. Doose syndrome (myoclonic–astatic epilepsy): 40 years of progress. Dev Med Child Neurol. 2010;52:988–93.

    Article  PubMed  Google Scholar 

  31. Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, Van Emde Boas W, Engel J, French J, Glauser TA, Mathern GW, Moshé SL, Nordli D, Plouin P, Scheffer IE. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE commission on classification and terminology, 2005–2009. Epilepsia. 2010;51:676–85.

    Article  PubMed  Google Scholar 

  32. Covanis A. Photosensitivity in idiopathic generalized epilepsies. Epilepsia. 2005;46:67–72.

    Article  PubMed  Google Scholar 

  33. Grosso S, Galimberti D, Vezzosi P, Farnetani M, Di Bartolo RM, Bazzotti S, Morgese G, Balestri P. Childhood absence epilepsy: evolution and prognostic factors. Epilepsia. 2005;46:1796–801.

    Article  PubMed  Google Scholar 

  34. Covanis A, Skiadas K, Loli N, Lada C, Theodorou V. Absence epilepsy: early prognostic signs. Seizure Eur J Epilep. 1992;1:281–9.

    Article  CAS  Google Scholar 

  35. Taylor I, Berkovic SF, Scheffer IE. Genetics of epilepsy syndromes in families with photosensitivity. Neurology. 2013;80:1322–9.

    Article  PubMed  Google Scholar 

  36. Jeavons PM. Nosological problems of myoclonic epilepsies in childhood and adolescence. Dev Med Child Neurol. 1977;19:3–8.

    Article  CAS  PubMed  Google Scholar 

  37. Galizia EC, Myers CT, Leu C, De Kovel CGF, Afrikanova T, Cordero-Maldonado ML, Martins TG, Jacmin M, Drury S, Krishna Chinthapalli V, Muhle H, Pendziwiat M, Sander T, Ruppert A-K, Møller RS, Thiele H, Krause R, Schubert J, Lehesjoki A-E, Nürnberg P, Lerche H, For the Euro, ECC, Palotie A, Coppola A, Striano S, Gaudio LD, Boustred C, Schneider AL, Lench N, Jocic-Jakubi B, Covanis A, Capovilla G, Veggiotti P, Piccioli M, Parisi P, Cantonetti L, Sadleir LG, Mullen SA, Berkovic SF, Stephani U, Helbig I, Crawford AD, Esguerra CV, Kasteleijn-Nolst Trenité DGA, Koeleman BPC, Mefford HC, Scheffer IE, Sisodiya SM. CHD2 variants are a risk factor for photosensitivity in epilepsy. Brain. 2015;138:1198–208.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Viravan S, Go C, Ochi A, Akiyama T, Snead OC, Otsubo H. Jeavons syndrome existing as occipital cortex initiating generalized epilepsy. Epilepsia. 2011;52:1273–9.

    Article  PubMed  Google Scholar 

  39. Striano S, Capovilla G, Sofia V, Romeo A, Rubboli G, Striano P, Kasteleijn-Nolst Trenité D. Eyelid myoclonia with absences (Jeavons syndrome): a well-defined idiopathic generalized epilepsy syndrome or a spectrum of photosensitive conditions? Epilepsia. 2009;50:15–9.

    Article  PubMed  Google Scholar 

  40. Takahashi S, Yamamoto S, Tanaka R, Okayama A, Araki A, Azuma H. Focal frontal epileptiform discharges in a patient with eyelid myoclonia and absence seizures. Epilepsy Behav Case Rep. 2015;4:35–7.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Guerrini R, Dravet C, Genton P, Bureau M, Bonanni P, Ferrari Anna R, Roger J. Idiopathic photosensitive occipital lobe epilepsy. Epilepsia. 1995;36:883–91.

    Article  CAS  PubMed  Google Scholar 

  42. Janz D. Epilepsy with impulsive petit mal (juvenile myoclonic epilepsy). Acta Neurol Scand. 1985;72:449–59.

    Article  CAS  PubMed  Google Scholar 

  43. Bauer PR, Gorgels K, Spetgens W, Van Klink NEC, Leijten FSS, Sander JW, Visser GH, Zijlmans M. The topographical distribution of epileptic spikes in juvenile myoclonic epilepsy with and without photosensitivity. Clin Neurophysiol. 2017;128:176–82.

    Article  CAS  PubMed  Google Scholar 

  44. Höfler J, Unterberger I, Dobesberger J, Kuchukhidze G, Walser G, Trinka E. Seizure outcome in 175 patients with juvenile myoclonic epilepsy—a long-term observational study. Epilepsy Res. 2014;108:1817–24.

    Article  PubMed  Google Scholar 

  45. Geithner J, Schneider F, Wang Z, Berneiser J, Herzer R, Kessler C, Runge U. Predictors for long-term seizure outcome in juvenile myoclonic epilepsy: 25–63 years of follow-up. Epilepsia. 2012;53:1379–86.

    Article  PubMed  Google Scholar 

  46. Janz D. The grand mal epilepsies and the sleeping-waking cycle. Epilepsia. 1962;3:69–109.

    Article  CAS  PubMed  Google Scholar 

  47. Andermann F, Berkovic S. The idiopathic generalized epilepsies across life. In: Hallett M, Phillips LH, Schomer DL, Massey JM, editors. Supplements to clinical neurophysiology. Amsterdam: Elsevier; 2004.

    Google Scholar 

  48. Koutroumanidis M, Aggelakis K, Panayiotopoulos CP. Idiopathic epilepsy with generalized tonic–clonic seizures only versus idiopathic epilepsy with phantom absences and generalized tonic–clonic seizures: one or two syndromes? Epilepsia. 2008;49:2050–62.

    Article  PubMed  Google Scholar 

  49. Carvill GL, Heavin SB, Yendle SC, Mcmahon JM, O’Roak BJ, Cook J, Khan A, Dorschner MO, Weaver M, Calvert S, Malone S, Wallace G, Stanley T, Bye AME, Bleasel A, Howell KB, Kivity S, Mackay MT, Rodriguez-Casero V, Webster R, Korczyn A, Afawi Z, Zelnick N, Lerman-Sagie T, Lev D, Møller RS, Gill D, Andrade DM, Freeman JL, Sadleir LG, Shendure J, Berkovic SF, Scheffer IE, Mefford HC. Targeted resequencing in epileptic encephalopathies identifies de novo mutations in CHD2 and SYNGAP1. Nat Genet. 2013;45:825.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Thomas RH, Zhang LM, Carvill GL, Archer JS, Heavin SB, Mandelstam SA, Craiu D, Berkovic SF, Gill DS, Mefford HC, Scheffer IE. CHD2 myoclonic encephalopathy is frequently associated with self-induced seizures. Neurology. 2015;84:951–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Verbeek N, Kasteleijn-Nolst Trenité D, Wassenaar M, Van Campen J, Sonsma A, Gunning WB, De Weerd A, Knoers N, Spetgens W, Gutter T, Leijten F, Brilstra E. Photosensitivity in Dravet syndrome is under-recognized and related to prognosis. Clin Neurophysiol. 2017;128:323–30.

    Article  PubMed  Google Scholar 

  52. Michelucci R, Pasini E, Riguzzi P, Andermann E, Kälviäinen R, Genton P. Myoclonus and seizures in progressive myoclonus epilepsies: pharmacology and therapeutic trials. Epileptic Disord. 2016;18:S145–53.

    Article  Google Scholar 

  53. Lehesjoki, A. & Gardiner, M. 2012. Progressive myoclonus epilepsy: Unverricht-Lundborg disease and neuronal ceroid lipofuscinoses. In: Noebels JL, Rogawski MA, et al. (ed.) Jasper’s basic mechanisms of the epilepsies. 4th edn. Bethesda, MD: National Center for Biotechnology Information (US).

    Google Scholar 

  54. Ferlazzo E, Magaudda A, Striano P, Vi N, Serra S, Genton P. Long-term evolution of EEG in Unverricht-Lundborg disease. Epilepsy Res. 2007;73:219.

    Article  PubMed  Google Scholar 

  55. Magaudda A, Ferlazzo E, Nguyen VH, Genton P. Unverricht-Lundborg disease, a condition with self-limited progression: long-term follow-up of 20 patients. Epilepsia. 2006;47:860–6.

    Article  PubMed  Google Scholar 

  56. Minassian BA. The progressive myoclonus epilepsies. In: Steinlein OK, editor. Progress in brain research. Ansterdam: Elsevier; 2014.

    Google Scholar 

  57. Specchio N, Bellusci M, Pietrafusa N, Trivisano M, Palma L, Vigevano F. Photosensitivity is an early marker of neuronal ceroid lipofuscinosis type 2 disease. Epilepsia. 2017;58:1380–8.

    Article  CAS  PubMed  Google Scholar 

  58. Albert DV, Yin H, De Los Reyes EC, Vidaurre J. Unique characteristics of the Photoparoxysmal response in patients with neuronal ceroid lipofuscinosis type 2: can Eeg be a biomarker? J Child Neurol. 2016;31:1475–82.

    Article  PubMed  Google Scholar 

  59. Xin W, Mullen TE, Kiely R, Min J, Feng X, Cao Y, O’Malley L, Shen Y, Chu-Shore C, Mole SE, Goebel HH, Sims K. CLN5 mutations are frequent in juvenile and late-onset non-Finnish patients with NCL. Neurology. 2010;74:565–71.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Stefano Seri .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Seri, S., Carr, B., Cerquiglini, A. (2021). Photosensitivity in Epilepsy Syndromes: Age Differences?. In: Kasteleijn-Nolst Trenite, D. (eds) The Importance of Photosensitivity for Epilepsy. Springer, Cham. https://doi.org/10.1007/978-3-319-05080-5_22

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-05080-5_22

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-05079-9

  • Online ISBN: 978-3-319-05080-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics