Skip to main content

Epidemiology of Epilepsy in Persons with Intellectual Disabilities

  • Chapter

The epilepsies represent one of the most significant groups of conditions that may be associated with intellectual disabilities (ID). There is a general consensus that the incidence (new cases arising in a population in a period of time) and prevalence (the number of people in a population with the diagnosis at a given point in time) of seizure disorders in people with ID are greatly increased compared to the general population, and that the spectrum of epilepsy syndromes and seizure types represented are more refractory and difficult to treat. However, such generalized statements disguise a complex and varied range of presentations, and this chapter will attempt to disentangle some of these relationships in the present state of knowledge. When interpreting the results of studies it is important to remember that not all epileptologists are specialists in the psychiatry of ID, and not all researchers who work in the ID field are epilepsy specialists. Learning disabilities and seizure disorders may be defined or classified in different ways in different studies, and there may be geographical variations in presentation for various reasons. Also, other factors, including developments in prenatal screening, obstetrics, and postnatal care, may yet have a significant impact on the epidemiology of epilepsy in the ID population.

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

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   109.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. . Internationl Statistical Classification of Disease and Related Health Problems. Tenth Revision. (1992) Geneva World Health Organization.

    Google Scholar 

  2. American Psychiatric Association. (1994) Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition. Washington, DC, American Psychiatric Association.

    Google Scholar 

  3. Brown SW (2003) Towards definitions: Learning disability, mental handicap and intelligence. In Trimble MR (ed) Learning disability and epilepsy: An integrative approach. Clarius Press, London, pp. 1-16.

    Google Scholar 

  4. Beange OH, Taplin JE. (1996) Prevalence of intellectual disability in northern Sydney adults. J Intellect Disabil Res 40: 191-7.

    Article  PubMed  Google Scholar 

  5. Bhasin TK, Brocksen S, Avchen RN et al. (2006) Prevalence of four developmental disabilities among children aged 8 years — Metropolitan Atlanta Developmental Disabilities Surveillance Program, 1996 and 2000. MMWR — Morbidity & Mortality Weekly Report. 55 (SS-1):1-9.

    Google Scholar 

  6. Anonymous. (1996) State-specific rates of mental retardation— United States, 1993. MMWR — Morbidity & Mortality Weekly Report. 45: 61-5.

    Google Scholar 

  7. McConkey R, Mulvany F, Barron S. (2006) Adult persons with intellectual disabilities on the island of Ireland. J Intellect Disabil Res. 50: 227-36.

    Article  CAS  PubMed  Google Scholar 

  8. Valuing people: A new strategy for learning disability for the 21st century. (2001) London, The Stationery Office.

    Google Scholar 

  9. Hauser WA, Hesdorffer DC. (1990) Epilepsy: Frequency, causes and consequences. Demos Press. New York:

    Google Scholar 

  10. Hauser WA, Kurland LT. (1975) The epidemiology of epilepsy in Rochester Minnesota 1935 through 1967. Epilepsia 16: 1-66.

    Article  CAS  PubMed  Google Scholar 

  11. Joint Epilepsy Council. (2005) Epilepsy prevalence, incidence and other statistics. Joint Epilepsy Council, Leeds.

    Google Scholar 

  12. EUCARE. (2003) European White Paper on Epilepsy. Epilepsia. 44 Suppl 6: 1 - 88.

    Google Scholar 

  13. Illingworth RS. (1959) Convulsions in mentally retarded children with or without cerebral palsy. J Ment Defic Res. 3: 88-93

    Google Scholar 

  14. Iavanainen M. (1974) A study of origins of mental retardation, Heinemann London.

    Google Scholar 

  15. Corbett JA. (1979) Epilepsy and the electroencephalogram in early childhood psychoses. In: Wing JK (ed), Handbook of Psychiatry, Vol. 3. Cambridge University Press. Cambridge:

    Google Scholar 

  16. Corbett JA. (1988) Epilepsy and mental handicap. In: Laidlaw J, Richens A, Oxley J (eds) . A textbook of epilepsy, 3rd ed. Churchill Livingstone, Edinburgh: 533-8.

    Google Scholar 

  17. Corbett JA. (1983) Epilepsy and mental retardation. In: Parsonage M (ed). Proceedings of the 6th International Symposium on Epilepsy, Raven Press, London. New York, 207-14.

    Google Scholar 

  18. Richardson SA, Koller H, Katz M et al. (1981) A functional classification of seizures and its distribution in a mentally retarded population. Am J Ment Defic 815: 457-66.

    Google Scholar 

  19. Goulden KJ, Shinnar S, Koller H, et al. (1991) Epilepsy in children with mental retardation: A cohort study. Epilepsia 32: 690-7.

    Article  CAS  PubMed  Google Scholar 

  20. Forsgren L, Edvinsson SO, Blomquist HK, et al. (1990) Epilepsy in a population of mentally retarded children and adults. Epilepsy Res 6: 234-48.

    Article  CAS  PubMed  Google Scholar 

  21. Steffenburg U, Hagberg G, Viggedal G, et al. (1995) Active epilepsy in mentally retarded children. I. Prevalence and additional neuro-impairments. Acta Paediatrica 84: 1147-52.

    Article  CAS  PubMed  Google Scholar 

  22. Steffenburg U, Hagberg G, Kyllerman M (1996) Characteristics of seizures in a populationbased series of mentally retarded children with active epilepsy. Epilepsia 37: 850-6.

    Article  CAS  PubMed  Google Scholar 

  23. Sillanpää M. (1999) Definitions and epidemiology. In: Sillanpää M, Gram L, Johannessen SI, Tomson T (eds). Epilepsy and mental retardation. UK, Wrightson Biomedical Publishing, Petersfield, Hampshire, 1-6.

    Google Scholar 

  24. Lhatoo SD, Sander JWAS. (2001) The epidemiology of epilepsy and learning disability. Epilepsia. 42 (Suppl 1): 6-9.

    Article  PubMed  Google Scholar 

  25. Cockerell OC, Johnson A, Sander JWAS, et al. (1994) Mortality from epilepsy: Results from a prospective population-based study. Lancet 344: 918-21.

    Article  CAS  PubMed  Google Scholar 

  26. Hanna N J, Black M, Sander JWS, et al. (2002) The National Sentinel Clinical Audit of Epilepsy-Related Death: Epilepsy — death in the shadows. The Stationery Office. London,

    Google Scholar 

  27. Walczak TS, Hauser WA, Leppik IE, et al. ( 1998) I ncidence and risk factors for sudden unexpected death in epilepsy: A prospective cohort study. Neurology 50 [Suppl 4]: 443-4.

    Google Scholar 

  28. Nashef L, Fish DR, Garner S et al. (1995) Sudden death in epilepsy: A study of incidence in a young cohort with epilepsy and learning disability. Epilepsia 36: 1187-94.

    Article  CAS  PubMed  Google Scholar 

  29. Walczak T. (2002) Sudden unexpected death in epilepsy: An update. In: Devinsky O, Westbrook LE (eds). Epilepsy and developmental disabilities, Butterworth Heinemann, Boston: 41-7.

    Google Scholar 

  30. Brown S, Sullivan H, Hooper M (2004) Reduction of epilepsy-related mortality in a population with intellectual disability (ID). J Intellect Disabil Res 48: 340.

    Article  Google Scholar 

  31. Krishnamoorthy ES. ( 2002) Neuropsychiatric disorders in epilepsy: Epidemiology and clas- sification. In: Trimble MR, Schmitz EB (eds). The neuropsychiatry of epilepsy. Cambridge University Press, Cambridge, UK: 5-17.

    Google Scholar 

  32. Kerker BD. Owens PL. Zigler E, et al. (2004) Mental health disorders among individuals with mental retardation: Challenges to accurate prevalence estimates. Public Health Reports 119: 409-17.

    Article  PubMed  Google Scholar 

  33. Krishnamoorthy ES. (2003) Neuropsychiatric epidemiology at the interface between learning disability and epilepsy. In: Trimble MR (ed). Learning disability and epilepsy: An integrative approach. Clarius Press, London: 17-26.

    Google Scholar 

  34. Steffenburg S, Gillberg C, Steffenburg U. (1996) Psychiatric disorders in children and adolescents with mental retardation and active epilepsy. Arch Neurol 53: 904-12.

    CAS  PubMed  Google Scholar 

  35. Lund J. (1985) Epilepsy and psychiatric disorder in mentally retarded adults. Acta Psychiatrica Scandinavica 72: 557-62.

    Article  CAS  PubMed  Google Scholar 

  36. Lund J. (1985) The prevalence of psychiatric morbidity in mentally retarded adults. Acta Psychiatrica Scandinavica 72: 563-70.

    Article  CAS  PubMed  Google Scholar 

  37. Bax MCO. (1964) Terminology and classification of cerebral palsy. Dev Med Child Neurology 6: 295-7.

    CAS  Google Scholar 

  38. Aicardi J (1990) Epilepsy in brain-injured children. Dev Med Child Neurol 32: 191-202.

    CAS  PubMed  Google Scholar 

  39. Kudrjavcev T, Schoenberg BS, Kurland LT, et al. (1985) Cerebral palsy: Survival rates, associated handicaps, and distribution by clinical subtype (Rochester, MN, 1950 -1976). Neurology 35: 900-3.

    CAS  PubMed  Google Scholar 

  40. Delgado MR, Riela AR, Mills J, et al. (1996) Discontinuation of antiepileptic drug treatment after two seizure-free years in children with cerebral palsy. Pediatrics 97: 192-197.

    CAS  PubMed  Google Scholar 

  41. Corbett JA, Harris R, Robinson R. (1975) Epilepsy. In: Wortis J (ed). Mental retardation and developmental disabilities, Vol VII. Raven Press, New York: 79 - 111.

    Google Scholar 

  42. Arpino C, Curatolo P, Stazi MA, et al. ( 1999) Differing risk factors for cerebral palsy in the presence of mental retardation and epilepsy. J Child Neurol 14: 151-5.

    Article  CAS  PubMed  Google Scholar 

  43. D’Amelio M, Shinnar S, Hauser WA (2002) Epilepsy in children with mental retardation and cerebral palsy. In: Devinsky O, Westbrook LE (eds). Epilepsy and developmental disabilities. Butterworth-Heinemann, Woburn, MA: 3 - 16.

    Google Scholar 

  44. McDermott S, Moran R, Platt T, et al. (2005) Prevalence of epilepsy in adults with mental retardation and related disabilities in primary care. Am J Ment Retard 110: 48-56.

    Article  PubMed  Google Scholar 

  45. Webb TP, Bundey SE, Thake AI, et al. (1986) Population incidence and segregation ratios in the Martin-Bell syndrome. Am J Med Genet 23: 573-80.

    Article  CAS  PubMed  Google Scholar 

  46. Vries BB, de Ouweland AM, van den Mohkamsing S et al. (1997) Screening and diagnosis for the fragile X syndrome among the mentally retarded: An epidemiological and psychological survey. Collaborative Fragile X Study Group. Am J Human Genetics 61: 660-7.

    Article  Google Scholar 

  47. Wisniewski KE, Segan SM, Miezejeski CM, et al. (1991) The Fra(X) syndrome: Neurological, electrophysiological, and neuropathological abnormalities. Am J Med Genet 38: 476-80.

    Article  CAS  PubMed  Google Scholar 

  48. Musumeci SA, Hagerman RJ, Ferri R, et al. (1999) Epilepsy and EEG findings in males with fragile X syndrome. Epilepsia 40: 1092-1099.

    Article  CAS  PubMed  Google Scholar 

  49. Gobbi G, Genton P, Pini A, et al. (2005) Epilepsies and chromosomal disorders. In: Roger J, Bureau M, Dravet C et al. (eds). Epileptic syndromes in infancy childhood and adolescence, 4t h ed., John Libbey & Co Ltd., Eastleigh, UK: 467 -92.

    Google Scholar 

  50. Hagberg B (ed). (1993) Rett Syndrome — Clinical & Biological Aspects. McKeith Press. London:

    Google Scholar 

  51. Stafstrom. CE (1999) Mechanism of epilepsy in mental retardation: Insights from Angelman syndrome, Down syndrome and fragile X syndrome. In: Sillanpää M, Gram L, Johannessen SI, Tomson T (eds). Epilepsy and mental retardation. Wrightson Biomedical Publishing, Petersfield, Hampshire, UK: 7-41.

    Google Scholar 

  52. Matsumoto A, Kumagai T, Miura K et al. (1992) Epilepsy in Angelman syndrome associated with chromosome 15q deletion. Epilepsia 33: 1083-1090.

    Article  CAS  PubMed  Google Scholar 

  53. Guerrini R, Lorey TM, De Bonanni P, et al. (1996) Cortical myoclonus in Angelman syndrome. Ann Neurol 40: 39- 48.

    Article  CAS  PubMed  Google Scholar 

  54. Minassian BA, DeLorey TM, Olsen RW, et al. (1998) Angelman syndrome: Correlations between epilepsy phenotypes and genotypes. Ann Neurol 43: 485-93.

    Article  CAS  PubMed  Google Scholar 

  55. Kumada T, Ito M, Miyajima T, et al. (2005) Multi-institutional study on the correlation between chromosomal abnormalities and epilepsy. Brain Dev —NPN 27: 127-34.

    Article  Google Scholar 

  56. Butler JV, Whittington JE, Holland AJ. (2002) Prevalence of, and risk factors for, physical illhealth in people with Prader-Willi syndrome: A population-based study. Dev Med Child Neurol 44: 248-55.

    Article  CAS  PubMed  Google Scholar 

  57. Shapira NA, Lessig MC, Murphy TK, et al. (2002) Topiramate attenuates self-injurious behavior in Prader-Willi Syndrome. Inter J Neuropsychopharmacol 5: 141-5.

    CAS  Google Scholar 

  58. Smathers SA, Wilson JG, Nigro MA, et al. (2003) Topiramate effectiveness in Prader-Willi syndrome. Pediatr Neurol 28: 130-3.

    Article  PubMed  Google Scholar 

  59. Shapira NA, Lessig MC, Lewis MH, et al. (2004) Effects of topiramate in adults with PraderWilli syndrome. Am J Ment Retard 109: 301-9

    Article  PubMed  Google Scholar 

  60. Battaglia A, Battaglia A. (2005) The inv dup(15) or idic(15) syndrome: A clinically recognisable neurogenetic disorder. Brain and Development. 27: 365-9.

    Article  PubMed  Google Scholar 

  61. Ahlsén G, Gillberg IC, Lindblom R, et al. (1994) Tuberous sclerosis in Western Sweden. A population study of cases with early childhood onset. Arch Neurology. 51: 76-81.

    Google Scholar 

  62. Hunt A. (1993) Development, behaviour and seizures in 300 cases of tuberous sclerosis. J Intellect Disabil Res 37: 41-51.

    PubMed  Google Scholar 

  63. Gillberg C. (1995) Clinical child neuropsychiatry. Cambridge University Press. Cambridge, UK:

    Google Scholar 

  64. Riikonen R, Simell O. (1990) Tuberous sclerosis and infantile spasms. Dev Med Child Neurol 32: 203-9.

    CAS  PubMed  Google Scholar 

  65. Guerreiro MM, Andermann F, Andermann E, et al. (1998) Surgical treatment of epilepsy in tuberous sclerosis: strategies and results in 18 patients. Neurology 51: 1263-9.

    CAS  PubMed  Google Scholar 

  66. Stafstrom CE, Patxot OF, Gilmore HE, et al. (1991) Seizures in children with Down syndrome: Etiology, characteristics and outcome. Dev Med Child Neurol 33: 191-200.

    Article  CAS  PubMed  Google Scholar 

  67. Lazarowski A, Lubieniecki F, Camarero S, et al. (2006). New proteins configure a brain drug resistance map in tuberous sclerosis. Pediatr Neurol 34: 20-4.

    Article  PubMed  Google Scholar 

  68. Riikonen R, Donner M. (1979) Incidence and etiology of infantile spasms from 1960 to 1976: A population study in Finland. Dev Med Child Neurol 21: 333-43.

    Article  CAS  PubMed  Google Scholar 

  69. Guerrini R, Genton P, Bureau M, et al. (1990) Reflex seizures are frequent in patients with Down syndrome and epilepsy. Epilepsia 31: 406-17.

    Article  CAS  PubMed  Google Scholar 

  70. Brown SW. (2002) Epilepsy. In: Prasher VP, Janicki MP, (eds) Physical health of adults with intellectual disabilities. Blackwell Publishing; Oxford: 133-59.

    Chapter  Google Scholar 

  71. Pueschel SM, Louis S. McKnight P. (1991) Seizure disorders in Down syndrome. Arch Neurol 84: 318-20.

    Google Scholar 

  72. Lai F, Williams RS. (1989) A prospective study of Alzheimer disease in Down syndrome. Arch Neurol 46: 849-53.

    CAS  PubMed  Google Scholar 

  73. Evenhuis HM. (1990) The natural history of dementia in Down’s syndrome. Arch Neurol 47: 263-7.

    CAS  PubMed  Google Scholar 

  74. Genton P, Paglia G. (1994) É pilepsie myoclonique s é nile (senile myoclonic epilepsy). Epilepsies 6: 5 - 11.

    Google Scholar 

  75. Sabers A, (1999) Epilepsy in Down Syndrome. In: Sillanpaa M, Gram L, Johannessen SI, Tomsom T (eds). Epilepsy & mental retardation. Wrightson Biomedical Publishing, Petersfield, Hampshire, UK: 41 - 5.

    Google Scholar 

  76. Glauser TA, Morita DA. (2002) Lennox-Gastaut syndrome. In: Devinsky O, Westbrook LE (eds). Epilepsy and developmental disabilities. Butterworth-Heinemann, Woburn, MA: 65 - 78.

    Google Scholar 

  77. Trevathan E, Murphy CC, Yeargin-Allsopp M. (1997) Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia 38: 1283-8.

    Article  CAS  PubMed  Google Scholar 

  78. Jenkins LK, Brown SW. (1990) The value of syndrome classification in childhood epilepsy. Acta Neurologica Scandinavica 82 (Suppl) 133: - 10.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer Science + Business Media, LLC

About this chapter

Cite this chapter

Brown, S.W. (2008). Epidemiology of Epilepsy in Persons with Intellectual Disabilities. In: Prasher, V.P., Kerr, M.P. (eds) Epilepsy and Intellectual Disabilities. Springer, London. https://doi.org/10.1007/978-1-84800-259-3_3

Download citation

  • DOI: https://doi.org/10.1007/978-1-84800-259-3_3

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84800-258-6

  • Online ISBN: 978-1-84800-259-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics