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Epidemiology of Epilepsy in Persons with Intellectual Disabilities

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Abstract

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.

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References

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

    Google Scholar 

  2. Brown SW. Towards definitions: learning disability, mental handicap and intelligence. In: Trimble MR, editor. Learning disability and epilepsy: an integrative approach. London: Clarius Press; 2003. p. 1–16.

    Google Scholar 

  3. American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 5th ed. Washington, DC: American Psychiatric Association; 2013.

    Book  Google Scholar 

  4. Disorders of intellectual development. Available from: http://id.who.int/icd/entity/605267007. Accessed Jan 2016.

  5. Beange OH, Taplin JE. Prevalence of intellectual disability in northern Sydney adults. J Intellect Disabil Res. 1996;40:191–7.

    Article  PubMed  Google Scholar 

  6. Bhasin TK, Brocksen S, Avchen RN, Van Naarden Braun K. Prevalence of four developmental disabilities among children aged 8 years–Metropolitan Atlanta Developmental Disabilities Surveillance Program, 1996 and 2000. MMWR Surveill Summ. 2006;55(1):1–9.

    PubMed  Google Scholar 

  7. Centers for Disease Control and Prevention (CDC). State-specific rates of mental retardation–United States, 1993. MMWR Morb Mortal Wkly Rep. 1996;45(3):61–5.

    Google Scholar 

  8. McConkey R, Mulvany F, Barron S. Adult persons with intellectual disabilities on the island of Ireland. J Intellect Disabil Res. 2006;50:227–36.

    Article  CAS  PubMed  Google Scholar 

  9. Department of Health. Valuing people: a new strategy for learning disability for the 21st century. London: The Stationery Office; 2001.

    Google Scholar 

  10. Hauser WA, Hesdorffer DC. Epilepsy: frequency, causes and consequences. New York: Demos Press; 1990.

    Google Scholar 

  11. Hauser WA, Kurland LT. The epidemiology of epilepsy in Rochester Minnesota 1935 through 1967. Epilepsia. 1975;16:1–66.

    Article  CAS  PubMed  Google Scholar 

  12. Joint Epilepsy Council. Epilepsy prevalence, incidence and other statistics. Leeds: Joint Epilepsy Council; 2005.

    Google Scholar 

  13. EUCARE. European white paper on epilepsy. Epilepsia. 2003;44 Suppl 6:1–88.

    Google Scholar 

  14. Illingworth RS. Convulsions in mentally retarded children with or without cerebral palsy. J Ment Defic Res. 1969;3:88–93.

    Google Scholar 

  15. Iavanainen M. A study of origins of mental retardation. London: Heinemann; 1974.

    Google Scholar 

  16. Corbett JA. Epilepsy and the electroencephalogram in early childhood psychoses. In: Wing JK, editor. Handbook of psychiatry, vol. 3. Cambridge: Cambridge University Press; 1979.

    Google Scholar 

  17. Corbett JA. Epilepsy and mental handicap. In: Laidlaw J, Richens A, Oxley J, editors. A textbook of epilepsy. 3rd ed. Edinburgh: Churchill Livingstone; 1988. p. 533–8.

    Google Scholar 

  18. Corbett JA. Epilepsy and mental retardation. In: Parsonage M, editor. Proceedings of the 6th International Symposium on Epilepsy. New York: Raven Press; 1983. p. 207–14.

    Google Scholar 

  19. Richardson SA, Koller H, Katz M, McLaren J. A functional classification of seizures and its distribution in a mentally retarded population. Am J Ment Defic. 1981;85(5):457–66.

    CAS  PubMed  Google Scholar 

  20. Goulden KJ, Shinnar S, Koller H, Katz M, Richardson SA. Epilepsy in children with mental retardation: a cohort study. Epilepsia. 1991;32(5):690–7.

    Article  CAS  PubMed  Google Scholar 

  21. Forsgren L, Edvinsson SO, Blomquist HK, Heijbel J, Sidenvall R. Epilepsy in a population of mentally retarded children and adults. Epilepsy Res. 1990;6(3):234–48.

    Article  CAS  PubMed  Google Scholar 

  22. Steffenburg U, Hagberg G, Viggedal G, Kyllerman M. Active epilepsy in mentally retarded children. I. Prevalence and additional neuro-impairments. Acta Paediatr. 1995;84(10):1147–52.

    Article  CAS  PubMed  Google Scholar 

  23. Steffenburg U, Hagberg G, Kyllerman M. Characteristics of seizures in a population-based series of mentally retarded children with active epilepsy. Epilepsia. 1996;37:850–6.

    Article  CAS  PubMed  Google Scholar 

  24. Oeseburg B, Dijkstra GJ, Groothoff JW, Reijneveld SA, Jansen DE. Prevalence of chronic health conditions in children with intellectual disability: a systematic literature review. Intellect Dev Disabil. 2011;49:59–85.

    Article  PubMed  Google Scholar 

  25. Sillanpää M. Definitions and epidemiology. In: Sillanpää M, Gram L, Johannessen SI, Tomson T, editors. Epilepsy and mental retardation. Petersfield/Hampshire: Wrightson Biomedical Publishing; 1999. p. 1–6.

    Google Scholar 

  26. Lhatoo SD, Sander JW. The epidemiology of epilepsy and learning disability. Epilepsia. 2001;42 Suppl 1:6–9.

    Article  PubMed  Google Scholar 

  27. Young C, Shankar R, Palmer J, Craig J, Hargreaves C, McLean B, et al. Does intellectual disability increase sudden unexpected death in epilepsy (SUDEP) risk? Seizure. 2015;25:112–6.

    Article  PubMed  Google Scholar 

  28. Cockerell OC, Johnson AL, Sander JW, Hart YM, Goodridge DM, Shorvon SD. Mortality from epilepsy: results from a prospective population-based study. Lancet. 1994;344:918–21.

    Article  CAS  PubMed  Google Scholar 

  29. Hanna NJ, Black M, Sander JW. The National Sentinel Clinical Audit of epilepsy-related death: epilepsy—death in the shadows. London: The Stationery Office; 2002.

    Google Scholar 

  30. Walczak TS, Hauser WA, Leppik IE. Incidence and risk factors for sudden unexpected death in epilepsy: a prospective cohort study. Neurology. 1998;50 Suppl 4:443–4.

    Google Scholar 

  31. Nashef L, Fish DR, Garner S, Sander JW, Shorvon SD. Sudden death in epilepsy: a study of incidence in a young cohort with epilepsy and learning disability. Epilepsia. 1995;36(12):1187–94.

    Article  CAS  PubMed  Google Scholar 

  32. Walczak T. Sudden unexpected death in epilepsy: an update. In: Devinsky O, Westbrook LE, editors. Epilepsy and developmental disabilities. Boston: Butterworth Heinemann; 2002. p. 41–7.

    Google Scholar 

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

    Article  Google Scholar 

  34. Vlooswijk MC, Majoie HJ, De Krom MC, Tan IY, Aldenkamp AP. SUDEP in the Netherlands: a retrospective study in a tertiary referral center. Seizure. 2007;16(2):153–9.

    Article  CAS  PubMed  Google Scholar 

  35. Kiani R, Tyrer F, Jesu A. Mortality from sudden unexpected death in epilepsy (SUDEP) in a cohort of adults with intellectual disability. J Intellect Disabil Res. 2014;58:508–20.

    Article  CAS  PubMed  Google Scholar 

  36. Cooper SA, Smiley A, Morrison J, Allan L, Williamson A, Finlayson J, et al. Psychosis and adults with intellectual disabilities. Prevalence, incidence, and related factors. Soc Psychiatry Psychiatr Epidemiol. 2007;42(7):530–6.

    Article  PubMed  Google Scholar 

  37. Krishnamoorthy ES. Neuropsychiatric disorders in epilepsy: epidemiology and classification. In: Trimble MR, Schmitz EB, editors. The neuropsychiatry of epilepsy. Cambridge, UK: Cambridge University Press; 2002. p. 5–17.

    Chapter  Google Scholar 

  38. Kerker BD, Owens PL, Zigler E, Horwitz SM. Mental health disorders among individuals with mental retardation: challenges to accurate prevalence estimates. Public Health Rep. 2004;119(4):409–17.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Krishnamoorthy ES. Neuropsychiatric epidemiology at the interface between learning disability and epilepsy. In: Trimble MR, editor. Learning disability and epilepsy: an integrative approach. London: Clarius Press; 2003. p. 17–26.

    Google Scholar 

  40. Steffenburg S, Gillberg C, Steffenburg U. Psychiatric disorders in children and adolescents with mental retardation and active epilepsy. Arch Neurol. 1996;53:904–12.

    Article  CAS  PubMed  Google Scholar 

  41. Lund J. Epilepsy and psychiatric disorder in mentally retarded adults. Acta Psychiatr Scand. 1985;72:557–62.

    Article  CAS  PubMed  Google Scholar 

  42. Lund J. The prevalence of psychiatric morbidity in mentally retarded adults. Acta Psychiatr Scand. 1985;72:563–70.

    Article  CAS  PubMed  Google Scholar 

  43. Bax MC. Terminology and classification of cerebral palsy. Dev Med Child Neurol. 1965;6:295–7.

    Article  Google Scholar 

  44. Aicardi J. Epilepsy in brain-injured children. Dev Med Child Neurol. 1990;32:191–202.

    Article  CAS  PubMed  Google Scholar 

  45. Andersen GL, Irgens LM, Haagaas I, Skranes JS, Meberg AE, Vik T. Cerebral palsy in Norway: prevalence, subtypes and severity. Eur J Paediatr Neurol. 2008;12(1):4–13.

    Article  PubMed  Google Scholar 

  46. Kudrjavcev T, Schoenberg BS, Kurland LT, Groover RV. Cerebral palsy: survival rates, associated handicaps, and distribution by clinical subtype (Rochester, MN, 1950–1976). Neurology. 1985;35(6):900–3.

    Article  CAS  PubMed  Google Scholar 

  47. Delgado MR, Riela AR, Mills J, Pitt A, Browne R. Discontinuation of antiepileptic drug treatment after two seizure-free years in children with cerebral palsy. Pediatrics. 1996;97:192–7.

    CAS  PubMed  Google Scholar 

  48. Corbett JA, Harris R, Robinson R. Epilepsy. In: Wortis J, editor. Mental retardation and developmental disabilities, vol. VII. New York: Raven Press; 1975. p. 79–111.

    Google Scholar 

  49. Arpino C, Curatolo P, Stazi MA, Pellegri A, Vlahov D. Differing risk factors for cerebral palsy in the presence of mental retardation and epilepsy. J Child Neurol. 1999;14(3):151–5.

    Article  CAS  PubMed  Google Scholar 

  50. D’Amelio M, Shinnar S, Hauser WA. Epilepsy in children with mental retardation and cerebral palsy. In: Devinsky O, Westbrook LE, editors. Epilepsy and developmental disabilities. Woburn: Butterworth-Heinemann; 2002. p. 3–16.

    Google Scholar 

  51. McDermott S, Moran R, Platt T, Wood H, Isaac T, Dasari S. Prevalence of epilepsy in adults with mental retardation and related disabilities in primary care. Am J Ment Retard. 2005;110:48–56.

    Article  PubMed  Google Scholar 

  52. Webb TP, Bundey SE, Thake AI, Todd J. Population incidence and segregation ratios in the Martin-Bell syndrome. Am J Med Genet. 1986;23:573–80.

    Article  CAS  PubMed  Google Scholar 

  53. de Vries BB, de Ouweland AM, van den Mohkamsing S, Duivenvoorden HJ, Mol E, Gelsema K, et al. Screening and diagnosis for the fragile X syndrome among the mentally retarded: An epidemiological and psychological survey. Collaborative Fragile X Study Group. Am J Hum Genet. 1997;61:660–7.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Wisniewski KE, Segan SM, Miezejeski CM, Sersen EA, Rudelli RD. The Fra(X) syndrome: neurological, electrophysiological, and neuropathological abnormalities. Am J Med Genet. 1991;38:476–80.

    Article  CAS  PubMed  Google Scholar 

  55. Musumeci SA, Hagerman RJ, Ferri R, Bosco P, Dalla Bernardina B, Tassinari CA, et al. Epilepsy and EEG findings in males with fragile X syndrome. Epilepsia. 1999;40(8):1092–9.

    Article  CAS  PubMed  Google Scholar 

  56. Berry-Kravis E, Raspa M, Loggin-Hester L, Bishop E, Holiday D, Bailey DB. Seizures in fragile X syndrome: characteristics and comorbid diagnoses. Am J Intellect Dev Disabil. 2010;115(6):461–72.

    Article  PubMed  Google Scholar 

  57. Gobbi G, Genton P, Pini A. Epilepsies and chromosomal disorders. In: Roger J, Bureau M, Dravet C, editors. Epileptic syndromes in infancy childhood and adolescence. 4th ed. Eastleigh: John Libbey & Co Ltd; 2005. p. 467–92.

    Google Scholar 

  58. Hagberg B. Rett syndrome—clinical & biological aspects. London: McKeith Press; 1993.

    Google Scholar 

  59. Cardoza B, Clarke A, Wilcox J, Gibbon F, Smith PE, Archer H, et al. Epilepsy in Rett syndrome: association between phenotype and genotype, and implications for practice. Seizure. 2011;20(8):646–9.

    Article  PubMed  Google Scholar 

  60. Stafstrom CE. 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, editors. Epilepsy and mental retardation. Petersfield/Hampshire: Wrightson Biomedical Publishing; 1999. p. 7–41.

    Google Scholar 

  61. Matsumoto A, Kumagai T, Miura K, Miyazaki S, Hayakawa C, Yamanaka T. Epilepsy in Angelman syndrome associated with chromosome 15q deletion. Epilepsia. 1992;33(6):1083–90.

    Article  CAS  PubMed  Google Scholar 

  62. Guerrini R, De Lorey TM, Bonanni P, Moncla A, Dravet C, Suisse G, et al. Cortical myoclonus in Angelman syndrome. Ann Neurol. 1996;40(1):39–48.

    Article  CAS  PubMed  Google Scholar 

  63. Minassian BA, DeLorey TM, Olsen RW, Philippart M, Bronstein Y, Zhang Q, et al. Angelman syndrome: correlations between epilepsy phenotypes and genotypes. Ann Neurol. 1998;43(4):485–93.

    Article  CAS  PubMed  Google Scholar 

  64. Kumada T, Ito M, Miyajima T, Fujii T, Okuno T, Go T, et al. Multi-institutional study on the correlation between chromosomal abnormalities and epilepsy. Brain Dev. 2005;27(2):127–34.

    Article  PubMed  Google Scholar 

  65. Butler JV, Whittington JE, Holland AJ. Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol. 2002;44:248–55.

    Article  CAS  PubMed  Google Scholar 

  66. Shapira NA, Lessig MC, Murphy TK, Driscoll DJ, Goodman WK. Topiramate attenuates self-injurious behavior in Prader-Willi Syndrome. Int J Neuropsychopharmacol. 2002;5(2):141–5.

    Article  CAS  PubMed  Google Scholar 

  67. Smathers SA, Wilson JG, Nigro MA. Topiramate effectiveness in Prader-Willi syndrome. Pediatr Neurol. 2003;28(2):130–3.

    Article  PubMed  Google Scholar 

  68. Shapira NA, Lessig MC, Lewis MH, Goodman WK, Driscoll DJ. Effects of topiramate in adults with Prader-Willi syndrome. Am J Ment Retard. 2004;109(4):301–9.

    Article  PubMed  Google Scholar 

  69. Battaglia A. The inv dup(15) or idic(15) syndrome: a clinically recognisable neurogenetic disorder. Brain Dev. 2005;27(5):365–9.

    Article  PubMed  Google Scholar 

  70. Cox DM, Butler MG. The 15q11.2 BP1-BP2 microdeletion syndrome: a review. Int J Mol Sci. 2015;16:4068–82.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  71. Cafferkey M, Ahn JW, Flinter F, Ogilvie C. Phenotypic features in patients with 15q11.2(BP1-BP2) deletion: further delineation of an emerging syndrome. Am J Med Genet A. 2014;164A(8):1916–22.

    Article  PubMed  Google Scholar 

  72. Lowther C, Costain G, Stavropoulos DJ, Melvin R, Silversides CK, Andrade DM, et al. Delineating the 15q13.3 microdeletion phenotype: a case series and comprehensive review of the literature. Genet Med. 2015;17(2):149–57.

    Article  PubMed  Google Scholar 

  73. Ahlsén G, Gillberg IC, Lindblom R, Gillberg C. Tuberous sclerosis in Western Sweden. A population study of cases with early childhood onset. Arch Neurol. 1994;51(1):76–81.

    Article  PubMed  Google Scholar 

  74. Hunt A. Development, behaviour and seizures in 300 cases of tuberous sclerosis. J Intellect Disabil Res. 1993;37(Pt 1):41–51.

    PubMed  Google Scholar 

  75. Kingswood C, Bolton P, Crawford P, Harland C, Johnson SR, Sampson JR, et al. The clinical profile of tuberous sclerosis complex (TSC) in the United Kingdom: a retrospective cohort study in the Clinical Practice Research Datalink (CPRD). Eur J Paediatr Neurol. 2016;20(2):296–308.

    Article  PubMed  Google Scholar 

  76. Gillberg C. Clinical child neuropsychiatry. Cambridge, UK: Cambridge University Press; 1995.

    Book  Google Scholar 

  77. Riikonen R, Simell O. Tuberous sclerosis and infantile spasms. Dev Med Child Neurol. 1990;32:203–9.

    Article  CAS  PubMed  Google Scholar 

  78. Guerreiro MM, Andermann F, Andermann E, Palmini A, Hwang P, Hoffman HJ, et al. Surgical treatment of epilepsy in tuberous sclerosis: strategies and results in 18 patients. Neurology. 1998;51(5):1263–9.

    Article  CAS  PubMed  Google Scholar 

  79. Lazarowski A, Lubieniecki F, Camarero S, Pomata HH, Bartuluchi MA, Sevlever G, et al. New proteins configure a brain drug resistance map in tuberous sclerosis. Pediatr Neurol. 2006;34(1):20–4.

    Article  PubMed  Google Scholar 

  80. Stafstrom CE, Patxot OF, Gilmore HE, Wisniewski KE. Seizures in children with Down syndrome: etiology, characteristics and outcome. Dev Med Child Neurol. 1991;33(3):191–200.

    Article  CAS  PubMed  Google Scholar 

  81. Riikonen R, Donner M. Incidence and etiology of infantile spasms from 1960 to 1976: a population study in Finland. Dev Med Child Neurol. 1979;21:333–43.

    Article  CAS  PubMed  Google Scholar 

  82. Guerrini R, Genton P, Bureau M, Dravet C, Roger J. Reflex seizures are frequent in patients with Down syndrome and epilepsy. Epilepsia. 1990;31(4):406–17.

    Article  CAS  PubMed  Google Scholar 

  83. Brown SW. Epilepsy. In: Prasher VP, Janicki MP, editors. Physical health of adults with intellectual disabilities. Oxford: Blackwell Publishing; 2002. p. 133–59.

    Google Scholar 

  84. Pueschel SM, Louis S, McKnight P. Seizure disorders in Down syndrome. Arch Neurol. 1991;84:318–20.

    Article  Google Scholar 

  85. Lai F, Williams RS. A prospective study of Alzheimer disease in Down syndrome. Arch Neurol. 1989;46:849–53.

    Article  CAS  PubMed  Google Scholar 

  86. Evenhuis HM. The natural history of dementia in Down’s syndrome. Arch Neurol. 1990;7:263–7.

    Article  Google Scholar 

  87. Genton P, Paglia G. Épilepsie myoclonique sénile (senile myoclonic epilepsy). Epilepsies. 1994;6:5–11. Article in French.

    Google Scholar 

  88. Sabers A. Epilepsy in Down syndrome. In: Sillanpaa M, Gram L, Johannessen SI, Tomsom T, editors. Epilepsy & mental retardation. Petersfield/Hampshire: Wrightson Biomedical Publishing; 1999. p. 41–5.

    Google Scholar 

  89. Glauser TA, Morita DA. Lennox-Gastaut syndrome. In: Devinsky O, Westbrook LE, editors. Epilepsy and developmental disabilities. Woburn: Butterworth-Heinemann; 2002. p. 65–78.

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  91. Jenkins LK, Brown SW. The value of syndrome classification in childhood epilepsy. Acta Neurol Scand. 1990;82(Suppl)133:10.

    Google Scholar 

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Brown, S.W. (2016). Epidemiology of Epilepsy in Persons with Intellectual Disabilities. In: Prasher, V., Kerr, M. (eds) Epilepsy and Intellectual Disabilities. Springer, Cham. https://doi.org/10.1007/978-3-319-39144-1_3

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