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
. Internationl Statistical Classification of Disease and Related Health Problems. Tenth Revision. (1992) Geneva World Health Organization.
American Psychiatric Association. (1994) Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition. Washington, DC, American Psychiatric Association.
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.
Beange OH, Taplin JE. (1996) Prevalence of intellectual disability in northern Sydney adults. J Intellect Disabil Res 40: 191-7.
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.
Anonymous. (1996) State-specific rates of mental retardation— United States, 1993. MMWR — Morbidity & Mortality Weekly Report. 45: 61-5.
McConkey R, Mulvany F, Barron S. (2006) Adult persons with intellectual disabilities on the island of Ireland. J Intellect Disabil Res. 50: 227-36.
Valuing people: A new strategy for learning disability for the 21st century. (2001) London, The Stationery Office.
Hauser WA, Hesdorffer DC. (1990) Epilepsy: Frequency, causes and consequences. Demos Press. New York:
Hauser WA, Kurland LT. (1975) The epidemiology of epilepsy in Rochester Minnesota 1935 through 1967. Epilepsia 16: 1-66.
Joint Epilepsy Council. (2005) Epilepsy prevalence, incidence and other statistics. Joint Epilepsy Council, Leeds.
EUCARE. (2003) European White Paper on Epilepsy. Epilepsia. 44 Suppl 6: 1 - 88.
Illingworth RS. (1959) Convulsions in mentally retarded children with or without cerebral palsy. J Ment Defic Res. 3: 88-93
Iavanainen M. (1974) A study of origins of mental retardation, Heinemann London.
Corbett JA. (1979) Epilepsy and the electroencephalogram in early childhood psychoses. In: Wing JK (ed), Handbook of Psychiatry, Vol. 3. Cambridge University Press. Cambridge:
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.
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.
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.
Goulden KJ, Shinnar S, Koller H, et al. (1991) Epilepsy in children with mental retardation: A cohort study. Epilepsia 32: 690-7.
Forsgren L, Edvinsson SO, Blomquist HK, et al. (1990) Epilepsy in a population of mentally retarded children and adults. Epilepsy Res 6: 234-48.
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.
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.
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.
Lhatoo SD, Sander JWAS. (2001) The epidemiology of epilepsy and learning disability. Epilepsia. 42 (Suppl 1): 6-9.
Cockerell OC, Johnson A, Sander JWAS, et al. (1994) Mortality from epilepsy: Results from a prospective population-based study. Lancet 344: 918-21.
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,
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.
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.
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.
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.
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.
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.
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.
Steffenburg S, Gillberg C, Steffenburg U. (1996) Psychiatric disorders in children and adolescents with mental retardation and active epilepsy. Arch Neurol 53: 904-12.
Lund J. (1985) Epilepsy and psychiatric disorder in mentally retarded adults. Acta Psychiatrica Scandinavica 72: 557-62.
Lund J. (1985) The prevalence of psychiatric morbidity in mentally retarded adults. Acta Psychiatrica Scandinavica 72: 563-70.
Bax MCO. (1964) Terminology and classification of cerebral palsy. Dev Med Child Neurology 6: 295-7.
Aicardi J (1990) Epilepsy in brain-injured children. Dev Med Child Neurol 32: 191-202.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Musumeci SA, Hagerman RJ, Ferri R, et al. (1999) Epilepsy and EEG findings in males with fragile X syndrome. Epilepsia 40: 1092-1099.
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.
Hagberg B (ed). (1993) Rett Syndrome — Clinical & Biological Aspects. McKeith Press. London:
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.
Matsumoto A, Kumagai T, Miura K et al. (1992) Epilepsy in Angelman syndrome associated with chromosome 15q deletion. Epilepsia 33: 1083-1090.
Guerrini R, Lorey TM, De Bonanni P, et al. (1996) Cortical myoclonus in Angelman syndrome. Ann Neurol 40: 39- 48.
Minassian BA, DeLorey TM, Olsen RW, et al. (1998) Angelman syndrome: Correlations between epilepsy phenotypes and genotypes. Ann Neurol 43: 485-93.
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.
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.
Shapira NA, Lessig MC, Murphy TK, et al. (2002) Topiramate attenuates self-injurious behavior in Prader-Willi Syndrome. Inter J Neuropsychopharmacol 5: 141-5.
Smathers SA, Wilson JG, Nigro MA, et al. (2003) Topiramate effectiveness in Prader-Willi syndrome. Pediatr Neurol 28: 130-3.
Shapira NA, Lessig MC, Lewis MH, et al. (2004) Effects of topiramate in adults with PraderWilli syndrome. Am J Ment Retard 109: 301-9
Battaglia A, Battaglia A. (2005) The inv dup(15) or idic(15) syndrome: A clinically recognisable neurogenetic disorder. Brain and Development. 27: 365-9.
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.
Hunt A. (1993) Development, behaviour and seizures in 300 cases of tuberous sclerosis. J Intellect Disabil Res 37: 41-51.
Gillberg C. (1995) Clinical child neuropsychiatry. Cambridge University Press. Cambridge, UK:
Riikonen R, Simell O. (1990) Tuberous sclerosis and infantile spasms. Dev Med Child Neurol 32: 203-9.
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.
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.
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.
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.
Guerrini R, Genton P, Bureau M, et al. (1990) Reflex seizures are frequent in patients with Down syndrome and epilepsy. Epilepsia 31: 406-17.
Brown SW. (2002) Epilepsy. In: Prasher VP, Janicki MP, (eds) Physical health of adults with intellectual disabilities. Blackwell Publishing; Oxford: 133-59.
Pueschel SM, Louis S. McKnight P. (1991) Seizure disorders in Down syndrome. Arch Neurol 84: 318-20.
Lai F, Williams RS. (1989) A prospective study of Alzheimer disease in Down syndrome. Arch Neurol 46: 849-53.
Evenhuis HM. (1990) The natural history of dementia in Down’s syndrome. Arch Neurol 47: 263-7.
Genton P, Paglia G. (1994) É pilepsie myoclonique s é nile (senile myoclonic epilepsy). Epilepsies 6: 5 - 11.
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.
Glauser TA, Morita DA. (2002) Lennox-Gastaut syndrome. In: Devinsky O, Westbrook LE (eds). Epilepsy and developmental disabilities. Butterworth-Heinemann, Woburn, MA: 65 - 78.
Trevathan E, Murphy CC, Yeargin-Allsopp M. (1997) Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia 38: 1283-8.
Jenkins LK, Brown SW. (1990) The value of syndrome classification in childhood epilepsy. Acta Neurologica Scandinavica 82 (Suppl) 133: - 10.
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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
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