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Cited by 99Down syndrome and intellectual disability researchEpilepsy research and treatmentDementia and Cognitive Impairment Research

Epilepsy in Down Syndrome: A Highly Prevalent Comorbidity

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Altuna, Miren, Giménez, Sandra, Fortea, Juan·2021·Journal of Clinical Medicine
DOI 10.3390/jcm10132776PMC PMC8268854
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Abstract

Individuals with Down syndrome (DS) have an increased risk for epilepsy during the whole lifespan, but especially after age 40 years. The increase in the number of individuals with DS living into late middle age due to improved health care is resulting in an increase in epilepsy prevalence in this population. However, these epileptic seizures are probably underdiagnosed and inadequately treated. This late onset epilepsy is linked to the development of symptomatic Alzheimer’s disease (AD), which is the main comorbidity in adults with DS with a cumulative incidence of more than 90% of adults by the seventh decade. More than 50% of patients with DS and AD dementia will most likely develop epilepsy, which in this context has a specific clinical presentation in the form of generalized myoclonic epilepsy. This epilepsy, named late onset myoclonic epilepsy (LOMEDS) affects the quality of life, might be associated with worse cognitive and functional outcomes in patients with AD dementia and has an impact on mortality. This review aims to summarize the current knowledge about the clinical and electrophysiological characteristics, diagnosis and treatment of epileptic seizures in the DS population, with a special emphasis on LOMEDS. Raised awareness and a better understanding of epilepsy in DS from families, caregivers and clinicians could enable earlier diagnoses and better treatments for individuals with DS.

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  1. [1]Kinnear D. Morrison J. Allan L. Henderson A. Smiley E. Cooper S.-A. Prevalence of physical conditions and multimorbidity in a cohort of adults with intellectual disabilities with and without Down syndrome: Cross-sectional study BMJ Open 2018 8 e018292 10.1136/bmjopen-2017-018292 PMC5829598 29431619 · DOI
  2. [2]Lott I.T. Doran E. Nguyen V.Q. Tournay A. Movsesyan N. Gillen D.L. Down Syndrome and Dementia: Seizures and Cognitive Decline J. Alzheimer’s Dis. 2012 29 177 185 10.3233/JAD-2012-111613 22214782 PMC3406603 · DOI
  3. [3]Breia P. Mendes R. Silvestre A. Gonçalves M.J. Figueira M.J. Bispo R. Adults with Down syndrome: Characterization of a Portuguese sample Acta Méd. Port. 2014 27 357 363 10.20344/amp.4898 25017348 · DOI
  4. [4]Startin C.M. D’Souza H. Ball G. Hamburg S. Hithersay R. Hughes K.M. Massand E. Karmiloff-Smith A. Thomas M.S. LonDownS Consortium Health comorbidities and cognitive abilities across the lifespan in Down syndrome J. Neurodev. Disord. 2020 12 4 10.1186/s11689-019-9306-9 31973697 PMC6979347 · DOI
  5. [5]Arvio M. Sillanpää M. Prevalence, aetiology and comorbidity of severe and profound intellectual disability in Finland J. Intellect. Disabil. Res. 2003 47 Pt 2 108 112 10.1046/j.1365-2788.2003.00447.x 12542576 · DOI
  6. [6]Tenenbaum A. Chavkin M. Wexler I.D. Korem M. Merrick J. Morbidity and hospitalizations of adults with Down syndrome Res. Dev. Disabil. 2012 33 435 441 10.1016/j.ridd.2011.09.026 22137940 · DOI
  7. [7]Gaete B. Mellado C. Hernández M. Prevalence of neurological disorders among children with Down syndrome Rev. Med. Chile 2012 140 214 218 10.4067/S0034-98872012000200010 22739951 · DOI
  8. [8]Glasson E.J. Dye D.E. Bittles A.H. The triple challenges associated with age-related comorbidities in Down syndrome J. Intellect. Disabil. Res. 2013 58 393 398 10.1111/jir.12026 23510031 · DOI
  9. [9]McVicker R.W. Shanks O.E.P. McClelland R.J. Prevalence and Associated Features of Epilepsy in Adults with Down’s Syndrome Br. J. Psychiatry 1994 164 528 532 10.1192/bjp.164.4.528 8038944 · DOI
  10. [10]Rafii M.S. Santoro S.L. Prevalence and Severity of Alzheimer Disease in Individuals with Down Syndrome JAMA Neurol. 2019 76 142 10.1001/jamaneurol.2018.3443 30452497 · DOI
  11. [11]D’Orsi G. Specchio L.M. Progressive myoclonus epilepsy in Down syndrome patients with dementia J. Neurol. 2014 261 1584 1597 10.1007/s00415-014-7376-x 24893590 · DOI
  12. [12]Aller-Alvarez J. Menéndez-González M. Ribacoba-Montero R. Salvado M. Vega V. Suárez-Moro R. Sueiras M. Toledo M. Salas-Puig J. Álvarez-Sabin J. Myoclonic epilepsy in Down syndrome and Alzheimer disease Neurología 2017 32 69 73 10.1016/j.nrl.2014.12.008 25661268 · DOI
  13. [13]Ohwada H. Nakayama T. Tomono Y. Yamanaka K. Predictors, including blood, urine, anthropometry, and nutritional indices, of all-cause mortality among institutionalized individuals with intellectual disability Res. Dev. Disabil. 2013 34 650 655 10.1016/j.ridd.2012.10.006 23123878 · DOI
  14. [14]Dyggve H. Kodahl T. Disease pattern among 942 mentally retarded persons in a Danish county Acta Psychiatr. Scand. 1979 59 381 394 10.1111/j.1600-0447.1979.tb04481.x 155388 · DOI
  15. [15]Hermans H. Evenhuis H.M. Multimorbidity in older adults with intellectual disabilities Res. Dev. Disabil. 2014 35 776 783 10.1016/j.ridd.2014.01.022 24529858 · DOI
  16. [16]Vignoli A. Zambrelli E. Chiesa V. Savini M. La Briola F. Gardella E. Canevini M.P. Epilepsy in adult patients with Down syndrome: A clinical-video EEG study Epileptic Disord. 2011 13 125 132 10.1684/epd.2011.0426 21561839 · DOI
  17. [17]Lefter S. Costello D.J. McNamara B. Sweeney B. Clinical and EEG Features of Seizures in Adults with Down Syndrome J. Clin. Neurophysiol. 2011 28 469 473 10.1097/WNP.0b013e318230da76 21946360 · DOI
  18. [18]Pueschel S.M. Louis S. McKnight P. Seizure Disorders in Down Syndrome Arch. Neurol. 1991 48 318 320 10.1001/archneur.1991.00530150088024 1825777 · DOI
  19. [19]Prasher V. Epilepsy and associated effects on adaptive behaviour in adults with Down syndrome Seizure 1995 4 53 56 10.1016/S1059-1311(05)80079-2 7788109 · DOI
  20. [20]Johannsen P. Christensen J.E.J. Goldstein H. Nielsen V.K. Mai J. Epilepsy in Down syndrome—Prevalence in three age groups Seizure 1996 5 121 125 10.1016/s1059-1311(96)80105-1 8795127 · DOI
  21. [21]Inokuchi R. Mizuno N. Shinohara K. Matsubara T. Doi K. Gunshin M. Nakajima S. Nakamura K. Sato H. Ishii T. Adult-onset seizures in a patient with Down syndrome and portosystemic shunt Brain Dev. 2014 36 626 629 10.1016/j.braindev.2013.08.007 24035599 · DOI
  22. [22]Chaanine A. Hugonenq C. Lena G. Mancini J. Les complications neurologiques liées à la trisomie 21 [Neurological complications in Down syndrome] Arch. Pediatr. 2008 15 388 396 (In French) 10.1016/j.arcped.2008.01.015 18329863 · DOI
  23. [23]Lista S. O’Bryant S. Blennow K. Dubois B. Hugon J. Zetterberg H. Hampel H. Biomarkers in Sporadic and Familial Alzheimer’s Disease J. Alzheimer’s Dis. 2015 47 291 317 10.3233/JAD-143006 26401553 · DOI · Paper page
  24. [24]Crespel A. Gonzalez V. Coubes P. Gelisse P. Senile myoclonic epilepsy of Genton: Two cases in Down syndrome with dementia and late onset epilepsy Epilepsy Res. 2007 77 165 168 10.1016/j.eplepsyres.2007.09.002 17961983 · DOI
  25. [25]Veall R.M. The Prevalence of Epilepsy among Mongols Related to Age J. Intellect. Disabil. Res. 1974 18 99 106 10.1111/j.1365-2788.1974.tb01224.x 4281033 · DOI
  26. [26]Määttä T. Määttä J. Tervo-Määttä T. Taanila A. Kaski M. Iivanainen M. Healthcare and guidelines: A population-based survey of recorded medical problems and health surveillance for people with Down syndrome J. Intellect. Dev. Disabil. 2011 36 118 126 10.1080/13668250.2011.570253 21501111 · DOI
  27. [27]Collacott R.A. Epilepsy, dementia and adaptive behaviour in Down’s syndrome J. Intellect. Disabil. Res. 2008 37 153 160 10.1111/j.1365-2788.1993.tb00582.x 8481613 · DOI
  28. [28]Hithersay R. Hamburg S. Knight B. Strydom A. Cognitive decline and dementia in Down syndrome Curr. Opin. Psychiatry 2017 30 102 107 10.1097/YCO.0000000000000307 28009725 · DOI
  29. [29]Gholipour T. Mitchell S. Sarkis R.A. Chemali Z. The clinical and neurobehavioral course of Down syndrome and dementia with or without new-onset epilepsy Epilepsy Behav. 2017 68 11 16 10.1016/j.yebeh.2016.12.014 28109983 · DOI
  30. [30]Brodtkorb E. The diversity of epilepsy in adults with severe developmental disabilities: Age at seizure onset and other prognostic factors Seizure 1994 3 277 285 10.1016/S1059-1311(05)80175-X 7894838 · DOI
  31. [31]Arai Y. Iwasaki Y. Suzuki T. Ide S. Kaga M. Elimination of amyloid precursor protein in senile plaques in the brain of a patient with Alzheimer-type dementia and Down syndrome Brain Dev. 2019 41 106 110 10.1016/j.braindev.2018.07.017 30086988 · DOI
  32. [32]De Simone R. Daquin G. Genton P. Senile myoclonic epilepsy in Down syndrome: A video and EEG presentation of two cases Epileptic Disord. 2006 8 223 227 16987746
  33. [33]Fisher R.S. Acevedo C. Arzimanoglou A. Bogacz A. Cross J.H. Elger C.E. Engel J. Jr. Forsgren L. French J.A. Glynn M. ILAE Official Report: A practical clinical definition of epilepsy Epilepsia 2014 55 475 482 10.1111/epi.12550 24730690 · DOI · Paper page
  34. [34]Misra U.K. Kalita J. Management of provoked seizure Ann. Indian Acad. Neurol. 2011 14 2 8 10.4103/0972-2327.78041 21633606 PMC3098518 · DOI
  35. [35]Arya R. Kabra M. Gulati S. Epilepsy in children with Down syndrome Epileptic Disord 2011 13 1 7 10.1684/epd.2011.0415 21398208 · DOI
  36. [36]Ulate-Campos A. Nascimento A. Ortez C. Down’s syndrome and epilepsy Int. Med. Rev. Down Syndr. 2014 18 3 8 Available online: https://www.sciencedirect.com/science/article/pii/S2171974814700443 (accessed on 21 June 2021) 10.1016/S2171-9748(14)70044-3 · DOI
  37. [37]Gaete B. Mellado C. Hernández M. Trastornos neurológicos en niños con síndrome de Down Rev. Méd. Chile 2012 140 214 218 10.4067/S0034-98872012000200010 22739951 · DOI
  38. [38]Shimakawa S. Tanabe T. Ono M. Nonaka M. Nambu M. Shinohara T. Nishikubo T. Fukui M. Nomura S. Azumagawa K. Incidence of febrile seizure in patients with Down syndrome Pediatr. Int. 2015 57 670 672 10.1111/ped.12601 25676606 · DOI
  39. [39]Daniels D. Knupp K. Benke T. Wolter-Warmerdam K. Moran M. Hickey F. Infantile Spasms in Children with Down Syndrome: Identification and Treatment Response Glob. Pediatr. Health 2019 6 10.1177/2333794X18821939 30671494 PMC6328947 · DOI
  40. [40]Araujo B. Torres L.B. Guilhoto L.M.F. Cerebal overinhibition could be the basis for the high prevalence of epilepsy in persons with Down syndrome Epilepsy Behav. 2015 53 120 125 10.1016/j.yebeh.2015.10.004 26558714 · DOI
  41. [41]Takashima S. Becker L.E. Armstrong D.L. Chan F. Abnormal neuronal development in the visual cortex of the human fetus and infant with Down’s syndrome. A quantitative and qualitative Golgi study Brain Res. 1981 225 1 21 10.1016/0006-8993(81)90314-0 6457667 · DOI · Paper page
  42. [42]Stafstrom C.E. Patxot O.F. Gilmore H.E. Wisniewski K.E. Seizures in Children with Down Syndrome: Etiology, Characteristics and Outcome Dev. Med. Child Neurol. 1991 33 191 200 10.1111/j.1469-8749.1991.tb05108.x 1827417 · DOI
  43. [43]Gupta A. Febrile Seizures Continuum 2016 22 51 59 10.1212/CON.0000000000000274 26844730 · DOI
  44. [44]Romano C. Tiné A. Fazio G. Rizzo R. Colognola R.M. Sorge G. Bergonzi P. Pavone L. Seizures in patients with trisomy 21 Am. J. Med. Genet. 2005 37 298 300 10.1002/ajmg.1320370758 2149964 · DOI
  45. [45]Anderson T. Visootsak J. Tapp S. Neurodevelopmental Outcomes in Children with Down Syndrome and Infantile Spasms J. Pediatr. Neurol. 2015 13 74 77 10.1055/s-0035-1556768 26523121 PMC4627704 · DOI
  46. [46]Stafstrom C.E. Konkol R.J. Infantile Spasms in Children with Down Syndrome Dev. Med. Child Neurol. 2008 36 576 585 10.1111/j.1469-8749.1994.tb11894.x 8034119 · DOI
  47. [47]Gandy K.C. Castillo H.A. Ouellette L. Castillo J. Lupo P.J. Jacola L.M. Rabin K.R. Raghubar K.P. Gramatges M.M. The relationship between chronic health conditions and cognitive deficits in children, adolescents, and young adults with Down syndrome: A systematic review PLoS ONE 2020 15 e0239040 10.1371/journal.pone.0239040 32915911 PMC7485757 · DOI
  48. [48]Beatty C.W. Wrede J.E. Blume H.K. Diagnosis, treatment, and outcomes of infantile spasms in the Trisomy 21 population Seizure 2017 45 184 188 10.1016/j.seizure.2016.12.016 28088035 · DOI
  49. [49]Kats D.J. Roche K.J. Skotko B.G. Epileptic spasms in individuals with Down syndrome: A review of the current literature Epilepsia Open 2020 5 344 353 10.1002/epi4.12412 32913943 PMC7469826 · DOI
  50. [50]Pueschel S.M. Louis S. Reflex seizures in Down syndrome Child’s Nerv. Syst. 1993 9 23 24 10.1007/BF00301930 8481939 · DOI
  51. [51]Giménez-Roldán S. Martin M. Startle epilepsy complicating down syndrome during adulthood Ann. Neurol. 1980 7 78 80 10.1002/ana.410070114 6444792 · DOI
  52. [52]Guerrini R. Genton P. Bureau M. Dravet C. Roger J. Reflex seizures are frequent in patients with Down syndrome and epilepsy Epilepsia 1990 31 406 417 10.1111/j.1528-1157.1990.tb05496.x 2142449 · DOI
  53. [53]Abdelmoity S. Ilyas M. Polyspike ictal-onset absence seizures in a pediatric patient with Down syndrome Epilepsy Behav. Case Rep. 2020 14 100376 10.1016/j.ebr.2020.100376 PMC7393451 32760906 · DOI
  54. [54]Ferlazzo E. Adjien C.K. Guerrini R. Calarese T. Crespel A. Elia M. Striano P. Gelisse P. Bramanti P. Di Bella P. Lennox-Gastaut syndrome with late-onset and prominent reflex seizures in trisomy 21 patients Epilepsia 2009 50 1587 1595 10.1111/j.1528-1167.2008.01944.x 19187280 · DOI
  55. [55]Hsieh K. Rimmer J. Heller T. Prevalence of Falls and Risk Factors in Adults with Intellectual Disability Am. J. Intellect. Dev. Disabil. 2012 117 442 454 10.1352/1944-7558-117.6.442 23167484 · DOI
  56. [56]Barca D. Tarta-Arsene O. Dica A. Iliescu C. Budisteanu M. Motoescu C. Butoianu N. Craiu D. Intellectual Disability and Epilepsy in Down Syndrome Maedica 2014 9 344 350 25705303 PMC4316878
  57. [57]Ellingson R.J. Eisen J.D. Ottersberg G. Clinical electroencephalographic observations on institutionalized mongoloids confirmed by karyotype Electroencephalogr. Clin. Neurophysiol. 1973 34 193 196 10.1016/0013-4694(73)90047-3 4119533 · DOI
  58. [58]Śmigielska-Kuzia J. Sobaniec W. Kułak W. Boćkowski L. Clinical and EEG Features of Epilepsy in Children and Adolescents in Down Syndrome J. Child Neurol. 2009 24 416 420 10.1177/0883073808324542 19339285 · DOI
  59. [59]Verrotti A. Cusmai R. Nicita F. Pizzolorusso A. Elia M. Zamponi N. Cesaroni E. Granata T. De Giorgi I. Giordano L. Electroclinical Features and Long-Term Outcome of Cryptogenic Epilepsy in Children with Down Syndrome J. Pediatr. 2013 163 1754 1758 10.1016/j.jpeds.2013.07.022 23992680 · DOI
  60. [60]Rahman M.M. Fatema K. Seizures in Down Syndrome: An Update Mymensingh Med. J. 2019 28 712 715 31391451
  61. [61]McCarron M. Gill M. McCallion P. Begley C. Health co-morbidities in ageing persons with Down syndrome and Alzheimer’s dementia J. Intellect. Disabil. Res. 2005 49 560 566 10.1111/j.1365-2788.2005.00704.x 15966964 · DOI
  62. [62]Palop J. Chin J. Roberson E.D. Wang J. Thwin M.T. Bien-Ly N. Yoo J. Ho K.O. Yu G.-Q. Kreitzer A. Aberrant Excitatory Neuronal Activity and Compensatory Remodeling of Inhibitory Hippocampal Circuits in Mouse Models of Alzheimer’s Disease Neuron 2007 55 697 711 10.1016/j.neuron.2007.07.025 17785178 PMC8055171 · DOI · Paper page
  63. [63]Giorgi F.S. Saccaro L.F. Busceti C.L. Biagioni F. Fornai F. Epilepsy and Alzheimer’s Disease: Potential mechanisms for an association Brain Res. Bull. 2020 160 107 120 10.1016/j.brainresbull.2020.04.009 32380189 · DOI
  64. [64]Carmona-Iragui M. Balasa M. Benejam B. Alcolea D. Fernández S. Videla L. Sala I. Sánchez-Saudinós M.B. Morenas-Rodriguez E. Ribosa-Nogué R. Cerebral amyloid angiopathy in Down syndrome and sporadic and autosomal-dominant Alzheimer’s disease Alzheimer’s Dement. 2017 13 1251 1260 10.1016/j.jalz.2017.03.007 28463681 PMC5660938 · DOI
  65. [65]Zarea A. Charbonnier C. Rovelet-Lecrux A. Nicolas G. Rousseau S. Borden A. Pariente J. Le Ber I. Pasquier F. Formaglio M. Seizures in dominantly inherited Alzheimer disease Neurology 2016 87 912 919 10.1212/WNL.0000000000003048 27466472 · DOI
  66. [66]Costa C. Romoli M. Liguori C. Farotti L. Eusebi P. Bedetti C. Siliquini S. Cesarini E.N. Romigi A. Mercuri N.B. Alzheimer’s disease and late-onset epilepsy of unknown origin: Two faces of beta amyloid pathology Neurobiol. Aging 2019 73 61 67 10.1016/j.neurobiolaging.2018.09.006 30317034 · DOI
  67. [67]Toniolo S. Sen A. Husain M. Modulation of Brain Hyperexcitability: Potential New Therapeutic Approaches in Alzheimer’s Disease Int. J. Mol. Sci. 2020 21 9318 10.3390/ijms21239318 PMC7730926 33297460 · DOI
  68. [68]Liguori C. Spanetta M. Romoli M. Placidi F. Cesarini E.N. Mercuri N.B. Costa C. Sleep disorders and late-onset epilepsy of unknown origin: Understanding new trajectories to brain amyloidopathy Mech. Ageing Dev. 2021 194 111434 10.1016/j.mad.2021.111434 33444630 · DOI
  69. [69]Johnson E.L. Krauss G.L. Kucharska-Newton A. Albert M.S. Brandt J. Walker K.A. Yasar S. Knopman D.S. Vossel K.A. Gottesman R.F. Dementia in late-onset epilepsy: The Atherosclerosis Risk in Communities study Neurology 2020 95 e3248 e3256 10.1212/WNL.0000000000011080 33097597 PMC7836657 · DOI
  70. [70]Joutsa J. Rinne J.O. Hermann B. Karrasch M. Anttinen A. Shinnar S. Sillanpää M. Association Between Childhood-Onset Epilepsy and Amyloid Burden 5 Decades Later JAMA Neurol. 2017 74 583 590 10.1001/jamaneurol.2016.6091 28346588 PMC5822199 · DOI
  71. [71]Menéndez M. Down syndrome, Alzheimer’s disease and seizures Brain Dev. 2005 27 246 252 10.1016/j.braindev.2004.07.008 15862185 · DOI · Paper page
  72. [72]Bayen E. Possin K.L. Chen Y. De Langavant L.C. Yaffe K. Prevalence of Aging, Dementia, and Multimorbidity in Older Adults with Down Syndrome JAMA Neurol. 2018 75 1399 1406 10.1001/jamaneurol.2018.2210 30032260 PMC6248113 · DOI
  73. [73]Speller C. Johnston S. Kanagaratum S. Epilepsy in Down’s syndrome Br. J. Psychiatry J. Ment. Sci. 1994 165 270 10.1192/bjp.165.2.270a 7953048 · DOI
  74. [74]Vossel A.K. Tartaglia M.C. Nygaard H.B. Zeman A.Z. Miller B.L. Epileptic activity in Alzheimer’s disease: Causes and clinical relevance Lancet Neurol. 2017 16 311 322 10.1016/S1474-4422(17)30044-3 28327340 PMC5973551 · DOI · Paper page
  75. [75]Lam A.D. Sarkis R.A. Pellerin K.R. Jing J. Dworetzky B.A. Hoch D.B. Jacobs C.S. Lee J.W. Weisholtz D.S. Zepeda R. Association of epileptiform abnormalities and seizures in Alzheimer disease Neurology 2020 95 e2259 e2270 10.1212/WNL.0000000000010612 32764101 PMC7713786 · DOI · Paper page
  76. [76]Cortini F. Cantoni C. Villa C. Epileptic seizures in autosomal dominant forms of Alzheimer’s disease Seizure 2018 61 4 7 10.1016/j.seizure.2018.07.015 30041064 · DOI
  77. [77]Lai F. Williams R.S. A prospective study of Alzheimer disease in Down syndrome Arch. Neurol. 1989 46 849 853 10.1001/archneur.1989.00520440031017 2527024 · DOI · Paper page
  78. [78]Vöglein J. Ricard I. Noachtar S. Kukull W.A. Dieterich M. Levin J. Danek A. Seizures in Alzheimer’s disease are highly recurrent and associated with a poor disease course J. Neurol. 2020 267 2941 2948 10.1007/s00415-020-09937-7 32488295 PMC7501095 · DOI
  79. [79]Evenhuis H.M. The natural history of dementia in Down’s syndrome Arch. Neurol. 1990 47 263 267 10.1001/archneur.1990.00530030029011 2138013 · DOI
  80. [80]Sangani M. Shahid A. Amina S. Koubeissi M. Improvement of myoclonic epilepsy in Down syndrome treated with levetiracetam Epileptic Disord. 2010 12 151 154 10.1684/epd.2010.0306 20483713 · DOI
  81. [81]Larner A. Senile myoclonic epilepsy in Down syndrome Seizure 2011 20 512 10.1016/j.seizure.2011.01.020 21334927 · DOI
  82. [82]Kleinschmidt-DeMasters B. Damek D.M. Lillehei K.O. Dogan A. Giannini C. Epstein Barr Virus-Associated Primary CNS Lymphomas in Elderly Patients on Immunosuppressive Medications J. Neuropathol. Exp. Neurol. 2008 67 1103 1111 10.1097/NEN.0b013e31818beaea 18957891 · DOI
  83. [83]Forsgren L. Edvinsson S.O. Blomquist H.K. Heijbel J. Sidenvall R. Epilepsy in a population of mentally retarded children and adults Epilepsy Res. 1990 6 234 248 10.1016/0920-1211(90)90079-B 2148725 · DOI
  84. [84]Moller J.C. Hamer H.M. Oertel W.H. Rosenow F. Late-onset myoclonic epilepsy in Down’s syndrome (LOMEDS) Seizure 2001 10 303 306 10.1053/seiz.2000.0500 11466028 · DOI
  85. [85]Li L.M. O’Donoghue M.F. Sander J.W. Myoclonic epilepsy of late onset in trisomy 21 Arq. Neuropsiquiatr. 1995 53 792 794 10.1590/S0004-282X1995000500014 8729775 · DOI
  86. [86]Xu Y. Lavrencic L. Radford K. Booth A. Yoshimura S. Anstey K.J. Anderson C.S. Peters R. Systematic review of coexistent epileptic seizures and Alzheimer’s disease: Incidence and prevalence J. Am. Geriatr. Soc. 2021 10.1111/jgs.17101 33740274 · DOI
  87. [87]Hithersay R. Startin C.M. Hamburg S. Mok K.Y. Hardy J. Fisher E.M.C. Tybulewicz V.L.J. Nizetic D. Strydom A. Association of Dementia with Mortality among Adults with Down Syndrome Older than 35 Years JAMA Neurol. 2019 76 152 160 10.1001/jamaneurol.2018.3616 30452522 PMC6439956 · DOI · Paper page
  88. [88]McCarron M. McCallion P. Reilly E. Dunne P. Carroll R. Mulryan N. A prospective 20-year longitudinal follow-up of dementia in persons with Down syndrome J. Intellect. Disabil. Res. 2017 61 843 852 10.1111/jir.12390 28664561 · DOI · Paper page
  89. [89]Uppal H. Chandran S. Potluri R. Risk factors for mortality in Down syndrome J. Intellect. Disabil. Res. 2015 59 873 881 10.1111/jir.12196 25851193 · DOI
  90. [90]McLoughlin I.J. A Study of Mortality Experiences in a Mental-Handicap Hospital Br. J. Psychiatry 1988 153 645 649 10.1192/bjp.153.5.645 2978380 · DOI
  91. [91]Reppermund S. Srasuebkul P. Dean K. Trollor J.N. Factors associated with death in people with intellectual disability J. Appl. Res. Intellect. Disabil. 2019 33 420 429 10.1111/jar.12684 31786826 · DOI
  92. [92]Cooper S.-A. Allan L. Greenlaw N. McSkimming P. Jasilek A. Henderson A. McCowan C. Kinnear D. Melville C. Rates, causes, place and predictors of mortality in adults with intellectual disabilities with and without Down syndrome: Cohort study with record linkage BMJ Open 2020 10 e036465 10.1136/bmjopen-2019-036465 32423940 PMC7239521 · DOI
  93. [93]Lott I.T. Lai F. Dementia in Down’s syndrome: Observations from a neurology clinic Appl. Res. Ment. Retard. 1982 3 233 239 10.1016/0270-3092(82)90017-0 6216849 · DOI
  94. [94]Tangye S.R. The EEG and incidence of epilepsy in Down’s syndrome J. Ment. Defic. Res. 1979 23 17 24 10.1111/j.1365-2788.1979.tb00849.x 158092 · DOI
  95. [95]Seppäläinen A.M. Kivalo E. EEG Findings and Epilepsy in Down’s Syndrome J. Intellect. Disabil. Res. 2008 11 116 125 10.1111/j.1365-2788.1967.tb00212.x 4226941 · DOI
  96. [96]Horváth A. Szűcs A. Hidasi Z. Csukly G. Barcs G. Kamondi A. Prevalence, Semiology, and Risk Factors of Epilepsy in Alzheimer’s Disease: An Ambulatory EEG Study J. Alzheimer’s Dis. 2018 63 1045 1054 10.3233/JAD-170925 29710705 · DOI
  97. [97]Tsiouris J.A. Patti P.J. Tipu O. Raguthu S. Adverse effects of phenytoin given for late-onset seizures in adults with Down syndrome Neurology 2002 59 779 780 10.1212/WNL.59.5.779 12221182 · DOI
  98. [98]Fortea J. Zaman S. Hartley S. Rafii M. Head E. Carmona-Iragui M. Down syndrome-associated Alzheimer’s disease Lancet Neurol. 2021 in press PMC9387748 34687637
  99. [99]Giménez S. Videla L. Romero S. Benejam B. Clos S. Fernández S. Martínez M. Carmona-Iragui M. Antonijoan R.M. Mayos M. Prevalence of Sleep Disorders in Adults with Down Syndrome: A Comparative Study of Self-Reported, Actigraphic, and Polysomnographic Findings J. Clin. Sleep Med. 2018 14 1725 1733 10.5664/jcsm.7382 30353801 PMC6175810 · DOI
  100. [100]Bubu O.M. Andrade A.G. Umasabor-Bubu O.Q. Hogan M.M. Turner A.D. de Leon M. Ogedegbe G. Ayappa I. Girardin J.-L.G. Jackson M.L. Obstructive sleep apnea, cognition and Alzheimer’s disease: A systematic review integrating three decades of multidisciplinary research Sleep Med. Rev. 2020 50 101250 10.1016/j.smrv.2019.101250 31881487 PMC7593825 · DOI · Paper page

Authors
Altuna, M., Giménez, S., & Fortea, J.
Affiliations
Universitat Autònoma de Barcelona (ES)
Corresponding author
  • Altuna, Miren
  • Fortea, Juan
ORCID
Venue
Journal of Clinical Medicine
Publisher
MDPI AG
Published
2021-06-24
Volume
10
Issue
13
Pages
2776
ISSN
2077-0383 (ISSN-L)
DOI
10.3390/jcm10132776
PMC
PMC8268854
OpenAlex
W3176315765
Cited by
99
References
102
Citations by year
2026: 15 · 2025: 22 · 2024: 24 · 2023: 15 · 2022: 17 · 2021: 6
FWCI
2.76
Type
journal article
Language
en
Open access
Licence
Locations
  • Journal of Clinical Medicine (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • Dipòsit Digital de Documents de la UAB (Universitat Autònoma de Barcelona) (submittedVersion, cc-by)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
  • Fondation Jérôme Lejeune (JLF1801)
  • National Institutes of Health
  • Instituto de Salud Carlos III (PI14/01126, PI14/, PI20/00836, PI17/01019)
  • National Institute on Aging (RF1AG056850, RF1AG061566, R21AG056974)
Topics
  • Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
  • Health Sciences › Medicine › Psychiatry and Mental health › Epilepsy research and treatment
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Keywords
Down syndrome, epilepsy, Alzheimer's disease, dementia, comorbidities, underdiagnosis, seizure treatment, electrophysiological characteristics, quality of life, mortality

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