Abstract
Early onset dementia (EOD) is a major diagnostic challenge as it often presents with atypical features and may be attributed to treatable diseases. Primary degenerative dementias (Alzheimer's disease-AD, frontotemporal lobar degeneration-FTLD, Lewy body dementia-LBD), although traditionally considered to affect older people, are still a main cause of EOD. 491 demented patients were assessed from January 1, 2003 to December 31, 2010 in the Neurology Department of a tertiary referral center. Patients were classified as AD, behavioral variant frontotemporal dementia (bvFTD), non-fluent agrammatic variant primary progressive aphasia (naPPA), semantic variant PPA (svPPA), corticobasal degeneration (CBD), or progressive supranuclear palsy (PSP) who also met criteria for naPPA and LBD. Finally, their demographic characteristics were analysed, according to age at onset (EOD <65 years, late onset dementia-LOD ≥65 years). From the 491 patients, 137 (27.9 %) were EOD. In the EOD group, 52 (38 %) were diagnosed with bvFTD, 34 (24.8 %) with AD, 27 (19.7 %) with naPPA, 10 (7.2 %) with svPPA, 12 (8.8 %) with CBD or PSP, and 2 (1.5 %) with LBD. Demographic characteristics did not differ significantly among diagnostic categories in the EOD group, while in the LOD group FTLD patients were younger and more frequently men compared to both AD and LBD patients. EOD patients had more years of education than LOD patients. Degenerative disorders as causes of EOD are not rare. High clinical alertness is warranted to achieve correct and timely diagnosis.
Similar content being viewed by others
Notes
Statistical analysis did not yield significant differences neither in age, age at onset, and education nor in sex among subgroups in the FTLD category (Table 2).
References
Ferri CP, Prince M, Brayne C, Brodaty H, Fratiglioni L, Ganguli M, Hall K, Hasegawa K, Hendrie H, Huang Y, Jorm A, Mathers C, Menezes PR, Rimmer E, Scazufca M, Alzheimer's Disease International (2005) Global prevalence of dementia: a Delphi consensus study. Lancet 366:2112–2117
van Vliet D, de Vugt ME, Bakker C, Koopmans RT, Verhey FR (2010) Impact of early onset dementia on caregivers: a review. Int J Geriatr Psychiatry 25:1091–1100
Ridha B, Josephs KA (2006) Young-onset dementia: a practical approach to diagnosis. Neurologist 12:2–13
Rossor MN, Fox NC, Mummery CJ, Schott JM, Warren JD (2010) The diagnosis of young-onset dementia. Lancet Neurol 9:793–806
McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan EM (1984) Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA work group under the auspices of Department of Health and Human Services Task Force on Alzheimer's disease. Neurology 34:939–944
Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, van Swieten JC, Seelaar H, Dopper EG, Onyike CU, Hillis AE, Josephs KA, Boeve BF, Kertesz A, Seeley WW, Rankin KP, Johnson JK, Gorno-Tempini ML, Rosen H, Prioleau-Latham CE, Lee A, Kipps CM, Lillo P, Piguet O, Rohrer JD, Rossor MN, Warren JD, Fox NC, Galasko D, Salmon DP, Black SE, Mesulam M, Weintraub S, Dickerson BC, Diehl-Schmid J, Pasquier F, Deramecourt V, Lebert F, Pijnenburg Y, Chow TW, Manes F, Grafman J, Cappa SF, Freedman M, Grossman M, Miller BL (2011) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134:2456–2477
Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M (2011) Classification of primary progressive aphasia and its variants. Neurology 76:1006–1014
Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554
Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54(Suppl. 5):S15–S19
Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, Goetz CG, Golbe LI, Grafman J, Growdon JH, Hallett M, Jankovic J, Quinn NP, Tolosa E, Zee DS (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47:1–9
McKeith IG, Perry EK, Perry RH (1999) Report of the second dementia with Lewy body International Workshop: diagnosis and treatment. Consortium on dementia with Lewy bodies. Neurology 53:902–905
Ioannidis P, Konstantinopoulou E, Maiovis P, Karacostas D (2012) The frontotemporal dementias in a tertiary referral center: classification and demographic characteristics in a series of 232 cases. J Neurol Sci 318(1–2):171–173
Papageorgiou SG, Kontaxis T, Bonakis A, Kalfakis N, Vassilopoulos D (2009) Frequency and causes of early-onset dementia in a tertiary referral center in Athens. Alzheimer Dis Assoc Disord 23:347–351
Picard C, Pasquier F, Martinaud O, Hannequin D, Godefroy O (2011) Early onset dementia characteristics in a large cohort from academic memory clinics. Alzheimer Dis Assoc Disord 25:203–205
van Vliet D, de Vugt ME, Bakker C, Pijnenburg YA, Vernooij-Dassen MJ, Koopmans RT, Verhey FR (2012) Time to diagnosis in young-onset dementia as compared with late-onset dementia. Psychol Med 28:1–10
Harvey RJ, Skelton-Robinson M, Rossor MN (2003) The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry 74:1206–1209
Ikejima C, Yasuno F, Mizukami K, Sasaki M, Tanimukai S, Asada T (2009) Prevalence and causes of early-onset dementia in Japan: a population-based study. Stroke 40:2709–2714
Renvoize E, Hanson M, Dale M (2011) Prevalence and causes of young onset dementia in an English health district. Int J Geriatr Psychiatry 26:106–108
Kelley BJ, Boeve BF, Josephs KA (2008) Young-onset dementia: demographic and etiologic characteristics of 235 patients. Arch Neurol 65:1502–1508
Borroni B, Alberici A, Grassi M, Rozzini L, Turla M, Zanetti O, Bianchetti A, Gilberti N, Bonvicini C, Volta GD, Rozzini R, Padovani A (2011) Prevalence and demographic features of early-onset neurodegenerative dementia in Brescia County, Italy. Alzheimer Dis Assoc Disord 25(4):341–344
Snowden JS, Thompson JC, Stopford CL, Richardson AM, Gerhard A, Neary D, Mann DM (2011) The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships. Brain 134:2478–2492
Shinagawa S, Ikeda M, Toyota Y, Matsumoto T, Matsumoto N, Mori T, Ishikawa T, Fukuhara R, Komori K, Hokoishi K, Tanabe H (2007) Frequency and clinical characteristics of early-onset dementia in consecutive patients in a memory clinic. Dement Geriatr Cogn Disord 24(1):42–47
Blennow K, de Leon MJ, Zetterberg H (2006) Alzheimer's disease. Lancet 368(9533):387–403
Gislason TB, Sjögren M, Larsson L, Skoog I (2003) The prevalence of frontal variant frontotemporal dementia and the frontal lobe syndrome in a population based sample of 85 year olds. J Neurol Neurosurg Psychiatry 74:867–871
Ibach B, Koch H, Koller M, Wolfersdorf M (2003) Hospital admission circumstances and prevalence of frontotemporal lobar degeneration: a multicenter psychiatric state hospital study in Germany. Dement Geriatr Cogn Disord 16:253–264
Pasquier F (2013) New behavioural variant FTD criteria and clinical practice. Rev Neur (Paris) 169(10):799–805
Tola-Arribas MA, Yugueros MI, Garea MJ, Ortega-Valín F, Cerón-Fernández A, Fernández-Malvido B, San José-Gallegos A, González-Touya M, Botrán-Velicia A, Iglesias-Rodríguez V, Díaz-Gómez B (2013) Prevalence of Dementia and Subtypes in Valladolid, Northwestern Spain: The DEMINVALL Study. PLoS One 8(10):e77688
Fereshtehnejad SM, Dorota Religa D, Westman E, Aarsland D, Lökk J, Eriksdotter M (2013) Demography, diagnostics, and medication in dementia with Lewy bodies and Parkinson's disease with dementia: data from the Swedish Dementia Quality Registry (SveDem). Neuropsychiatr Dis Treat 9:927–935
Mackenzie CS, Gekoski WL, Knox VJ (2006) Age, gender, and the underutilization of mental health services: the influence of help-seeking attitudes. Aging Ment Health 10:574–582
Tedstone Doherty D, Kartalova-O'Doherty Y (2010) Gender and self-reported mental health problems: predictors of help seeking from a general practitioner. Br J Health Psychol 15:213–228
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Maiovis, P., Ioannidis, P., Konstantinopoulou, E. et al. Early onset degenerative dementias: demographic characteristics and etiologic classification in a tertiary referral center. Acta Neurol Belg 115, 27–31 (2015). https://doi.org/10.1007/s13760-014-0310-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13760-014-0310-2