Genetics of dementia: Update and guidelines for the clinician

With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzh...

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Vydané v:American journal of medical genetics. Part B, Neuropsychiatric genetics Ročník 159B; číslo 6; s. 628 - 643
Hlavní autori: Cohn-Hokke, Petra E., Elting, Mariet W., Pijnenburg, Yolande A.L., van Swieten, John C.
Médium: Journal Article
Jazyk:English
Vydavateľské údaje: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.09.2012
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Abstract With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X‐linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia. © 2012 Wiley Periodicals, Inc.
AbstractList With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X-linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia.
With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X-linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia.With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X-linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia.
With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X‐linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia. © 2012 Wiley Periodicals, Inc.
With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X-linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia. © 2012 Wiley Periodicals, Inc. [PUBLICATION ABSTRACT]
With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a complex disease, but may be autosomal dominant inherited. Mutations in the PSEN1 gene are the most common genetic cause of early onset Alzheimer's disease, whereas APP and PSEN2 gene mutations are less frequent. Familial frontotemporal dementia may be associated with a mutation in the MAPT or GRN gene, or with a repeat expansion in the C9orf72 gene. All these genes show autosomal dominant inheritance with a high penetrance. Although Alzheimer's disease and frontotemporal dementia are clinically distinguishable entities, phenotypical overlap may occur. Rarely, dementia is caused by mutations in other autosomal dominant genes or by genetic defects with autosomal recessive, X-linked dominant or mitochondrial inheritance. The inherited forms of frontotemporal dementia and Alzheimer's disease show a large phenotypic variability also within families, resulting in many remaining uncertainties for mutation carriers. Therefore, genetic counseling before performing genetic testing is essential in both symptomatic individuals and healthy at risk relatives. This review provides an overview of the genetic causes of dementia and discusses all aspects relevant for genetic counseling and testing. Furthermore, based on current knowledge, we provide algorithms for genetic testing in patients with early onset Alzheimer's disease or frontotemporal dementia. copyright 2012 Wiley Periodicals, Inc.
Author Pijnenburg, Yolande A.L.
Elting, Mariet W.
van Swieten, John C.
Cohn-Hokke, Petra E.
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  organization: Department of Clinical Genetics, VU University Medical Center, Amsterdam, The Netherlands
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  givenname: Mariet W.
  surname: Elting
  fullname: Elting, Mariet W.
  organization: Department of Clinical Genetics, VU University Medical Center, Amsterdam, The Netherlands
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  givenname: Yolande A.L.
  surname: Pijnenburg
  fullname: Pijnenburg, Yolande A.L.
  organization: Alzheimer Center, Department of Neurology, VU University Medical Center, Amsterdam, The Netherlands
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  givenname: John C.
  surname: van Swieten
  fullname: van Swieten, John C.
  organization: Department of Clinical Genetics, VU University Medical Center, Amsterdam, The Netherlands
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Cites_doi 10.1212/01.wnl.0000171397.32851.bc
10.1093/brain/awr195
10.1093/brain/awh356
10.1097/GIM.0b013e318164e4cf
10.1093/brain/awl226
10.1192/bjp.bp.109.068981
10.1038/nature10353
10.1212/WNL.34.7.939
10.1212/WNL.47.5.1113
10.1212/WNL.58.11.1615
10.1038/ng.440
10.1111/j.1468-1331.2007.01664.x
10.1002/ana.10267
10.1016/j.neurobiolaging.2007.05.022
10.1016/j.jns.2009.07.011
10.1126/science.1566067
10.1016/j.jalz.2011.03.005
10.1001/archneurol.2009.285
10.3233/JAD-2010-1337
10.1016/j.expneurol.2007.08.016
10.1177/0891988705281883
10.1212/WNL.0b013e3181bf997a
10.1074/jbc.M705115200
10.1001/archneurol.2009.328
10.1093/brain/awl271
10.1038/ng.439
10.1080/13554790903456209
10.1097/WAD.0b013e3181eff860
10.3233/JAD-2010-100837
10.1212/WNL.51.6.1546
10.1136/jnnp.74.9.1206
10.1007/s00415-002-0896-9
10.1001/archneur.62.7.1091
10.1001/archneur.58.11.1803
10.1001/archneurol.2010.201
10.1093/hmg/ddm309
10.1001/archneurol.2011.53
10.1097/WAD.0b013e31819e6b28
10.1007/s00415-005-0019-5
10.1126/science.1199214
10.1002/ana.20366
10.1093/brain/awl212
10.1007/s10897-009-9222-3
10.1161/STROKEAHA.108.542308
10.1001/jama.287.3.329
10.3233/JAD-2010-101032
10.1111/j.1750-3639.2007.00052.x
10.1016/j.jalz.2011.02.004
10.1212/WNL.0b013e3181ccc732
10.1038/nature05017
10.1016/j.neurobiolaging.2009.08.016
10.1038/sj.ejhg.5201966
10.1016/S1474-4422(10)70299-4
10.1093/brain/awr355
10.1212/01.wnl.0000343851.46573.67
10.7326/0003-4819-601
10.1371/journal.pone.0009872
10.1038/ng1609
10.1002/mds.22697
10.1038/nature05016
10.1073/pnas.95.13.7737
10.1016/j.neurobiolaging.2007.11.002
10.1212/WNL.56.suppl_4.S21
10.1002/1096-8628(20011001)103:2<138::AID-AJMG1529>3.0.CO;2-8
10.1212/01.wnl.0000172911.39167.b6
10.1038/ng1718
10.1093/brain/awl203
10.1002/ana.22264
10.1016/S1474-4422(10)70159-9
10.1038/mp.2011.52
10.1001/archneur.58.11.1828
10.1212/01.wnl.0000311445.21321.fc
10.1002/humu.9484
10.1111/j.1468-1331.2010.02985.x
10.1002/ana.10721
10.1212/WNL.44.9.1683
10.1093/brain/awp062
10.1016/S1474-4422(11)70261-7
10.1212/01.wnl.0000187068.92184.63
10.1002/ajmg.b.30410
10.1001/archneur.62.6.925
10.1093/hmg/7.1.43
10.1177/1533317506290448
10.1001/archneurol.2010.319
10.1002/ajmg.b.31099
10.1007/s00401-010-0698-6
10.1002/ana.20083
10.1016/S0304-3940(96)13138-4
10.1212/01.wnl.0000267642.41594.9d
10.1212/01.wnl.0000334277.16896.fa
10.1093/brain/awr216
10.1016/j.neurobiolaging.2011.08.005
10.1001/archneur.65.4.506
10.1093/brain/awn352
10.1186/1471-2377-8-48
10.1523/JNEUROSCI.4305-10.2010
10.1212/WNL.0b013e31821103e6
10.1038/349704a0
10.1097/NEN.0b013e3182270c54
10.1001/archneur.58.3.383
10.1038/ng1095-219
10.1177/1533317507302320
10.1136/jmg.2005.033456
10.1097/GIM.0b013e31821d69b8
10.1016/j.jns.2006.11.013
10.1093/hmg/ddl241
10.1002/ana.21099
10.1111/j.1468-1331.2008.02069.x
10.1001/archneur.57.1.72
10.1136/jnnp.2007.121137
10.1093/brain/awg204
10.1212/01.wnl.0000244749.20056.d4
10.1038/375754a0
10.1016/j.neuron.2011.09.011
10.1001/archneur.59.11.1759
10.1212/01.WNL.0000086811.39675.79
10.1007/s00415-009-5163-x
10.1016/j.neuron.2011.09.010
10.1126/science.1072994
10.1001/archneur.63.9.1307
10.1093/brain/awn012
10.1056/NEJMc1113592
10.1212/WNL.57.4.621
10.1212/01.wnl.0000319702.37497.72
10.1056/NEJMoa0809578
10.1002/1531-8249(20010201)49:2<263::AID-ANA50>3.0.CO;2-K
10.1002/mds.22642
10.1093/brain/awr179
10.1159/000077158
10.1523/JNEUROSCI.0730-07.2007
10.1093/brain/awr353
10.1136/jnnp.2006.113803
10.1086/302702
10.1038/31508
10.1007/s00401-011-0816-0
10.1007/s00401-009-0501-8
10.1002/humu.21100
10.1016/j.neuron.2010.11.036
10.3233/JAD-2004-6503
10.1016/S0197-4580(00)00202-5
10.1007/s12031-011-9557-8
10.1038/ng1332
10.1007/s00401-011-0907-y
10.1002/ana.1009
10.1212/01.wnl.0000325058.10218.fc
10.1007/s00259-008-0833-y
10.1093/brain/awm331
10.1007/s12031-011-9637-9
10.1093/brain/awq033
10.3988/jcn.2011.7.1.1
10.1001/archneurol.2010.113
10.1074/jbc.R800019200
10.1212/WNL.0b013e3181b389d9
10.1002/1531-8249(199908)46:2<243::AID-ANA14>3.0.CO;2-L
10.1002/humu.20520
10.1093/brain/awq216
10.1016/j.neurobiolaging.2011.10.014
10.1212/01.WNL.0000042088.22694.E3
10.1212/WNL.56.11.1545
10.1093/hmg/ddh134
10.1212/WNL.59.8.1134
10.1212/01.wnl.0000334278.11022.42
10.1007/s10897-005-4063-1
10.1093/brain/awp009
10.1007/s10048-008-0127-3
10.1086/302553
10.1006/exnr.2000.7613
10.1212/WNL.55.10.1577
10.1159/000058328
10.1136/jnnp.72.2.266
10.1001/archpsyc.63.2.168
10.1038/ng.801
10.1016/j.neuron.2008.11.007
10.1007/s00415-009-5404-z
10.1038/ng0892-345
10.1212/WNL.0b013e3181f07e0c
10.1177/0891988710383570
10.1017/S1462399409001136
10.1016/j.neulet.2007.03.044
10.1212/WNL.50.6.1541
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IsScholarly true
Issue 6
Keywords Nervous system diseases
Alzheimer disease
early onset
Recommendation
Cerebral disorder
Genetic counseling
genetic testing
Central nervous system disease
Genetics
Early
Degenerative disease
Frontotemporal dementia
Alzheimer's disease
Language English
License http://onlinelibrary.wiley.com/termsAndConditions#vor
CC BY 4.0
Copyright © 2012 Wiley Periodicals, Inc.
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How to Cite this Article: Cohn-Hokke PE, Elting MW, Pijnenburg YAL, van Swieten JC. 2012. Genetics of Dementia: Update and Guidelines for the Clinician. Am J Med Genet Part B 159B:628-643.
ArticleID:AJMG32080
istex:DCDC3317C05817D17461EF9EDA4D6DA20F31E465
How to Cite this Article: Cohn‐Hokke PE, Elting MW, Pijnenburg YAL, van Swieten JC. 2012. Genetics of Dementia: Update and Guidelines for the Clinician. Am J Med Genet Part B 159B:628–643.
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PublicationTitle American journal of medical genetics. Part B, Neuropsychiatric genetics
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PublicationYear 2012
Publisher Wiley Subscription Services, Inc., A Wiley Company
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References Halliday GM, Song YJ, Lepar G, Brooks WS, Kwok JB, Kersaitis C, Gregory G, Shepherd CE, Rahimi F, Schofield PR, Kril JJ. 2005. Pick bodies in a family with presenilin-1 Alzheimer's disease. Ann Neurol 57: 139-143.
Herve D, Chabriat H. 2010. CADASIL. J Geriatr Psychiatry Neurol 23: 269-276.
Deng H, Chen W, Hong S, Boycott K, Gorrie G, Siddique N, Yang Y, Fecto F, Shi y, Zhai H, Jiang H, Hirano M, Rampersaud E, Jansen G, Donkervoort S, Bigio E, Brooks B, Ajroud K, Sufit R, Haines J, Mugnaini E, Pericak-Vance M, Siddique T. 2011. Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature 477: 211-215.
Gijselinck I, Van Langenhove T, van der Zee J, Sleegers K, Philtjens S, Kleinberger G, Janssens J, Bettens K, Van Cauwenberghe C, Pereson S, Engelborghs S, Sieben A, De Jonghe P, Vandenberghe R, Santens P, De Bleecker J, Maes G, Baumer V, Dillen L, Joris G, Cuijt I, Corsmit E, Elinck E, Van Dongen J, Vermeulen S, Van den Broeck M, Vaerenberg C, Mattheijssens M, Peeters K, Robberecht W, Cras P, Martin JJ, De Deyn PP, Cruts M, Van Broeckhoven C. 2012. A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: A gene identification study. Lancet Neurol 11: 54-65.
Cannella M, Martino T, Simonelli M, Ciammola A, Gradini R, Ciarmiello A, Gianfrancesco F, Squitieri F. 2007. De novo seven extra repeat expanded mutation in the PRNP gene in an Italian patient with early onset dementia. J Neurol Neurosurg Psychiatry 78: 1411-1413.
McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G, Quinn NP, Edwardson JA, Ince PG, Bergeron C, Burns A, Miller BL, Lovestone S, Collerton D, Jansen EN, Ballard C, de Vos RA, Wilcock GK, Jellinger KA, Perry RH. 1996. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop. Neurology 47: 1113-1124.
Revesz T, Holton JL, Lashley T, Plant G, Frangione B, Rostagno A, Ghiso J. 2009. Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathies. Acta Neuropathol 118: 115-130.
Renton AE, Majounie E, Waite A, Simon-Sanchez J, Rollinson S, Gibbs JR, Schymick JC, Laaksovirta H, van Swieten JC, Myllykangas L, Kalimo H, Paetau A, Abramzon Y, Remes AM, Kaganovich A, Scholz SW, Duckworth J, Ding J, Harmer DW, Hernandez DG, Johnson JO, Mok K, Ryten M, Trabzuni D, Guerreiro RJ, Orrell RW, Neal J, Murray A, Pearson J, Jansen IE, Sondervan D, Seelaar H, Blake D, Young K, Halliwell N, Callister JB, Toulson G, Richardson A, Gerhard A, Snowden J, Mann D, Neary D, Nalls MA, Peuralinna T, Jansson L, Isoviita VM, Kaivorinne AL, Holtta-Vuori M, Ikonen E, Sulkava R, Benatar M, Wuu J, Chio A, Restagno G, Borghero G, Sabatelli M, Heckerman D, Rogaeva E, Zinman L, Rothstein JD, Sendtner M, Drepper C, Eichler EE, Alkan C, Abdullaev Z, Pack SD, Dutra A, Pak E, Hardy J, Singleton A, Williams NM, Heutink P, Pickering-Brown S, Morris HR, Tienari PJ, Traynor BJ. 2011. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72: 257-268.
Devi G, Ottman R, Tang MX, Marder K, Stern Y, Mayeux R. 2000. Familial aggregation of Alzheimer disease among whites, African Americans, and Caribbean Hispanics in northern Manhattan. Arch Neurol 57: 72-77.
Mackenzie IR, Baker M, Pickering-Brown S, Hsiung GY, Lindholm C, Dwosh E, Gass J, Cannon A, Rademakers R, Hutton M, Feldman HH. 2006. The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene. Brain 129: 3081-3090.
Peters N, Opherk C, Bergmann T, Castro M, Herzog J, Dichgans M. 2005. Spectrum of mutations in biopsy-proven CADASIL: Implications for diagnostic strategies. Arch Neurol 62: 1091-1094.
Queralt R, Ezquerra M, Lleo A, Castellvi M, Gelpi J, Ferrer I, Acarin N, Pasarin L, Blesa R, Oliva R. 2002. A novel mutation (V89L) in the presenilin 1 gene in a family with early onset Alzheimer's disease and marked behavioural disturbances. J Neurol Neurosurg Psychiatry 72: 266-269.
Schofield EC, Halliday GM, Kwok J, Loy C, Double KL, Hodges JR. 2010. Low serum progranulin predicts the presence of mutations: A prospective study. J Alzheimers Dis 22: 981-984.
Finch N, Baker M, Crook R, Swanson K, Kuntz K, Surtees R, Bisceglio G, Rovelet-Lecrux A, Boeve B, Petersen RC, Dickson DW, Younkin SG, Deramecourt V, Crook J, Graff-Radford NR, Rademakers R. 2009. Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members. Brain 132: 583-591.
Meeus B, Verstraeten A, Crosiers D, Engelborghs S, Van den Broeck M, Mattheijssens M, Peeters K, Corsmit E, Elinck E, Pickut B, Vandenberghe R, Cras P, De Deyn P, Van Broeckhoven C, Theuns J. 2012. DLB and PDD: A role for mutations in dementia and Parkinson disease genes? Neurobiol Aging 33: 629.
Piguet O, Hornberger M, Mioshi E, Hodges JR. 2011. Behavioural-variant frontotemporal dementia: Diagnosis, clinical staging, and management. Lancet Neurol 10: 162-172.
Yan J, Deng HX, Siddique N, Fecto F, Chen W, Yang Y, Liu E, Donkervoort S, Zheng JG, Shi Y, Ahmeti KB, Brooks B, Engel WK, Siddique T. 2010. Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia. Neurology 75: 807-814.
Munoz DG, Ros R, Fatas M, Bermejo F, de Yebenes JG. 2007. Progressive nonfluent aphasia associated with a new mutation V363I in tau gene. Am J Alzheimers Dis Other Demen 22: 294-299.
Cox LE, Ferraiuolo L, Goodall EF, Heath PR, Higginbottom A, Mortiboys H, Hollinger HC, Hartley JA, Brockington A, Burness CE, Morrison KE, Wharton SB, Grierson AJ, Ince PG, Kirby J, Shaw PJ. 2010. Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS). PLoS ONE 5: e9872.
Rohrer JD, Guerreiro R, Vandrovcova J, Uphill J, Reiman D, Beck J, Isaacs AM, Authier A, Ferrari R, Fox NC, Mackenzie IR, Warren JD, de Silva R, Holton J, Revesz T, Hardy J, Mead S, Rossor MN. 2009. The heritability and genetics of frontotemporal lobar degeneration. Neurology 73: 1451-1456.
Riedijk SR, Niermeijer MFN, Dooijes D, Tibben A. 2009. A decade of genetic counseling in frontotemporal dementia affected families: Few counseling requests and much familial opposition to testing. J Genet Couns 18: 350-356.
Ostojic J, Elfgren C, Passant U, Nilsson K, Gustafson L, Lannfelt L, Froelich Fabre S. 2004. The tau R406W mutation causes progressive presenile dementia with bitemporal atrophy. Dement Geriatr Cogn Disord 17: 298-2301.
Rohrer JD, Warren JD, Omar R, Mead S, Beck J, Revesz T, Holton J, Stevens JM, Al-Sarraj S, Pickering-Brown SM, Hardy J, Fox NC, Collinge J, Warrington EK, Rossor MN. 2008. Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene. Arch Neurol 65: 506-513.
Spillantini MG, Murrell JR, Goedert M, Farlow MR, Klug A, Ghetti B. 1998. Mutation in the tau gene in familial multiple system tauopathy with presenile dementia. Proc Natl Acad Sci USA 95: 7737-7741.
Ladogana A, Sanchez-Juan P, Mitrova E, Green A, Cuadrado-Corrales N, Sanchez-Valle R, Koscova S, Aguzzi A, Sklaviadis T, Kulczycki J, Gawinecka J, Saiz A, Calero M, van Duijn CM, Pocchiari M, Knight R, Zerr I. 2009. Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies. J Neurol 256: 1620-1628.
Mok K, Traynor B, Schymick J, Tienari P, Laaksovirta H, Peuralinna T, Myllykangas L, Chio A, Shatunov A, Boeve B, Boxer A, Dejesus-Hernandez M, Mackenzie I, Waite A, Williams N, Morris H, Simon-Sanchez J, van Swieten J, Heutink P, Restagno G, Mora G, Morrison K, Shaw P, Rollinson P, Al-Chalabi A, Rademakers R, Pickering-Brown S, Orrell R, Nalls M, Hardy J. 2012. The chromosome 9 ALS and FTD locus is probably derived from a single founder. Neurobiol Aging 209.e3-209.e8.
Lambert JC, Heath S, Even G, Campion D, Sleegers K, Hiltunen M, Combarros O, Zelenika D, Bullido MJ, Tavernier B, Letenneur L, Bettens K, Berr C, Pasquier F, Fievet N, Barberger-Gateau P, Engelborghs S, de Deyn P, Mateo I, Franck A, Helisalmi S, Porcellini E, Hanon O, de Pancorbo MM, Lendon C, Dufouil C, Jaillard C, Leveillard T, Alvarez V, Bosco P, Mancuso M, Panza F, Nacmias B, Bossu P, Piccardi P, Annoni G, Seripa D, Galimberti D, Hannequin D, Licastro F, Soininen H, Ritchie K, Blanche H, Dartigues JF, Tzourio C, Gut I, Van BC, Alperovitch A, Lathrop M, Amouyel P. 2009. Genome-wide association study identifies variants at CLU and CR1 associated with Alzheimer's disease. Nat Genet 41: 1094-1099.
Edison P, Archer HA, Hinz R, Hammers A, Pavese N, Tai YF, Hotton G, Cutler D, Fox N, Kennedy A, Rossor M, Brooks DJ. 2007. Amyloid, hypometabolism, and cognition in Alzheimer disease: An [11C]PIB and [18F]FDG PET study. Neurology 68: 501-508.
Chen-Plotkin AS, Martinez-Lage M, Sleiman PMA, Hu W, Greene R, Wood EM, Bing S, Grossman M, Schellenberg GD, Hatanpaa KJ, Weiner MF, White CL, Brooks WS, Halliday GM, Kril JJ, Gearing M, Beach TG, Graff-Radford NR, Dickson DW, Rademakers R, Boeve BF, Pickering-Brown SM, Snowden J, van Swieten JC, Heutink P, Seelaar H, Murrell JR, Ghetti B, Spina S, Grafman J, Kaye JA, Woltjer RL, Mesulam M, Bigio E, Llado A, Miller BL, Alzualde A, Moreno F, Rohrer JD, Mackenzie IRA, Feldman HH, Hamilton RL, Cruts M, Engelborghs S, de Deyn PP, Van Broeckhoven C, Bird TD, Cairns NJ, Goate A, Frosch MP, Riederer PF, Bogdanovic N, Lee VMY, Trojanowski JQ, Van Deerlin VM. 2011. Genetic and clinical features of progranulin-associated frontotemporal lobar degeneration. Arch Neurol 68: 488-497.
Clark RF, Hutton M, Fuldner M, Froelich S, Karran E, Talbot C, Crook R, Lendon C, Prihar G, He C, Korenblat K, Martinez A, Wragg W, Busfield F, Behrens MI, Myers A, Norton J, Morris J, Mehta N, Pearson C, Lincoln S, Baker M, Duff K, Zehr C, Perez-Tur J, Houlden H, Ruiz A, Ossa J, Lopera F, Acros M, Madrigal L, Collinge J, Humphreys
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2002; 14
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2012; 366
2010; 19
2010; 17
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1999; 46
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2004; 6
2005; 65
2008; 35
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1991; 115
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2011; 7
2006; 141B
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2008; 60
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2011; 332
2008; 283
2009; 30
2004; 17
2010; 257
2002; 287
2004; 13
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2011; 43
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References_xml – reference: Ghidoni R, Benussi L, Glionna M, Franzoni M, Binetti G. 2008. Low plasma progranulin levels predict progranulin mutations in frontotemporal lobar degeneration. Neurology 71: 1235-1239.
– reference: Small SA, Duff K. 2008. Linking Abeta and tau in late-onset Alzheimer's disease: A dual pathway hypothesis. Neuron 60: 534-542.
– reference: 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.
– reference: Tanahashi H, Kawakatsu S, Kaneko M, Yamanaka H, Takahashi K, Tabira T. 1996. Sequence analysis of presenilin-1 gene mutation in Japanese Alzheimer's disease patients. Neurosci Lett 218: 139-141.
– reference: Halliday GM, Song YJ, Lepar G, Brooks WS, Kwok JB, Kersaitis C, Gregory G, Shepherd CE, Rahimi F, Schofield PR, Kril JJ. 2005. Pick bodies in a family with presenilin-1 Alzheimer's disease. Ann Neurol 57: 139-143.
– reference: Munoz DG, Ros R, Fatas M, Bermejo F, de Yebenes JG. 2007. Progressive nonfluent aphasia associated with a new mutation V363I in tau gene. Am J Alzheimers Dis Other Demen 22: 294-299.
– reference: Nervi A, Reitz C, Tang MX, Santana V, Piriz A, Reyes D, Lantigua R, Medrano M, Jimenez-Velazquez IZ, Lee JH, Mayeux R. 2011. Familial aggregation of dementia with Lewy bodies. Arch Neurol 68: 90-93.
– reference: Rizzu P, van Mil SE, Anar B, Rosso SM, Donker Kaat L, Heutink P, van Swieten JC. 2006. CHMP2B mutations are not a cause of dementia in Dutch patients with familial and sporadic frontotemporal dementia. Am J Med Genet Part B 141B: 944-946.
– reference: Schumacher A, Friedrich P, Diehl-Schmid J, Ibach B, Pernechzky R, Eisele T, Vukovich R, Foerstl H, Riemenschneider M. 2009. No association of TDP-43 with sporadic frontotemporal dementia. Neurobiol Aging 30: 157-159.
– reference: Clark RF, Hutton M, Fuldner M, Froelich S, Karran E, Talbot C, Crook R, Lendon C, Prihar G, He C, Korenblat K, Martinez A, Wragg W, Busfield F, Behrens MI, Myers A, Norton J, Morris J, Mehta N, Pearson C, Lincoln S, Baker M, Duff K, Zehr C, Perez-Tur J, Houlden H, Ruiz A, Ossa J, Lopera F, Acros M, Madrigal L, Collinge J, Humphreys C, Ashwordth A, Sarner S, Fox N, Harvey R, Kennedy A, Roques P, Cline RT, Philips CA, Venter JC, Forsell L, Axelman K, Lilius L, Johnston J, Cowburn R, Viitanen M, Winblad B, Kosik K, Haltia M, Poyhonen M, Dickson D, Mann D, Neary D, Snowden J, Lantos P, Lannfelt L, Rossor M, Roberts GW, Adams MD, Hardy J, Goate A. 1995. The structure of the presenilin 1 (S182) gene and identification of six novel mutations in early onset AD families. Alzheimer's Disease Collaborative Group. Nat Genet 11: 219-222.
– reference: Mead S, Webb TEF, Campbell TA, Beck J, Linehan JM, Rutherfoord S, Joiner S, Wadsworth JDF, Heckmann J, Wroe S, Doey L, King A, Collinge J. 2007. Inherited prion disease with 5-OPRI: Phenotype modification by repeat length and codon 129. Neurology 69: 730-738.
– reference: 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.
– reference: Lindquist SG, Holm IE, Schwartz M, Law I, Stokholm J, Batbayli M, Waldemar G, Nielsen JE. 2008. Alzheimer disease-like clinical phenotype in a family with FTDP-17 caused by a MAPT R406W mutation. Eur J Neurol 15: 377-385.
– reference: McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CRJ, Kawas CH, Klunk WE, Koroshetz WJ, Manly JJ, Mayeux R, Mohs RC, Morris JC, Rossor MN, Scheltens P, Carrillo MC, Thies B, Weintraub S, Phelps CH. 2011. The diagnosis of dementia due to Alzheimer's disease: Recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement 7: 263-269.
– reference: Raux G, Gantier R, Thomas-Anterion C, Boulliat J, Verpillat P, Hannequin D, Brice A, Frebourg T, Campion D. 2000. Dementia with prominent frontotemporal features associated with L113P presenilin 1 mutation. Neurology 55: 1577-1578.
– reference: Ostojic J, Elfgren C, Passant U, Nilsson K, Gustafson L, Lannfelt L, Froelich Fabre S. 2004. The tau R406W mutation causes progressive presenile dementia with bitemporal atrophy. Dement Geriatr Cogn Disord 17: 298-2301.
– reference: van der Zee J, Pirici D, van Langenhove T, Engelborghs S, Vandenberghe R, Hoffmann M, Pusswald G, Van den Broeck M, Peeters K, Mattheijssens M, Martin JJ, de Deyn PP, Cruts M, Haubenberger D, Kumar-Singh S, Zimprich A, Van Broeckhoven C. 2009. Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His. Neurology 73: 626-632.
– reference: Finch N, Baker M, Crook R, Swanson K, Kuntz K, Surtees R, Bisceglio G, Rovelet-Lecrux A, Boeve B, Petersen RC, Dickson DW, Younkin SG, Deramecourt V, Crook J, Graff-Radford NR, Rademakers R. 2009. Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members. Brain 132: 583-591.
– reference: Neumann M, Tolnay M, Mackenzie IRA. 2009. The molecular basis of frontotemporal dementia. Expert Rev Mol Med 11: e23.
– reference: Alzualde A, Moreno F, Martinez-Lage P, Ferrer I, Gorostidi A, Otaegui D, Blazquez L, Atares B, Cardoso S, Martinez de Pancorbo M, Juste R, Rodriguez-Martinez AB, Indakoetxea B, Lopez de Munain A. 2010. Somatic mosaicism in a case of apparently sporadic Creutzfeldt-Jakob disease carrying a de novo D178N mutation in the PRNP gene. Am J Med Genet Part B 153B: 1283-1291.
– reference: Burgunder JM, Finsterer J, Szolnoki Z, Fontaine B, Baets J, Van Broeckhoven C, Di Donato S, de Jonghe P, Lynch T, Mariotti C, Schols L, Spinazzola A, Tabrizi SJ, Tallaksen C, Zeviani M, Harbo HF, Gasser T. 2010. EFNS guidelines on the molecular diagnosis of channelopathies, epilepsies, migraine, stroke, and dementias. Eur J Neurol 17: 641-648.
– reference: Yu CE, Bird TD, Bekris LM, Montine TJ, Leverenz JB, Steinbart E, Galloway NM, Feldman H, Woltjer R, Miller CA, Wood EM, Grossman M, McCluskey L, Clark CM, Neumann M, Danek A, Galasko DR, Arnold SE, Chen-Plotkin A, Karydas A, Miller BL, Trojanowski JQ, Lee VMY, Schellenberg GD, Van Deerlin VM. 2010. The spectrum of mutations in progranulin: A collaborative study screening 545 cases of neurodegeneration. Arch Neurol 67: 161-170.
– reference: Schofield EC, Halliday GM, Kwok J, Loy C, Double KL, Hodges JR. 2010. Low serum progranulin predicts the presence of mutations: A prospective study. J Alzheimers Dis 22: 981-984.
– reference: Green RC, Cupples LA, Go R, Benke KS, Edeki T, Griffith PA, Williams M, Hipps Y, Graff-Radford N, Bachman D, Farrer LA. 2002. Risk of dementia among white and African American relatives of patients with Alzheimer disease. JAMA 287: 329-336.
– reference: Portet F, Dauvilliers Y, Campion D, Raux G, Hauw JJ, Lyon-Caen O, Camu W, Touchon J. 2003. Very early onset AD with a de novo mutation in the presenilin 1 gene (Met 233 Leu). Neurology 61: 1136-1137.
– reference: Rohrer JD, Guerreiro R, Vandrovcova J, Uphill J, Reiman D, Beck J, Isaacs AM, Authier A, Ferrari R, Fox NC, Mackenzie IR, Warren JD, de Silva R, Holton J, Revesz T, Hardy J, Mead S, Rossor MN. 2009. The heritability and genetics of frontotemporal lobar degeneration. Neurology 73: 1451-1456.
– reference: Boeve BF, Tremont-Lukats IW, Waclawik AJ, Murrell JR, Hermann B, Jack CRJ, Shiung MM, Smith GE, Nair AR, Lindor N, Koppikar V, Ghetti B. 2005. Longitudinal characterization of two siblings with frontotemporal dementia and parkinsonism linked to chromosome 17 associated with the S305N tau mutation. Brain 128: 752-772.
– reference: Biffi A, Greenberg SM. 2011. Cerebral amyloid angiopathy: A systematic review. J Clin Neurol 7: 1-9.
– reference: Hardy J, Selkoe DJ. 2002. The amyloid hypothesis of Alzheimer's disease: Progress and problems on the road to therapeutics. Science 297: 353-356.
– reference: Brouwers N, Sleegers K, Engelborghs S, Bogaerts V, Serneels S, Kamali K, Corsmit E, de Leenheir E, Martin JJ, de Deyn PP, Van Broeckhoven C, Theuns J. 2006. Genetic risk and transcriptional variability of amyloid precursor protein in Alzheimer's disease. Brain 129: 2984-2991.
– reference: Klunk WE, Price JC, Mathis CA, Tsopelas ND, Lopresti BJ, Ziolko SK, Bi W, Hoge JA, Cohen AD, Ikonomovic MD, Saxton JA, Snitz BE, Pollen DA, Moonis M, Lippa CF, Swearer JM, Johnson KA, Rentz DM, Fischman AJ, Aizenstein HJ, DeKosky ST. 2007. Amyloid deposition begins in the striatum of presenilin-1 mutation carriers from two unrelated pedigrees. J Neurosci 27: 6174-6184.
– reference: Le Ber I, Camuzat A, Hannequin D, Pasquier F, Guedj E, Rovelet-Lecrux A, Hahn-Barma V, van der Zee J, Clot F, Bakchine S, Puel M, Ghanim M, Lacomblez L, Mikol J, Deramecourt V, Lejeune P, de la Sayette V, Belliard S, Vercelletto M, Meyrignac C, Van Broeckhoven C, Lambert JC, Verpillat P, Campion D, Habert MO, Dubois B, Brice A. 2008. Phenotype variability in progranulin mutation carriers: A clinical, neuropsychological, imaging and genetic study. Brain 131: 732-746.
– reference: Li Y, Rinne JO, Mosconi L, Pirraglia E, Rusinek H, DeSanti S, Kemppainen N, Nagren K, Kim BC, Tsui W, de Leon MJ. 2008. Regional analysis of FDG and PIB-PET images in normal aging, mild cognitive impairment, and Alzheimer's disease. Eur J Nucl Med Mol Imaging 35: 2169-2181.
– reference: 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.
– reference: Sleegers K, Brouwers N, Gijselinck I, Theuns J, Goossens D, Wauters J, Del-Favero J, Cruts M, van Duijn CM, Van Broeckhoven C. 2006. APP duplication is sufficient to cause early onset Alzheimer's dementia with cerebral amyloid angiopathy. Brain 129: 2977-2983.
– reference: Scholl M, Almkvist O, Axelman K, Stefanova E, Wall A, Westman E, Langstrom B, Lannfelt L, Graff C, Nordberg A. 2011. Glucose metabolism and PIB binding in carriers of a His163Tyr presenilin 1 mutation. Neurobiol Aging 32: 1388-1399.
– reference: Anfossi M, Bernardi L, Gallo M, Geracitano S, Colao R, Puccio G, Curcio SA, Frangipane F, Mirabelli M, Tomaino C, Smirne N, Maletta R, Bruni AC. 2011. MAPT V363I variation in a sporadic case of frontotemporal dementia: Variable penetrant mutation or rare polymorphism? Alzheimer Dis Assoc Disord 25: 96-99.
– reference: Moonis M, Swearer JM, Dayaw MPE, St George-Hyslop P, Rogaeva E, Kawarai T, Pollen DA. 2005. Familial Alzheimer disease: Decreases in CSF Abeta42 levels precede cognitive decline. Neurology 65: 323-325.
– reference: Borroni B, Archetti S, Alberici A, Agosti C, Gennarelli M, Bigni B, Bonvicini C, Ferrari M, Bellelli G, Galimberti D, Scarpini E, Di Lorenzo D, Caimi L, Caltagirone C, Di Luca M, Padovani A. 2008. Progranulin genetic variations in frontotemporal lobar degeneration: Evidence for low mutation frequency in an Italian clinical series. Neurogenetics 9: 197-205.
– reference: Jayadev S, Nochlin D, Poorkaj P, Steinbart EJ, Mastrianni JA, Montine TJ, Ghetti B, Schellenberg GD, Bird TD, Leverenz JB. 2011. Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype. Ann Neurol 69: 712-720.
– reference: Kovacs GG, Murrell JR, Horvath S, Haraszti L, Majtenyi K, Molnar MJ, Budka H, Ghetti B, Spina S. 2009. TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea. Mov Disord 24: 1843-1847.
– reference: Quaid K. 2011. Genetic counseling for frontotemporal dementias. J Mol Neurosci 45: 706-709.
– reference: Rollinson S, Snowden JS, Neary D, Morrison KE, Mann DM, Pickering-Brown SM. 2007. TDP-43 gene analysis in frontotemporal lobar degeneration. Neurosci Lett 419: 1-4.
– reference: Rovelet-Lecrux A, Hannequin D, Raux G, Le Meur N, Laquerriere A, Vital A, Dumanchin C, Feuillette S, Brice A, Vercelletto M, Dubas F, Frebourg T, Campion D. 2006. APP locus duplication causes autosomal dominant early-onset Alzheimer disease with cerebral amyloid angiopathy. Nat Genet 38: 24-26.
– reference: Menendez M. 2004. Pathological and clinical heterogeneity of presenilin 1 gene mutations. J Alzheimers Dis 6: 475-482.
– reference: Ladogana A, Sanchez-Juan P, Mitrova E, Green A, Cuadrado-Corrales N, Sanchez-Valle R, Koscova S, Aguzzi A, Sklaviadis T, Kulczycki J, Gawinecka J, Saiz A, Calero M, van Duijn CM, Pocchiari M, Knight R, Zerr I. 2009. Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies. J Neurol 256: 1620-1628.
– reference: Gijselinck I, Van Langenhove T, van der Zee J, Sleegers K, Philtjens S, Kleinberger G, Janssens J, Bettens K, Van Cauwenberghe C, Pereson S, Engelborghs S, Sieben A, De Jonghe P, Vandenberghe R, Santens P, De Bleecker J, Maes G, Baumer V, Dillen L, Joris G, Cuijt I, Corsmit E, Elinck E, Van Dongen J, Vermeulen S, Van den Broeck M, Vaerenberg C, Mattheijssens M, Peeters K, Robberecht W, Cras P, Martin JJ, De Deyn PP, Cruts M, Van Broeckhoven C. 2012. A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: A gene identification study. Lancet Neurol 11: 54-65.
– reference: Skibinski G, Parkinson NJ, Brown JM, Chakrabarti L, Lloyd SL, Hummerich H, Nielsen JE, Hodges JR, Spillantini MG, Thusgaard T, Brandner S, Brun A, Rossor MN, Gade A, Johannsen P, Sorensen SA, Gydesen S, Fisher EM, Collinge J. 2005. Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet 37: 806-808.
– reference: McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G, Quinn NP, Edwardson JA, Ince PG, Bergeron C, Burns A, Miller BL, Lovestone S, Collerton D, Jansen EN, Ballard C, de Vos RA, Wilcock GK, Jellinger KA, Perry RH. 1996. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop. Neurology 47: 1113-1124.
– reference: Baker M, Mackenzie IR, Pickering-Brown SM, Gass J, Rademakers R, Lindholm C, Snowden J, Adamson J, Sadovnick AD, Rollinson S, Cannon A, Dwosh E, Neary D, Melquist S, Richardson A, Dickson D, Berger Z, Eriksen J, Robinson T, Zehr C, Dickey CA, Crook R, McGowan E, Mann D, Boeve B, Feldman H, Hutton M. 2006. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature 442: 916-919.
– reference: Poorkaj P, Grossman M, Steinbart E, Payami H, Sadovnick A, Nochlin D, Tabira T, Trojanowski JQ, Borson S, Galasko D, Reich S, Quinn B, Schellenberg G, Bird TD. 2001. Frequency of tau gene mutations in familial and sporadic cases of non-Alzheimer dementia. Arch Neurol 58: 383-387.
– reference: Momeni P, DeTucci K, Straub RE, Weinberger DR, Davies P, Grafman J, Hardy J, Huey ED. 2010. Progranulin (GRN) in two siblings of a Latino family and in other patients with schizophrenia. Neurocase 16: 273-279.
– reference: Majounie E, Abramzon Y, Renton AE, Perry R, Bassett SS, Pletnikova O, Troncoso JC, Hardy J, Singleton AB, Traynor BJ. 2012. Repeat expansion in C9ORF72 in Alzheimer's disease. N Engl J Med 366: 283-284.
– reference: Chen-Plotkin AS, Martinez-Lage M, Sleiman PMA, Hu W, Greene R, Wood EM, Bing S, Grossman M, Schellenberg GD, Hatanpaa KJ, Weiner MF, White CL, Brooks WS, Halliday GM, Kril JJ, Gearing M, Beach TG, Graff-Radford NR, Dickson DW, Rademakers R, Boeve BF, Pickering-Brown SM, Snowden J, van Swieten JC, Heutink P, Seelaar H, Murrell JR, Ghetti B, Spina S, Grafman J, Kaye JA, Woltjer RL, Mesulam M, Bigio E, Llado A, Miller BL, Alzualde A, Moreno F, Rohrer JD, Mackenzie IRA, Feldman HH, Hamilton RL, Cruts M, Engelborghs S, de Deyn PP, Van Broeckhoven C, Bird TD, Cairns NJ, Goate A, Frosch MP, Riederer PF, Bogdanovic N, Lee VMY, Trojanowski JQ, Van Deerlin VM. 2011. Genetic and clinical features of progranulin-associated frontotemporal lobar degeneration. Arch Neurol 68: 488-497.
– reference: Devi G, Ottman R, Tang MX, Marder K, Stern Y, Mayeux R. 2000. Familial aggregation of Alzheimer disease among whites, African Americans, and Caribbean Hispanics in northern Manhattan. Arch Neurol 57: 72-77.
– reference: Dermaut B, Kumar-Singh S, Engelborghs S, Theuns J, Rademakers R, Saerens J, Pickut BA, Peeters K, Van den Broeck M, Vennekens K, Claes S, Cruts M, Cras P, Martin JJ, Van BC, de Deyn PP. 2004. A novel presenilin 1 mutation associated with Pick's disease but not beta-amyloid plaques. Ann Neurol 55: 617-626.
– reference: Lleo A, Blesa R, Queralt R, Ezquerra M, Molinuevo JL, Pena-Casanova J, Rojo A, Oliva R. 2002. Frequency of mutations in the presenilin and amyloid precursor protein genes in early-onset Alzheimer disease in Spain. Arch Neurol 59: 1759-1763.
– reference: Nozaki I, Hamaguchi T, Sanjo N, Noguchi-Shinohara M, Sakai K, Nakamura Y, Sato T, Kitamoto T, Mizusawa H, Moriwaka F, Shiga Y, Kuroiwa Y, Nishizawa M, Kuzuhara S, Inuzuka T, Takeda M, Kuroda S, Abe K, Murai H, Murayama S, Tateishi J, Takumi I, Shirabe S, Harada M, Sadakane A, Yamada M. 2010. Prospective 10-year surveillance of human prion diseases in Japan. Brain 133: 3043-3057.
– reference: Alberici A, Bonato C, Borroni B, Cotelli M, Mattioli F, Binetti G, Gennarelli M, Luca MD, Simonati A, Perani D, Rossini P, Padovani A. 2007. Dementia, delusions and seizures: Storage disease or genetic AD? Eur J Neurol 14: 1057-1059.
– reference: Reed LA, Wszolek ZK, Hutton M. 2001. Phenotypic correlations in FTDP-17. Neurobiol Aging 22: 89-107.
– reference: Larner AJ, Doran M. 2006. Clinical phenotypic heterogeneity of Alzheimer's disease associated with mutations of the presenilin-1 gene. J Neurol 253: 139-158.
– reference: Murray M, Dejesus-Hernandez M, Rutherford N, Baker M, Duara R, Graff-Radford N, Wszolek Z, Ferman T, Josephs K, Boylan K, Rademakers R, Dickson D. 2011. Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol 122: 673-690.
– reference: Jun G, Naj AC, Beecham GW, Wang LS, Buros J, Gallins PJ, Buxbaum JD, Ertekin-Taner N, Fallin MD, Friedland R, Inzelberg R, Kramer P, Rogaeva E, St George-Hyslop P, Cantwell LB, Dombroski BA, Saykin AJ, Reiman EM, Bennett DA, Morris JC, Lunetta KL, Martin ER, Montine TJ, Goate AM, Blacker D, Tsuang DW, Beekly D, Cupples LA, Hakonarson H, Kukull W, Foroud TM, Haines J, Mayeux R, Farrer LA, Pericak-Vance MA, Schellenberg GD. 2010. Meta-analysis confirms CR1, CLU, and PICALM as alzheimer disease risk loci and reveals interactions with APOE genotypes. Arch Neurol 67: 1473-1484.
– reference: Kauwe JSK, Jacquart S, Chakraverty S, Wang J, Mayo K, Fagan AM, Holtzman DM, Morris JC, Goate AM. 2007. Extreme cerebrospinal fluid amyloid beta levels identify family with late-onset Alzheimer's disease presenilin 1 mutation. Ann Neurol 61: 446-453.
– reference: Chapman J, Ben-Israel J, Goldhammer Y, Korczyn AD. 1994. The risk of developing Creutzfeldt-Jakob disease in subjects with the PRNP gene codon 200 point mutation. Neurology 44: 1683-1686.
– reference: Harold D, Abraham R, Hollingworth P, Sims R, Gerrish A, Hamshere ML, Pahwa JS, Moskvina V, Dowzell K, Williams A, Jones N, Thomas C, Stretton A, Morgan AR, Lovestone S, Powell J, Proitsi P, Lupton MK, Brayne C, Rubinsztein DC, Gill M, Lawlor B, Lynch A, Morgan K, Brown KS, Passmore PA, Craig D, McGuinness B, Todd S, Holmes C, Mann D, Smith AD, Love S, Kehoe PG, Hardy J, Mead S, Fox N, Rossor M, Collinge J, Maier W, Jessen F, Schurmann B, van den Bussche H, Heuser I, Kornhuber J, Wiltfang J, Dichgans M, Frolich L, Hampel H, Hull M, Rujescu D, Goate AM, Kauwe JS, Cruchaga C, Nowotny P, Morris JC, Mayo K, Sleegers K, Bettens K, Engelborghs S, de Deyn PP, Van BC, Livingston G, Bass NJ, Gurling H, McQuillin A, Gwilliam R, Deloukas P, Al-Chalabi A, Shaw CE, Tsolaki M, Singleton AB, Guerreiro R, Muhleisen TW, Nothen MM, Moebus S, Jockel KH, Klopp N, Wichmann HE, Carrasquillo MM, Pankratz VS, Younkin SG, Holmans PA, O'Donovan M, Owen MJ, Williams J. 2009. Genome-wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease. Nat Genet 41: 1088-1093.
– reference: Spillantini MG, Murrell JR, Goedert M, Farlow MR, Klug A, Ghetti B. 1998. Mutation in the tau gene in familial multiple system tauopathy with presenile dementia. Proc Natl Acad Sci USA 95: 7737-7741.
– reference: Rosso SM, Donker Kaat L, Baks T, Joosse M, de Koning I, Pijnenburg Y, de Jong D, Dooijes D, Kamphorst W, Ravid R, Niermeijer MF, Verheij F, Kremer HP, Scheltens P, van Duijn CM, Heutink P, van Swieten JC. 2003. Frontotemporal dementia in The Netherlands: Patient characteristics and prevalence estimates from a population-based study. Brain 126: 2016-2022.
– reference: Urwin H, Josephs KA, Rohrer JD, Mackenzie IR, Neumann M, Authier A, Seelaar H, van Swieten JC, Brown JM, Johannsen P, Nielsen JE, Holm IE, Dickson DW, Rademakers R, Graff-Radford NR, Parisi JE, Petersen RC, Hatanpaa KJ, White CL III, Weiner MF, Geser F, Van Deerlin VM, Trojanowski JQ, Miller BL, Seeley WW, van der Zee J, Kumar-Singh S, Engelborghs S, de Deyn PP, Van Broeckhoven C, Bigio EH, Deng HX, Halliday GM, Kril JJ, Munoz DG, Mann DM, Pickering-Brown SM, Doodeman V, Adamson G, Ghazi-Noori S, Fisher EM, Holton JL, Revesz T, Rossor MN, Collinge J, Mead S, Isaacs AM. 2010. FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol 120: 33-41.
– reference: Leyton C, Villemagne V, Savage S, Pike K, Ballard K, Piguet O, Burrell J, Rowe C, Hodges J. 2011. Subtypes of progressive aphasia: Application of the international consensus criteria and validation using {beta}-amyloid imaging. Brain 134: 3030-3043.
– reference: Seelaar H, Kamphorst W, Rosso SM, Azmani A, Masdjedi R, de Koning I, Maat-Kievit JA, Anar B, Donker KL, Breedveld GJ, Dooijes D, Rozemuller JM, Bronner IF, Rizzu P, van Swieten JC. 2008. Distinct genetic forms of frontotemporal dementia. Neurology 71: 1220-1226.
– reference: Schoder D, Hannequin D, Martinaud O, Opolczynski G, Guyant-Marechal L, Le Ber I, Campion D. 2010. Morbid risk for schizophrenia in first-degree relatives of people with frontotemporal dementia. Br J Psychiatry 197: 28-35.
– reference: Kaivorinne AL, Kruger J, Kuivaniemi K, Tuominen H, Moilanen V, Majamaa K, Remes AM. 2008. Role of MAPT mutations and haplotype in frontotemporal lobar degeneration in Northern Finland. BMC Neurol 8: 48.
– reference: van der Zee J, Urwin H, Engelborghs S, Bruyland M, Vandenberghe R, Dermaut B, de Pooter T, Peeters K, Santens P, de Deyn PP, Fisher EM, Collinge J, Isaacs AM, Van Broeckhoven C. 2008. CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro. Hum Mol Genet 17: 313-322.
– reference: Broustal O, Camuzat A, Guillot-Noel L, Guy N, Millecamps S, Deffond D, Lacomblez L, Golfier V, Hannequin D, Salachas F, Camu W, Didic M, Dubois B, Meininger V, Le B I, Brice A. 2010. FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis. J Alzheimers Dis 22: 765-769.
– reference: Queralt R, Ezquerra M, Lleo A, Castellvi M, Gelpi J, Ferrer I, Acarin N, Pasarin L, Blesa R, Oliva R. 2002. A novel mutation (V89L) in the presenilin 1 gene in a family with early onset Alzheimer's disease and marked behavioural disturbances. J Neurol Neurosurg Psychiatry 72: 266-269.
– reference: Genin E, Hannequin D, Wallon D, Sleegers K, Hiltunen M, Combarros O, Bullido MJ, Engelborghs S, de Deyn P, Berr C, Pasquier F, Dubois B, Tognoni G, Fievet N, Brouwers N, Bettens K, Arosio B, Coto E, Del Zompo M, Mateo I, Epelbaum J, Frank-Garcia A, Helisalmi S, Porcellini E, Pilotto A, Forti P, Ferri R, Scarpini E, Siciliano G, Solfrizzi V, Sorbi S, Spalletta G, Valdivieso F, Vepsalainen S, Alvarez V, Bosco P, Mancuso M, Panza F, Nacmias B, Bossu P, Hanon O, Piccardi P, Annoni G, Seripa D, Galimberti D, Licastro F, Soininen H, Dartigues JF, Kamboh MI, Van Broeckhoven C, Lambert JC, Amouyel P, Campion D. 2011. APOE and Alzheimer disease: A major gene with semi-dominant inheritance. Mol Psychiatry 16: 903-907.
– reference: Alzheimer's Association. 2011. 2011 Alzheimer's disease facts and figures. Alzheimers Dement 7: 208-244.
– reference: Helzner EP, Scarmeas N, Cosentino S, Tang MX, Schupf N, Stern Y. 2008. Survival in Alzheimer disease: A multiethnic, population-based study of incident cases. Neurology 71: 1489-1495.
– reference: Hutton M, Lendon CL, Rizzu P, Baker M, Froelich S, Houlden H, Pickering-Brown S, Chakraverty S, Isaacs A, Grover A, Hackett J, Adamson J, Lincoln S, Dickson D, Davies P, Petersen RC, Stevens M, de Graaff E, Wauters E, van Baren J, Hillebrand M, Joosse M, Kwon JM, Nowotny P, Che LK, Norton J, Morris JC, Reed LA, Trojanowski J, Basun H, Lannfelt L, Neystat M, Fahn S, Dark F, Tannenberg T, Dodd PR, Hayward N, Kwok JB, Schofield PR, Andreadis A, Snowden J, Craufurd D, Neary D, Owen F, Oostra BA, Hardy J, Goate A, van Swieten J, Mann D, Lynch T, Heutink P. 1998. Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 393: 702-705.
– reference: Roberts JS, Cupples LA, Relkin NR, Whitehouse PJ, Green RC. 2005. Genetic risk assessment for adult children of people with Alzheimer's disease: The Risk Evaluation and Education for Alzheimer's Disease (REVEAL) study. J Geriatr Psychiatry Neurol 18: 250-255.
– reference: Wardle M, Morris HR, Robertson NP. 2009. Clinical and genetic characteristics of non-Asian dentatorubral-pallidoluysian atrophy: A systematic review. Mov Disord 24: 1636-1640.
– reference: Bian H, van Swieten JC, Leight S, Massimo L, Wood E, Forman M, Moore P, de Koning I, Clark CM, Rosso S, Trojanowski J, Lee VMY, Grossman M. 2008. CSF biomarkers in frontotemporal lobar degeneration with known pathology. Neurology 70: 1827-1835.
– reference: Oberstein SA. 2003. Diagnostic strategies in CADASIL. Neurology 60: 2020.
– reference: Burrell JR, Kiernan MC, Vucic S, Hodges JR. 2011. Motor neuron dysfunction in frontotemporal dementia. Brain 134: 2582-2594.
– reference: 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.
– reference: McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ. 2001. Clinical and pathological diagnosis of frontotemporal dementia: Report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 58: 1803-1809.
– reference: Renton AE, Majounie E, Waite A, Simon-Sanchez J, Rollinson S, Gibbs JR, Schymick JC, Laaksovirta H, van Swieten JC, Myllykangas L, Kalimo H, Paetau A, Abramzon Y, Remes AM, Kaganovich A, Scholz SW, Duckworth J, Ding J, Harmer DW, Hernandez DG, Johnson JO, Mok K, Ryten M, Trabzuni D, Guerreiro RJ, Orrell RW, Neal J, Murray A, Pearson J, Jansen IE, Sondervan D, Seelaar H, Blake D, Young K, Halliwell N, Callister JB, Toulson G, Richardson A, Gerhard A, Snowden J, Mann D, Neary D, Nalls MA, Peuralinna T, Jansson L, Isoviita VM, Kaivorinne AL, Holtta-Vuori M, Ikonen E, Sulkava R, Benatar M, Wuu J, Chio A, Restagno G, Borghero G, Sabatelli M, Heckerman D, Rogaeva E, Zinman L, Rothstein JD, Sendtner M, Drepper C, Eichler EE, Alkan C, Abdullaev Z, Pack SD, Dutra A, Pak E, Hardy J, Singleton A, Williams NM, Heutink P, Pickering-Brown S, Morris HR, Tienari PJ, Traynor BJ. 2011. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72: 257-268.
– reference: Mead S, Poulter M, Beck J, Webb TEF, Campbell TA, Linehan JM, Desbruslais M, Joiner S, Wadsworth JDF, King A, Lantos P, Collinge J. 2006. Inherited prion disease with six octapeptide repeat insertional mutation-Molecular analysis of phenotypic heterogeneity. Brain 129: 2297-2317.
– reference: Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. 2005. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology 65: 586-590.
– reference: Cruts M, van Duijn CM, Backhovens H, Van den Broeck M, Wehnert A, Serneels S, Sherrington R, Hutton M, Hardy J, St George-Hyslop PH, Hofman A, Van BC. 1998. Estimation of the genetic contribution of presenilin-1 and -2 mutations in a population-based study of presenile Alzheimer disease. Hum Mol Genet 7: 43-51.
– reference: Yan J, Deng HX, Siddique N, Fecto F, Chen W, Yang Y, Liu E, Donkervoort S, Zheng JG, Shi Y, Ahmeti KB, Brooks B, Engel WK, Siddique T. 2010. Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia. Neurology 75: 807-814.
– reference: Rascovsky K, Hodges J, Knopman D, Mendez M, Kramer J, Neuhaus J, van Swieten J, Seelaar H, Dopper E, Onyike C, Hillis A, Josephs K, Boeve B, Kertesz A, Seeley W, Rankin K, Johnson J, Gorno-Tempini M, Rosen H, Prioleau-Latham C, Lee A, Kipps C, Lillo P, Piguet O, Rohrer J, Rossor M, Warren J, Fox N, Galasko D, Salmon D, Black S, Mesulam M, Weintraub S, Dickerson B, Diehl-Schmid J, Pasquier F, Deramecourt V, Lebert F, Pijnenburg Y, Chow T, Manes F, Grafman J, Cappa S, Freedman M, Grossman M, Miller B. 2011. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134: 2456-2477.
– reference: Mendez MF, McMurtray A. 2006. Frontotemporal dementia-like phenotypes associated with presenilin-1 mutations. Am J Alzheimers Dis Other Demen 21: 281-286.
– reference: Watts GD, Wymer J, Kovach MJ, Mehta SG, Mumm S, Darvish D, Pestronk A, Whyte MP, Kimonis VE. 2004. Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat Genet 36: 377-381.
– reference: Fabre SF, Forsell C, Viitanen M, Sjogren M, Wallin A, Blennow K, Blomberg M, Andersen C, Wahlund LO, Lannfelt L. 2001. Clinic-based cases with frontotemporal dementia show increased cerebrospinal fluid tau and high apolipoprotein E epsilon4 frequency, but no tau gene mutations. Exp Neurol 168: 413-418.
– reference: Mullan M, Crawford F, Axelman K, Houlden H, Lilius L, Winblad B, Lannfelt L. 1992. A pathogenic mutation for probable Alzheimer's disease in the APP gene at the N-terminus of beta-amyloid. Nat Genet 1: 345-347.
– reference: Arango D, Cruts M, Torres O, Backhovens H, Serrano ML, Villareal E, Montanes P, Matallana D, Cano C, Van Broeckhoven C, Jacquier M. 2001. Systematic genetic study of Alzheimer disease in Latin America: Mutation frequencies of the amyloid beta precursor protein and presenilin genes in Colombia. Am J Med Genet 103: 138-143.
– reference: Piguet O, Hornberger M, Mioshi E, Hodges JR. 2011. Behavioural-variant frontotemporal dementia: Diagnosis, clinical staging, and management. Lancet Neurol 10: 162-172.
– reference: Mok K, Traynor B, Schymick J, Tienari P, Laaksovirta H, Peuralinna T, Myllykangas L, Chio A, Shatunov A, Boeve B, Boxer A, Dejesus-Hernandez M, Mackenzie I, Waite A, Williams N, Morris H, Simon-Sanchez J, van Swieten J, Heutink P, Restagno G, Mora G, Morrison K, Shaw P, Rollinson P, Al-Chalabi A, Rademakers R, Pickering-Brown S, Orrell R, Nalls M, Hardy J. 2012. The chromosome 9 ALS and FTD locus is probably derived from a single founder. Neurobiol Aging 209.e3-209.e8.
– reference: Seelaar H, Klijnsma KY, de Koning I, van der Lugt A, Chiu WZ, Azmani A, Rozemuller AJ, van Swieten JC. 2010. Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration. J Neurol 257: 747-753.
– reference: Pimplikar SW, Nixon RA, Robakis NK, Shen J, Tsai LH. 2010. Amyloid-independent mechanisms in Alzheimer's disease pathogenesis. J Neurosci 30: 14946-14954.
– reference: Gatz M, Reynolds CA, Fratiglioni L, Johansson B, Mortimer JA, Berg S, Fiske A, Pedersen NL. 2006. Role of genes and environments for explaining Alzheimer disease. Arch Gen Psychiatry 63: 168-174.
– reference: Goldman JS, Farmer JM, Wood EM, Johnson JK, Boxer A, Neuhaus J, Lomen-Hoerth C, Wilhelmsen KC, Lee VMY, Grossman M, Miller BL. 2005. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology 65: 1817-1819.
– reference: Jack CRJ, Lowe VJ, Weigand SD, Wiste HJ, Senjem ML, Knopman DS, Shiung MM, Gunter JL, Boeve BF, Kemp BJ, Weiner M, Petersen RC. 2009. Serial PIB and MRI in normal, mild cognitive impairment and Alzheimer's disease: Implications for sequence of pathological events in Alzheimer's disease. Brain 132: 1355-1365.
– reference: Blom ES, Viswanathan J, Kilander L, Helisalmi S, Soininen H, Lannfelt L, Ingelsson M, Glaser A, Hiltunen M. 2008. Low prevalence of APP duplications in Swedish and Finnish patients with early-onset Alzheimer's disease. Eur J Hum Genet 16: 171-175.
– reference: Whitwell JL, Jack CR Jr, Boeve BF, Senjem ML, Baker M, Rademakers R, Ivnik RJ, Knopman DS, Wszolek ZK, Petersen RC, Josephs KA. 2009. Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN. Neurology 72: 813-820.
– reference: Carecchio M, Fenoglio C, de Riz M, Guidi I, Comi C, Cortini F, Venturelli E, Restelli I, Cantoni C, Bresolin N, Monaco F, Scarpini E, Galimberti D. 2009. Progranulin plasma levels as potential biomarker for the identification of GRN deletion carriers. A case with atypical onset as clinical amnestic Mild Cognitive Impairment converted to Alzheimer's disease. J Neurol Sci 287: 291-293.
– reference: Jayadev S, Leverenz JB, Steinbart E, Stahl J, Klunk W, Yu CE, Bird TD. 2010. Alzheimer's disease phenotypes and genotypes associated with mutations in presenilin 2. Brain 133: 1143-1154.
– reference: Romero LJ, Garry PJ, Schuyler M, Bennahum DA, Qualls C, Ballinger L, Kelly V, Schmitt C, Skipper B, Ortiz IE, Rhyne RL. 2005. Emotional responses to APO E genotype disclosure for Alzheimer disease. J Genet Couns 14: 141-150.
– reference: Finckh U, Muller-Thomsen T, Mann U, Eggers C, Marksteiner J, Meins W, Binetti G, Alberici A, Hock C, Nitsch RM, Gal A. 2000. High prevalence of pathogenic mutations in patients with early-onset dementia detected by sequence analyses of four different genes. Am J Hum Genet 66: 110-117.
– reference: Rogaeva EA, Fafel KC, Song YQ, Medeiros H, Sato C, Liang Y, Richard E, Rogaev EI, Frommelt P, Sadovnick AD, Meschino W, Rockwood K, Boss MA, Mayeux R, St George-Hyslop P. 2001. Screening for PS1 mutations in a referral-based series of AD cases: 21 novel mutations. Neurology 57: 621-625.
– reference: Janssen JC, Beck JA, Campbell TA, Dickinson A, Fox NC, Harvey RJ, Houlden H, Rossor MN, Collinge J. 2003. Early onset familial Alzheimer's disease: Mutation frequency in 31 families. Neurology 60: 235-239.
– reference: Stevens M, van Duijn CM, Kamphorst W, de Knijff P, Heutink P, van Gool WA, Scheltens P, Ravid R, Oostra BA, Niermeijer MF, van Swieten JC. 1998. Familial aggregation in frontotemporal dementia. Neurology 50: 1541-1545.
– reference: Dagvadorj A, Petersen RB, Lee HS, Cervenakova L, Shatunov A, Budka H, Brown P, Gambetti P, Goldfarb LG. 2002. Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy. Ann Neurol 52: 355-359.
– reference: 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.
– reference: 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.
– reference: Mackenzie IR, Baker M, Pickering-Brown S, Hsiung GY, Lindholm C, Dwosh E, Gass J, Cannon A, Rademakers R, Hutton M, Feldman HH. 2006. The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene. Brain 129: 3081-3090.
– reference: van Langenhove T, van der Zee J, Sleegers K, Engelborghs S, Vandenberghe R, Gijselinck I, Van den Broeck M, Mattheijssens M, Peeters K, de Deyn PP, Cruts M, Van Broeckhoven C. 2010. Genetic contribution of FUS to frontotemporal lobar degeneration. Neurology 74: 366-371.
– reference: Ratnavalli E, Brayne C, Dawson K, Hodges JR. 2002. The prevalence of frontotemporal dementia. Neurology 58: 1615-1621.
– reference: Breitner JC. 1991. Clinical genetics and genetic counseling in Alzheimer disease. Ann Intern Med 115: 601-606.
– reference: Koedam ELGE, Lauffer V, van der Vlies AE, van der Flier WM, Scheltens P, Pijnenburg YAL. 2010. Early-versus late-onset Alzheimer's disease: More than age alone. J Alzheimers Dis 19: 1401-1408.
– reference: Johnson JK, Diehl J, Mendez MF, Neuhaus J, Shapira JS, Forman M, Chute DJ, Roberson ED, Pace-Savitsky C, Neumann M, Chow TW, Rosen HJ, Forstl H, Kurz A, Miller BL. 2005. Frontotemporal lobar degeneration: Demographic characteristics of 353 patients. Arch Neurol 62: 925-930.
– reference: Villemagne VL, Ataka S, Mizuno T, Brooks WS, Wada Y, Kondo M, Jones G, Watanabe Y, Mulligan R, Nakagawa M, Miki T, Shimada H, O'Keefe GJ, Masters CL, Mori H, Rowe CC. 2009. High striatal amyloid beta-peptide deposition across different autosomal Alzheimer disease mutation types. Arch Neurol 66: 1537-1544.
– reference: Borroni B, Bonvicini C, Alberici A, Buratti E, Agosti C, Archetti S, Papetti A, Stuani C, Di Luca M, Gennarelli M, Padovani A. 2009. Mutation within TARDBP leads to frontotemporal dementia without motor neuron disease. Hum Mutat 30: E974-E983.
– reference: Rohrer JD, Warren JD, Omar R, Mead S, Beck J, Revesz T, Holton J, Stevens JM, Al-Sarraj S, Pickering-Brown SM, Hardy J, Fox NC, Collinge J, Warrington EK, Rossor MN. 2008. Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene. Arch Neurol 65: 506-513.
– reference: Kovacs GG, Trabattoni G, Hainfellner JA, Ironside JW, Knight RS, Budka H. 2002. Mutations of the prion protein gene phenotypic spectrum. J Neurol 249: 1567-1582.
– reference: Shankaran SS, Capell A, Hruscha AT, Fellerer K, Neumann M, Schmid B, Haass C. 2008. Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion. J Biol Chem 283: 1744-1753.
– reference: Green RC, Roberts JS, Cupples LA, Relkin NR, Whitehouse PJ, Brown T, Eckert SL, Butson M, Sadovnick AD, Quaid KA, Chen C, Cook-Deegan R, Farrer LA. 2009. Disclosure of APOE genotype for risk of Alzheimer's disease. N Engl J Med 361: 245-254.
– reference: Herve D, Chabriat H. 2010. CADASIL. J Geriatr Psychiatry Neurol 23: 269-276.
– reference: Johnson JO, Mandrioli J, Benatar M, Abramzon Y, Van Deerlin VM, Trojanowski JQ, Gibbs JR, Brunetti M, Gronka S, Wuu J, Ding J, McCluskey L, Martinez-Lage M, Falcone D, Hernandez DG, Arepalli S, Chong S, Schymick JC, Rothstein J, Landi F, Wang YD, Calvo A, Mora G, Sabatelli M, Monsurro MR, Battistini S, Salvi F, Spataro R, Sola P, Borghero G, Galassi G, Scholz SW, Taylor JP, Restagno G, Chio A, Traynor BJ. 2010. Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 68: 857-864.
– reference: EUROCJD. 2001. Genetic epidemiology of Creutzfeldt-Jakob disease in Europe. Rev Neurol (Paris) 157: 633-637.
– reference: Gijselinck I, Sleegers K, Engelborghs S, Robberecht W, Martin JJ, Vandenberghe R, Sciot R, Dermaut B, Goossens D, van der Zee J, de Pooter T, Del-Favero J, Santens P, de Jonghe P, de Deyn PP, Van Broeckhoven C, Cruts M. 2009. Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS. Neurobiol Aging 30: 1329-1331.
– reference: Fecto F, Siddique T. 2011. Making Connections: Pathology and Genetics Link Amyotrophic Lateral Sclerosis with Frontotemporal Lobe Dementia. J Mol Neurosci 45: 663-675.
– reference: Le Ber I, van der Zee J, Hannequin D, Gijselinck I, Campion D, Puel M, Laquerriere A, de Pooter T, Camuzat A, Van den Broeck M, Dubois B, Sellal F, Lacomblez L, Vercelletto M, Thomas-Anterion C, Michel BF, Golfier V, Didic M, Salachas F, Duyckaerts C, Cruts M, Verpillat P, Van Broeckhoven C, Brice A. 2007. Progranulin null mutations in both sporadic and familial frontotemporal dementia. Hum Mutat 28: 846-855.
– reference: Steinbart EJ, Smith CO, Poorkaj P, Bird TD. 2001. Impact of DNA testing for early-onset familial Alzheimer disease and frontotemporal dementia. Arch Neurol 58: 1828-1831.
– reference: Deng H, Chen W, Hong S, Boycott K, Gorrie G, Siddique N, Yang Y, Fecto F, Shi y, Zhai H, Jiang H, Hirano M, Rampersaud E, Jansen G, Donkervoort S, Bigio E, Brooks B, Ajroud K, Sufit R, Haines J, Mugnaini E, Pericak-Vance M, Siddique T. 2011. Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature 477: 211-215.
– reference: Gass J, Cannon A, Mackenzie IR, Boeve B, Baker M, Adamson J, Crook R, Melquist S, Kuntz K, Petersen R, Josephs K, Pickering-Brown SM, Graff-Radford N, Uitti R, Dickson D, Wszolek Z, Gonzalez J, Beach TG, Bigio E, Johnson N, Weintraub S, Mesulam M, White CL III, Woodruff B, Caselli R, Hsiung GY, Feldman H, Knopman D, Hutton M, Rademakers R. 2006. Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet 15: 2988-3001.
– reference: Snowden J, Rollinson S, Thompson J, Harris J, Stopford C, Richardson A, Jones M, Gerhard A, Davidson Y, Robinson A, Gibbons L, Hu Q, Duplessis D, Neary D, Mann D, Pickering-Brown S. 2012. Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain 135: 693-708.
– reference: Brickell KL, Leverenz JB, Steinbart EJ, Rumbaugh M, Schellenberg GD, Nochlin D, Lampe TH, Holm IE, Van D V, Yuan W, Bird TD. 2007. Clinicopathological concordance and discordance in three monozygotic twin pairs with familial Alzheimer's disease. J Neurol Neurosurg Psychiatry 78: 1050-1055.
– reference: Meeus B, Verstraeten A, Crosiers D, Engelborghs S, Van den Broeck M, Mattheijssens M, Peeters K, Corsmit E, Elinck E, Pickut B, Vandenberghe R, Cras P, De Deyn P, Van Broeckhoven C, Theuns J. 2012. DLB and PDD: A role for mutations in dementia and Parkinson disease genes? Neurobiol Aging 33: 629.
– reference: Cannella M, Martino T, Simonelli M, Ciammola A, Gradini R, Ciarmiello A, Gianfrancesco F, Squitieri F. 2007. De novo seven extra repeat expanded mutation in the PRNP gene in an Italian patient with early onset dementia. J Neurol Neurosurg Psychiatry 78: 1411-1413.
– reference: Pastor P, Pastor E, Carnero C, Vela R, Garcia T, Amer G, Tolosa E, Oliva R. 2001. Familial atypical progressive supranuclear palsy associated with homozigosity for the delN296 mutation in the tau gene. Ann Neurol 49: 263-267.
– reference: Hardy JA, Higgins GA. 1992. Alzheimer's disease: The amyloid cascade hypothesis. Science 256: 184-185.
– reference: Johnson KA, Lopera F, Jones K, Becker A, Sperling R, Hilson J, Londono J, Siegert I, Arcos M, Moreno S, Madrigal L, Ossa J, Pineda N, Ardila A, Roselli M, Albert MS, Kosik KS, Rios A. 2001. Presenilin-1-associated abnormalities in regional cerebral perfusion. Neurology 56: 1545-1551.
– reference: Sherrington R, Rogaev EI, Liang Y, Rogaeva EA, Levesque G, Ikeda M, Chi H, Lin C, Li G, Holman K, Tsuda T, Mar L, Foncin JF, Bruni AC, Montesi MP, Sorbi S, Rainero I, Pinessi L, Nee L, Chumakov I, Pollen D, Brookes A, Sanseau P, Polinsky RJ, Wasco W, Da Silva HA, Haines JL, Perkicak-Vance MA, Tanzi RE, Roses AD, Fraser PE, Rommens JM, St George-Hyslop PH. 1995. Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease. Nature 375: 754-760.
– reference: Edison P, Archer HA, Hinz R, Hammers A, Pavese N, Tai YF, Hotton G, Cutler D, Fox N, Kennedy A, Rossor M, Brooks DJ. 2007. Amyloid, hypometabolism, and cognition in Alzheimer disease: An [11C]PIB and [18F]FDG PET study. Neurology 68: 501-508.
– reference: Larner AJ, Ray PS, Doran M. 2007. The R269H mutation in presenilin-1 presenting as late-onset autosomal dominant Alzheimer's disease. J Neurol Sci 252: 173-176.
– reference: Mercy L, Hodges JR, Dawson K, Barker RA, Brayne C. 2008. Incidence of early-onset dementias in Cambridgeshire, United Kingdom. Neurology 71: 1496-1499.
– reference: Simon-Sanchez J, Dopper E, Cohn-Hokke P, Hukema R, Nicolaou N, Seelaar H, de Graaf J, de Koning I, van Schoor N, Deeg D, Smits M, Raaphorst J, van den Berg L, Schelhaas H, De Die-Smulders C, Majoor-Krakauer D, Rozemuller A, Willemsen R, Pijnenburg Y, Heutink P, van Swieten J. 2012. The clinical and pathological phenotype of C9orf72 hexanucleotide repeat expansions. Brain 135: 723-735.
– reference: Revesz T, Holton JL, Lashley T, Plant G, Frangione B, Rostagno A, Ghiso J. 2009. Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathies. Acta Neuropathol 118: 115-130.
– reference: Van Swieten J, Spillantini MG. 2007. Hereditary frontotemporal dementia caused by Tau gene mutations. Brain Pathol 17: 63-73.
– reference: Thinakaran G, Koo EH. 2008. Amyloid precursor protein trafficking, processing, and function. J Biol Chem 283: 29615-29619.
– reference: Beck JA, Poulter M, Campbell TA, Uphill JB, Adamson G, Geddes JF, Revesz T, Davis MB, Wood NW, Collinge J, Tabrizi SJ. 2004. Somatic and germline mosaicism in sporadic early-onset Alzheimer's disease. Hum Mol Genet 13: 1219-1224.
– reference: Markus HS, Martin RJ, Simpson MA, Dong YB, Ali N, Crosby AH, Powell JF. 2002. Diagnostic strategies in CADASIL. Neurology 59: 1134-1138.
– reference: Goldman JS, Hahn SE, Catania JW, LaRusse-Eckert S, Butson MB, Rumbaugh M, Strecker MN, Roberts JS, Burke W, Mayeux R, Bird T. 2011. Genetic counseling and testing for Alzheimer disease: Joint practice guidelines of the American College of Medical Genetics and the National Society of Genetic Counselors. Genet Med 13: 597-605.
– reference: Raux G, Guyant-Marechal L, Martin C, Bou J, Penet C, Brice A, Hannequin D, Frebourg T, Campion D. 2005. Molecular diagnosis of autosomal dominant early onset Alzheimer's disease: An update. J Med Genet 42: 793-795.
– reference: Houlden H, Baker M, Adamson J, Grover A, Waring S, Dickson D, Lynch T, Boeve B, Petersen RC, Pickering-Brown S, Owen F, Neary D, Craufurd D, Snowden J, Mann D, Hutton M. 1999. Frequency of tau mutations in three series of non-Alzheimer's degenerative dementia. Ann Neurol 46: 243-248.
– reference: Riedijk SR, Niermeijer MFN, Dooijes D, Tibben A. 2009. A decade of genetic counseling in frontotemporal dementia affected families: Few counseling requests and much familial opposition to testing. J Genet Couns 18: 350-356.
– reference: Cruts M, Gijselinck I, van der Zee J, Engelborghs S, Wils H, Pirici D, Rademakers R, Vandenberghe R, Dermaut B, Martin JJ, van Duijn C, Peeters K, Sciot R, Santens P, de Pooter T, Mattheijssens M, Van den Broeck M, Cuijt I, Vennekens K, de Deyn PP, Kumar-Singh S, Van Broeckhoven C. 2006. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature 442: 920-924.
– reference: Lambert JC, Heath S, Even G, Campion D, Sleegers K, Hiltunen M, Combarros O, Zelenika D, Bullido MJ, Tavernier B, Letenneur L, Bettens K, Berr C, Pasquier F, Fievet N, Barberger-Gateau P, Engelborghs S, de Deyn P, Mateo I, Franck A, Helisalmi S, Porcellini E, Hanon O, de Pancorbo MM, Lendon C, Dufouil C, Jaillard C, Leveillard T, Alvarez V, Bosco P, Mancuso M, Panza F, Nacmias B, Bossu P, Piccardi P, Annoni G, Seripa D, Galimberti D, Hannequin D, Licastro F, Soininen H, Ritchie K, Blanche H, Dartigues JF, Tzourio C, Gut I, Van BC, Alperovitch A, Lathrop M, Amouyel P. 2009. Genome-wide association study identifies variants at CLU and CR1 associated with Alzheimer's disease. Nat Genet 41: 1094-1099.
– reference: Peters N, Opherk C, Bergmann T, Castro M, Herzog J, Dichgans M. 2005. Spectrum of mutations in biopsy-proven CADASIL: Implications for diagnostic strategies. Arch Neurol 62: 1091-1094.
– reference: Snowden JS, Hu Q, Rollinson S, Halliwell N, Robinson A, Davidson YS, Momeni P, Baborie A, Griffiths TD, Jaros E, Perry RH, Richardson A, Pickering-Brown SM, Neary D, Mann DM. 2011. The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene. Acta Neuropathol 122: 99-110.
– reference: Tang-Wai D, Lewis P, Boeve B, Hutton M, Golde T, Baker M, Hardy J, Michels V, Ivnik R, Jack C, Petersen R. 2002. Familial frontotemporal dementia associated with a novel presenilin-1 mutation. Dement Geriatr Cogn Disord 14: 13-21.
– reference: Golan MP, Styczynska M, Jozwiak K, Walecki J, Maruszak A, Pniewski J, Lugiewicz R, Filipek S, Zekanowski C, Barcikowska M. 2007. Early-onset Alzheimer's disease with a de novo mutation in the presenilin 1 gene. Exp Neurol 208: 264-268.
– reference: van der Zee J, Le Ber I, Maurer-Stroh S, Engelborghs S, Gijselinck I, Camuzat A, Brouwers N, Vandenberghe R, Sleegers K, Hannequin D, Dermaut B, Schymkowitz J, Campion D, Santens P, Martin JJ, Lacomblez L, de Pooter T, Peeters K, Mattheijssens M, Vercelletto M, Van den Broeck M, Cruts M, de Deyn PP, Rousseau F, Brice A, Van Broeckhoven C. 2007. Mutations other than null mutations producing a pathogenic loss of progranulin in frontotemporal dementia. Hum Mutat 28: 416.
– reference: Grabowski TJ, Cho HS, Vonsattel JP, Rebeck GW, Greenberg SM. 2001. Novel amyloid precursor protein mutation in an Iowa family with dementia and severe cerebral amyloid angiopathy. Ann Neurol 49: 697-705.
– reference: Kelley BJ, Haidar W, Boeve BF, Baker M, Shiung M, Knopman DS, Rademakers R, Hutton M, Adamson J, Kuntz KM, Dickson DW, Parisi JE, Smith GE, Petersen RC. 2010. Alzheimer disease-like phenotype associated with the c.154delA mutation in progranulin. Arch Neurol 67: 171-177.
– reference: Naj AC, Jun G, Beecham GW, Wang LS, Vardarajan BN, Buros J, Gallins PJ, Buxbaum JD, Jarvik GP, Crane PK, Larson EB, Bird TD, Boeve BF, Graff-Radford NR, de Jager PL, Evans D, Schneider JA, Carrasquillo MM, Ertekin-Taner N, Younkin SG, Cruchaga C, Kauwe JS, Nowotny P, Kramer P, Hardy J, Huentelman MJ, Myers AJ, Barmada MM, Demirci FY, Baldwin CT, Green RC, Rogaeva E, George-Hyslop PS, Arnold SE, Barber R, Beach T, Bigio EH, Bowen JD, Boxer A, Burke JR, Cairns NJ, Carlson CS, Carney RM, Carroll SL, Chui HC, Clark DG, Corneveaux J, Cotman CW, Cummings JL, DeCarli C, DeKosky ST, Diaz-Arrastia R, Dick M, Dickson DW, Ellis WG, Faber KM, Fallon KB, Farlow MR, Ferris S, Frosch MP, Galasko DR, Ganguli M, Gearing M, Geschwind DH, Ghetti B, Gilbert JR, Gilman S, Giordani B, Glass JD, Growdon JH, Hamilton RL, Harrell LE, Head E, Honig LS, Hulette CM, Hyman BT, Jicha GA, Jin LW, Johnson N, Karlawish J, Karydas A, Kaye JA, Kim R, Koo EH, Kowall NW, Lah JJ, Levey AI, Lieberman AP, Lopez OL, Mack WJ, Marson DC, Martiniuk F, Mash DC, Masliah E, McCormick WC, McCurry SM, McDavid AN, McKee AC, Mesulam M, et al. 2011. Common variants at MS4A4/MS4A6E, CD2AP, CD33 and EPHA1 are associated with late-onset Alzheimer's disease. Nat Genet 43: 436-441.
– reference: Brickell KL, Steinbart EJ, Rumbaugh M, Payami H, Schellenberg GD, Van Deerlin V, Yuan W, Bird TD. 2006. Early-onset Alzheimer disease in families with late-onset Alzheimer disease: A potential important subtype of familial Alzheimer disease. Arch Neurol 63: 1307-1311.
– reference: Goate A, Chartier-Harlin MC, Mullan M, Brown J, Crawford F, Fidani L, Giuffra L, Haynes A, Irving N, James L. 1991. Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease. Nature 349: 704-706.
– reference: van Herpen E, Rosso SM, Serverijnen LA, Yoshida H, Breedveld G, van de Graaf R, Kamphorst W, Ravid R, Willemsen R, Dooijes D, Majoor-Krakauer D, Kros JM, Crowther RA, Goedert M, Heutink P, van Swieten JC. 2003. Variable phenotypic expression and extensive tau pathology in two families with the novel tau mutation L315R. Ann Neurol 54: 573-581.
– reference: Cox LE, Ferraiuolo L, Goodall EF, Heath PR, Higginbottom A, Mortiboys H, Hollinger HC, Hartley JA, Brockington A, Burness CE, Morrison KE, Wharton SB, Grierson AJ, Ince PG, Kirby J, Shaw PJ. 2010. Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS). PLoS ONE 5: e9872.
– reference: Bird TD. 2009. Progranulin plasma levels in the diagnosis of frontotemporal dementia. Brain 132: 568-569.
– reference: Jansen C, Parchi P, Capellari S, Strammiello R, Dopper EGP, van Swieten JC, Kamphorst W, Rozemuller AJM. 2011. A second case of Gerstmann-Straussler-Scheinker disease linked to the G131V mutation in the prion protein gene in a Dutch patient. J Neuropathol Exp Neurol 70: 698-702.
– reference: Hutton M. 2001. Missense and splice site mutations in tau associated with FTDP-17: Multiple pathogenic mechanisms. Neurology 56: S21-S25.
– reference: Dejesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, Nicholson AM, Finch NA, Flynn H, Adamson J, Kouri N, Wojtas A, Sengdy P, Hsiung GY, Karydas A, Seeley WW, Josephs KA, Coppola G, Geschwind DH, Wszolek ZK, Feldman H, Knopman DS, Petersen RC, Miller BL, Dickson DW, Boylan KB, Graff-Radford NR, Rademakers R. 2011. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72: 245-256.
– reference: Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AMT, Neary D, Snowden JS, Mann DMA. 2008. Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: Comparison with patients with MAPT and no known mutations. Brain 131: 721-731.
– reference: Tang W, Lu Y, Tian QY, Zhang Y, Guo FJ, Liu GY, Syed NM, Lai Y, Lin EA, Kong L, Su J, Yin F, Ding AH, Zanin-Zhorov A, Dustin ML, Tao J, Craft J, Yin Z, Feng JQ, Abramson SB, Yu XP, Liu CJ. 2011. The growth factor progranulin binds to TNF receptors and is therapeutic against inflammatory arthritis in mice. Science 332: 478-484.
– reference: Campion D, Dumanchin C, Hannequin D, Dubois B, Belliard S, Puel M, Thomas-Anterion C, Michon A, Martin C, Charbonnier F, Raux G, Camuzat A, Penet C, Mesnage V, Martinez M, Clerget-Darpoux F, Brice A, Frebourg T. 1999. Early-onset autosomal dominant Alzheimer disease: Prevalence, genetic heterogeneity, and mutation spectrum. Am J Hum Genet 65: 664-670.
– reference: Christensen KD, Roberts JS, Royal CDM, Fasaye GA, Obisesan T, Cupples LA, Whitehouse PJ, Butson MB, Linnenbringer E, Relkin NR, Farrer L, Cook-Deegan R, Green RC. 2008. Incorporating ethnicity into genetic risk assessment for Alzheimer disease: The REVEAL study experience. Genet Med 10: 207-214.
– reference: Rossor MN, Fox NC, Mummery CJ, Schott JM, Warren JD. 2010. The diagnosis of young-onset dementia. Lancet Neurol 9: 793-806.
– volume: 51
  start-page: 1546
  year: 1998
  end-page: 1554
  article-title: Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria
  publication-title: Neurology
– volume: 44
  start-page: 1683
  year: 1994
  end-page: 1686
  article-title: The risk of developing Creutzfeldt–Jakob disease in subjects with the PRNP gene codon 200 point mutation
  publication-title: Neurology
– volume: 115
  start-page: 601
  year: 1991
  end-page: 606
  article-title: Clinical genetics and genetic counseling in Alzheimer disease
  publication-title: Ann Intern Med
– volume: 128
  start-page: 752
  year: 2005
  end-page: 772
  article-title: Longitudinal characterization of two siblings with frontotemporal dementia and parkinsonism linked to chromosome 17 associated with the S305N tau mutation
  publication-title: Brain
– volume: 122
  start-page: 99
  year: 2011
  end-page: 110
  article-title: The most common type of FTLD‐FUS (aFTLD‐U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene
  publication-title: Acta Neuropathol
– volume: 65
  start-page: 323
  year: 2005
  end-page: 325
  article-title: Familial Alzheimer disease: Decreases in CSF Abeta42 levels precede cognitive decline
  publication-title: Neurology
– volume: 71
  start-page: 1220
  year: 2008
  end-page: 1226
  article-title: Distinct genetic forms of frontotemporal dementia
  publication-title: Neurology
– volume: 40
  start-page: 2709
  year: 2009
  end-page: 2714
  article-title: Prevalence and causes of early‐onset dementia in Japan: A population‐based study
  publication-title: Stroke
– volume: 62
  start-page: 1091
  year: 2005
  end-page: 1094
  article-title: Spectrum of mutations in biopsy‐proven CADASIL: Implications for diagnostic strategies
  publication-title: Arch Neurol
– volume: 67
  start-page: 1473
  year: 2010
  end-page: 1484
  article-title: Meta‐analysis confirms CR1, CLU, and PICALM as alzheimer disease risk loci and reveals interactions with APOE genotypes
  publication-title: Arch Neurol
– volume: 63
  start-page: 168
  year: 2006
  end-page: 174
  article-title: Role of genes and environments for explaining Alzheimer disease
  publication-title: Arch Gen Psychiatry
– volume: 43
  start-page: 436
  year: 2011
  end-page: 441
  article-title: Common variants at MS4A4/MS4A6E, CD2AP, CD33 and EPHA1 are associated with late‐onset Alzheimer's disease
  publication-title: Nat Genet
– volume: 8
  start-page: 48
  year: 2008
  article-title: Role of MAPT mutations and haplotype in frontotemporal lobar degeneration in Northern Finland
  publication-title: BMC Neurol
– volume: 71
  start-page: 1235
  year: 2008
  end-page: 1239
  article-title: Low plasma progranulin levels predict progranulin mutations in frontotemporal lobar degeneration
  publication-title: Neurology
– volume: 21
  start-page: 281
  year: 2006
  end-page: 286
  article-title: Frontotemporal dementia‐like phenotypes associated with presenilin‐1 mutations
  publication-title: Am J Alzheimers Dis Other Demen
– volume: 47
  start-page: 1113
  year: 1996
  end-page: 1124
  article-title: Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop
  publication-title: Neurology
– volume: 65
  start-page: 586
  year: 2005
  end-page: 590
  article-title: Prevalence and patterns of cognitive impairment in sporadic ALS
  publication-title: Neurology
– volume: 28
  start-page: 416
  year: 2007
  article-title: Mutations other than null mutations producing a pathogenic loss of progranulin in frontotemporal dementia
  publication-title: Hum Mutat
– volume: 131
  start-page: 721
  year: 2008
  end-page: 731
  article-title: Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: Comparison with patients with MAPT and no known mutations
  publication-title: Brain
– volume: 17
  start-page: 313
  year: 2008
  end-page: 322
  article-title: CHMP2B C‐truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro
  publication-title: Hum Mol Genet
– volume: 16
  start-page: 171
  year: 2008
  end-page: 175
  article-title: Low prevalence of APP duplications in Swedish and Finnish patients with early‐onset Alzheimer's disease
  publication-title: Eur J Hum Genet
– volume: 15
  start-page: 377
  year: 2008
  end-page: 385
  article-title: Alzheimer disease‐like clinical phenotype in a family with FTDP‐17 caused by a MAPT R406W mutation
  publication-title: Eur J Neurol
– volume: 55
  start-page: 1577
  year: 2000
  end-page: 1578
  article-title: Dementia with prominent frontotemporal features associated with L113P presenilin 1 mutation
  publication-title: Neurology
– volume: 22
  start-page: 294
  year: 2007
  end-page: 299
  article-title: Progressive nonfluent aphasia associated with a new mutation V363I in tau gene
  publication-title: Am J Alzheimers Dis Other Demen
– volume: 23
  start-page: 347
  year: 2009
  end-page: 351
  article-title: Frequency and causes of early‐onset dementia in a tertiary referral center in Athens
  publication-title: Alzheimer Dis Assoc Disord
– volume: 78
  start-page: 1411
  year: 2007
  end-page: 1413
  article-title: De novo seven extra repeat expanded mutation in the PRNP gene in an Italian patient with early onset dementia
  publication-title: J Neurol Neurosurg Psychiatry
– volume: 41
  start-page: 1094
  year: 2009
  end-page: 1099
  article-title: Genome‐wide association study identifies variants at CLU and CR1 associated with Alzheimer's disease
  publication-title: Nat Genet
– volume: 70
  start-page: 698
  year: 2011
  end-page: 702
  article-title: A second case of Gerstmann‐Straussler‐Scheinker disease linked to the G131V mutation in the prion protein gene in a Dutch patient
  publication-title: J Neuropathol Exp Neurol
– volume: 249
  start-page: 1567
  year: 2002
  end-page: 1582
  article-title: Mutations of the prion protein gene phenotypic spectrum
  publication-title: J Neurol
– volume: 135
  start-page: 723
  year: 2012
  end-page: 735
  article-title: The clinical and pathological phenotype of C9orf72 hexanucleotide repeat expansions
  publication-title: Brain
– volume: 132
  start-page: 1355
  year: 2009
  end-page: 1365
  article-title: Serial PIB and MRI in normal, mild cognitive impairment and Alzheimer's disease: Implications for sequence of pathological events in Alzheimer's disease
  publication-title: Brain
– volume: 62
  start-page: 925
  year: 2005
  end-page: 930
  article-title: Frontotemporal lobar degeneration: Demographic characteristics of 353 patients
  publication-title: Arch Neurol
– volume: 19
  start-page: 1401
  year: 2010
  end-page: 1408
  article-title: Early‐versus late‐onset Alzheimer's disease: More than age alone
  publication-title: J Alzheimers Dis
– volume: 442
  start-page: 916
  year: 2006
  end-page: 919
  article-title: Mutations in progranulin cause tau‐negative frontotemporal dementia linked to chromosome 17
  publication-title: Nature
– volume: 366
  start-page: 283
  year: 2012
  end-page: 284
  article-title: Repeat expansion in C9ORF72 in Alzheimer's disease
  publication-title: N Engl J Med
– volume: 38
  start-page: 24
  year: 2006
  end-page: 26
  article-title: APP locus duplication causes autosomal dominant early‐onset Alzheimer disease with cerebral amyloid angiopathy
  publication-title: Nat Genet
– volume: 76
  start-page: 1006
  year: 2011
  end-page: 1014
  article-title: Classification of primary progressive aphasia and its variants
  publication-title: Neurology
– volume: 131
  start-page: 732
  year: 2008
  end-page: 746
  article-title: Phenotype variability in progranulin mutation carriers: A clinical, neuropsychological, imaging and genetic study
  publication-title: Brain
– volume: 135
  start-page: 693
  year: 2012
  end-page: 708
  article-title: Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations
  publication-title: Brain
– volume: 393
  start-page: 702
  year: 1998
  end-page: 705
  article-title: Association of missense and 5′‐splice‐site mutations in tau with the inherited dementia FTDP‐17
  publication-title: Nature
– volume: 7
  start-page: 263
  year: 2011
  end-page: 269
  article-title: The diagnosis of dementia due to Alzheimer's disease: Recommendations from the National Institute on Aging‐Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease
  publication-title: Alzheimers Dement
– volume: 10
  start-page: 162
  year: 2011
  end-page: 172
  article-title: Behavioural‐variant frontotemporal dementia: Diagnosis, clinical staging, and management
  publication-title: Lancet Neurol
– volume: 14
  start-page: 141
  year: 2005
  end-page: 150
  article-title: Emotional responses to APO E genotype disclosure for Alzheimer disease
  publication-title: J Genet Couns
– volume: 36
  start-page: 377
  year: 2004
  end-page: 381
  article-title: Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin‐containing protein
  publication-title: Nat Genet
– volume: 67
  start-page: 161
  year: 2010
  end-page: 170
  article-title: The spectrum of mutations in progranulin: A collaborative study screening 545 cases of neurodegeneration
  publication-title: Arch Neurol
– volume: 16
  start-page: 273
  year: 2010
  end-page: 279
  article-title: Progranulin (GRN) in two siblings of a Latino family and in other patients with schizophrenia
  publication-title: Neurocase
– volume: 59
  start-page: 1759
  year: 2002
  end-page: 1763
  article-title: Frequency of mutations in the presenilin and amyloid precursor protein genes in early‐onset Alzheimer disease in Spain
  publication-title: Arch Neurol
– volume: 133
  start-page: 3043
  year: 2010
  end-page: 3057
  article-title: Prospective 10‐year surveillance of human prion diseases in Japan
  publication-title: Brain
– volume: 18
  start-page: 250
  year: 2005
  end-page: 255
  article-title: Genetic risk assessment for adult children of people with Alzheimer's disease: The Risk Evaluation and Education for Alzheimer's Disease (REVEAL) study
  publication-title: J Geriatr Psychiatry Neurol
– volume: 73
  start-page: 626
  year: 2009
  end-page: 632
  article-title: Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His
  publication-title: Neurology
– volume: 27
  start-page: 6174
  year: 2007
  end-page: 6184
  article-title: Amyloid deposition begins in the striatum of presenilin‐1 mutation carriers from two unrelated pedigrees
  publication-title: J Neurosci
– volume: 57
  start-page: 621
  year: 2001
  end-page: 625
  article-title: Screening for PS1 mutations in a referral‐based series of AD cases: 21 novel mutations
  publication-title: Neurology
– volume: 24
  start-page: 1636
  year: 2009
  end-page: 1640
  article-title: Clinical and genetic characteristics of non‐Asian dentatorubral‐pallidoluysian atrophy: A systematic review
  publication-title: Mov Disord
– volume: 283
  start-page: 29615
  year: 2008
  end-page: 29619
  article-title: Amyloid precursor protein trafficking, processing, and function
  publication-title: J Biol Chem
– volume: 218
  start-page: 139
  year: 1996
  end-page: 141
  article-title: Sequence analysis of presenilin‐1 gene mutation in Japanese Alzheimer's disease patients
  publication-title: Neurosci Lett
– volume: 54
  start-page: 573
  year: 2003
  end-page: 581
  article-title: Variable phenotypic expression and extensive tau pathology in two families with the novel tau mutation L315R
  publication-title: Ann Neurol
– volume: 132
  start-page: 568
  year: 2009
  end-page: 569
  article-title: Progranulin plasma levels in the diagnosis of frontotemporal dementia
  publication-title: Brain
– volume: 256
  start-page: 184
  year: 1992
  end-page: 185
  article-title: Alzheimer's disease: The amyloid cascade hypothesis
  publication-title: Science
– volume: 65
  start-page: 1817
  year: 2005
  end-page: 1819
  article-title: Comparison of family histories in FTLD subtypes and related tauopathies
  publication-title: Neurology
– volume: 72
  start-page: 245
  year: 2011
  end-page: 256
  article-title: Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p‐linked FTD and ALS
  publication-title: Neuron
– volume: 68
  start-page: 501
  year: 2007
  end-page: 508
  article-title: Amyloid, hypometabolism, and cognition in Alzheimer disease: An [11C]PIB and [18F]FDG PET study
  publication-title: Neurology
– volume: 442
  start-page: 920
  year: 2006
  end-page: 924
  article-title: Null mutations in progranulin cause ubiquitin‐positive frontotemporal dementia linked to chromosome 17q21
  publication-title: Nature
– volume: 297
  start-page: 353
  year: 2002
  end-page: 356
  article-title: The amyloid hypothesis of Alzheimer's disease: Progress and problems on the road to therapeutics
  publication-title: Science
– volume: 34
  start-page: 939
  year: 1984
  end-page: 944
  article-title: 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
  publication-title: Neurology
– volume: 257
  start-page: 747
  year: 2010
  end-page: 753
  article-title: Frequency of ubiquitin and FUS‐positive, TDP‐43‐negative frontotemporal lobar degeneration
  publication-title: J Neurol
– volume: 49
  start-page: 697
  year: 2001
  end-page: 705
  article-title: Novel amyloid precursor protein mutation in an Iowa family with dementia and severe cerebral amyloid angiopathy
  publication-title: Ann Neurol
– volume: 71
  start-page: 1496
  year: 2008
  end-page: 1499
  article-title: Incidence of early‐onset dementias in Cambridgeshire, United Kingdom
  publication-title: Neurology
– volume: 30
  start-page: 14946
  year: 2010
  end-page: 14954
  article-title: Amyloid‐independent mechanisms in Alzheimer's disease pathogenesis
  publication-title: J Neurosci
– volume: 126
  start-page: 2016
  year: 2003
  end-page: 2022
  article-title: Frontotemporal dementia in The Netherlands: Patient characteristics and prevalence estimates from a population‐based study
  publication-title: Brain
– volume: 13
  start-page: 597
  year: 2011
  end-page: 605
  article-title: Genetic counseling and testing for Alzheimer disease: Joint practice guidelines of the American College of Medical Genetics and the National Society of Genetic Counselors
  publication-title: Genet Med
– volume: 59
  start-page: 1134
  year: 2002
  end-page: 1138
  article-title: Diagnostic strategies in CADASIL
  publication-title: Neurology
– volume: 332
  start-page: 478
  year: 2011
  end-page: 484
  article-title: The growth factor progranulin binds to TNF receptors and is therapeutic against inflammatory arthritis in mice
  publication-title: Science
– volume: 66
  start-page: 1537
  year: 2009
  end-page: 1544
  article-title: High striatal amyloid beta‐peptide deposition across different autosomal Alzheimer disease mutation types
  publication-title: Arch Neurol
– volume: 17
  start-page: 298
  year: 2004
  end-page: 2301
  article-title: The tau R406W mutation causes progressive presenile dementia with bitemporal atrophy
  publication-title: Dement Geriatr Cogn Disord
– volume: 15
  start-page: 2988
  year: 2006
  end-page: 3001
  article-title: Mutations in progranulin are a major cause of ubiquitin‐positive frontotemporal lobar degeneration
  publication-title: Hum Mol Genet
– volume: 141B
  start-page: 944
  year: 2006
  end-page: 946
  article-title: CHMP2B mutations are not a cause of dementia in Dutch patients with familial and sporadic frontotemporal dementia
  publication-title: Am J Med Genet Part B
– volume: 118
  start-page: 115
  year: 2009
  end-page: 130
  article-title: Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathies
  publication-title: Acta Neuropathol
– volume: 153B
  start-page: 1283
  year: 2010
  end-page: 1291
  article-title: Somatic mosaicism in a case of apparently sporadic Creutzfeldt–Jakob disease carrying a de novo D178N mutation in the PRNP gene
  publication-title: Am J Med Genet Part B
– volume: 69
  start-page: 730
  year: 2007
  end-page: 738
  article-title: Inherited prion disease with 5‐OPRI: Phenotype modification by repeat length and codon 129
  publication-title: Neurology
– volume: 1
  start-page: 345
  year: 1992
  end-page: 347
  article-title: A pathogenic mutation for probable Alzheimer's disease in the APP gene at the N‐terminus of beta‐amyloid
  publication-title: Nat Genet
– volume: 120
  start-page: 33
  year: 2010
  end-page: 41
  article-title: FUS pathology defines the majority of tau‐ and TDP‐43‐negative frontotemporal lobar degeneration
  publication-title: Acta Neuropathol
– volume: 78
  start-page: 1050
  year: 2007
  end-page: 1055
  article-title: Clinicopathological concordance and discordance in three monozygotic twin pairs with familial Alzheimer's disease
  publication-title: J Neurol Neurosurg Psychiatry
– volume: 28
  start-page: 846
  year: 2007
  end-page: 855
  article-title: Progranulin null mutations in both sporadic and familial frontotemporal dementia
  publication-title: Hum Mutat
– volume: 7
  start-page: 43
  year: 1998
  end-page: 51
  article-title: Estimation of the genetic contribution of presenilin‐1 and ‐2 mutations in a population‐based study of presenile Alzheimer disease
  publication-title: Hum Mol Genet
– volume: 56
  start-page: 1545
  year: 2001
  end-page: 1551
  article-title: Presenilin‐1‐associated abnormalities in regional cerebral perfusion
  publication-title: Neurology
– volume: 35
  start-page: 2169
  year: 2008
  end-page: 2181
  article-title: Regional analysis of FDG and PIB‐PET images in normal aging, mild cognitive impairment, and Alzheimer's disease
  publication-title: Eur J Nucl Med Mol Imaging
– volume: 56
  start-page: S21
  year: 2001
  end-page: S25
  article-title: Missense and splice site mutations in tau associated with FTDP‐17: Multiple pathogenic mechanisms
  publication-title: Neurology
– volume: 57
  start-page: 139
  year: 2005
  end-page: 143
  article-title: Pick bodies in a family with presenilin‐1 Alzheimer's disease
  publication-title: Ann Neurol
– volume: 52
  start-page: 355
  year: 2002
  end-page: 359
  article-title: Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy
  publication-title: Ann Neurol
– volume: 55
  start-page: 617
  year: 2004
  end-page: 626
  article-title: A novel presenilin 1 mutation associated with Pick's disease but not beta‐amyloid plaques
  publication-title: Ann Neurol
– volume: 25
  start-page: 96
  year: 2011
  end-page: 99
  article-title: MAPT V363I variation in a sporadic case of frontotemporal dementia: Variable penetrant mutation or rare polymorphism?
  publication-title: Alzheimer Dis Assoc Disord
– start-page: 209.e3
  year: 2012
  end-page: 209.e8
  article-title: The chromosome 9 ALS and FTD locus is probably derived from a single founder
  publication-title: Neurobiol Aging
– volume: 13
  start-page: 1219
  year: 2004
  end-page: 1224
  article-title: Somatic and germline mosaicism in sporadic early‐onset Alzheimer's disease
  publication-title: Hum Mol Genet
– volume: 253
  start-page: 139
  year: 2006
  end-page: 158
  article-title: Clinical phenotypic heterogeneity of Alzheimer's disease associated with mutations of the presenilin‐1 gene
  publication-title: J Neurol
– volume: 5
  start-page: e9872
  year: 2010
  article-title: Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS)
  publication-title: PLoS ONE
– volume: 60
  start-page: 2020
  year: 2003
  article-title: Diagnostic strategies in CADASIL
  publication-title: Neurology
– volume: 129
  start-page: 2977
  year: 2006
  end-page: 2983
  article-title: APP duplication is sufficient to cause early onset Alzheimer's dementia with cerebral amyloid angiopathy
  publication-title: Brain
– volume: 66
  start-page: 110
  year: 2000
  end-page: 117
  article-title: High prevalence of pathogenic mutations in patients with early‐onset dementia detected by sequence analyses of four different genes
  publication-title: Am J Hum Genet
– volume: 133
  start-page: 1143
  year: 2010
  end-page: 1154
  article-title: Alzheimer's disease phenotypes and genotypes associated with mutations in presenilin 2
  publication-title: Brain
– volume: 11
  start-page: 219
  year: 1995
  end-page: 222
  article-title: The structure of the presenilin 1 (S182) gene and identification of six novel mutations in early onset AD families. Alzheimer's Disease Collaborative Group
  publication-title: Nat Genet
– volume: 287
  start-page: 329
  year: 2002
  end-page: 336
  article-title: Risk of dementia among white and African American relatives of patients with Alzheimer disease
  publication-title: JAMA
– volume: 9
  start-page: 197
  year: 2008
  end-page: 205
  article-title: Progranulin genetic variations in frontotemporal lobar degeneration: Evidence for low mutation frequency in an Italian clinical series
  publication-title: Neurogenetics
– volume: 74
  start-page: 366
  year: 2010
  end-page: 371
  article-title: Genetic contribution of FUS to frontotemporal lobar degeneration
  publication-title: Neurology
– volume: 6
  start-page: 475
  year: 2004
  end-page: 482
  article-title: Pathological and clinical heterogeneity of presenilin 1 gene mutations
  publication-title: J Alzheimers Dis
– volume: 122
  start-page: 673
  year: 2011
  end-page: 690
  article-title: Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72
  publication-title: Acta Neuropathol
– volume: 287
  start-page: 291
  year: 2009
  end-page: 293
  article-title: Progranulin plasma levels as potential biomarker for the identification of GRN deletion carriers. A case with atypical onset as clinical amnestic Mild Cognitive Impairment converted to Alzheimer's disease
  publication-title: J Neurol Sci
– volume: 72
  start-page: 257
  year: 2011
  end-page: 268
  article-title: A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21‐linked ALS‐FTD
  publication-title: Neuron
– volume: 197
  start-page: 28
  year: 2010
  end-page: 35
  article-title: Morbid risk for schizophrenia in first‐degree relatives of people with frontotemporal dementia
  publication-title: Br J Psychiatry
– volume: 30
  start-page: E974
  year: 2009
  end-page: E983
  article-title: Mutation within TARDBP leads to frontotemporal dementia without motor neuron disease
  publication-title: Hum Mutat
– volume: 75
  start-page: 807
  year: 2010
  end-page: 814
  article-title: Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia
  publication-title: Neurology
– volume: 41
  start-page: 1088
  year: 2009
  end-page: 1093
  article-title: Genome‐wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease
  publication-title: Nat Genet
– volume: 129
  start-page: 3081
  year: 2006
  end-page: 3090
  article-title: The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene
  publication-title: Brain
– volume: 72
  start-page: 266
  year: 2002
  end-page: 269
  article-title: A novel mutation (V89L) in the presenilin 1 gene in a family with early onset Alzheimer's disease and marked behavioural disturbances
  publication-title: J Neurol Neurosurg Psychiatry
– volume: 17
  start-page: 641
  year: 2010
  end-page: 648
  article-title: EFNS guidelines on the molecular diagnosis of channelopathies, epilepsies, migraine, stroke, and dementias
  publication-title: Eur J Neurol
– volume: 375
  start-page: 754
  year: 1995
  end-page: 760
  article-title: Cloning of a gene bearing missense mutations in early‐onset familial Alzheimer's disease
  publication-title: Nature
– volume: 17
  start-page: 63
  year: 2007
  end-page: 73
  article-title: Hereditary frontotemporal dementia caused by Tau gene mutations
  publication-title: Brain Pathol
– volume: 57
  start-page: 72
  year: 2000
  end-page: 77
  article-title: Familial aggregation of Alzheimer disease among whites, African Americans, and Caribbean Hispanics in northern Manhattan
  publication-title: Arch Neurol
– volume: 208
  start-page: 264
  year: 2007
  end-page: 268
  article-title: Early‐onset Alzheimer's disease with a de novo mutation in the presenilin 1 gene
  publication-title: Exp Neurol
– volume: 129
  start-page: 2984
  year: 2006
  end-page: 2991
  article-title: Genetic risk and transcriptional variability of amyloid precursor protein in Alzheimer's disease
  publication-title: Brain
– volume: 14
  start-page: 1057
  year: 2007
  end-page: 1059
  article-title: Dementia, delusions and seizures: Storage disease or genetic AD?
  publication-title: Eur J Neurol
– volume: 134
  start-page: 2582
  year: 2011
  end-page: 2594
  article-title: Motor neuron dysfunction in frontotemporal dementia
  publication-title: Brain
– volume: 132
  start-page: 583
  year: 2009
  end-page: 591
  article-title: Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members
  publication-title: Brain
– volume: 58
  start-page: 1803
  year: 2001
  end-page: 1809
  article-title: Clinical and pathological diagnosis of frontotemporal dementia: Report of the Work Group on Frontotemporal Dementia and Pick's Disease
  publication-title: Arch Neurol
– volume: 134
  start-page: 2456
  year: 2011
  end-page: 2477
  article-title: Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
  publication-title: Brain
– volume: 71
  start-page: 1489
  year: 2008
  end-page: 1495
  article-title: Survival in Alzheimer disease: A multiethnic, population‐based study of incident cases
  publication-title: Neurology
– volume: 58
  start-page: 1828
  year: 2001
  end-page: 1831
  article-title: Impact of DNA testing for early‐onset familial Alzheimer disease and frontotemporal dementia
  publication-title: Arch Neurol
– volume: 33
  start-page: 629
  year: 2012
  article-title: DLB and PDD: A role for mutations in dementia and Parkinson disease genes?
  publication-title: Neurobiol Aging
– volume: 32
  start-page: 1388
  year: 2011
  end-page: 1399
  article-title: Glucose metabolism and PIB binding in carriers of a His163Tyr presenilin 1 mutation
  publication-title: Neurobiol Aging
– volume: 60
  start-page: 235
  year: 2003
  end-page: 239
  article-title: Early onset familial Alzheimer's disease: Mutation frequency in 31 families
  publication-title: Neurology
– volume: 95
  start-page: 7737
  year: 1998
  end-page: 7741
  article-title: Mutation in the tau gene in familial multiple system tauopathy with presenile dementia
  publication-title: Proc Natl Acad Sci USA
– volume: 134
  start-page: 3030
  year: 2011
  end-page: 3043
  article-title: Subtypes of progressive aphasia: Application of the international consensus criteria and validation using {beta}‐amyloid imaging
  publication-title: Brain
– volume: 419
  start-page: 1
  year: 2007
  end-page: 4
  article-title: TDP‐43 gene analysis in frontotemporal lobar degeneration
  publication-title: Neurosci Lett
– volume: 10
  start-page: 207
  year: 2008
  end-page: 214
  article-title: Incorporating ethnicity into genetic risk assessment for Alzheimer disease: The REVEAL study experience
  publication-title: Genet Med
– volume: 46
  start-page: 243
  year: 1999
  end-page: 248
  article-title: Frequency of tau mutations in three series of non‐Alzheimer's degenerative dementia
  publication-title: Ann Neurol
– volume: 61
  start-page: 1136
  year: 2003
  end-page: 1137
  article-title: Very early onset AD with a de novo mutation in the presenilin 1 gene (Met 233 Leu)
  publication-title: Neurology
– volume: 103
  start-page: 138
  year: 2001
  end-page: 143
  article-title: Systematic genetic study of Alzheimer disease in Latin America: Mutation frequencies of the amyloid beta precursor protein and presenilin genes in Colombia
  publication-title: Am J Med Genet
– volume: 23
  start-page: 269
  year: 2010
  end-page: 276
  article-title: CADASIL
  publication-title: J Geriatr Psychiatry Neurol
– volume: 60
  start-page: 534
  year: 2008
  end-page: 542
  article-title: Linking Abeta and tau in late‐onset Alzheimer's disease: A dual pathway hypothesis
  publication-title: Neuron
– volume: 65
  start-page: 664
  year: 1999
  end-page: 670
  article-title: Early‐onset autosomal dominant Alzheimer disease: Prevalence, genetic heterogeneity, and mutation spectrum
  publication-title: Am J Hum Genet
– volume: 349
  start-page: 704
  year: 1991
  end-page: 706
  article-title: Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease
  publication-title: Nature
– volume: 18
  start-page: 350
  year: 2009
  end-page: 356
  article-title: A decade of genetic counseling in frontotemporal dementia affected families: Few counseling requests and much familial opposition to testing
  publication-title: J Genet Couns
– volume: 256
  start-page: 1620
  year: 2009
  end-page: 1628
  article-title: Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies
  publication-title: J Neurol
– volume: 129
  start-page: 2297
  year: 2006
  end-page: 2317
  article-title: Inherited prion disease with six octapeptide repeat insertional mutation—Molecular analysis of phenotypic heterogeneity
  publication-title: Brain
– volume: 74
  start-page: 1206
  year: 2003
  end-page: 1209
  article-title: The prevalence and causes of dementia in people under the age of 65 years
  publication-title: J Neurol Neurosurg Psychiatry
– volume: 67
  start-page: 171
  year: 2010
  end-page: 177
  article-title: Alzheimer disease‐like phenotype associated with the c.154delA mutation in progranulin
  publication-title: Arch Neurol
– volume: 7
  start-page: 208
  year: 2011
  end-page: 244
  article-title: 2011 Alzheimer's disease facts and figures
  publication-title: Alzheimers Dement
– volume: 68
  start-page: 90
  year: 2011
  end-page: 93
  article-title: Familial aggregation of dementia with Lewy bodies
  publication-title: Arch Neurol
– volume: 73
  start-page: 1451
  year: 2009
  end-page: 1456
  article-title: The heritability and genetics of frontotemporal lobar degeneration
  publication-title: Neurology
– volume: 283
  start-page: 1744
  year: 2008
  end-page: 1753
  article-title: Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin‐immunoreactive inclusions reduce progranulin production and secretion
  publication-title: J Biol Chem
– volume: 58
  start-page: 383
  year: 2001
  end-page: 387
  article-title: Frequency of tau gene mutations in familial and sporadic cases of non‐Alzheimer dementia
  publication-title: Arch Neurol
– volume: 24
  start-page: 1843
  year: 2009
  end-page: 1847
  article-title: TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea
  publication-title: Mov Disord
– volume: 69
  start-page: 712
  year: 2011
  end-page: 720
  article-title: Familial prion disease with Alzheimer disease‐like tau pathology and clinical phenotype
  publication-title: Ann Neurol
– volume: 22
  start-page: 89
  year: 2001
  end-page: 107
  article-title: Phenotypic correlations in FTDP‐17
  publication-title: Neurobiol Aging
– volume: 49
  start-page: 263
  year: 2001
  end-page: 267
  article-title: Familial atypical progressive supranuclear palsy associated with homozigosity for the delN296 mutation in the tau gene
  publication-title: Ann Neurol
– volume: 30
  start-page: 1329
  year: 2009
  end-page: 1331
  article-title: Neuronal inclusion protein TDP‐43 has no primary genetic role in FTD and ALS
  publication-title: Neurobiol Aging
– volume: 22
  start-page: 765
  year: 2010
  end-page: 769
  article-title: FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis
  publication-title: J Alzheimers Dis
– volume: 168
  start-page: 413
  year: 2001
  end-page: 418
  article-title: Clinic‐based cases with frontotemporal dementia show increased cerebrospinal fluid tau and high apolipoprotein E epsilon4 frequency, but no tau gene mutations
  publication-title: Exp Neurol
– volume: 65
  start-page: 506
  year: 2008
  end-page: 513
  article-title: Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene
  publication-title: Arch Neurol
– volume: 16
  start-page: 903
  year: 2011
  end-page: 907
  article-title: APOE and Alzheimer disease: A major gene with semi‐dominant inheritance
  publication-title: Mol Psychiatry
– volume: 45
  start-page: 706
  year: 2011
  end-page: 709
  article-title: Genetic counseling for frontotemporal dementias
  publication-title: J Mol Neurosci
– volume: 37
  start-page: 806
  year: 2005
  end-page: 808
  article-title: Mutations in the endosomal ESCRTIII‐complex subunit CHMP2B in frontotemporal dementia
  publication-title: Nat Genet
– volume: 7
  start-page: 1
  year: 2011
  end-page: 9
  article-title: Cerebral amyloid angiopathy: A systematic review
  publication-title: J Clin Neurol
– volume: 361
  start-page: 245
  year: 2009
  end-page: 254
  article-title: Disclosure of APOE genotype for risk of Alzheimer's disease
  publication-title: N Engl J Med
– volume: 14
  start-page: 13
  year: 2002
  end-page: 21
  article-title: Familial frontotemporal dementia associated with a novel presenilin‐1 mutation
  publication-title: Dement Geriatr Cogn Disord
– volume: 45
  start-page: 663
  year: 2011
  end-page: 675
  article-title: Making Connections: Pathology and Genetics Link Amyotrophic Lateral Sclerosis with Frontotemporal Lobe Dementia
  publication-title: J Mol Neurosci
– volume: 11
  start-page: e23
  year: 2009
  article-title: The molecular basis of frontotemporal dementia
  publication-title: Expert Rev Mol Med
– volume: 72
  start-page: 813
  year: 2009
  end-page: 820
  article-title: Voxel‐based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN
  publication-title: Neurology
– volume: 477
  start-page: 211
  year: 2011
  end-page: 215
  article-title: Mutations in UBQLN2 cause dominant X‐linked juvenile and adult‐onset ALS and ALS/dementia
  publication-title: Nature
– volume: 63
  start-page: 1307
  year: 2006
  end-page: 1311
  article-title: Early‐onset Alzheimer disease in families with late‐onset Alzheimer disease: A potential important subtype of familial Alzheimer disease
  publication-title: Arch Neurol
– volume: 9
  start-page: 793
  year: 2010
  end-page: 806
  article-title: The diagnosis of young‐onset dementia
  publication-title: Lancet Neurol
– volume: 30
  start-page: 157
  year: 2009
  end-page: 159
  article-title: No association of TDP‐43 with sporadic frontotemporal dementia
  publication-title: Neurobiol Aging
– volume: 68
  start-page: 857
  year: 2010
  end-page: 864
  article-title: Exome sequencing reveals VCP mutations as a cause of familial ALS
  publication-title: Neuron
– volume: 22
  start-page: 981
  year: 2010
  end-page: 984
  article-title: Low serum progranulin predicts the presence of mutations: A prospective study
  publication-title: J Alzheimers Dis
– volume: 252
  start-page: 173
  year: 2007
  end-page: 176
  article-title: The R269H mutation in presenilin‐1 presenting as late‐onset autosomal dominant Alzheimer's disease
  publication-title: J Neurol Sci
– volume: 70
  start-page: 1827
  year: 2008
  end-page: 1835
  article-title: CSF biomarkers in frontotemporal lobar degeneration with known pathology
  publication-title: Neurology
– volume: 61
  start-page: 446
  year: 2007
  end-page: 453
  article-title: Extreme cerebrospinal fluid amyloid beta levels identify family with late‐onset Alzheimer's disease presenilin 1 mutation
  publication-title: Ann Neurol
– volume: 157
  start-page: 633
  year: 2001
  end-page: 637
  article-title: Genetic epidemiology of Creutzfeldt–Jakob disease in Europe
  publication-title: Rev Neurol (Paris)
– volume: 68
  start-page: 488
  year: 2011
  end-page: 497
  article-title: Genetic and clinical features of progranulin‐associated frontotemporal lobar degeneration
  publication-title: Arch Neurol
– volume: 58
  start-page: 1615
  year: 2002
  end-page: 1621
  article-title: The prevalence of frontotemporal dementia
  publication-title: Neurology
– volume: 50
  start-page: 1541
  year: 1998
  end-page: 1545
  article-title: Familial aggregation in frontotemporal dementia
  publication-title: Neurology
– volume: 11
  start-page: 54
  year: 2012
  end-page: 65
  article-title: A C9orf72 promoter repeat expansion in a Flanders‐Belgian cohort with disorders of the frontotemporal lobar degeneration‐amyotrophic lateral sclerosis spectrum: A gene identification study
  publication-title: Lancet Neurol
– volume: 42
  start-page: 793
  year: 2005
  end-page: 795
  article-title: Molecular diagnosis of autosomal dominant early onset Alzheimer's disease: An update
  publication-title: J Med Genet
– ident: e_1_2_13_112_1
  doi: 10.1212/01.wnl.0000171397.32851.bc
– ident: e_1_2_13_24_1
  doi: 10.1093/brain/awr195
– ident: e_1_2_13_15_1
  doi: 10.1093/brain/awh356
– ident: e_1_2_13_30_1
  doi: 10.1097/GIM.0b013e318164e4cf
– ident: e_1_2_13_104_1
  doi: 10.1093/brain/awl226
– ident: e_1_2_13_152_1
  doi: 10.1192/bjp.bp.109.068981
– ident: e_1_2_13_37_1
  doi: 10.1038/nature10353
– ident: e_1_2_13_101_1
  doi: 10.1212/WNL.34.7.939
– ident: e_1_2_13_100_1
  doi: 10.1212/WNL.47.5.1113
– ident: e_1_2_13_134_1
  doi: 10.1212/WNL.58.11.1615
– ident: e_1_2_13_63_1
  doi: 10.1038/ng.440
– ident: e_1_2_13_2_1
  doi: 10.1111/j.1468-1331.2007.01664.x
– ident: e_1_2_13_35_1
  doi: 10.1002/ana.10267
– ident: e_1_2_13_5_1
– ident: e_1_2_13_155_1
  doi: 10.1016/j.neurobiolaging.2007.05.022
– ident: e_1_2_13_27_1
  doi: 10.1016/j.jns.2009.07.011
– ident: e_1_2_13_61_1
  doi: 10.1126/science.1566067
– ident: e_1_2_13_103_1
  doi: 10.1016/j.jalz.2011.03.005
– ident: e_1_2_13_180_1
  doi: 10.1001/archneurol.2009.285
– ident: e_1_2_13_84_1
  doi: 10.3233/JAD-2010-1337
– ident: e_1_2_13_53_1
  doi: 10.1016/j.expneurol.2007.08.016
– ident: e_1_2_13_143_1
  doi: 10.1177/0891988705281883
– ident: e_1_2_13_146_1
  doi: 10.1212/WNL.0b013e3181bf997a
– ident: e_1_2_13_158_1
  doi: 10.1074/jbc.M705115200
– ident: e_1_2_13_185_1
  doi: 10.1001/archneurol.2009.328
– ident: e_1_2_13_97_1
  doi: 10.1093/brain/awl271
– volume: 60
  start-page: 2020
  year: 2003
  ident: e_1_2_13_121_1
  article-title: Diagnostic strategies in CADASIL
  publication-title: Neurology
– ident: e_1_2_13_88_1
  doi: 10.1038/ng.439
– ident: e_1_2_13_111_1
  doi: 10.1080/13554790903456209
– ident: e_1_2_13_7_1
  doi: 10.1097/WAD.0b013e3181eff860
– volume: 22
  start-page: 765
  year: 2010
  ident: e_1_2_13_21_1
  article-title: FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis
  publication-title: J Alzheimers Dis
  doi: 10.3233/JAD-2010-100837
– ident: e_1_2_13_117_1
  doi: 10.1212/WNL.51.6.1546
– ident: e_1_2_13_64_1
  doi: 10.1136/jnnp.74.9.1206
– ident: e_1_2_13_85_1
  doi: 10.1007/s00415-002-0896-9
– ident: e_1_2_13_125_1
  doi: 10.1001/archneur.62.7.1091
– ident: e_1_2_13_102_1
  doi: 10.1001/archneur.58.11.1803
– ident: e_1_2_13_79_1
  doi: 10.1001/archneurol.2010.201
– ident: e_1_2_13_175_1
  doi: 10.1093/hmg/ddm309
– ident: e_1_2_13_29_1
  doi: 10.1001/archneurol.2011.53
– ident: e_1_2_13_3_1
– ident: e_1_2_13_123_1
  doi: 10.1097/WAD.0b013e31819e6b28
– ident: e_1_2_13_89_1
  doi: 10.1007/s00415-005-0019-5
– ident: e_1_2_13_170_1
  doi: 10.1126/science.1199214
– ident: e_1_2_13_60_1
  doi: 10.1002/ana.20366
– ident: e_1_2_13_22_1
  doi: 10.1093/brain/awl212
– ident: e_1_2_13_140_1
  doi: 10.1007/s10897-009-9222-3
– ident: e_1_2_13_70_1
  doi: 10.1161/STROKEAHA.108.542308
– ident: e_1_2_13_58_1
  doi: 10.1001/jama.287.3.329
– ident: e_1_2_13_153_1
  doi: 10.3233/JAD-2010-101032
– ident: e_1_2_13_179_1
  doi: 10.1111/j.1750-3639.2007.00052.x
– ident: e_1_2_13_4_1
  doi: 10.1016/j.jalz.2011.02.004
– ident: e_1_2_13_178_1
  doi: 10.1212/WNL.0b013e3181ccc732
– ident: e_1_2_13_34_1
  doi: 10.1038/nature05017
– ident: e_1_2_13_154_1
  doi: 10.1016/j.neurobiolaging.2009.08.016
– ident: e_1_2_13_14_1
  doi: 10.1038/sj.ejhg.5201966
– ident: e_1_2_13_127_1
  doi: 10.1016/S1474-4422(10)70299-4
– ident: e_1_2_13_165_1
  doi: 10.1093/brain/awr355
– ident: e_1_2_13_183_1
  doi: 10.1212/01.wnl.0000343851.46573.67
– ident: e_1_2_13_18_1
  doi: 10.7326/0003-4819-601
– ident: e_1_2_13_32_1
  doi: 10.1371/journal.pone.0009872
– ident: e_1_2_13_161_1
  doi: 10.1038/ng1609
– ident: e_1_2_13_86_1
  doi: 10.1002/mds.22697
– ident: e_1_2_13_9_1
  doi: 10.1038/nature05016
– ident: e_1_2_13_166_1
  doi: 10.1073/pnas.95.13.7737
– ident: e_1_2_13_50_1
  doi: 10.1016/j.neurobiolaging.2007.11.002
– ident: e_1_2_13_68_1
  doi: 10.1212/WNL.56.suppl_4.S21
– ident: e_1_2_13_8_1
  doi: 10.1002/1096-8628(20011001)103:2<138::AID-AJMG1529>3.0.CO;2-8
– ident: e_1_2_13_141_1
  doi: 10.1212/01.wnl.0000172911.39167.b6
– ident: e_1_2_13_151_1
  doi: 10.1038/ng1718
– ident: e_1_2_13_162_1
  doi: 10.1093/brain/awl203
– ident: e_1_2_13_75_1
  doi: 10.1002/ana.22264
– ident: e_1_2_13_150_1
  doi: 10.1016/S1474-4422(10)70159-9
– ident: e_1_2_13_48_1
  doi: 10.1038/mp.2011.52
– ident: e_1_2_13_167_1
  doi: 10.1001/archneur.58.11.1828
– ident: e_1_2_13_11_1
  doi: 10.1212/01.wnl.0000311445.21321.fc
– ident: e_1_2_13_174_1
  doi: 10.1002/humu.9484
– ident: e_1_2_13_23_1
  doi: 10.1111/j.1468-1331.2010.02985.x
– ident: e_1_2_13_177_1
  doi: 10.1002/ana.10721
– ident: e_1_2_13_28_1
  doi: 10.1212/WNL.44.9.1683
– ident: e_1_2_13_71_1
  doi: 10.1093/brain/awp062
– ident: e_1_2_13_51_1
  doi: 10.1016/S1474-4422(11)70261-7
– ident: e_1_2_13_54_1
  doi: 10.1212/01.wnl.0000187068.92184.63
– ident: e_1_2_13_142_1
  doi: 10.1002/ajmg.b.30410
– ident: e_1_2_13_77_1
  doi: 10.1001/archneur.62.6.925
– ident: e_1_2_13_33_1
  doi: 10.1093/hmg/7.1.43
– ident: e_1_2_13_107_1
  doi: 10.1177/1533317506290448
– ident: e_1_2_13_118_1
  doi: 10.1001/archneurol.2010.319
– ident: e_1_2_13_6_1
  doi: 10.1002/ajmg.b.31099
– ident: e_1_2_13_173_1
  doi: 10.1007/s00401-010-0698-6
– ident: e_1_2_13_38_1
  doi: 10.1002/ana.20083
– ident: e_1_2_13_169_1
  doi: 10.1016/S0304-3940(96)13138-4
– ident: e_1_2_13_105_1
  doi: 10.1212/01.wnl.0000267642.41594.9d
– ident: e_1_2_13_109_1
  doi: 10.1212/01.wnl.0000334277.16896.fa
– ident: e_1_2_13_93_1
  doi: 10.1093/brain/awr216
– ident: e_1_2_13_110_1
  doi: 10.1016/j.neurobiolaging.2011.08.005
– ident: e_1_2_13_145_1
  doi: 10.1001/archneur.65.4.506
– ident: e_1_2_13_44_1
  doi: 10.1093/brain/awn352
– ident: e_1_2_13_80_1
  doi: 10.1186/1471-2377-8-48
– ident: e_1_2_13_128_1
  doi: 10.1523/JNEUROSCI.4305-10.2010
– ident: e_1_2_13_56_1
  doi: 10.1212/WNL.0b013e31821103e6
– ident: e_1_2_13_52_1
  doi: 10.1038/349704a0
– ident: e_1_2_13_72_1
  doi: 10.1097/NEN.0b013e3182270c54
– ident: e_1_2_13_129_1
  doi: 10.1001/archneur.58.3.383
– ident: e_1_2_13_31_1
  doi: 10.1038/ng1095-219
– ident: e_1_2_13_114_1
  doi: 10.1177/1533317507302320
– ident: e_1_2_13_136_1
  doi: 10.1136/jmg.2005.033456
– ident: e_1_2_13_55_1
  doi: 10.1097/GIM.0b013e31821d69b8
– ident: e_1_2_13_90_1
  doi: 10.1016/j.jns.2006.11.013
– ident: e_1_2_13_46_1
  doi: 10.1093/hmg/ddl241
– ident: e_1_2_13_81_1
  doi: 10.1002/ana.21099
– ident: e_1_2_13_95_1
  doi: 10.1111/j.1468-1331.2008.02069.x
– ident: e_1_2_13_39_1
  doi: 10.1001/archneur.57.1.72
– ident: e_1_2_13_26_1
  doi: 10.1136/jnnp.2007.121137
– ident: e_1_2_13_149_1
  doi: 10.1093/brain/awg204
– ident: e_1_2_13_40_1
  doi: 10.1212/01.wnl.0000244749.20056.d4
– ident: e_1_2_13_159_1
  doi: 10.1038/375754a0
– ident: e_1_2_13_36_1
  doi: 10.1016/j.neuron.2011.09.011
– ident: e_1_2_13_96_1
  doi: 10.1001/archneur.59.11.1759
– ident: e_1_2_13_130_1
  doi: 10.1212/01.WNL.0000086811.39675.79
– ident: e_1_2_13_87_1
  doi: 10.1007/s00415-009-5163-x
– ident: e_1_2_13_138_1
  doi: 10.1016/j.neuron.2011.09.010
– ident: e_1_2_13_62_1
  doi: 10.1126/science.1072994
– ident: e_1_2_13_19_1
  doi: 10.1001/archneur.63.9.1307
– ident: e_1_2_13_92_1
  doi: 10.1093/brain/awn012
– ident: e_1_2_13_98_1
  doi: 10.1056/NEJMc1113592
– ident: e_1_2_13_144_1
  doi: 10.1212/WNL.57.4.621
– ident: e_1_2_13_156_1
  doi: 10.1212/01.wnl.0000319702.37497.72
– ident: e_1_2_13_59_1
  doi: 10.1056/NEJMoa0809578
– ident: e_1_2_13_124_1
  doi: 10.1002/1531-8249(20010201)49:2<263::AID-ANA50>3.0.CO;2-K
– ident: e_1_2_13_181_1
  doi: 10.1002/mds.22642
– ident: e_1_2_13_133_1
  doi: 10.1093/brain/awr179
– ident: e_1_2_13_122_1
  doi: 10.1159/000077158
– ident: e_1_2_13_83_1
  doi: 10.1523/JNEUROSCI.0730-07.2007
– ident: e_1_2_13_160_1
  doi: 10.1093/brain/awr353
– volume: 78
  start-page: 1050
  year: 2007
  ident: e_1_2_13_20_1
  article-title: Clinicopathological concordance and discordance in three monozygotic twin pairs with familial Alzheimer's disease
  publication-title: J Neurol Neurosurg Psychiatry
  doi: 10.1136/jnnp.2006.113803
– ident: e_1_2_13_45_1
  doi: 10.1086/302702
– ident: e_1_2_13_69_1
  doi: 10.1038/31508
– ident: e_1_2_13_164_1
  doi: 10.1007/s00401-011-0816-0
– ident: e_1_2_13_139_1
  doi: 10.1007/s00401-009-0501-8
– ident: e_1_2_13_17_1
  doi: 10.1002/humu.21100
– ident: e_1_2_13_78_1
  doi: 10.1016/j.neuron.2010.11.036
– volume: 6
  start-page: 475
  year: 2004
  ident: e_1_2_13_108_1
  article-title: Pathological and clinical heterogeneity of presenilin 1 gene mutations
  publication-title: J Alzheimers Dis
  doi: 10.3233/JAD-2004-6503
– ident: e_1_2_13_137_1
  doi: 10.1016/S0197-4580(00)00202-5
– ident: e_1_2_13_131_1
  doi: 10.1007/s12031-011-9557-8
– ident: e_1_2_13_182_1
  doi: 10.1038/ng1332
– ident: e_1_2_13_115_1
  doi: 10.1007/s00401-011-0907-y
– ident: e_1_2_13_57_1
  doi: 10.1002/ana.1009
– ident: e_1_2_13_49_1
  doi: 10.1212/01.wnl.0000325058.10218.fc
– ident: e_1_2_13_94_1
  doi: 10.1007/s00259-008-0833-y
– ident: e_1_2_13_126_1
  doi: 10.1093/brain/awm331
– ident: e_1_2_13_43_1
  doi: 10.1007/s12031-011-9637-9
– ident: e_1_2_13_74_1
  doi: 10.1093/brain/awq033
– ident: e_1_2_13_12_1
  doi: 10.3988/jcn.2011.7.1.1
– ident: e_1_2_13_82_1
  doi: 10.1001/archneurol.2010.113
– ident: e_1_2_13_172_1
  doi: 10.1074/jbc.R800019200
– ident: e_1_2_13_176_1
  doi: 10.1212/WNL.0b013e3181b389d9
– ident: e_1_2_13_67_1
  doi: 10.1002/1531-8249(199908)46:2<243::AID-ANA14>3.0.CO;2-L
– ident: e_1_2_13_91_1
  doi: 10.1002/humu.20520
– ident: e_1_2_13_120_1
  doi: 10.1093/brain/awq216
– ident: e_1_2_13_106_1
  doi: 10.1016/j.neurobiolaging.2011.10.014
– ident: e_1_2_13_73_1
  doi: 10.1212/01.WNL.0000042088.22694.E3
– ident: e_1_2_13_76_1
  doi: 10.1212/WNL.56.11.1545
– ident: e_1_2_13_10_1
  doi: 10.1093/hmg/ddh134
– ident: e_1_2_13_99_1
  doi: 10.1212/WNL.59.8.1134
– ident: e_1_2_13_65_1
  doi: 10.1212/01.wnl.0000334278.11022.42
– ident: e_1_2_13_148_1
  doi: 10.1007/s10897-005-4063-1
– ident: e_1_2_13_13_1
  doi: 10.1093/brain/awp009
– ident: e_1_2_13_16_1
  doi: 10.1007/s10048-008-0127-3
– ident: e_1_2_13_25_1
  doi: 10.1086/302553
– ident: e_1_2_13_42_1
  doi: 10.1006/exnr.2000.7613
– ident: e_1_2_13_135_1
  doi: 10.1212/WNL.55.10.1577
– ident: e_1_2_13_171_1
  doi: 10.1159/000058328
– ident: e_1_2_13_132_1
  doi: 10.1136/jnnp.72.2.266
– ident: e_1_2_13_47_1
  doi: 10.1001/archpsyc.63.2.168
– ident: e_1_2_13_116_1
  doi: 10.1038/ng.801
– ident: e_1_2_13_163_1
  doi: 10.1016/j.neuron.2008.11.007
– ident: e_1_2_13_157_1
  doi: 10.1007/s00415-009-5404-z
– ident: e_1_2_13_113_1
  doi: 10.1038/ng0892-345
– ident: e_1_2_13_184_1
  doi: 10.1212/WNL.0b013e3181f07e0c
– volume: 157
  start-page: 633
  year: 2001
  ident: e_1_2_13_41_1
  article-title: Genetic epidemiology of Creutzfeldt–Jakob disease in Europe
  publication-title: Rev Neurol (Paris)
– ident: e_1_2_13_66_1
  doi: 10.1177/0891988710383570
– ident: e_1_2_13_119_1
  doi: 10.1017/S1462399409001136
– ident: e_1_2_13_147_1
  doi: 10.1016/j.neulet.2007.03.044
– ident: e_1_2_13_168_1
  doi: 10.1212/WNL.50.6.1541
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Snippet With increased frequency, clinical geneticists are asked for genetic advice on the heredity of dementia in families. Alzheimer's disease is in most cases a...
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SubjectTerms Adult and adolescent clinical studies
Age of Onset
Algorithms
Alzheimer's disease
Biological and medical sciences
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Dementia - genetics
early onset
frontotemporal dementia
General aspects. Genetic counseling
Genetic Association Studies
genetic counseling
Genetic Predisposition to Disease
genetic testing
Genetics
Humans
Medical genetics
Medical sciences
Neurology
Organic mental disorders. Neuropsychology
Physicians
Practice Guidelines as Topic
Psychology. Psychoanalysis. Psychiatry
Psychopathology. Psychiatry
Title Genetics of dementia: Update and guidelines for the clinician
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https://onlinelibrary.wiley.com/doi/abs/10.1002%2Fajmg.b.32080
https://www.ncbi.nlm.nih.gov/pubmed/22815225
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https://www.proquest.com/docview/1033451716
https://www.proquest.com/docview/1492622501
Volume 159B
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