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. 2002 Jun;72(6):691–700. doi: 10.1136/jnnp.72.6.691

Presenile dementia syndromes: an update on taxonomy and diagnosis

M Greicius 1, M Geschwind 1, B Miller 1
PMCID: PMC1737933  PMID: 12023408

Abstract

The four major degenerative dementias that often begin in presenescence: are reviewed. These are Alzheimer's disease, frontotemporal dementia, dementia with Lewy bodies, and Creutzfeldt–Jakob disease. Their epidemiological, genetic, and clinical features are reviewed, and controversies in taxonomy arising from recent discoveries described. Particular attention is given to the pathological role of protein aggregation, which appears to be a factor in each disease.

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Selected References

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  1. Arriagada P. V., Marzloff K., Hyman B. T. Distribution of Alzheimer-type pathologic changes in nondemented elderly individuals matches the pattern in Alzheimer's disease. Neurology. 1992 Sep;42(9):1681–1688. doi: 10.1212/wnl.42.9.1681. [DOI] [PubMed] [Google Scholar]
  2. Barber R., Ballard C., McKeith I. G., Gholkar A., O'Brien J. T. MRI volumetric study of dementia with Lewy bodies: a comparison with AD and vascular dementia. Neurology. 2000 Mar 28;54(6):1304–1309. doi: 10.1212/wnl.54.6.1304. [DOI] [PubMed] [Google Scholar]
  3. Belay E. D. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol. 1999;53:283–314. doi: 10.1146/annurev.micro.53.1.283. [DOI] [PubMed] [Google Scholar]
  4. Belichenko P. V., Miklossy J., Belser B., Budka H., Celio M. R. Early destruction of the extracellular matrix around parvalbumin-immunoreactive interneurons in Creutzfeldt-Jakob disease. Neurobiol Dis. 1999 Aug;6(4):269–279. doi: 10.1006/nbdi.1999.0245. [DOI] [PubMed] [Google Scholar]
  5. Benson D. F., Davis R. J., Snyder B. D. Posterior cortical atrophy. Arch Neurol. 1988 Jul;45(7):789–793. doi: 10.1001/archneur.1988.00520310107024. [DOI] [PubMed] [Google Scholar]
  6. Bethwaite P. B., Delahunt B., Holloway L. J., Thornton A. Comparison of silver-staining nucleolar organizer region (AgNOR) counts and proliferating cell nuclear antigen (PCNA) expression in reactive mesothelial hyperplasia and malignant mesothelioma. Pathology. 1995 Jan;27(1):1–4. doi: 10.1080/00313029500169362. [DOI] [PubMed] [Google Scholar]
  7. Blacker D., Albert M. S., Bassett S. S., Go R. C., Harrell L. E., Folstein M. F. Reliability and validity of NINCDS-ADRDA criteria for Alzheimer's disease. The National Institute of Mental Health Genetics Initiative. Arch Neurol. 1994 Dec;51(12):1198–1204. doi: 10.1001/archneur.1994.00540240042014. [DOI] [PubMed] [Google Scholar]
  8. Blacker D., Wilcox M. A., Laird N. M., Rodes L., Horvath S. M., Go R. C., Perry R., Watson B., Jr, Bassett S. S., McInnis M. G. Alpha-2 macroglobulin is genetically associated with Alzheimer disease. Nat Genet. 1998 Aug;19(4):357–360. doi: 10.1038/1243. [DOI] [PubMed] [Google Scholar]
  9. Blessed G., Tomlinson B. E., Roth M. The association between quantitative measures of dementia and of senile change in the cerebral grey matter of elderly subjects. Br J Psychiatry. 1968 Jul;114(512):797–811. doi: 10.1192/bjp.114.512.797. [DOI] [PubMed] [Google Scholar]
  10. Bookheimer S. Y., Strojwas M. H., Cohen M. S., Saunders A. M., Pericak-Vance M. A., Mazziotta J. C., Small G. W. Patterns of brain activation in people at risk for Alzheimer's disease. N Engl J Med. 2000 Aug 17;343(7):450–456. doi: 10.1056/NEJM200008173430701. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Braak H., Braak E. Staging of Alzheimer's disease-related neurofibrillary changes. Neurobiol Aging. 1995 May-Jun;16(3):271–284. doi: 10.1016/0197-4580(95)00021-6. [DOI] [PubMed] [Google Scholar]
  12. Brown J., Ashworth A., Gydesen S., Sorensen A., Rossor M., Hardy J., Collinge J. Familial non-specific dementia maps to chromosome 3. Hum Mol Genet. 1995 Sep;4(9):1625–1628. doi: 10.1093/hmg/4.9.1625. [DOI] [PubMed] [Google Scholar]
  13. Brown P., Cathala F., Castaigne P., Gajdusek D. C. Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol. 1986 Nov;20(5):597–602. doi: 10.1002/ana.410200507. [DOI] [PubMed] [Google Scholar]
  14. Brown P., Gibbs C. J., Jr, Rodgers-Johnson P., Asher D. M., Sulima M. P., Bacote A., Goldfarb L. G., Gajdusek D. C. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol. 1994 May;35(5):513–529. doi: 10.1002/ana.410350504. [DOI] [PubMed] [Google Scholar]
  15. Brun A. Frontal lobe degeneration of non-Alzheimer type revisited. Dementia. 1993 May-Aug;4(3-4):126–131. doi: 10.1159/000107311. [DOI] [PubMed] [Google Scholar]
  16. Brun A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr. 1987 Sep;6(3):193–208. doi: 10.1016/0167-4943(87)90021-5. [DOI] [PubMed] [Google Scholar]
  17. Bugiani O., Murrell J. R., Giaccone G., Hasegawa M., Ghigo G., Tabaton M., Morbin M., Primavera A., Carella F., Solaro C. Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. J Neuropathol Exp Neurol. 1999 Jun;58(6):667–677. doi: 10.1097/00005072-199906000-00011. [DOI] [PubMed] [Google Scholar]
  18. Burke W. J., Pfeiffer R. F., McComb R. D. Neuroleptic sensitivity to clozapine in dementia with Lewy bodies. J Neuropsychiatry Clin Neurosci. 1998 Spring;10(2):227–229. doi: 10.1176/jnp.10.2.227. [DOI] [PubMed] [Google Scholar]
  19. Burns A., Rossor M., Hecker J., Gauthier S., Petit H., Möller H. J., Rogers S. L., Friedhoff L. T. The effects of donepezil in Alzheimer's disease - results from a multinational trial. Dement Geriatr Cogn Disord. 1999 May-Jun;10(3):237–244. doi: 10.1159/000017126. [DOI] [PubMed] [Google Scholar]
  20. Chen L., Baum L., Ng H. K., Chan L. Y., Sastre I., Artíga M. J., Valdivieso F., Bullido M. J., Chiu H. F., Pang C. P. Apolipoprotein E promoter and alpha2-macroglobulin polymorphisms are not genetically associated with Chinese late onset Alzheimer's disease. Neurosci Lett. 1999 Jul 16;269(3):173–177. doi: 10.1016/s0304-3940(99)00421-8. [DOI] [PubMed] [Google Scholar]
  21. Chow T. W., Miller B. L., Hayashi V. N., Geschwind D. H. Inheritance of frontotemporal dementia. Arch Neurol. 1999 Jul;56(7):817–822. doi: 10.1001/archneur.56.7.817. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Clark L. N., Poorkaj P., Wszolek Z., Geschwind D. H., Nasreddine Z. S., Miller B., Li D., Payami H., Awert F., Markopoulou K. Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17. Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):13103–13107. doi: 10.1073/pnas.95.22.13103. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry. 1994 Apr;57(4):416–418. doi: 10.1136/jnnp.57.4.416. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Collie D. A., Sellar R. J., Zeidler M., Colchester A. C., Knight R., Will R. G. MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. Clin Radiol. 2001 Sep;56(9):726–739. doi: 10.1053/crad.2001.0771. [DOI] [PubMed] [Google Scholar]
  25. Conway K. A., Harper J. D., Lansbury P. T. Accelerated in vitro fibril formation by a mutant alpha-synuclein linked to early-onset Parkinson disease. Nat Med. 1998 Nov;4(11):1318–1320. doi: 10.1038/3311. [DOI] [PubMed] [Google Scholar]
  26. Cummings J. L., Miller B., Hill M. A., Neshkes R. Neuropsychiatric aspects of multi-infarct dementia and dementia of the Alzheimer type. Arch Neurol. 1987 Apr;44(4):389–393. doi: 10.1001/archneur.1987.00520160031010. [DOI] [PubMed] [Google Scholar]
  27. Delacourte A. Biochemical and molecular characterization of neurofibrillary degeneration in frontotemporal dementias. Dement Geriatr Cogn Disord. 1999;10 (Suppl 1):75–79. doi: 10.1159/000051218. [DOI] [PubMed] [Google Scholar]
  28. Detoledo-Morrell L., Sullivan M. P., Morrell F., Wilson R. S., Bennett D. A., Spencer S. Alzheimer's disease: in vivo detection of differential vulnerability of brain regions. Neurobiol Aging. 1997 Sep-Oct;18(5):463–468. doi: 10.1016/s0197-4580(97)00114-0. [DOI] [PubMed] [Google Scholar]
  29. Di Maria E., Tabaton M., Vigo T., Abbruzzese G., Bellone E., Donati C., Frasson E., Marchese R., Montagna P., Munoz D. G. Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Ann Neurol. 2000 Mar;47(3):374–377. doi: 10.1002/1531-8249(200003)47:3<374::aid-ana15>3.3.co;2-#. [DOI] [PubMed] [Google Scholar]
  30. Dickson D. W., Schmidt M. L., Lee V. M., Zhao M. L., Yen S. H., Trojanowski J. Q. Immunoreactivity profile of hippocampal CA2/3 neurites in diffuse Lewy body disease. Acta Neuropathol. 1994;87(3):269–276. doi: 10.1007/BF00296742. [DOI] [PubMed] [Google Scholar]
  31. Doody R. S., Geldmacher D. S., Gordon B., Perdomo C. A., Pratt R. D., Donepezil Study Group Open-label, multicenter, phase 3 extension study of the safety and efficacy of donepezil in patients with Alzheimer disease. Arch Neurol. 2001 Mar;58(3):427–433. doi: 10.1001/archneur.58.3.427. [DOI] [PubMed] [Google Scholar]
  32. Dovey H. F., John V., Anderson J. P., Chen L. Z., de Saint Andrieu P., Fang L. Y., Freedman S. B., Folmer B., Goldbach E., Holsztynska E. J. Functional gamma-secretase inhibitors reduce beta-amyloid peptide levels in brain. J Neurochem. 2001 Jan;76(1):173–181. doi: 10.1046/j.1471-4159.2001.00012.x. [DOI] [PubMed] [Google Scholar]
  33. Duda J. E., Lee V. M., Trojanowski J. Q. Neuropathology of synuclein aggregates. J Neurosci Res. 2000 Jul 15;61(2):121–127. doi: 10.1002/1097-4547(20000715)61:2<121::AID-JNR1>3.0.CO;2-4. [DOI] [PubMed] [Google Scholar]
  34. Edwards-Lee T., Miller B. L., Benson D. F., Cummings J. L., Russell G. L., Boone K., Mena I. The temporal variant of frontotemporal dementia. Brain. 1997 Jun;120(Pt 6):1027–1040. doi: 10.1093/brain/120.6.1027. [DOI] [PubMed] [Google Scholar]
  35. Esmonde T., Giles E., Gibson M., Hodges J. R. Neuropsychological performance, disease severity, and depression in progressive supranuclear palsy. J Neurol. 1996 Sep;243(9):638–643. doi: 10.1007/BF00878659. [DOI] [PubMed] [Google Scholar]
  36. Farrer M., Gwinn-Hardy K., Hutton M., Hardy J. The genetics of disorders with synuclein pathology and parkinsonism. Hum Mol Genet. 1999;8(10):1901–1905. doi: 10.1093/hmg/8.10.1901. [DOI] [PubMed] [Google Scholar]
  37. Feany M. B., Dickson D. W. Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Ann Neurol. 1996 Aug;40(2):139–148. doi: 10.1002/ana.410400204. [DOI] [PubMed] [Google Scholar]
  38. Ferrer I., Casas R., Rivera R. Parvalbumin-immunoreactive cortical neurons in Creutzfeldt-Jakob disease. Ann Neurol. 1993 Dec;34(6):864–866. doi: 10.1002/ana.410340617. [DOI] [PubMed] [Google Scholar]
  39. Filley C. M. Neuropsychiatric features of Lewy body disease. Brain Cogn. 1995 Aug;28(3):229–239. doi: 10.1006/brcg.1995.1254. [DOI] [PubMed] [Google Scholar]
  40. Fox N. C., Warrington E. K., Seiffer A. L., Agnew S. K., Rossor M. N. Presymptomatic cognitive deficits in individuals at risk of familial Alzheimer's disease. A longitudinal prospective study. Brain. 1998 Sep;121(Pt 9):1631–1639. doi: 10.1093/brain/121.9.1631. [DOI] [PubMed] [Google Scholar]
  41. Francis P. T., Holmes C., Webster M. T., Stratmann G. C., Procter A. W., Bowen D. M. Preliminary neurochemical findings in non-Alzheimer dementia due to lobar atrophy. Dementia. 1993 May-Aug;4(3-4):172–177. doi: 10.1159/000107319. [DOI] [PubMed] [Google Scholar]
  42. Frisoni G. B., Laakso M. P., Beltramello A., Geroldi C., Bianchetti A., Soininen H., Trabucchi M. Hippocampal and entorhinal cortex atrophy in frontotemporal dementia and Alzheimer's disease. Neurology. 1999 Jan 1;52(1):91–100. doi: 10.1212/wnl.52.1.91. [DOI] [PubMed] [Google Scholar]
  43. Gajdusek D. C. Unconventional viruses and the origin and disappearance of kuru. Science. 1977 Sep 2;197(4307):943–960. doi: 10.1126/science.142303. [DOI] [PubMed] [Google Scholar]
  44. Galasko D., Saitoh T., Xia Y., Thal L. J., Katzman R., Hill L. R., Hansen L. The apolipoprotein E allele epsilon 4 is overrepresented in patients with the Lewy body variant of Alzheimer's disease. Neurology. 1994 Oct;44(10):1950–1951. doi: 10.1212/wnl.44.10.1950. [DOI] [PubMed] [Google Scholar]
  45. Gambetti P., Parchi P. Insomnia in prion diseases: sporadic and familial. N Engl J Med. 1999 May 27;340(21):1675–1677. doi: 10.1056/NEJM199905273402111. [DOI] [PubMed] [Google Scholar]
  46. Gearing M., Olson D. A., Watts R. L., Mirra S. S. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology. 1994 Jun;44(6):1015–1024. doi: 10.1212/wnl.44.6.1015. [DOI] [PubMed] [Google Scholar]
  47. Goate A., Chartier-Harlin M. C., Mullan M., Brown J., Crawford F., Fidani L., Giuffra L., Haynes A., Irving N., James L. Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease. Nature. 1991 Feb 21;349(6311):704–706. doi: 10.1038/349704a0. [DOI] [PubMed] [Google Scholar]
  48. Goedert M., Crowther R. A., Spillantini M. G. Tau mutations cause frontotemporal dementias. Neuron. 1998 Nov;21(5):955–958. doi: 10.1016/s0896-6273(00)80615-7. [DOI] [PubMed] [Google Scholar]
  49. Grimes D. A., Lang A. E., Bergeron C. B. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology. 1999 Dec 10;53(9):1969–1974. doi: 10.1212/wnl.53.9.1969. [DOI] [PubMed] [Google Scholar]
  50. Guentchev M., Groschup M. H., Kordek R., Liberski P. P., Budka H. Severe, early and selective loss of a subpopulation of GABAergic inhibitory neurons in experimental transmissible spongiform encephalopathies. Brain Pathol. 1998 Oct;8(4):615–623. doi: 10.1111/j.1750-3639.1998.tb00188.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  51. Guentchev M., Wanschitz J., Voigtländer T., Flicker H., Budka H. Selective neuronal vulnerability in human prion diseases. Fatal familial insomnia differs from other types of prion diseases. Am J Pathol. 1999 Nov;155(5):1453–1457. doi: 10.1016/S0002-9440(10)65459-4. [DOI] [PMC free article] [PubMed] [Google Scholar]
  52. Gustafson L. Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Arch Gerontol Geriatr. 1987 Sep;6(3):209–223. doi: 10.1016/0167-4943(87)90022-7. [DOI] [PubMed] [Google Scholar]
  53. Hashimoto M., Hsu L. J., Xia Y., Takeda A., Sisk A., Sundsmo M., Masliah E. Oxidative stress induces amyloid-like aggregate formation of NACP/alpha-synuclein in vitro. Neuroreport. 1999 Mar 17;10(4):717–721. doi: 10.1097/00001756-199903170-00011. [DOI] [PubMed] [Google Scholar]
  54. Hashimoto M., Kitagaki H., Imamura T., Hirono N., Shimomura T., Kazui H., Tanimukai S., Hanihara T., Mori E. Medial temporal and whole-brain atrophy in dementia with Lewy bodies: a volumetric MRI study. Neurology. 1998 Aug;51(2):357–362. doi: 10.1212/wnl.51.2.357. [DOI] [PubMed] [Google Scholar]
  55. Haxby J. V., Grady C. L., Koss E., Horwitz B., Heston L., Schapiro M., Friedland R. P., Rapoport S. I. Longitudinal study of cerebral metabolic asymmetries and associated neuropsychological patterns in early dementia of the Alzheimer type. Arch Neurol. 1990 Jul;47(7):753–760. doi: 10.1001/archneur.1990.00530070043010. [DOI] [PubMed] [Google Scholar]
  56. Hendrie H. C. Epidemiology of dementia and Alzheimer's disease. Am J Geriatr Psychiatry. 1998 Spring;6(2 Suppl 1):S3–18. doi: 10.1097/00019442-199821001-00002. [DOI] [PubMed] [Google Scholar]
  57. Heutink P. Untangling tau-related dementia. Hum Mol Genet. 2000 Apr 12;9(6):979–986. doi: 10.1093/hmg/9.6.979. [DOI] [PubMed] [Google Scholar]
  58. Hill A. F., Butterworth R. J., Joiner S., Jackson G., Rossor M. N., Thomas D. J., Frosh A., Tolley N., Bell J. E., Spencer M. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet. 1999 Jan 16;353(9148):183–189. doi: 10.1016/s0140-6736(98)12075-5. [DOI] [PubMed] [Google Scholar]
  59. Hodges J. R., Miller B. The classification, genetics and neuropathology of frontotemporal dementia. Introduction to the special topic papers: Part I. Neurocase. 2001;7(1):31–35. doi: 10.1093/neucas/7.1.31. [DOI] [PubMed] [Google Scholar]
  60. Hodges J. R., Patterson K., Oxbury S., Funnell E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain. 1992 Dec;115(Pt 6):1783–1806. doi: 10.1093/brain/115.6.1783. [DOI] [PubMed] [Google Scholar]
  61. Holmes C., Cairns N., Lantos P., Mann A. Validity of current clinical criteria for Alzheimer's disease, vascular dementia and dementia with Lewy bodies. Br J Psychiatry. 1999 Jan;174:45–50. doi: 10.1192/bjp.174.1.45. [DOI] [PubMed] [Google Scholar]
  62. Hong M., Zhukareva V., Vogelsberg-Ragaglia V., Wszolek Z., Reed L., Miller B. I., Geschwind D. H., Bird T. D., McKeel D., Goate A. Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17. Science. 1998 Dec 4;282(5395):1914–1917. doi: 10.1126/science.282.5395.1914. [DOI] [PubMed] [Google Scholar]
  63. Hosler B. A., Siddique T., Sapp P. C., Sailor W., Huang M. C., Hossain A., Daube J. R., Nance M., Fan C., Kaplan J. Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. JAMA. 2000 Oct 4;284(13):1664–1669. doi: 10.1001/jama.284.13.1664. [DOI] [PubMed] [Google Scholar]
  64. Houlden H., Baker M., Adamson J., Grover A., Waring S., Dickson D., Lynch T., Boeve B., Petersen R. C., Pickering-Brown S. Frequency of tau mutations in three series of non-Alzheimer's degenerative dementia. Ann Neurol. 1999 Aug;46(2):243–248. doi: 10.1002/1531-8249(199908)46:2<243::aid-ana14>3.0.co;2-l. [DOI] [PubMed] [Google Scholar]
  65. Houlden H., Baker M., Morris H. R., MacDonald N., Pickering-Brown S., Adamson J., Lees A. J., Rossor M. N., Quinn N. P., Kertesz A. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology. 2001 Jun 26;56(12):1702–1706. doi: 10.1212/wnl.56.12.1702. [DOI] [PubMed] [Google Scholar]
  66. Hutton M., Lendon C. L., Rizzu P., Baker M., Froelich S., Houlden H., Pickering-Brown S., Chakraverty S., Isaacs A., Grover A. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature. 1998 Jun 18;393(6686):702–705. doi: 10.1038/31508. [DOI] [PubMed] [Google Scholar]
  67. Ince P. G., McArthur F. K., Bjertness E., Torvik A., Candy J. M., Edwardson J. A. Neuropathological diagnoses in elderly patients in Oslo: Alzheimer's disease, Lewy body disease, vascular lesions. Dementia. 1995 May-Jun;6(3):162–168. doi: 10.1159/000106940. [DOI] [PubMed] [Google Scholar]
  68. Jensen P. H., Nielsen M. S., Jakes R., Dotti C. G., Goedert M. Binding of alpha-synuclein to brain vesicles is abolished by familial Parkinson's disease mutation. J Biol Chem. 1998 Oct 9;273(41):26292–26294. doi: 10.1074/jbc.273.41.26292. [DOI] [PubMed] [Google Scholar]
  69. Jeste D. V., Rockwell E., Harris M. J., Lohr J. B., Lacro J. Conventional vs. newer antipsychotics in elderly patients. Am J Geriatr Psychiatry. 1999 Winter;7(1):70–76. [PubMed] [Google Scholar]
  70. Johnson J. K., Head E., Kim R., Starr A., Cotman C. W. Clinical and pathological evidence for a frontal variant of Alzheimer disease. Arch Neurol. 1999 Oct;56(10):1233–1239. doi: 10.1001/archneur.56.10.1233. [DOI] [PubMed] [Google Scholar]
  71. Johnson K. A., Albert M. S. Perfusion abnormalities in prodromal AD. Neurobiol Aging. 2000 Mar-Apr;21(2):289–292. doi: 10.1016/s0197-4580(00)00137-8. [DOI] [PubMed] [Google Scholar]
  72. Kazee A. M., Eskin T. A., Lapham L. W., Gabriel K. R., McDaniel K. D., Hamill R. W. Clinicopathologic correlates in Alzheimer disease: assessment of clinical and pathologic diagnostic criteria. Alzheimer Dis Assoc Disord. 1993 Fall;7(3):152–164. doi: 10.1097/00002093-199307030-00004. [DOI] [PubMed] [Google Scholar]
  73. Kertesz A. Frontotemporal dementia, Pick disease, and corticobasal degeneration. One entity or 3? 1. Arch Neurol. 1997 Nov;54(11):1427–1429. doi: 10.1001/archneur.1997.00550230090024. [DOI] [PubMed] [Google Scholar]
  74. Kertesz A., Kawarai T., Rogaeva E., St George-Hyslop P., Poorkaj P., Bird T. D., Munoz D. G. Familial frontotemporal dementia with ubiquitin-positive, tau-negative inclusions. Neurology. 2000 Feb 22;54(4):818–827. doi: 10.1212/wnl.54.4.818. [DOI] [PubMed] [Google Scholar]
  75. Kertesz A., Martinez-Lage P., Davidson W., Munoz D. G. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology. 2000 Nov 14;55(9):1368–1375. doi: 10.1212/wnl.55.9.1368. [DOI] [PubMed] [Google Scholar]
  76. Korth C., May B. C., Cohen F. E., Prusiner S. B. Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proc Natl Acad Sci U S A. 2001 Aug 14;98(17):9836–9841. doi: 10.1073/pnas.161274798. [DOI] [PMC free article] [PubMed] [Google Scholar]
  77. Kramer J. H., Miller B. L. Alzheimer's disease and its focal variants. Semin Neurol. 2000;20(4):447–454. doi: 10.1055/s-2000-13177. [DOI] [PubMed] [Google Scholar]
  78. Kretzschmar H. A., Ironside J. W., DeArmond S. J., Tateishi J. Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol. 1996 Sep;53(9):913–920. doi: 10.1001/archneur.1996.00550090125018. [DOI] [PubMed] [Google Scholar]
  79. Kukull W. A., Larson E. B., Reifler B. V., Lampe T. H., Yerby M., Hughes J. Interrater reliability of Alzheimer's disease diagnosis. Neurology. 1990 Feb;40(2):257–260. doi: 10.1212/wnl.40.2.257. [DOI] [PubMed] [Google Scholar]
  80. Lanctôt K. L., Herrmann N. Donepezil for behavioural disorders associated with Lewy bodies: a case series. Int J Geriatr Psychiatry. 2000 Apr;15(4):338–345. doi: 10.1002/(sici)1099-1166(200004)15:4<338::aid-gps119>3.0.co;2-u. [DOI] [PubMed] [Google Scholar]
  81. Lippa C. F., Smith T. W., Saunders A. M., Crook R., Pulaski-Salo D., Davies P., Hardy J., Roses A. D., Dickson D. Apolipoprotein E genotype and Lewy body disease. Neurology. 1995 Jan;45(1):97–103. doi: 10.1212/wnl.45.1.97. [DOI] [PubMed] [Google Scholar]
  82. Lopez O. L., Litvan I., Catt K. E., Stowe R., Klunk W., Kaufer D. I., Becker J. T., DeKosky S. T. Accuracy of four clinical diagnostic criteria for the diagnosis of neurodegenerative dementias. Neurology. 1999 Oct 12;53(6):1292–1299. doi: 10.1212/wnl.53.6.1292. [DOI] [PubMed] [Google Scholar]
  83. Lorains J. W., Henry C., Agbamu D. A., Rossi M., Bishop M., Will R. G., Ironside J. W. Variant Creutzfeldt-Jakob disease in an elderly patient. Lancet. 2001 Apr 28;357(9265):1339–1340. doi: 10.1016/s0140-6736(00)04504-9. [DOI] [PubMed] [Google Scholar]
  84. Masters C. L., Gajdusek D. C., Gibbs C. J., Jr The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer's disease. Brain. 1981 Sep;104(3):535–558. doi: 10.1093/brain/104.3.535. [DOI] [PubMed] [Google Scholar]
  85. Mastrianni J. A., Roos R. P. The prion diseases. Semin Neurol. 2000;20(3):337–352. doi: 10.1055/s-2000-9396. [DOI] [PubMed] [Google Scholar]
  86. Mathuranath P. S., Xuereb J. H., Bak T., Hodges J. R. Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature. J Neurol Neurosurg Psychiatry. 2000 Mar;68(3):304–312. doi: 10.1136/jnnp.68.3.304. [DOI] [PMC free article] [PubMed] [Google Scholar]
  87. Matoba M., Tonami H., Miyaji H., Yokota H., Yamamoto I. Creutzfeldt-Jakob disease: serial changes on diffusion-weighted MRI. J Comput Assist Tomogr. 2001 Mar-Apr;25(2):274–277. doi: 10.1097/00004728-200103000-00022. [DOI] [PubMed] [Google Scholar]
  88. Mayeux R., Sano M. Treatment of Alzheimer's disease. N Engl J Med. 1999 Nov 25;341(22):1670–1679. doi: 10.1056/NEJM199911253412207. [DOI] [PubMed] [Google Scholar]
  89. Mayeux R., Saunders A. M., Shea S., Mirra S., Evans D., Roses A. D., Hyman B. T., Crain B., Tang M. X., Phelps C. H. Utility of the apolipoprotein E genotype in the diagnosis of Alzheimer's disease. Alzheimer's Disease Centers Consortium on Apolipoprotein E and Alzheimer's Disease. N Engl J Med. 1998 Feb 19;338(8):506–511. doi: 10.1056/NEJM199802193380804. [DOI] [PubMed] [Google Scholar]
  90. McKeith I. G., Ballard C. G., Harrison R. W. Neuroleptic sensitivity to risperidone in Lewy body dementia. Lancet. 1995 Sep 9;346(8976):699–699. doi: 10.1016/s0140-6736(95)92307-1. [DOI] [PubMed] [Google Scholar]
  91. McKeith I. G., Galasko D., Kosaka K., Perry E. K., Dickson D. W., Hansen L. A., Salmon D. P., Lowe J., Mirra S. S., Byrne E. J. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology. 1996 Nov;47(5):1113–1124. doi: 10.1212/wnl.47.5.1113. [DOI] [PubMed] [Google Scholar]
  92. McKeith I. G., Perry E. K., Perry R. H. Report of the second dementia with Lewy body international workshop: diagnosis and treatment. Consortium on Dementia with Lewy Bodies. Neurology. 1999 Sep 22;53(5):902–905. doi: 10.1212/wnl.53.5.902. [DOI] [PubMed] [Google Scholar]
  93. McKeith I., Del Ser T., Spano P., Emre M., Wesnes K., Anand R., Cicin-Sain A., Ferrara R., Spiegel R. Efficacy of rivastigmine in dementia with Lewy bodies: a randomised, double-blind, placebo-controlled international study. Lancet. 2000 Dec 16;356(9247):2031–2036. doi: 10.1016/S0140-6736(00)03399-7. [DOI] [PubMed] [Google Scholar]
  94. McKeith I., Fairbairn A., Perry R., Thompson P., Perry E. Neuroleptic sensitivity in patients with senile dementia of Lewy body type. BMJ. 1992 Sep 19;305(6855):673–678. doi: 10.1136/bmj.305.6855.673. [DOI] [PMC free article] [PubMed] [Google Scholar]
  95. McKhann G., Drachman D., Folstein M., Katzman R., Price D., Stadlan E. M. 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. 1984 Jul;34(7):939–944. doi: 10.1212/wnl.34.7.939. [DOI] [PubMed] [Google Scholar]
  96. McShane R., Keene J., Gedling K., Fairburn C., Jacoby R., Hope T. Do neuroleptic drugs hasten cognitive decline in dementia? Prospective study with necropsy follow up. BMJ. 1997 Jan 25;314(7076):266–270. doi: 10.1136/bmj.314.7076.266. [DOI] [PMC free article] [PubMed] [Google Scholar]
  97. Mega M. S., Masterman D. L., Benson D. F., Vinters H. V., Tomiyasu U., Craig A. H., Foti D. J., Kaufer D., Scharre D. W., Fairbanks L. Dementia with Lewy bodies: reliability and validity of clinical and pathologic criteria. Neurology. 1996 Dec;47(6):1403–1409. doi: 10.1212/wnl.47.6.1403. [DOI] [PubMed] [Google Scholar]
  98. Mentis M. J., Horwitz B., Grady C. L., Alexander G. E., VanMeter J. W., Maisog J. M., Pietrini P., Schapiro M. B., Rapoport S. I. Visual cortical dysfunction in Alzheimer's disease evaluated with a temporally graded "stress test" during PET. Am J Psychiatry. 1996 Jan;153(1):32–40. doi: 10.1176/ajp.153.1.32. [DOI] [PubMed] [Google Scholar]
  99. Mesulam M. M. Slowly progressive aphasia without generalized dementia. Ann Neurol. 1982 Jun;11(6):592–598. doi: 10.1002/ana.410110607. [DOI] [PubMed] [Google Scholar]
  100. Miller B. L., Chang L., Mena I., Boone K., Lesser I. M. Progressive right frontotemporal degeneration: clinical, neuropsychological and SPECT characteristics. Dementia. 1993 May-Aug;4(3-4):204–213. doi: 10.1159/000107324. [DOI] [PubMed] [Google Scholar]
  101. Miller B. L., Gearhart R. Neuroimaging in the diagnosis of frontotemporal dementia. Dement Geriatr Cogn Disord. 1999;10 (Suppl 1):71–74. doi: 10.1159/000051217. [DOI] [PubMed] [Google Scholar]
  102. Miller B. L., Ikonte C., Ponton M., Levy M., Boone K., Darby A., Berman N., Mena I., Cummings J. L. A study of the Lund-Manchester research criteria for frontotemporal dementia: clinical and single-photon emission CT correlations. Neurology. 1997 Apr;48(4):937–942. doi: 10.1212/wnl.48.4.937. [DOI] [PubMed] [Google Scholar]
  103. Muenter M. D., Forno L. S., Hornykiewicz O., Kish S. J., Maraganore D. M., Caselli R. J., Okazaki H., Howard F. M., Jr, Snow B. J., Calne D. B. Hereditary form of parkinsonism--dementia. Ann Neurol. 1998 Jun;43(6):768–781. doi: 10.1002/ana.410430612. [DOI] [PubMed] [Google Scholar]
  104. Mölsä P. K., Marttila R. J., Rinne U. K. Survival and cause of death in Alzheimer's disease and multi-infarct dementia. Acta Neurol Scand. 1986 Aug;74(2):103–107. doi: 10.1111/j.1600-0404.1986.tb04634.x. [DOI] [PubMed] [Google Scholar]
  105. Nasreddine Z. S., Loginov M., Clark L. N., Lamarche J., Miller B. L., Lamontagne A., Zhukareva V., Lee V. M., Wilhelmsen K. C., Geschwind D. H. From genotype to phenotype: a clinical pathological, and biochemical investigation of frontotemporal dementia and parkinsonism (FTDP-17) caused by the P301L tau mutation. Ann Neurol. 1999 Jun;45(6):704–715. doi: 10.1002/1531-8249(199906)45:6<704::aid-ana4>3.0.co;2-x. [DOI] [PubMed] [Google Scholar]
  106. Neary D., Snowden J. S., Gustafson L., Passant U., Stuss D., Black S., Freedman M., Kertesz A., Robert P. H., Albert M. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998 Dec;51(6):1546–1554. doi: 10.1212/wnl.51.6.1546. [DOI] [PubMed] [Google Scholar]
  107. Neary D., Snowden J. S., Northen B., Goulding P. Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry. 1988 Mar;51(3):353–361. doi: 10.1136/jnnp.51.3.353. [DOI] [PMC free article] [PubMed] [Google Scholar]
  108. Neary D., Snowden J. S., Shields R. A., Burjan A. W., Northen B., MacDermott N., Prescott M. C., Testa H. J. Single photon emission tomography using 99mTc-HM-PAO in the investigation of dementia. J Neurol Neurosurg Psychiatry. 1987 Sep;50(9):1101–1109. doi: 10.1136/jnnp.50.9.1101. [DOI] [PMC free article] [PubMed] [Google Scholar]
  109. Nestor P., Hodges J. Non-Alzheimer dementias. Semin Neurol. 2000;20(4):439–446. doi: 10.1055/s-2000-13176. [DOI] [PubMed] [Google Scholar]
  110. Oppenheim C., Brandel J. P., Hauw J. J., Deslys J. P., Fontaine B. MRI and the second French case of vCJD. Lancet. 2000 Jul 15;356(9225):253–254. doi: 10.1016/S0140-6736(05)74505-0. [DOI] [PubMed] [Google Scholar]
  111. Pals P., Van Everbroeck B., Sciot R., Godfraind C., Robberecht W., Dom R., Laterre C., Martin J. J., Cras P. A retrospective study of Creutzfeldt-Jakob disease in Belgium. Eur J Epidemiol. 1999 Jul;15(6):517–519. doi: 10.1023/a:1007581313067. [DOI] [PubMed] [Google Scholar]
  112. Papka M., Rubio A., Schiffer R. B. A review of Lewy body disease, an emerging concept of cortical dementia. J Neuropsychiatry Clin Neurosci. 1998 Summer;10(3):267–279. doi: 10.1176/jnp.10.3.267. [DOI] [PubMed] [Google Scholar]
  113. Parchi P., Giese A., Capellari S., Brown P., Schulz-Schaeffer W., Windl O., Zerr I., Budka H., Kopp N., Piccardo P. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol. 1999 Aug;46(2):224–233. [PubMed] [Google Scholar]
  114. Perry R. H., Irving D., Blessed G., Fairbairn A., Perry E. K. Senile dementia of Lewy body type. A clinically and neuropathologically distinct form of Lewy body dementia in the elderly. J Neurol Sci. 1990 Feb;95(2):119–139. doi: 10.1016/0022-510x(90)90236-g. [DOI] [PubMed] [Google Scholar]
  115. Perry R. H., Irving D., Blessed G., Perry E. K., Fairbairn A. F. Clinically and neuropathologically distinct form of dementia in the elderly. Lancet. 1989 Jan 21;1(8630):166–166. doi: 10.1016/s0140-6736(89)91187-2. [DOI] [PubMed] [Google Scholar]
  116. Perry R. J., Miller B. L. Behavior and treatment in frontotemporal dementia. Neurology. 2001 Jun;56(11 Suppl 4):S46–S51. doi: 10.1212/wnl.56.suppl_4.s46. [DOI] [PubMed] [Google Scholar]
  117. Petersen R. C., Smith G. E., Waring S. C., Ivnik R. J., Tangalos E. G., Kokmen E. Mild cognitive impairment: clinical characterization and outcome. Arch Neurol. 1999 Mar;56(3):303–308. doi: 10.1001/archneur.56.3.303. [DOI] [PubMed] [Google Scholar]
  118. Plassman B. L., Breitner J. C. Recent advances in the genetics of Alzheimer's disease and vascular dementia with an emphasis on gene-environment interactions. J Am Geriatr Soc. 1996 Oct;44(10):1242–1250. doi: 10.1111/j.1532-5415.1996.tb01377.x. [DOI] [PubMed] [Google Scholar]
  119. Polymeropoulos M. H., Lavedan C., Leroy E., Ide S. E., Dehejia A., Dutra A., Pike B., Root H., Rubenstein J., Boyer R. Mutation in the alpha-synuclein gene identified in families with Parkinson's disease. Science. 1997 Jun 27;276(5321):2045–2047. doi: 10.1126/science.276.5321.2045. [DOI] [PubMed] [Google Scholar]
  120. Poon M. A., Stuckey S., Storey E. MRI evidence of cerebellar and hippocampal involvement in Creutzfeldt-Jakob disease. Neuroradiology. 2001 Sep;43(9):746–749. doi: 10.1007/s002340100587. [DOI] [PubMed] [Google Scholar]
  121. Prusiner S. B. Genetic and infectious prion diseases. Arch Neurol. 1993 Nov;50(11):1129–1153. doi: 10.1001/archneur.1993.00540110011002. [DOI] [PubMed] [Google Scholar]
  122. Prusiner S. B. Molecular biology of prion diseases. Science. 1991 Jun 14;252(5012):1515–1522. doi: 10.1126/science.1675487. [DOI] [PubMed] [Google Scholar]
  123. Prusiner S. B. Novel proteinaceous infectious particles cause scrapie. Science. 1982 Apr 9;216(4542):136–144. doi: 10.1126/science.6801762. [DOI] [PubMed] [Google Scholar]
  124. Prusiner S. B. Prions. Proc Natl Acad Sci U S A. 1998 Nov 10;95(23):13363–13383. doi: 10.1073/pnas.95.23.13363. [DOI] [PMC free article] [PubMed] [Google Scholar]
  125. Raber J., Wong D., Buttini M., Orth M., Bellosta S., Pitas R. E., Mahley R. W., Mucke L. Isoform-specific effects of human apolipoprotein E on brain function revealed in ApoE knockout mice: increased susceptibility of females. Proc Natl Acad Sci U S A. 1998 Sep 1;95(18):10914–10919. doi: 10.1073/pnas.95.18.10914. [DOI] [PMC free article] [PubMed] [Google Scholar]
  126. Raskind M. A. Evaluation and management of aggressive behavior in the elderly demented patient. J Clin Psychiatry. 1999;60 (Suppl 15):45–49. [PubMed] [Google Scholar]
  127. Rizzu P., Van Swieten J. C., Joosse M., Hasegawa M., Stevens M., Tibben A., Niermeijer M. F., Hillebrand M., Ravid R., Oostra B. A. High prevalence of mutations in the microtubule-associated protein tau in a population study of frontotemporal dementia in the Netherlands. Am J Hum Genet. 1999 Feb;64(2):414–421. doi: 10.1086/302256. [DOI] [PMC free article] [PubMed] [Google Scholar]
  128. Rogers S. L., Farlow M. R., Doody R. S., Mohs R., Friedhoff L. T. A 24-week, double-blind, placebo-controlled trial of donepezil in patients with Alzheimer's disease. Donepezil Study Group. Neurology. 1998 Jan;50(1):136–145. doi: 10.1212/wnl.50.1.136. [DOI] [PubMed] [Google Scholar]
  129. Ropper A. H., Williams R. S. Relationship between plaques, tangles, and dementia in Down syndrome. Neurology. 1980 Jun;30(6):639–644. doi: 10.1212/wnl.30.6.639. [DOI] [PubMed] [Google Scholar]
  130. Rosen H. J., Lengenfelder J., Miller B. Frontotemporal dementia. Neurol Clin. 2000 Nov;18(4):979–992. doi: 10.1016/s0733-8619(05)70235-8. [DOI] [PubMed] [Google Scholar]
  131. Roses A. D. Apolipoprotein E affects the rate of Alzheimer disease expression: beta-amyloid burden is a secondary consequence dependent on APOE genotype and duration of disease. J Neuropathol Exp Neurol. 1994 Sep;53(5):429–437. doi: 10.1097/00005072-199409000-00002. [DOI] [PubMed] [Google Scholar]
  132. Rossor M. N. Differential diagnosis of frontotemporal dementia: Pick's disease. Dement Geriatr Cogn Disord. 1999;10 (Suppl 1):43–45. doi: 10.1159/000051211. [DOI] [PubMed] [Google Scholar]
  133. Sano M., Ernesto C., Thomas R. G., Klauber M. R., Schafer K., Grundman M., Woodbury P., Growdon J., Cotman C. W., Pfeiffer E. A controlled trial of selegiline, alpha-tocopherol, or both as treatment for Alzheimer's disease. The Alzheimer's Disease Cooperative Study. N Engl J Med. 1997 Apr 24;336(17):1216–1222. doi: 10.1056/NEJM199704243361704. [DOI] [PubMed] [Google Scholar]
  134. Saunders A. M., Strittmatter W. J., Schmechel D., George-Hyslop P. H., Pericak-Vance M. A., Joo S. H., Rosi B. L., Gusella J. F., Crapper-MacLachlan D. R., Alberts M. J. Association of apolipoprotein E allele epsilon 4 with late-onset familial and sporadic Alzheimer's disease. Neurology. 1993 Aug;43(8):1467–1472. doi: 10.1212/wnl.43.8.1467. [DOI] [PubMed] [Google Scholar]
  135. Schellenberg G. D. Genetic dissection of Alzheimer disease, a heterogeneous disorder. Proc Natl Acad Sci U S A. 1995 Sep 12;92(19):8552–8559. doi: 10.1073/pnas.92.19.8552. [DOI] [PMC free article] [PubMed] [Google Scholar]
  136. Schenk D., Barbour R., Dunn W., Gordon G., Grajeda H., Guido T., Hu K., Huang J., Johnson-Wood K., Khan K. Immunization with amyloid-beta attenuates Alzheimer-disease-like pathology in the PDAPP mouse. Nature. 1999 Jul 8;400(6740):173–177. doi: 10.1038/22124. [DOI] [PubMed] [Google Scholar]
  137. Scott M. R., Will R., Ironside J., Nguyen H. O., Tremblay P., DeArmond S. J., Prusiner S. B. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A. 1999 Dec 21;96(26):15137–15142. doi: 10.1073/pnas.96.26.15137. [DOI] [PMC free article] [PubMed] [Google Scholar]
  138. Selkoe D. J. Alzheimer's disease: genotypes, phenotypes, and treatments. Science. 1997 Jan 31;275(5300):630–631. doi: 10.1126/science.275.5300.630. [DOI] [PubMed] [Google Scholar]
  139. Selkoe D. J. The molecular pathology of Alzheimer's disease. Neuron. 1991 Apr;6(4):487–498. doi: 10.1016/0896-6273(91)90052-2. [DOI] [PubMed] [Google Scholar]
  140. Shea C., MacKnight C., Rockwood K. Donepezil for treatment of dementia with Lewy bodies: a case series of nine patients. Int Psychogeriatr. 1998 Sep;10(3):229–238. doi: 10.1017/s1041610298005341. [DOI] [PubMed] [Google Scholar]
  141. Sparks D. L., Woeltz V. M., Markesbery W. R. Alterations in brain monoamine oxidase activity in aging, Alzheimer's disease, and Pick's disease. Arch Neurol. 1991 Jul;48(7):718–721. doi: 10.1001/archneur.1991.00530190064017. [DOI] [PubMed] [Google Scholar]
  142. Spillantini M. G., Schmidt M. L., Lee V. M., Trojanowski J. Q., Jakes R., Goedert M. Alpha-synuclein in Lewy bodies. Nature. 1997 Aug 28;388(6645):839–840. doi: 10.1038/42166. [DOI] [PubMed] [Google Scholar]
  143. St George-Hyslop P. H., Tanzi R. E., Polinsky R. J., Haines J. L., Nee L., Watkins P. C., Myers R. H., Feldman R. G., Pollen D., Drachman D. The genetic defect causing familial Alzheimer's disease maps on chromosome 21. Science. 1987 Feb 20;235(4791):885–890. doi: 10.1126/science.2880399. [DOI] [PubMed] [Google Scholar]
  144. Swartz J. R., Miller B. L., Lesser I. M., Darby A. L. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors. J Clin Psychiatry. 1997 May;58(5):212–216. [PubMed] [Google Scholar]
  145. Verity C. M., Nicoll A., Will R. G., Devereux G., Stellitano L. Variant Creutzfeldt-Jakob disease in UK children: a national surveillance study. Lancet. 2000 Oct 7;356(9237):1224–1227. doi: 10.1016/s0140-6736(00)02785-9. [DOI] [PubMed] [Google Scholar]
  146. Vrancken A. F., Frijns C. J., Ramos L. M. FLAIR MRI in sporadic Creutzfeldt-Jakob disease. Neurology. 2000 Jul 12;55(1):147–148. doi: 10.1212/wnl.55.1.147. [DOI] [PubMed] [Google Scholar]
  147. Walker Z., Grace J., Overshot R., Satarasinghe S., Swan A., Katona C. L., McKeith I. G. Olanzapine in dementia with Lewy bodies: a clinical study. Int J Geriatr Psychiatry. 1999 Jun;14(6):459–466. [PubMed] [Google Scholar]
  148. Walsh J. S., Welch H. G., Larson E. B. Survival of outpatients with Alzheimer-type dementia. Ann Intern Med. 1990 Sep 15;113(6):429–434. doi: 10.7326/0003-4819-113-6-429. [DOI] [PubMed] [Google Scholar]
  149. Weiner M. F., Risser R. C., Cullum C. M., Honig L., White C., 3rd, Speciale S., Rosenberg R. N. Alzheimer's disease and its Lewy body variant: a clinical analysis of postmortem verified cases. Am J Psychiatry. 1996 Oct;153(10):1269–1273. doi: 10.1176/ajp.153.10.1269. [DOI] [PubMed] [Google Scholar]
  150. Wilhelmsen K. C., Lynch T., Pavlou E., Higgins M., Nygaard T. G. Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22. Am J Hum Genet. 1994 Dec;55(6):1159–1165. [PMC free article] [PubMed] [Google Scholar]
  151. Will R. G., Alperovitch A., Poser S., Pocchiari M., Hofman A., Mitrova E., de Silva R., D'Alessandro M., Delasnerie-Laupretre N., Zerr I. Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD. Ann Neurol. 1998 Jun;43(6):763–767. doi: 10.1002/ana.410430611. [DOI] [PubMed] [Google Scholar]
  152. Will R. G., Ironside J. W., Zeidler M., Cousens S. N., Estibeiro K., Alperovitch A., Poser S., Pocchiari M., Hofman A., Smith P. G. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996 Apr 6;347(9006):921–925. doi: 10.1016/s0140-6736(96)91412-9. [DOI] [PubMed] [Google Scholar]
  153. Will R. G., Zeidler M., Stewart G. E., Macleod M. A., Ironside J. W., Cousens S. N., Mackenzie J., Estibeiro K., Green A. J., Knight R. S. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol. 2000 May;47(5):575–582. [PubMed] [Google Scholar]
  154. Wolozin B., Behl C. Mechanisms of neurodegenerative disorders: Part 1: protein aggregates. Arch Neurol. 2000 Jun;57(6):793–796. doi: 10.1001/archneur.57.6.793. [DOI] [PubMed] [Google Scholar]
  155. Zeidler M., Collie D. A., Macleod M. A., Sellar R. J., Knight R. FLAIR MRI in sporadic Creutzfeldt-Jakob disease. Neurology. 2001 Jan 23;56(2):282–282. doi: 10.1212/wnl.56.2.282. [DOI] [PubMed] [Google Scholar]
  156. Zeidler M., Sellar R. J., Collie D. A., Knight R., Stewart G., Macleod M. A., Ironside J. W., Cousens S., Colchester A. C., Hadley D. M. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet. 2000 Apr 22;355(9213):1412–1418. doi: 10.1016/s0140-6736(00)02140-1. [DOI] [PubMed] [Google Scholar]
  157. Zhukareva V., Vogelsberg-Ragaglia V., Van Deerlin V. M., Bruce J., Shuck T., Grossman M., Clark C. M., Arnold S. E., Masliah E., Galasko D. Loss of brain tau defines novel sporadic and familial tauopathies with frontotemporal dementia. Ann Neurol. 2001 Feb;49(2):165–175. doi: 10.1002/1531-8249(20010201)49:2<165::aid-ana36>3.0.co;2-3. [DOI] [PubMed] [Google Scholar]
  158. van Slegtenhorst M., Lewis J., Hutton M. The molecular genetics of the tauopathies. Exp Gerontol. 2000 Jul;35(4):461–471. doi: 10.1016/s0531-5565(00)00114-5. [DOI] [PubMed] [Google Scholar]

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