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Disease relevance of Dementia

  • Hereditary dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurologic disorder characterized by variable combinations of myoclonus, epilepsy, cerebellar ataxia, choreoathetosis and dementia [1].
  • The outpatients refused life-sustaining treatments in 71 percent of their responses to options in the four scenarios (coma with chance of recovery, 57 percent; persistent vegetative state, 85 percent; dementia, 79 percent; and dementia with a terminal illness, 87 percent), with small differences between widely differing types of treatments [2].
  • Familial British dementia (FBD), previously designated familial cerebral amyloid angiopathy-British type, is an autosomal dominant disorder of undetermined origin characterized by progressive dementia, spasticity, and cerebellar ataxia, with onset at around the fifth decade of life [3].
  • Clinical studies over the past several years have correlated the use of anti-inflammatory drugs with a decrease in the incidence and progression of AD dementia and/or dysfunction, supporting a role for gliosis and inflammation in AD pathogenesis (reviewed in ref. 6). C5a, a product of C' activation, is chemotactic for microglia [4].
  • HIV-infected subjects with the E4 allele for APOE have excess dementia and peripheral neuropathy [5].

Psychiatry related information on Dementia


High impact information on Dementia


Chemical compound and disease context of Dementia


Biological context of Dementia


Anatomical context of Dementia


Gene context of Dementia

  • The most characteristic change in progressive dementia of Alzheimer's type is a tissue deposit of amyloid beta/A4 protein, which is derived from its precursor protein APP (ref.2) [31].
  • Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia [32].
  • The Notch family of transmembrane receptor proteins mediate developmental cell-fate decisions, and mutations in mammalian Notch genes have been implicated in leukaemia, breast cancer, stroke and dementia [33].
  • Furin mediates enhanced production of fibrillogenic ABri peptides in familial British dementia [34].
  • Means (SD) for CD14/CD16 in HIV-negative controls and in AIDS non-dementia and AIDS dementia patients were 6.5% (4), 16% (13), and 37% (21), respectively (p = 0.008 between the two groups of patients) [35].
  • Compared with men in the lowest quartile, men in the highest quartile of ApoA-I concentration had a significantly lower risk of dementia (hazard ratio = 0.25, 95% confidence interval: 0.08, 0.78) [36].

Analytical, diagnostic and therapeutic context of Dementia


  1. Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Koide, R., Ikeuchi, T., Onodera, O., Tanaka, H., Igarashi, S., Endo, K., Takahashi, H., Kondo, R., Ishikawa, A., Hayashi, T. Nat. Genet. (1994) [Pubmed]
  2. Advance directives for medical care--a case for greater use. Emanuel, L.L., Barry, M.J., Stoeckle, J.D., Ettelson, L.M., Emanuel, E.J. N. Engl. J. Med. (1991) [Pubmed]
  3. A stop-codon mutation in the BRI gene associated with familial British dementia. Vidal, R., Frangione, B., Rostagno, A., Mead, S., Révész, T., Plant, G., Ghiso, J. Nature (1999) [Pubmed]
  4. Aspartate residue 7 in amyloid beta-protein is critical for classical complement pathway activation: implications for Alzheimer's disease pathogenesis. Velazquez, P., Cribbs, D.H., Poulos, T.L., Tenner, A.J. Nat. Med. (1997) [Pubmed]
  5. HIV-infected subjects with the E4 allele for APOE have excess dementia and peripheral neuropathy. Corder, E.H., Robertson, K., Lannfelt, L., Bogdanovic, N., Eggertsen, G., Wilkins, J., Hall, C. Nat. Med. (1998) [Pubmed]
  6. Serpents on the road to dementia and death. Accumulating evidence from several studies points to the normal function of presenilin 1 and suggests how the mutant protein contributes to deposition of amyloid plaques in Alzheimer's disease. Beyreuther, K., Masters, C.L. Nat. Med. (1997) [Pubmed]
  7. Apolipoprotein E epsilon4 and the risk of dementia with stroke. A population-based investigation. Slooter, A.J., Tang, M.X., van Duijn, C.M., Stern, Y., Ott, A., Bell, K., Breteler, M.M., Van Broeckhoven, C., Tatemichi, T.K., Tycko, B., Hofman, A., Mayeux, R. JAMA (1997) [Pubmed]
  8. NIH Conference. Brain imaging: aging and dementia. Cutler, N.R., Duara, R., Creasey, H., Grady, C.L., Haxby, J.V., Schapiro, M.B., Rapoport, S.I. Ann. Intern. Med. (1984) [Pubmed]
  9. FGF-2 promotes neurogenesis and neuroprotection and prolongs survival in a transgenic mouse model of Huntington's disease. Jin, K., LaFevre-Bernt, M., Sun, Y., Chen, S., Gafni, J., Crippen, D., Logvinova, A., Ross, C.A., Greenberg, D.A., Ellerby, L.M. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  10. Prenatal diagnosis of mitochondrial DNA8993 T----G disease. Harding, A.E., Holt, I.J., Sweeney, M.G., Brockington, M., Davis, M.B. Am. J. Hum. Genet. (1992) [Pubmed]
  11. Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Skibinski, G., Parkinson, N.J., Brown, J.M., Chakrabarti, L., Lloyd, S.L., Hummerich, H., Nielsen, J.E., Hodges, J.R., Spillantini, M.G., Thusgaard, T., Brandner, S., Brun, A., Rossor, M.N., Gade, A., Johannsen, P., Sørensen, S.A., Gydesen, S., Fisher, E.M., Collinge, J. Nat. Genet. (2005) [Pubmed]
  12. A CBP binding transcriptional repressor produced by the PS1/epsilon-cleavage of N-cadherin is inhibited by PS1 FAD mutations. Marambaud, P., Wen, P.H., Dutt, A., Shioi, J., Takashima, A., Siman, R., Robakis, N.K. Cell (2003) [Pubmed]
  13. Apolipoprotein E and the neuropathology of dementia. Itoh, Y., Yamada, M. N. Engl. J. Med. (1996) [Pubmed]
  14. Nitric oxide and AIDS dementia. Fuchs, D., Baier-Bitterlich, G., Wachter, H. N. Engl. J. Med. (1995) [Pubmed]
  15. More on aluminum, pteridine, and dialysis dementia. Dhondt, J.L. N. Engl. J. Med. (1988) [Pubmed]
  16. Red-cell/plasma choline ratio in dementia. Barclay, L.L., Blass, J.P., Kopp, U., Hanin, I. N. Engl. J. Med. (1982) [Pubmed]
  17. Long-term treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid. Berginer, V.M., Salen, G., Shefer, S. N. Engl. J. Med. (1984) [Pubmed]
  18. Dementia induced by methyldopa with haloperidol. Thornton, W.E. N. Engl. J. Med. (1976) [Pubmed]
  19. Age-impaired impulse flow from nucleus basalis to cortex. Aston-Jones, G., Rogers, J., Shaver, R.D., Dinan, T.G., Moss, D.E. Nature (1985) [Pubmed]
  20. Oxidative damage linked to neurodegeneration by selective alpha-synuclein nitration in synucleinopathy lesions. Giasson, B.I., Duda, J.E., Murray, I.V., Chen, Q., Souza, J.M., Hurtig, H.I., Ischiropoulos, H., Trojanowski, J.Q., Lee, V.M. Science (2000) [Pubmed]
  21. Plasma cortisol and progression of dementia in subjects with Alzheimer-type dementia. Csernansky, J.G., Dong, H., Fagan, A.M., Wang, L., Xiong, C., Holtzman, D.M., Morris, J.C. Am. J. Psychiatry (2006) [Pubmed]
  22. Apolipoprotein E, dementia, and cortical deposition of beta-amyloid protein. Polvikoski, T., Sulkava, R., Haltia, M., Kainulainen, K., Vuorio, A., Verkkoniemi, A., Niinistö, L., Halonen, P., Kontula, K. N. Engl. J. Med. (1995) [Pubmed]
  23. Human Genetics. Notch, stroke and dementia. Gridley, T. Nature (1996) [Pubmed]
  24. Reproductive period and risk of dementia in postmenopausal women. Geerlings, M.I., Ruitenberg, A., Witteman, J.C., van Swieten, J.C., Hofman, A., van Duijn, C.M., Breteler, M.M., Launer, L.J. JAMA (2001) [Pubmed]
  25. Apolipoprotein E epsilon4 associated with chronic traumatic brain injury in boxing. Jordan, B.D., Relkin, N.R., Ravdin, L.D., Jacobs, A.R., Bennett, A., Gandy, S. JAMA (1997) [Pubmed]
  26. Elevated cerebrospinal fluid glutamate in patients with HIV-related dementia. Gurwitz, D., Kloog, Y. JAMA (1997) [Pubmed]
  27. Increased neocortical neurofibrillary tangle density in subjects with Alzheimer disease and psychosis. Farber, N.B., Rubin, E.H., Newcomer, J.W., Kinscherf, D.A., Miller, J.P., Morris, J.C., Olney, J.W., McKeel, D.W. Arch. Gen. Psychiatry (2000) [Pubmed]
  28. Assignment of the locus for PLO-SL, a frontal-lobe dementia with bone cysts, to 19q13. Pekkarinen, P., Hovatta, I., Hakola, P., Järvi, O., Kestilä, M., Lenkkeri, U., Adolfsson, R., Holmgren, G., Nylander, P.O., Tranebjaerg, L., Terwilliger, J.D., Lönnqvist, J., Peltonen, L. Am. J. Hum. Genet. (1998) [Pubmed]
  29. Induction of monocyte chemoattractant protein-1 in HIV-1 Tat-stimulated astrocytes and elevation in AIDS dementia. Conant, K., Garzino-Demo, A., Nath, A., McArthur, J.C., Halliday, W., Power, C., Gallo, R.C., Major, E.O. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  30. The gene for progressive myoclonus epilepsy of the Lafora type maps to chromosome 6q. Serratosa, J.M., Delgado-Escueta, A.V., Posada, I., Shih, S., Drury, I., Berciano, J., Zabala, J.A., Antúnez, M.C., Sparkes, R.S. Hum. Mol. Genet. (1995) [Pubmed]
  31. Alzheimer amyloid protein precursor complexes with brain GTP-binding protein G(o). Nishimoto, I., Okamoto, T., Matsuura, Y., Takahashi, S., Okamoto, T., Murayama, Y., Ogata, E. Nature (1993) [Pubmed]
  32. Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia. Joutel, A., Corpechot, C., Ducros, A., Vahedi, K., Chabriat, H., Mouton, P., Alamowitch, S., Domenga, V., Cécillion, M., Marechal, E., Maciazek, J., Vayssiere, C., Cruaud, C., Cabanis, E.A., Ruchoux, M.M., Weissenbach, J., Bach, J.F., Bousser, M.G., Tournier-Lasserve, E. Nature (1996) [Pubmed]
  33. Fringe modulates Notch-ligand interactions. Panin, V.M., Papayannopoulos, V., Wilson, R., Irvine, K.D. Nature (1997) [Pubmed]
  34. Furin mediates enhanced production of fibrillogenic ABri peptides in familial British dementia. Kim, S.H., Wang, R., Gordon, D.J., Bass, J., Steiner, D.F., Lynn, D.G., Thinakaran, G., Meredith, S.C., Sisodia, S.S. Nat. Neurosci. (1999) [Pubmed]
  35. Unique monocyte subset in patients with AIDS dementia. Pulliam, L., Gascon, R., Stubblebine, M., McGuire, D., McGrath, M.S. Lancet (1997) [Pubmed]
  36. The relation between apolipoprotein A-I and dementia: the Honolulu-Asia aging study. Saczynski, J.S., White, L., Peila, R.L., Rodriguez, B.L., Launer, L.J. Am. J. Epidemiol. (2007) [Pubmed]
  37. Single-photon emission computed tomography (SPECT). Applications and potential. Holman, B.L., Tumeh, S.S. JAMA (1990) [Pubmed]
  38. Estrogen therapy in postmenopausal women: effects on cognitive function and dementia. Yaffe, K., Sawaya, G., Lieberburg, I., Grady, D. JAMA (1998) [Pubmed]
  39. Suloctidil-induced hepatotoxicity. Chung, M.W., Komorowski, R.A., Varma, R.R. Gastroenterology (1988) [Pubmed]
  40. Plasmapheresis, aluminium, and dialysis dementia. Elliott, H.L., Macdougall, A.I., Fell, G.S., Gardiner, P.H. Lancet (1978) [Pubmed]
  41. Temporal and hippocampal metabolic rate during an olfactory memory task assessed by positron emission tomography in patients with dementia of the Alzheimer type and controls. Preliminary studies. Buchsbaum, M.S., Kesslak, J.P., Lynch, G., Chui, H., Wu, J., Sicotte, N., Hazlett, E., Teng, E., Cotman, C.W. Arch. Gen. Psychiatry (1991) [Pubmed]
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