Skip to main content
PMC home page
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 2004 Mar;80(941):125–139. doi: 10.1136/pgmj.2003.011171

Young onset dementia

E Sampson 1, J Warren 1, M Rossor 1
PMCID: PMC1742955  PMID: 15016933

Abstract

Young onset dementia is a challenging clinical problem with potentially devastating medical and social consequences. The differential diagnosis is wide, and includes a number of rare sporadic and hereditary diseases. However, accurate diagnosis is often possible, and all patients should be thoroughly investigated to identify treatable processes. This review presents an approach to the diagnosis, investigation, and management of patients with young onset dementia, with particular reference to common and treatable causes.

Full Text

The Full Text of this article is available as a PDF (419.1 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bak T. H., O'Donovan D. G., Xuereb J. H., Boniface S., Hodges J. R. Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome. Brain. 2001 Jan;124(Pt 1):103–120. doi: 10.1093/brain/124.1.103. [DOI] [PubMed] [Google Scholar]
  2. Bobholz Julie A., Rao Stephen M. Cognitive dysfunction in multiple sclerosis: a review of recent developments. Curr Opin Neurol. 2003 Jun;16(3):283–288. doi: 10.1097/01.wco.0000073928.19076.84. [DOI] [PubMed] [Google Scholar]
  3. Bradshaw C. B., Davis R. L., Shrimpton A. E., Holohan P. D., Rea C. B., Fieglin D., Kent P., Collins G. H. Cognitive deficits associated with a recently reported familial neurodegenerative disease: familial encephalopathy with neuroserpin inclusion bodies. Arch Neurol. 2001 Sep;58(9):1429–1434. doi: 10.1001/archneur.58.9.1429. [DOI] [PubMed] [Google Scholar]
  4. Brown P., Preece M., Brandel J. P., Sato T., McShane L., Zerr I., Fletcher A., Will R. G., Pocchiari M., Cashman N. R. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology. 2000 Oct 24;55(8):1075–1081. doi: 10.1212/wnl.55.8.1075. [DOI] [PubMed] [Google Scholar]
  5. Buttmann M., Marziniak M., Toyka K. V., Sommer C., Altland K. "Sporadic" familial amyloidotic polyneuropathy in a German patient with B cell lymphocytic leukaemia. J Neurol Neurosurg Psychiatry. 2002 Jul;73(1):86–87. doi: 10.1136/jnnp.73.1.86. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Bürk K., Globas C., Bösch S., Klockgether T., Zühlke C., Daum I., Dichgans J. Cognitive deficits in spinocerebellar ataxia type 1, 2, and 3. J Neurol. 2003 Feb;250(2):207–211. doi: 10.1007/s00415-003-0976-5. [DOI] [PubMed] [Google Scholar]
  7. Carrell R. W., Lomas D. A. Conformational disease. Lancet. 1997 Jul 12;350(9071):134–138. doi: 10.1016/S0140-6736(97)02073-4. [DOI] [PubMed] [Google Scholar]
  8. Caviness John N., Evidente Virgilio G. H. Cortical myoclonus during lithium exposure. Arch Neurol. 2003 Mar;60(3):401–404. doi: 10.1001/archneur.60.3.401. [DOI] [PubMed] [Google Scholar]
  9. Cengiz N., Ozbenli T., Onar M., Yildiz L., Ertaş B. Adult metachromatic leukodystrophy: three cases with normal nerve conduction velocities in a family. Acta Neurol Scand. 2002 Jun;105(6):454–457. doi: 10.1034/j.1600-0404.2002.01257.x. [DOI] [PubMed] [Google Scholar]
  10. Chan D., Fox N. C., Scahill R. I., Crum W. R., Whitwell J. L., Leschziner G., Rossor A. M., Stevens J. M., Cipolotti L., Rossor M. N. Patterns of temporal lobe atrophy in semantic dementia and Alzheimer's disease. Ann Neurol. 2001 Apr;49(4):433–442. [PubMed] [Google Scholar]
  11. Chavany C., Jendoubi M. Biology and potential strategies for the treatment of GM2 gangliosidoses. Mol Med Today. 1998 Apr;4(4):158–165. doi: 10.1016/s1357-4310(98)01227-1. [DOI] [PubMed] [Google Scholar]
  12. Chong Ji Y., Rowland Lewis P., Utiger Robert D. Hashimoto encephalopathy: syndrome or myth? Arch Neurol. 2003 Feb;60(2):164–171. doi: 10.1001/archneur.60.2.164. [DOI] [PubMed] [Google Scholar]
  13. Chui H. Vascular dementia, a new beginning: shifting focus from clinical phenotype to ischemic brain injury. Neurol Clin. 2000 Nov;18(4):951–978. doi: 10.1016/s0733-8619(05)70234-6. [DOI] [PubMed] [Google Scholar]
  14. 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]
  15. Collin P., Pirttilä T., Nurmikko T., Somer H., Erilä T., Keyriläinen O. Celiac disease, brain atrophy, and dementia. Neurology. 1991 Mar;41(3):372–375. doi: 10.1212/wnl.41.3.372. [DOI] [PubMed] [Google Scholar]
  16. Collinge J. Prion diseases of humans and animals: their causes and molecular basis. Annu Rev Neurosci. 2001;24:519–550. doi: 10.1146/annurev.neuro.24.1.519. [DOI] [PubMed] [Google Scholar]
  17. Collinge John, Brandner Sebastian, Kennedy Angus, Rossor Martin, Smith Peter, Stevens John, Rudge Peter. A 38-year-old man with a 9 month history of neurological and cognitive impairment. Lancet Neurol. 2003 Mar;2(3):189–194. doi: 10.1016/s1474-4422(03)00325-9. [DOI] [PubMed] [Google Scholar]
  18. Dalton C. M., Brex P. A., Jenkins R., Fox N. C., Miszkiel K. A., Crum W. R., O'Riordan J. I., Plant G. T., Thompson A. J., Miller D. H. Progressive ventricular enlargement in patients with clinically isolated syndromes is associated with the early development of multiple sclerosis. J Neurol Neurosurg Psychiatry. 2002 Aug;73(2):141–147. doi: 10.1136/jnnp.73.2.141. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. DeSousa Eduardo Adonias, Albert Ross H., Kalman Bernadette. Cognitive impairments in multiple sclerosis: a review. Am J Alzheimers Dis Other Demen. 2002 Jan-Feb;17(1):23–29. doi: 10.1177/153331750201700104. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Edwin D., Speedie L., Naidu S., Moser H. Cognitive impairment in adult-onset adrenoleukodystrophy. Mol Chem Neuropathol. 1990 Jun;12(3):167–176. doi: 10.1007/BF03159942. [DOI] [PubMed] [Google Scholar]
  21. Emre Murat. Dementia associated with Parkinson's disease. Lancet Neurol. 2003 Apr;2(4):229–237. doi: 10.1016/s1474-4422(03)00351-x. [DOI] [PubMed] [Google Scholar]
  22. Erkinjuntti Timo, Kurz Alexander, Gauthier Serge, Bullock Roger, Lilienfeld Sean, Damaraju ChandrasekharRao Venkata. Efficacy of galantamine in probable vascular dementia and Alzheimer's disease combined with cerebrovascular disease: a randomised trial. Lancet. 2002 Apr 13;359(9314):1283–1290. doi: 10.1016/S0140-6736(02)08267-3. [DOI] [PubMed] [Google Scholar]
  23. Ernst A., Zibrak J. D. Carbon monoxide poisoning. N Engl J Med. 1998 Nov 26;339(22):1603–1608. doi: 10.1056/NEJM199811263392206. [DOI] [PubMed] [Google Scholar]
  24. Fallon B. A., Nields J. A. Lyme disease: a neuropsychiatric illness. Am J Psychiatry. 1994 Nov;151(11):1571–1583. doi: 10.1176/ajp.151.11.1571. [DOI] [PubMed] [Google Scholar]
  25. Finkelstein J. E., Hauser E. R., Leonard C. O., Brusilow S. W. Late-onset ornithine transcarbamylase deficiency in male patients. J Pediatr. 1990 Dec;117(6):897–902. doi: 10.1016/s0022-3476(05)80129-5. [DOI] [PubMed] [Google Scholar]
  26. Fox N. C., Scahill R. I., Crum W. R., Rossor M. N. Correlation between rates of brain atrophy and cognitive decline in AD. Neurology. 1999 May 12;52(8):1687–1689. doi: 10.1212/wnl.52.8.1687. [DOI] [PubMed] [Google Scholar]
  27. 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]
  28. Garrard P., Hodges J. R. Semantic dementia: clinical, radiological and pathological perspectives. J Neurol. 2000 Jun;247(6):409–422. doi: 10.1007/s004150070169. [DOI] [PubMed] [Google Scholar]
  29. Gultekin S. H., Rosenfeld M. R., Voltz R., Eichen J., Posner J. B., Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000 Jul;123(Pt 7):1481–1494. doi: 10.1093/brain/123.7.1481. [DOI] [PubMed] [Google Scholar]
  30. Gusella J. F., MacDonald M. E., Ambrose C. M., Duyao M. P. Molecular genetics of Huntington's disease. Arch Neurol. 1993 Nov;50(11):1157–1163. doi: 10.1001/archneur.1993.00540110037003. [DOI] [PubMed] [Google Scholar]
  31. Gydesen S., Brown J. M., Brun A., Chakrabarti L., Gade A., Johannsen P., Rossor M., Thusgaard T., Grove A., Yancopoulou D. Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology. 2002 Nov 26;59(10):1585–1594. doi: 10.1212/01.wnl.0000034763.54161.1f. [DOI] [PubMed] [Google Scholar]
  32. Hardy John, Selkoe Dennis J. The amyloid hypothesis of Alzheimer's disease: progress and problems on the road to therapeutics. Science. 2002 Jul 19;297(5580):353–356. doi: 10.1126/science.1072994. [DOI] [PubMed] [Google Scholar]
  33. Harper P. S. The epidemiology of Huntington's disease. Hum Genet. 1992 Jun;89(4):365–376. doi: 10.1007/BF00194305. [DOI] [PubMed] [Google Scholar]
  34. Hayflick Susan J., Westaway Shawn K., Levinson Barbara, Zhou Bing, Johnson Monique A., Ching Katherine H. L., Gitschier Jane. Genetic, clinical, and radiographic delineation of Hallervorden-Spatz syndrome. N Engl J Med. 2003 Jan 2;348(1):33–40. doi: 10.1056/NEJMoa020817. [DOI] [PubMed] [Google Scholar]
  35. 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]
  36. Holland A. J., Oliver C. Down's syndrome and the links with Alzheimer's disease. J Neurol Neurosurg Psychiatry. 1995 Aug;59(2):111–114. doi: 10.1136/jnnp.59.2.111. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. 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]
  38. Høgh P., Smith S. J., Scahill R. I., Chan D., Harvey R. J., Fox N. C., Rossor M. N. Epilepsy presenting as AD: neuroimaging, electroclinical features, and response to treatment. Neurology. 2002 Jan 22;58(2):298–301. doi: 10.1212/wnl.58.2.298. [DOI] [PubMed] [Google Scholar]
  39. Imrie J., Vijayaraghaven S., Whitehouse C., Harris S., Heptinstall L., Church H., Cooper A., Besley G. T. N., Wraith J. E. Niemann-Pick disease type C in adults. J Inherit Metab Dis. 2002 Oct;25(6):491–500. doi: 10.1023/a:1021259403196. [DOI] [PubMed] [Google Scholar]
  40. Janssen J. C., Beck J. A., Campbell T. A., Dickinson A., Fox N. C., Harvey R. J., Houlden H., Rossor M. N., Collinge J. Early onset familial Alzheimer's disease: Mutation frequency in 31 families. Neurology. 2003 Jan 28;60(2):235–239. doi: 10.1212/01.wnl.0000042088.22694.e3. [DOI] [PubMed] [Google Scholar]
  41. Jardim L. B., Giugliani R., Pires R. F., Haussen S., Burin M. G., Rafi M. A., Wenger D. A. Protracted course of Krabbe disease in an adult patient bearing a novel mutation. Arch Neurol. 1999 Aug;56(8):1014–1017. doi: 10.1001/archneur.56.8.1014. [DOI] [PubMed] [Google Scholar]
  42. Jordan B. D. Chronic traumatic brain injury associated with boxing. Semin Neurol. 2000;20(2):179–185. doi: 10.1055/s-2000-9826. [DOI] [PubMed] [Google Scholar]
  43. Josephs Keith A., Holton Janice L., Rossor Martin N., Braendgaard Hans, Ozawa Tetsutaro, Fox Nick C., Petersen Ronald C., Pearl Gary S., Ganguly Milan, Rosa Pedro. Neurofilament inclusion body disease: a new proteinopathy? Brain. 2003 Jul 22;126(Pt 10):2291–2303. doi: 10.1093/brain/awg231. [DOI] [PubMed] [Google Scholar]
  44. Joutel A., Favrole P., Labauge P., Chabriat H., Lescoat C., Andreux F., Domenga V., Cécillon M., Vahedi K., Ducros A. Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis. Lancet. 2001 Dec 15;358(9298):2049–2051. doi: 10.1016/S0140-6736(01)07142-2. [DOI] [PubMed] [Google Scholar]
  45. Kennedy A. M., Newman S. K., Frackowiak R. S., Cunningham V. J., Roques P., Stevens J., Neary D., Bruton C. J., Warrington E. K., Rossor M. N. Chromosome 14 linked familial Alzheimer's disease. A clinico-pathological study of a single pedigree. Brain. 1995 Feb;118(Pt 1):185–205. doi: 10.1093/brain/118.1.185. [DOI] [PubMed] [Google Scholar]
  46. Klein C. M., Bosch E. P., Dyck P. J. Probable adult polyglucosan body disease. Mayo Clin Proc. 2000 Dec;75(12):1327–1331. doi: 10.4065/75.12.1327. [DOI] [PubMed] [Google Scholar]
  47. Knopman D. S., Mastri A. R., Frey W. H., 2nd, Sung J. H., Rustan T. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology. 1990 Feb;40(2):251–256. doi: 10.1212/wnl.40.2.251. [DOI] [PubMed] [Google Scholar]
  48. Koeppen Arnulf H., Robitaille Yves. Pelizaeus-Merzbacher disease. J Neuropathol Exp Neurol. 2002 Sep;61(9):747–759. doi: 10.1093/jnen/61.9.747. [DOI] [PubMed] [Google Scholar]
  49. Kovach M. J., Waggoner B., Leal S. M., Gelber D., Khardori R., Levenstien M. A., Shanks C. A., Gregg G., Al-Lozi M. T., Miller T. Clinical delineation and localization to chromosome 9p13.3-p12 of a unique dominant disorder in four families: hereditary inclusion body myopathy, Paget disease of bone, and frontotemporal dementia. Mol Genet Metab. 2001 Dec;74(4):458–475. doi: 10.1006/mgme.2001.3256. [DOI] [PMC free article] [PubMed] [Google Scholar]
  50. Krupp L. B., Lipton R. B., Swerdlow M. L., Leeds N. E., Llena J. Progressive multifocal leukoencephalopathy: clinical and radiographic features. Ann Neurol. 1985 Apr;17(4):344–349. doi: 10.1002/ana.410170407. [DOI] [PubMed] [Google Scholar]
  51. Lippa C. F., Swearer J. M., Kane K. J., Nochlin D., Bird T. D., Ghetti B., Nee L. E., St George-Hyslop P., Pollen D. A., Drachman D. A. Familial Alzheimer's disease: site of mutation influences clinical phenotype. Ann Neurol. 2000 Sep;48(3):376–379. [PubMed] [Google Scholar]
  52. Loscalzo Joseph. Homocysteine and dementias. N Engl J Med. 2002 Feb 14;346(7):466–468. doi: 10.1056/NEJM200202143460702. [DOI] [PubMed] [Google Scholar]
  53. Lovestone S., McLoughlin D. M. Protein aggregates and dementia: is there a common toxicity? J Neurol Neurosurg Psychiatry. 2002 Feb;72(2):152–161. doi: 10.1136/jnnp.72.2.152. [DOI] [PMC free article] [PubMed] [Google Scholar]
  54. Mann D. M., Iwatsubo T., Nochlin D., Sumi S. M., Levy-Lahad E., Bird T. D. Amyloid (Abeta) deposition in chromosome 1-linked Alzheimer's disease: the Volga German families. Ann Neurol. 1997 Jan;41(1):52–57. doi: 10.1002/ana.410410110. [DOI] [PubMed] [Google Scholar]
  55. Manzel K., Tranel D., Cooper G. Cognitive and behavioral abnormalities in a case of central nervous system Whipple disease. Arch Neurol. 2000 Mar;57(3):399–403. doi: 10.1001/archneur.57.3.399. [DOI] [PubMed] [Google Scholar]
  56. Markus H. S., Martin R. J., Simpson M. A., Dong Y. B., Ali N., Crosby A. H., Powell J. F. Diagnostic strategies in CADASIL. Neurology. 2002 Oct 22;59(8):1134–1138. doi: 10.1212/wnl.59.8.1134. [DOI] [PubMed] [Google Scholar]
  57. Mawrin C., Lins H., Koenig B., Heinrichs T., Murayama S., Kirches E., Boltze C., Dietzmann K. Spatial and temporal disease progression of adult-onset subacute sclerosing panencephalitis. Neurology. 2002 May 28;58(10):1568–1571. doi: 10.1212/wnl.58.10.1568. [DOI] [PubMed] [Google Scholar]
  58. 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]
  59. McKeith Ian G. Dementia with Lewy bodies. Br J Psychiatry. 2002 Feb;180:144–147. doi: 10.1192/bjp.180.2.144. [DOI] [PubMed] [Google Scholar]
  60. Mead S., James-Galton M., Revesz T., Doshi R. B., Harwood G., Pan E. L., Ghiso J., Frangione B., Plant G. Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings. Brain. 2000 May;123(Pt 5):975–991. doi: 10.1093/brain/123.5.975. [DOI] [PubMed] [Google Scholar]
  61. Mendez M. F., Stanley T. M., Medel N. M., Li Z., Tedesco D. T. The vascular dementia of Fabry's disease. Dement Geriatr Cogn Disord. 1997 Jul-Aug;8(4):252–257. doi: 10.1159/000106640. [DOI] [PubMed] [Google Scholar]
  62. Miller B. L., Darby A., Benson D. F., Cummings J. L., Miller M. H. Aggressive, socially disruptive and antisocial behaviour associated with fronto-temporal dementia. Br J Psychiatry. 1997 Feb;170:150–154. doi: 10.1192/bjp.170.2.150. [DOI] [PubMed] [Google Scholar]
  63. Minassian Berge A. Progressive myoclonus epilepsy with polyglucosan bodies: Lafora disease. Adv Neurol. 2002;89:199–210. [PubMed] [Google Scholar]
  64. Moulignier Antoine, Allo Stephanie, Zittoun Robert, Gout Oliver. Recombinant interferon-alpha-induced chorea and frontal subcortical dementia. Neurology. 2002 Jan 22;58(2):328–330. doi: 10.1212/wnl.58.2.328. [DOI] [PubMed] [Google Scholar]
  65. 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]
  66. Nijssen Peter C. G., Brusse Esther, Leyten Antonius C. M., Martin J. J., Teepen Johannes L. J. M., Roos Raymund A. C. Autosomal dominant adult neuronal ceroid lipofuscinosis: parkinsonism due to both striatal and nigral dysfunction. Mov Disord. 2002 May;17(3):482–487. doi: 10.1002/mds.10104. [DOI] [PubMed] [Google Scholar]
  67. O'Riordan S., McMonagle P., Janssen J. C., Fox N. C., Farrell M., Collinge J., Rossor M. N., Hutchinson M. Presenilin-1 mutation (E280G), spastic paraparesis, and cranial MRI white-matter abnormalities. Neurology. 2002 Oct 8;59(7):1108–1110. doi: 10.1212/wnl.59.7.1108. [DOI] [PubMed] [Google Scholar]
  68. Oktem-Tanör O., Baykan-Kurt B., Gürvit I. H., Akman-Demir G., Serdaroğlu P. Neuropsychological follow-up of 12 patients with neuro-Behçet disease. J Neurol. 1999 Feb;246(2):113–119. doi: 10.1007/s004150050317. [DOI] [PubMed] [Google Scholar]
  69. Palmeri S., Villanova M., Malandrini A., van Diggelen O. P., Huijmans J. G., Ceuterick C., Rufa A., DeFalco D., Ciacci G., Martin J. J. Type I sialidosis: a clinical, biochemical and neuroradiological study. Eur Neurol. 2000;43(2):88–94. doi: 10.1159/000008141. [DOI] [PubMed] [Google Scholar]
  70. Paloneva J., Autti T., Raininko R., Partanen J., Salonen O., Puranen M., Hakola P., Haltia M. CNS manifestations of Nasu-Hakola disease: a frontal dementia with bone cysts. Neurology. 2001 Jun 12;56(11):1552–1558. doi: 10.1212/wnl.56.11.1552. [DOI] [PubMed] [Google Scholar]
  71. Pasquier F., Lebert F., Lavenu I., Guillaume B. The clinical picture of frontotemporal dementia: diagnosis and follow-up. Dement Geriatr Cogn Disord. 1999;10 (Suppl 1):10–14. doi: 10.1159/000051206. [DOI] [PubMed] [Google Scholar]
  72. Paulsen J. S., Ready R. E., Hamilton J. M., Mega M. S., Cummings J. L. Neuropsychiatric aspects of Huntington's disease. J Neurol Neurosurg Psychiatry. 2001 Sep;71(3):310–314. doi: 10.1136/jnnp.71.3.310. [DOI] [PMC free article] [PubMed] [Google Scholar]
  73. Pfeil S. A., Lynn D. J. Wilson's disease: copper unfettered. J Clin Gastroenterol. 1999 Jul;29(1):22–31. doi: 10.1097/00004836-199907000-00007. [DOI] [PubMed] [Google Scholar]
  74. Pijnenburg Y. A. L., Sampson E. L., Harvey R. J., Fox N. C., Rossor M. N. Vulnerability to neuroleptic side effects in frontotemporal lobar degeneration. Int J Geriatr Psychiatry. 2003 Jan;18(1):67–72. doi: 10.1002/gps.774. [DOI] [PubMed] [Google Scholar]
  75. Queralt R., Ezquerra M., Lleó A., Castellví M., Gelpí J., Ferrer I., Acarín N., Pasarín L., Blesa R., Oliva R. 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. 2002 Feb;72(2):266–269. doi: 10.1136/jnnp.72.2.266. [DOI] [PMC free article] [PubMed] [Google Scholar]
  76. Rampoldi Luca, Danek Adrian, Monaco Anthony P. Clinical features and molecular bases of neuroacanthocytosis. J Mol Med (Berl) 2002 Jun 18;80(8):475–491. doi: 10.1007/s00109-002-0349-z. [DOI] [PubMed] [Google Scholar]
  77. Ratnavalli E., Brayne C., Dawson K., Hodges J. R. The prevalence of frontotemporal dementia. Neurology. 2002 Jun 11;58(11):1615–1621. doi: 10.1212/wnl.58.11.1615. [DOI] [PubMed] [Google Scholar]
  78. Revesz Tamas, Holton Janice L., Lashley Tammaryn, Plant Gordon, Rostagno Agueda, Ghiso Jorge, Frangione Blas. Sporadic and familial cerebral amyloid angiopathies. Brain Pathol. 2002 Jul;12(3):343–357. doi: 10.1111/j.1750-3639.2002.tb00449.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  79. Roos K. L. Mycobacterium tuberculosis meningitis and other etiologies of the aseptic meningitis syndrome. Semin Neurol. 2000;20(3):329–335. doi: 10.1055/s-2000-9428. [DOI] [PubMed] [Google Scholar]
  80. Rosen Howard J., Perry Richard J., Murphy Jennifer, Kramer Joel H., Mychack Paula, Schuff Norbert, Weiner Michael, Levenson Robert W., Miller Bruce L. Emotion comprehension in the temporal variant of frontotemporal dementia. Brain. 2002 Oct;125(Pt 10):2286–2295. doi: 10.1093/brain/awf225. [DOI] [PubMed] [Google Scholar]
  81. Rosso S. M., Kamphorst W., de Graaf B., Willemsen R., Ravid R., Niermeijer M. F., Spillantini M. G., Heutink P., van Swieten J. C. Familial frontotemporal dementia with ubiquitin-positive inclusions is linked to chromosome 17q21-22. Brain. 2001 Oct;124(Pt 10):1948–1957. doi: 10.1093/brain/124.10.1948. [DOI] [PubMed] [Google Scholar]
  82. Rosso Sonia M., van Swieten John C. New developments in frontotemporal dementia and parkinsonism linked to chromosome 17. Curr Opin Neurol. 2002 Aug;15(4):423–428. doi: 10.1097/00019052-200208000-00004. [DOI] [PubMed] [Google Scholar]
  83. Rossor M. N., Revesz T., Lantos P. L., Warrington E. K. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. Brain. 2000 Feb;123(Pt 2):267–276. doi: 10.1093/brain/123.2.267. [DOI] [PubMed] [Google Scholar]
  84. Sacktor N. C., Skolasky R. L., Lyles R. H., Esposito D., Selnes O. A., McArthur J. C. Improvement in HIV-associated motor slowing after antiretroviral therapy including protease inhibitors. J Neurovirol. 2000 Feb;6(1):84–88. doi: 10.3109/13550280009006385. [DOI] [PubMed] [Google Scholar]
  85. Schapira A. H. V. Primary and secondary defects of the mitochondrial respiratory chain. J Inherit Metab Dis. 2002 May;25(3):207–214. doi: 10.1023/a:1015629912477. [DOI] [PubMed] [Google Scholar]
  86. Schielke E., Nolte C., Müller W., Brück W. Sarcoidosis presenting as rapidly progressive dementia: clinical and neuropathological evaluation. J Neurol. 2001 Jun;248(6):522–524. doi: 10.1007/s004150170164. [DOI] [PubMed] [Google Scholar]
  87. Schott J. M., Crutch S. J., Fox N. C., Warrington E. K. Development of selective verbal memory impairment secondary to a left thalamic infarct: a longitudinal case study. J Neurol Neurosurg Psychiatry. 2003 Feb;74(2):255–257. doi: 10.1136/jnnp.74.2.255. [DOI] [PMC free article] [PubMed] [Google Scholar]
  88. Schott J. M., Harkness K., Barnes J., della Rocchetta A. Incisa, Vincent A., Rossor M. N. Amnesia, cerebral atrophy, and autoimmunity. Lancet. 2003 Apr 12;361(9365):1266–1266. doi: 10.1016/s0140-6736(03)12983-2. [DOI] [PubMed] [Google Scholar]
  89. Schott Jonathan M., Simon Jessica E., Fox Nick C., King Andrew P., Khan M. Nadeem, Cipolotti Lisa, Paviour Dominic C., Stevens John M., Rossor Martin N. Delineating the sites and progression of in vivo atrophy in multiple system atrophy using fluid-registered MRI. Mov Disord. 2003 Aug;18(8):955–958. doi: 10.1002/mds.10468. [DOI] [PubMed] [Google Scholar]
  90. Scolding N. J., Jayne D. R., Zajicek J. P., Meyer P. A., Wraight E. P., Lockwood C. M. Cerebral vasculitis--recognition, diagnosis and management. QJM. 1997 Jan;90(1):61–73. doi: 10.1093/qjmed/90.1.61. [DOI] [PubMed] [Google Scholar]
  91. Soliveri P., Monza D., Paridi D., Radice D., Grisoli M., Testa D., Savoiardo M., Girotti F. Cognitive and magnetic resonance imaging aspects of corticobasal degeneration and progressive supranuclear palsy. Neurology. 1999 Aug 11;53(3):502–507. doi: 10.1212/wnl.53.3.502. [DOI] [PubMed] [Google Scholar]
  92. Steiner M. C., Ward M. J., Ali N. J. Dementia and snoring. Lancet. 1999 Jan 16;353(9148):204–204. doi: 10.1016/s0140-6736(98)08303-2. [DOI] [PubMed] [Google Scholar]
  93. Takahashi H., Ohama E., Naito H., Takeda S., Nakashima S., Makifuchi T., Ikuta F. Hereditary dentatorubral-pallidoluysian atrophy: clinical and pathologic variants in a family. Neurology. 1988 Jul;38(7):1065–1070. doi: 10.1212/wnl.38.7.1065. [DOI] [PubMed] [Google Scholar]
  94. Talerico K. A., Evans L. K. Responding to safety issues in frontotemporal dementias. Neurology. 2001 Jun;56(11 Suppl 4):S52–S55. doi: 10.1212/wnl.56.suppl_4.s52. [DOI] [PubMed] [Google Scholar]
  95. Tuck R. R., Brew B. J., Britton A. M., Loewy J. Alcohol and brain damage. Br J Addict. 1984 Sep;79(3):251–259. doi: 10.1111/j.1360-0443.1984.tb00273.x. [DOI] [PubMed] [Google Scholar]
  96. Turner R. S., Kenyon L. C., Trojanowski J. Q., Gonatas N., Grossman M. Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia. Ann Neurol. 1996 Feb;39(2):166–173. doi: 10.1002/ana.410390205. [DOI] [PubMed] [Google Scholar]
  97. Tüzün E., Baykan B., Gürses C., Gökyigit A. Longterm follow-up of electroencephalographic and clinical findings of a case with Gaucher's disease type 3a. Seizure. 2000 Oct;9(7):469–472. doi: 10.1053/seiz.2000.0426. [DOI] [PubMed] [Google Scholar]
  98. Verrips A., Hoefsloot L. H., Steenbergen G. C., Theelen J. P., Wevers R. A., Gabreëls F. J., van Engelen B. G., van den Heuvel L. P. Clinical and molecular genetic characteristics of patients with cerebrotendinous xanthomatosis. Brain. 2000 May;123(Pt 5):908–919. doi: 10.1093/brain/123.5.908. [DOI] [PubMed] [Google Scholar]
  99. Victor M. Alcoholic dementia. Can J Neurol Sci. 1994 May;21(2):88–99. doi: 10.1017/s031716710004899x. [DOI] [PubMed] [Google Scholar]
  100. Vigliani M. C., Duyckaerts C., Hauw J. J., Poisson M., Magdelenat H., Delattre J. Y. Dementia following treatment of brain tumors with radiotherapy administered alone or in combination with nitrosourea-based chemotherapy: a clinical and pathological study. J Neurooncol. 1999 Jan;41(2):137–149. doi: 10.1023/a:1006183730847. [DOI] [PubMed] [Google Scholar]
  101. Visvanathan R. Is it truly dementia? Lancet. 2001 Mar 3;357(9257):684–684. doi: 10.1016/S0140-6736(00)04133-7. [DOI] [PubMed] [Google Scholar]
  102. Wenger David A., Coppola Stephanie, Liu Shu-Ling. Insights into the diagnosis and treatment of lysosomal storage diseases. Arch Neurol. 2003 Mar;60(3):322–328. doi: 10.1001/archneur.60.3.322. [DOI] [PubMed] [Google Scholar]
  103. Wesselingh S. L., Thompson K. A. Immunopathogenesis of HIV-associated dementia. Curr Opin Neurol. 2001 Jun;14(3):375–379. doi: 10.1097/00019052-200106000-00018. [DOI] [PubMed] [Google Scholar]
  104. Wilcock Gordon K. Memantine for the treatment of dementia. Lancet Neurol. 2003 Aug;2(8):503–505. doi: 10.1016/s1474-4422(03)00486-1. [DOI] [PubMed] [Google Scholar]
  105. 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]
  106. Wolozin B., Kellman W., Ruosseau P., Celesia G. G., Siegel G. Decreased prevalence of Alzheimer disease associated with 3-hydroxy-3-methyglutaryl coenzyme A reductase inhibitors. Arch Neurol. 2000 Oct;57(10):1439–1443. doi: 10.1001/archneur.57.10.1439. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES