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Diagnosis (2): Dementia Disorders

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Abstract

This chapter examines the various cognitive disorders (e.g. Alzheimer’s disease, frontotemporal lobar degenerations, parkinsonian disorders) which may be defined by clinical assessment and investigation, emphasizing their clinical heterogeneity.

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References

  • Abernethy Holland AJ, Larner AJ. Central nervous system/brain tumour 2-week referral guidelines: prospective 3-year audit. Clin Oncol. 2008;20:201–2.

    CAS  Google Scholar 

  • Adab N, Larner AJ. Adult-onset seizure disorder in 18q deletion syndrome. J Neurol. 2006;253:527–8.

    PubMed  CAS  Google Scholar 

  • Aisen PS, Andrieu S, Sampaio C, et al. Report of the task force on designing clinical trials in early (predementia) AD. Neurology. 2011;76: 280–6.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Aji BM, Medley G, O’Driscoll K, Larner AJ, Alusi SH. Perry syndrome: a disorder to consider in the differential diagnosis of parkinsonism. J Neurol Sci. 2013a;330:117–8.

    PubMed  CAS  Google Scholar 

  • Aji BM, Fratalia L, Alusi SH, Larner AJ. Perry syndrome: more common than previously thought and associated with early cognitive impairment. Abstract Book. Integration by Translation. XX World Congress on Parkinson’s Disease and Related Disorders, Geneva, 8–11 Dec; 2013b. p. 106–7 (abstract 393).

    Google Scholar 

  • Ala T, Hughes LF, Kyrouac GA, Ghobrial MW, Elble RJ. The Mini-Mental State exam may help in the differentiation of dementia with Lewy bodies and Alzheimer’s disease. Int J Geriatr Psychiatry. 2002;17:503–9.

    PubMed  Google Scholar 

  • Albert MS, DeKosky ST, Dickson D, et al. The diagnosis of mild cognitive impairment 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. 2011;7:270–9.

    PubMed Central  PubMed  Google Scholar 

  • Ali R, Barborie A, Larner AJ, White RP. Psychiatric presentation of sporadic Creutzfeldt-Jakob disease: a challenge to current diagnostic criteria. J Neuropsychiatry Clin Neurosci. 2013;25:335–8.

    PubMed  Google Scholar 

  • Amato MP, Langdon D, Montalban X, et al. Treatment of cognitive impairment in multiple sclerosis: position paper. J Neurol. 2013;260:1452–68.

    PubMed  CAS  Google Scholar 

  • American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 4th edition, text revision (DSM-IV-TR). Washington: American Psychiatric Association; 2000.

    Google Scholar 

  • Baborie A, Griffiths TD, Jaros E, Momeni P, McKeith IG, Burn DJ, Keir G, Larner AJ, Mann DM, Perry R. Frontotemporal dementia in elderly individuals. Arch Neurol. 2012;69:1052–60.

    PubMed  Google Scholar 

  • Baborie A, Griffiths TD, Jaros E, Momeni P, McKeith IG, Burn DJ, Keir G, Larner AJ, Mann DM, Perry R. Elderly individuals with FTLD. JAMA Neurol. 2013;70:412–3.

    PubMed  Google Scholar 

  • Ballard CG, Ayre G, O’Brien J, et al. Simple standardised neuropsychological assessments aid in the differential diagnosis of dementia with Lewy bodies from Alzheimer’s disease and vascular dementia. Dementia Geriatr Cogn Disord. 1999;10:104–8.

    CAS  Google Scholar 

  • Boeve BF, Lang AE, Litvan I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol. 2003;54 Suppl 5:S15–9.

    PubMed  Google Scholar 

  • Boeve BF, Boylan KB, Graff-Radford NR, et al. Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72. Brain. 2012;135:765–83.

    PubMed Central  PubMed  Google Scholar 

  • Cairns NJ, Grossman M, Arnold SE, et al. Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease. Neurology. 2004;63:1376–84.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Calderon J, Perry R, Erzinclioglu S, Berrios GE, Dening T, Hodges JR. Perception, attention and working memory are disproportionately impaired in dementia with Lewy body (LBD) compared to Alzheimer’s disease (AD). J Neurol Neurosurg Psychiatry. 2001;70:157–64.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Caselli RJ, Tariot PN. Alzheimer’s disease and its variants: a diagnostic and therapeutic guide. Oxford: Oxford University Press; 2010.

    Google Scholar 

  • Collinge J. Prion diseases of humans and animals: their causes and molecular basis. Annu Rev Neurosci. 2001;24:519–50.

    PubMed  CAS  Google Scholar 

  • Cruts M, van Duijn CM, Backhovens H, et al. Estimation of the genetic contribution of presenilin-1 and -2 mutations in a population-based study of presenile Alzheimer disease. Hum Mol Genet. 1998;7:43–51.

    Google Scholar 

  • Davies M, Larner AJ. Clinical misdiagnosis of Alzheimer’s disease: getting it wrong again. Eur J Neurol. 2009;16 Suppl 3:351 (abstract 2036).

    Google Scholar 

  • Davies M, Larner AJ. Frontotemporal dementias: development of an integrated care pathway through an experiential survey of patients and carers. Int J Care Pathways. 2010;14:65–9.

    Google Scholar 

  • Davies RR, Doran M, Larner AJ. Early-onset dementia. Prog Neurol Psychiatry. 2011;15(4):12–6.

    Google Scholar 

  • De Simone R, Puig XS, Gélisse P, Crespel A, Genton P. Senile myoclonic epilepsy: delineation of a common condition associated with Alzheimer’s disease in Down syndrome. Seizure. 2010;19:383–9.

    PubMed  Google Scholar 

  • Dobson-Stone C, Hallupp M, Bartley L, et al. C9ORF72 repeat expansion in clinical and neuropathologic frontotemporal dementia cohorts. Neurology. 2012;79:995–1001.

    PubMed Central  PubMed  Google Scholar 

  • Doran M. Diagnosis of presenile dementia. Br J Hosp Med. 1997;58:105–10.

    PubMed  CAS  Google Scholar 

  • Doran M, Larner AJ. Prominent behavioural and psychiatric symptoms in early-onset Alzheimer’s disease in a sib pair with the presenilin-1 gene R269G mutation. Eur Arch Psychiatry Clin Neurosci. 2004a;254:187–9.

    Google Scholar 

  • Doran M, Larner AJ. EEG findings in dementia with Lewy bodies causing diagnostic confusion with sporadic Creutzfeldt-Jakob disease. Eur J Neurol. 2004b;11:838–41.

    PubMed  CAS  Google Scholar 

  • Doran M, Larner AJ. Familial Alzheimer’s disease due to presenilin-1 Y115C mutation. J Neurol. 2006;253 Suppl 2:II91 (Poster P359).

    Google Scholar 

  • Doran M, Larner AJ. Monogenic Mendelian causes of dementia: ten-year survey of a dementia clinic. Eur J Neurol. 2009;16 Suppl 3:291 (abstract P1731).

    Google Scholar 

  • Doran M, du Plessis DG, Enevoldson TP, Fletcher NA, Ghadiali E, Larner AJ. Pathological heterogeneity of clinically diagnosed corticobasal degeneration. J Neurol Sci. 2003;216:127–34.

    PubMed  CAS  Google Scholar 

  • Doran M, Enevoldson TP, Ghadiali EJ, Larner AJ. Mills syndrome with dementia: broadening the phenotype of FTD/MND. J Neurol. 2005;252:846–7.

    PubMed  CAS  Google Scholar 

  • Downes JJ, Priestley NM, Doran M, Ferran J, Ghadiali E, Cooper P. Intellectual, mnemonic and frontal functions in dementia with Lewy bodies: a comparison with early and advanced Parkinson’s disease. Behav Neurol. 1998;11:173–83.

    PubMed  Google Scholar 

  • Du Plessis DG, Larner AJ. Phenotypic similarities causing clinical misdiagnosis of pathologically-confirmed sporadic Creutzfeldt-Jakob disease as dementia with Lewy bodies. Clin Neurol Neurosurg. 2008;110:194–7.

    PubMed  Google Scholar 

  • Dubois B, Feldman HH, Jacova C, et al. Research criteria for the diagnosis of Alzheimer’s disease: revising the NINCDS-ADRDA criteria. Lancet Neurol. 2007;6:734–46.

    PubMed  Google Scholar 

  • Filley CM. The behavioral neurology of white matter. 2nd ed. Oxford: Oxford University Press; 2012.

    Google Scholar 

  • Gaig C, Valledeoriola F, Gelpi E, et al. Rapidly progressive diffuse Lewy body disease. Mov Disord. 2011;26:1316–23.

    PubMed  Google Scholar 

  • Geschwind MD, Tan KM, Lennon VA, et al. Voltage-gated potassium channel autoimmunity mimicking Creutzfeldt-Jakob disease. Arch Neurol. 2008;65:1341–6.

    PubMed Central  PubMed  Google Scholar 

  • Gill ON, Spencer Y, Richard-Loendt A, et al. Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. BMJ. 2013;347:f5675.

    PubMed Central  PubMed  Google Scholar 

  • Gorelick PB, Scuteri A, Black SE, et al. Vascular contributions to cognitive impairment and dementia: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011;42:2672–713.

    PubMed Central  PubMed  Google Scholar 

  • Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76:1006–14.

    PubMed Central  PubMed  Google Scholar 

  • Haïk S, Brandel J-P, Sazdovitch V, et al. Dementia with Lewy bodies in a neuropathologic series of suspected Creutzfeldt-Jakob disease. Neurology. 2000;55:1401–4.

    PubMed  Google Scholar 

  • Hancock P, Larner AJ. A case of frontotemporal lobar degeneration with MND. Prog Neurol Psychiatry. 2008;12(3):15,18.

    Google Scholar 

  • Harvey RJ, Skelton-Robinson M, Rossor MN. The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry. 2003;74:1206–9.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Hodges JR, editor. Frontotemporal dementia syndromes. Cambridge: Cambridge University Press; 2007.

    Google Scholar 

  • Hsiung GY, DeJesus-Hernandez M, Feldman HH, et al. Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p. Brain. 2012;135:709–22.

    PubMed Central  PubMed  Google Scholar 

  • Kaul M. HIV-1 associated dementia: update on pathological mechanisms and therapeutic approaches. Curr Opin Neurol. 2009;22:315–20.

    PubMed Central  PubMed  Google Scholar 

  • Kurlan R, editor. Handbook of secondary dementias. New York: Taylor and Francis; 2006.

    Google Scholar 

  • Langa KM, Foster NL, Larson EB. Mixed dementia: emerging concepts and therapeutic implications. JAMA. 2004;292:2901–8.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Adult-onset dementia with prominent frontal lobe dysfunction in X-linked adrenoleukodystrophy with R152C mutation in ABCD1 gene. J Neurol. 2003;250:1253–4.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Getting it wrong: the clinical misdiagnosis of Alzheimer’s disease. Int J Clin Pract. 2004;58:1092–4.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Frequency of agnosic, apraxic and aphasic presentations of Alzheimer’s disease. Eur J Neurol. 2006a;13 Suppl 2:193 (abstract P2098).

    Google Scholar 

  • Larner AJ. Creutzfeldt-Jakob disease misdiagnosed as dementia with Lewy bodies: response to the paper by Kraemer et al. (J Neurol 2005;252:861–2). J Neurol. 2006b;253:960.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Down syndrome in the neurology clinic: Too much? Too little? Too late? Down Syndr Res Pract. 2007;12:69–71.

    Google Scholar 

  • Larner AJ. Neuropsychological neurology: the neurocognitive impairments of neurological disorders. Cambridge: Cambridge University Press; 2008a.

    Google Scholar 

  • Larner AJ. Alzheimer’s disease. In: Cappa SF, Abutalebi J, Démonet JF, Fletcher PC, Garrard P, editors. Cognitive neurology: a clinical textbook. Oxford: Oxford University Press; 2008b. p. 199–227.

    Google Scholar 

  • Larner AJ. Mutation negative early-onset familial Alzheimer disease: consider screening for tau gene mutations. Alzheimer Dis Assoc Disord. 2008c;22:194–5.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Delusion of pregnancy in frontotemporal lobar degeneration with motor neurone disease (FTLD/MND). Behav Neurol. 2008d;19:199–200.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Monogenic Mendelian disorders in general neurological practice. Int J Clin Pract. 2008e;62:744–6.

    PubMed  CAS  Google Scholar 

  • Larner AJ. A 50-year old man with deteriorating cognitive function and impaired movement. PLoS Med. 2009a;6(1):e1000019.

    Google Scholar 

  • Larner AJ. Deletion of 18q. In: Lang F, editor. Encyclopedia of molecular mechanisms of disease (3 volumes). Berlin: Springer; 2009b. p. 503–4.

    Google Scholar 

  • Larner AJ. What’s new in dementia? Clin Med. 2010a;10:391–4.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Neurological signs: lycanthropy. Adv Clin Neurosci Rehabil. 2010b;10(4):50.

    Google Scholar 

  • Larner AJ. Cholinesterase inhibitors – beyond Alzheimer’s disease. Exp Rev Neurotherapeutics. 2010c;10:1699–705.

    CAS  Google Scholar 

  • Larner AJ. Senile myoclonic epilepsy in Down syndrome. Seizure. 2011a;20:512.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Presenilin 1 mutation Alzheimer’s disease: a genetic epilepsy syndrome? Epilepsy Behav. 2011b;21:20–2.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Progressive nonfluent aphasia in a bilingual subject: relative preservation of mother tongue. J Neuropsychiatry Clin Neurosci. 2012a;24:E9–10.

    PubMed  Google Scholar 

  • Larner AJ. Intrafamilial clinical phenotypic heterogeneity with progranulin gene p.Glu498fs mutation. J Neurol Sci. 2012b;316: 189–90.

    PubMed  CAS  Google Scholar 

  • Larner AJ. FTDP-17: two-year follow-up of motor and cognitive features following autologous stem cell transplantation. J Neuropsychiatry Clin Neurosci. 2012c;24:E1–2.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Neuropsychological neurology: the neurocognitive impairments of neurological disorders. 2nd ed. Cambridge: Cambridge University Press; 2013a.

    Google Scholar 

  • Larner AJ. Delusion of pregnancy: a case revisited. Behav Neurol. 2013b;27:293–4.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Presenilin-1 mutations in Alzheimer’s disease: an update on genotype-phenotype relationships. J Alzheimers Dis. 2013c;37:653–9.

    PubMed  CAS  Google Scholar 

  • Larner AJ. Cerebral mass lesions presenting in a cognitive disorders clinic. Br J Hosp Med. 2013d;74:694–5.

    CAS  Google Scholar 

  • Larner AJ. Neurological update: dementia. J Neurol. 2014;261:635–9.

    Google Scholar 

  • Larner AJ, Doran M. Prion disease at a regional neuroscience centre: retrospective audit. J Neurol Neurosurg Psychiatry. 2004;75:1789–90.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Larner AJ, Doran M. Clinical phenotypic heterogeneity of Alzheimer’s disease associated with mutations of the presenilin-1 gene. J Neurol. 2006;253:139–58.

    PubMed  CAS  Google Scholar 

  • Larner AJ, Doran M. Clinical heterogeneity associated with tau gene mutations. Eur Neurol Rev. 2009a;3(2):31–2.

    Google Scholar 

  • Larner AJ, Doran M. Genotype-phenotype relationships of presenilin-1 mutations in Alzheimer’s disease: an update. J Alzheimers Dis. 2009b;17:259–65.

    PubMed  CAS  Google Scholar 

  • Larner AJ, du Plessis DG. Early-onset Alzheimer’s disease with presenilin-1 M139V mutation: clinical, neuropsychological and neuropathological study. Eur J Neurol. 2003;10:319–23.

    Google Scholar 

  • Larner AJ, Gardner-Thorpe C. Mills syndrome with dementia. Eur Neurol J. 2012;4(2):29–32.

    Google Scholar 

  • Larner AJ, Mathias CJ, Rossor MN. Autonomic failure preceding dementia with Lewy bodies. J Neurol. 2000;247:229–31.

    PubMed  CAS  Google Scholar 

  • Larner AJ, Brookfield K, Flynn A, Ghadiali EJ, Smith ETS, Doran M. The cerebral metabolic topography of semantic dementia. J Neurol. 2005a;252 Suppl 2:II106 (abstract P399).

    Google Scholar 

  • Larner AJ, Hart IK, Cresswell P, Doran M. REM sleep behaviour disorder in the cognitive function clinic. Eur J Neurol. 2005b;12 Suppl 2:218 (abstract 2211).

    Google Scholar 

  • Larner AJ, Ray PS, Doran M. The R269H mutation in presenilin-1 presenting as late-onset autosomal dominant Alzheimer’s disease. J Neurol Sci. 2007;252:173–6.

    PubMed  CAS  Google Scholar 

  • Lindquist SG, Holm IE, Schwartz M, et al. Alzheimer disease-like clinical phenotype in a family with FTDP-17 caused by a MAPT R406W mutation. Eur J Neurol. 2008;15:377–85.

    PubMed  CAS  Google Scholar 

  • Lorains JW, Henry C, Agbamu DA, Rossi M, Bishop M, Will RG, Ironside JW. Variant Creutzfeldt-Jakob disease in an elderly patient. Lancet. 2001;357:1339–40.

    PubMed  CAS  Google Scholar 

  • Mackenzie IR, Neumann M, Bigio EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 2010;119:1–4.

    PubMed Central  PubMed  Google Scholar 

  • Mahoney CJ, Beck J, Rohrer JD, et al. Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features. Brain. 2012;135:736–50.

    PubMed Central  PubMed  Google Scholar 

  • McKeith IG, Dickson DW, Lowe J, et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology. 2005;65:1863–72.

    PubMed  CAS  Google Scholar 

  • McKhann G, Drachman D, Folstein M, et al. Clinical diagnosis of Alzheimer’s disease. Report of the NINCDS-ADRDA work group under the auspices of the Department of Health and Human Service Task forces on Alzheimer’s disease. Neurology. 1984;34:939–44.

    PubMed  CAS  Google Scholar 

  • McKhann GM, Knopman DS, Chertkow H, et al. 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. 2011;7:263–9.

    PubMed Central  PubMed  Google Scholar 

  • Mendez MF, Cummings JL. Dementia: a clinical approach. 3rd ed. Philadelphia: Butterworth-Heinemann; 2003.

    Google Scholar 

  • Menon R, Barborie A, Jaros E, Mann DMA, Ray PS, Larner AJ. What’s in a name? Neuronal intermediate filament inclusion disease (NIFID), frontotemporal lobar degeneration-intermediate filament (FTLD-IF) or frontotemporal lobar degeneration-fused in sarcoma (FTLD-FUS)? J Neurol Neurosurg Psychiatry. 2011;82:1412–4.

    PubMed  CAS  Google Scholar 

  • Momjian-Mayor I, Pizzolato GP, Burkhardt K, et al. Fulminant Lewy body disease. Mov Disord. 2006;21:1748–51.

    PubMed  Google Scholar 

  • Mrak RE, Griffin WS. Trisomy 21 and the brain. J Neuropathol Exp Neurol. 2004;63:679–85.

    PubMed Central  PubMed  CAS  Google Scholar 

  • MRC CFAS. Pathological correlates of late-onset dementia in a multicentre, community-based population in England and Wales. Neuropathology Group of the Medical Research Council Cognitive Function and Ageing Study. Lancet. 2001;357:169–75.

    Google Scholar 

  • Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998;51:1546–54.

    PubMed  CAS  Google Scholar 

  • Neumann M, Roeber S, Kretzschmar HA, Rademakers R, Baker M, Mackenzie IRA. Abundant FUS pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol. 2009;118:605–16.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Nicholas RS, Partridge J, Donn RP, Hawkins C, Boggild MD. The role of the PTPRC (CD45) mutation in the development of multiple sclerosis in the North West region of the United Kingdom. J Neurol Neurosurg Psychiatry. 2003;74:944–5.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Prasher VP. Alzheimer’s disease and dementia in Down syndrome and intellectual disabilities. Oxford: Radcliffe Publishing; 2005.

    Google Scholar 

  • Rabinovici GD, Wang PN, Levin CM, et al. First symptom in sporadic Creutzfeldt-Jakob disease. Neurology. 2006;66:286–7.

    PubMed  CAS  Google Scholar 

  • Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134:2456–77.

    PubMed Central  PubMed  Google Scholar 

  • Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology. 2002;58:1615–21.

    PubMed  CAS  Google Scholar 

  • Reid WG, Hely MA, Morris JG, Loy C, Halliday GM. Dementia in Parkinson’s disease: a 20-year neuropsychological study (Sydney Multicentre Study). J Neurol Neurosurg Psychiatry. 2011;82:1033–7.

    PubMed  CAS  Google Scholar 

  • Rohrer JD, Guerriero R, Vandrovcova J, et al. The heritability and genetics of frontotemporal lobar degeneration. Neurology. 2009;73:1451–6.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Román GC, Erkinjuntti T, Wallin A, Pantoni L, Chui HC. Subcortical ischaemic vascular dementia. Lancet Neurol. 2002;1:426–36.

    PubMed  Google Scholar 

  • Rosness TA, Haugen PK, Passant U, Engedal K. Frontotemporal dementia – a clinically complex diagnosis. Int J Geriatr Psychiatry. 2008;23:837–42.

    PubMed  Google Scholar 

  • Rossor MN, Fox NC, Mummery CJ, Schott JM, Warren JD. The diagnosis of young-onset dementia. Lancet Neurol. 2010;9:793–806.

    PubMed Central  PubMed  Google Scholar 

  • Salmon DP, Galasko D, Hansen LA, et al. Neuropsychological deficits associated with diffuse Lewy body disease. Brain Cogn. 1996;31: 148–65.

    PubMed  CAS  Google Scholar 

  • Sathasivam S, Doran M, Larner AJ. Frontotemporal lobar degeneration with motor neurone disease (FTLD/MND): presentations in psychiatric practice. Int J Psychiatry Clin Pract. 2008;12:138–41.

    Google Scholar 

  • Schneider JA, Aggarwal NT, Barnes L, Boyle P, Bennett DA. The neuropathology of older persons with and without dementia from community versus clinic cohorts. J Alzheimers Dis. 2009;18:691–701.

    PubMed Central  PubMed  Google Scholar 

  • Schott JM, Warren JD, Rossor MN. The uncertain nosology of Hashimoto encephalopathy. Arch Neurol. 2003;60:1812.

    PubMed  Google Scholar 

  • Schott JM, Williams DR, Butterworth RJ, Janssen JC, Larner AJ, Holton JL, Rossor MN. Shunt responsive progressive supranuclear palsy? Mov Disord. 2007;22:902–3.

    PubMed  Google Scholar 

  • Seelaar H, Kamphorst W, Rosso SM, et al. Distinct genetic forms of frontotemporal dementia. Neurology. 2008;71:1220–6.

    PubMed  CAS  Google Scholar 

  • Seelaar H, Rohrer JD, Pijnenburg YA, Fox NC, van Swieten JC. Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry. 2011;82:476–86.

    PubMed  Google Scholar 

  • Sells RA, Larner AJ. Genetic causes of learning disability with epilepsy in the general neurology clinic. Eur J Neurol. 2011;18 Suppl 2:184 (abstract P1315).

    Google Scholar 

  • Silverdale M, Leach JP, Chadwick DW. New variant Creutzfeldt-Jakob disease presenting as localization-related epilepsy. Neurology. 2000;54:2188.

    PubMed  CAS  Google Scholar 

  • Simon-Sanchez J, Dopper EG, Cohn-Hokke PE, et al. The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions. Brain. 2012;135:723–35.

    PubMed  Google Scholar 

  • Snowden JS, Rollinson S, Thompson JC, et al. Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain. 2012;135:693–708.

    PubMed Central  PubMed  Google Scholar 

  • Snowdon DA, Greiner LH, Mortimer JA, et al. Brain infarction and the clinical expression of Alzheimer’s disease. The Nun Study. JAMA. 1997;277:813–7.

    PubMed  CAS  Google Scholar 

  • Sperling RA, Aisen PS, Beckett LA, et al. Toward defining the preclinical stages of Alzheimer’s disease: Recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011;7:280–92.

    PubMed Central  PubMed  Google Scholar 

  • Tschampa HJ, Neumann M, Zerr I, et al. Patients with Alzheimer’s disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry. 2001;71:33–9.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Van Everbroeck B, Dobbeleir I, De Waele M, De Deyn P, Martin J-J, Cras P. Differential diagnosis of 201 possible Creutzfeldt-Jakob disease patients. J Neurol. 2004;251:298–304.

    PubMed  Google Scholar 

  • Varma AR, Snowden JS, Lloyd JJ, et al. Evaluation of the NINCDS-ADRDA criteria in the differentiation of Alzheimer’s disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry. 1999;66: 184–8.

    PubMed Central  PubMed  CAS  Google Scholar 

  • Wahlund L-O, Erkinjuntti T, Gauthier S, editors. Vascular cognitive impairment in clinical practice. Cambridge: Cambridge University Press; 2009.

    Google Scholar 

  • Wall CA, Rummans TA, Aksamit AJ, Krah LE, Pankratz VS. Psychiatric manifestations of Creutzfeldt-Jakob disease: a 25-year analysis. J Neuropsychiatry Clin Neurosci. 2005;17:489–95.

    PubMed  Google Scholar 

  • Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996;347:921–5.

    PubMed  CAS  Google Scholar 

  • Williams-Gray CH, Mason SL, Evans JR, et al. The CamPaIGN study of Parkinson’s disease: 10-year outlook in an incident population-based cohort. J Neurol Neurosurg Psychiatry. 2012;84:1258–64.

    Google Scholar 

  • Wilson M, Doran M, Enevoldson TP, Larner AJ. Cognitive profiles associated with intracranial dural arteriovenous fistula. Age Ageing. 2010;39:389–92.

    PubMed  Google Scholar 

  • Young CA, Boggild M, Larner AJ. A familial syndrome of multiple sclerosis, early-onset dementia and epilepsy. Eur J Neurol. 2008;15 Suppl 3:142 (abstract P1428).

    Google Scholar 

  • Zaccai J, McCracken C, Brayne C. A systematic study of prevalence and incidence studies of dementia with Lewy bodies. Age Ageing. 2005;34:561–6.

    PubMed  Google Scholar 

  • Ziso B, Marsden D, Larner AJ, Alusi SH. “Undifferentiated schizophrenia” revisited. J Neuropsychiatry Clin Neurosci. 2014 (accepted).

    Google Scholar 

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Larner, A.J. (2014). Diagnosis (2): Dementia Disorders. In: Dementia in Clinical Practice: A Neurological Perspective. Springer, London. https://doi.org/10.1007/978-1-4471-6371-8_8

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