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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2000 Aug;69(2):178–186. doi: 10.1136/jnnp.69.2.178

Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease?

S Bozeat 1, C Gregory 1, M Ralph 1, J Hodges 1
PMCID: PMC1737062  PMID: 10896690

Abstract

OBJECTIVES—To investigate the prevalence of changes in mood, personality, and behaviour in frontotemporal dementia (FTD) and Alzheimer's disease (AD) and hence, which features reliably distinguish between them. To establish whether the frontal and temporal variants of FTD are characterised by different behavioural changes.
METHODS—A questionnaire was designed to assess a wide range of neuropsychiatric changes; it incorporated features reported in previous studies of FTD and components of the neuropsychiatric inventory.1 This was completed by 37 carers of patients with Alzheimer's disease (AD) and 33 patients with frontotemporal dementia (FTD), comprising 20 with temporal variant FTD (tv FTD) or semantic dementia and 13 with frontal variant FTD (fv FTD). An exploratory principal components factor analysis and discriminant function analysis was applied.
RESULTS—Factor analysis showed four robust and meaningful symptom clusters: factor 1—stereotypic and eating behaviour; factor 2—executive dysfunction and self care; factor 3—mood changes; factor 4—loss of social awareness. Only stereotypic and altered eating behaviour and loss of social awareness reliably differentiated AD from FTD with no effect of disease severity. By contrast, executive dysfunction, poor self care, and restlessness showed a significant effect of disease severity only, with the more impaired patients scoring more highly. Changes in mood were found to be equally prevalent in the three patient groups. Analysis of individual symptoms showed increased rates of mental rigidity and depression in the patients with semantic dementia compared with those with fv FTD. Conversely, the latter group showed greater disinhibition. Discriminant function analysis correctly classified 71.4% overall and 86.5% of the patients with AD.
CONCLUSIONS—This questionnaire disclosed striking differences between patients with FTD and AD, but only stereotypic behaviour, changes in eating preference, disinhibition, and features of poor social awareness reliably separated the groups. The patients with fv FTD and semantic dementia were behaviourally very similar, reflecting the involvement of a common network, the ventral frontal lobe, temporal pole, and amygdala. Dysexecutive symptoms and poor self care were found to be affected by the severity of the disease, reflecting perhaps spread to dorsolateral prefrontal areas relatively late in the course of both FTD and AD. This questionnaire may be of value in the diagnosis and the monitoring of therapies.



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Selected References

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  1. Ames D., Cummings J. L., Wirshing W. C., Quinn B., Mahler M. Repetitive and compulsive behavior in frontal lobe degenerations. J Neuropsychiatry Clin Neurosci. 1994 Spring;6(2):100–113. doi: 10.1176/jnp.6.2.100. [DOI] [PubMed] [Google Scholar]
  2. Bechara A., Damasio H., Tranel D., Anderson S. W. Dissociation Of working memory from decision making within the human prefrontal cortex. J Neurosci. 1998 Jan 1;18(1):428–437. doi: 10.1523/JNEUROSCI.18-01-00428.1998. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Berrios G. E., Brook P. Delusions and the psychopathology of the elderly with dementia. Acta Psychiatr Scand. 1985 Sep;72(3):296–301. doi: 10.1111/j.1600-0447.1985.tb02610.x. [DOI] [PubMed] [Google Scholar]
  4. Birkett D. P. The psychiatric differentiation of senility and arteriosclerosis. Br J Psychiatry. 1972 Mar;120(556):321–325. doi: 10.1192/bjp.120.556.321. [DOI] [PubMed] [Google Scholar]
  5. Braak H., Braak E. Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol. 1991;82(4):239–259. doi: 10.1007/BF00308809. [DOI] [PubMed] [Google Scholar]
  6. Brun A., Englund E. Regional pattern of degeneration in Alzheimer's disease: neuronal loss and histopathological grading. Histopathology. 1981 Sep;5(5):549–564. doi: 10.1111/j.1365-2559.1981.tb01818.x. [DOI] [PubMed] [Google Scholar]
  7. 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]
  8. Butter C. M., Snyder D. R., McDonald J. A. Effects of orbital frontal lesions on aversive and aggressive behaviors in rhesus monkeys. J Comp Physiol Psychol. 1970 Jul;72(1):132–144. doi: 10.1037/h0029303. [DOI] [PubMed] [Google Scholar]
  9. 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]
  10. Cooper J. K., Mungas D., Weiler P. G. Relation of cognitive status and abnormal behaviors in Alzheimer's disease. J Am Geriatr Soc. 1990 Aug;38(8):867–870. doi: 10.1111/j.1532-5415.1990.tb05701.x. [DOI] [PubMed] [Google Scholar]
  11. Cummings J. L., Mega M., Gray K., Rosenberg-Thompson S., Carusi D. A., Gornbein J. The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology. 1994 Dec;44(12):2308–2314. doi: 10.1212/wnl.44.12.2308. [DOI] [PubMed] [Google Scholar]
  12. 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]
  13. Cummings J. L. The Neuropsychiatric Inventory: assessing psychopathology in dementia patients. Neurology. 1997 May;48(5 Suppl 6):S10–S16. doi: 10.1212/wnl.48.5_suppl_6.10s. [DOI] [PubMed] [Google Scholar]
  14. Damasio A. R., Tranel D., Damasio H. Individuals with sociopathic behavior caused by frontal damage fail to respond autonomically to social stimuli. Behav Brain Res. 1990 Dec 14;41(2):81–94. doi: 10.1016/0166-4328(90)90144-4. [DOI] [PubMed] [Google Scholar]
  15. Gilley D. W., Whalen M. E., Wilson R. S., Bennett D. A. Hallucinations and associated factors in Alzheimer's disease. J Neuropsychiatry Clin Neurosci. 1991 Fall;3(4):371–376. doi: 10.1176/jnp.3.4.371. [DOI] [PubMed] [Google Scholar]
  16. Gregory C. A., Hodges J. R. Clinical features of frontal lobe dementia in comparison to Alzheimer's disease. J Neural Transm Suppl. 1996;47:103–123. doi: 10.1007/978-3-7091-6892-9_6. [DOI] [PubMed] [Google Scholar]
  17. Gregory C. A., Orrell M., Sahakian B., Hodges J. R. Can frontotemporal dementia and Alzheimer's disease be differentiated using a brief battery of tests? Int J Geriatr Psychiatry. 1997 Mar;12(3):375–383. doi: 10.1002/(sici)1099-1166(199703)12:3<375::aid-gps518>3.0.co;2-#. [DOI] [PubMed] [Google Scholar]
  18. 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]
  19. Hodges J. R., Graham N., Patterson K. Charting the progression in semantic dementia: implications for the organisation of semantic memory. Memory. 1995 Sep-Dec;3(3-4):463–495. doi: 10.1080/09658219508253161. [DOI] [PubMed] [Google Scholar]
  20. Hodges J. R., Patterson K. Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study. J Int Neuropsychol Soc. 1996 Nov;2(6):511–524. doi: 10.1017/s1355617700001685. [DOI] [PubMed] [Google Scholar]
  21. 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]
  22. Hodges J. R., Patterson K., Ward R., Garrard P., Bak T., Perry R., Gregory C. The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: a comparative neuropsychological study. Neuropsychology. 1999 Jan;13(1):31–40. doi: 10.1037//0894-4105.13.1.31. [DOI] [PubMed] [Google Scholar]
  23. Hughes C. P., Berg L., Danziger W. L., Coben L. A., Martin R. L. A new clinical scale for the staging of dementia. Br J Psychiatry. 1982 Jun;140:566–572. doi: 10.1192/bjp.140.6.566. [DOI] [PubMed] [Google Scholar]
  24. Jagust W. J., Reed B. R., Seab J. P., Kramer J. H., Budinger T. F. Clinical-physiologic correlates of Alzheimer's disease and frontal lobe dementia. Am J Physiol Imaging. 1989;4(3):89–96. [PubMed] [Google Scholar]
  25. Kumar A., Koss E., Metzler D., Moore A., Friedland R. P. Behavioral symptomatology in dementia of the Alzheimer type. Alzheimer Dis Assoc Disord. 1988;2(4):363–365. doi: 10.1097/00002093-198802040-00005. [DOI] [PubMed] [Google Scholar]
  26. Lebert F., Pasquier F., Souliez L., Petit H. Frontotemporal behavioral scale. Alzheimer Dis Assoc Disord. 1998 Dec;12(4):335–339. doi: 10.1097/00002093-199812000-00014. [DOI] [PubMed] [Google Scholar]
  27. Levy M. L., Miller B. L., Cummings J. L., Fairbanks L. A., Craig A. Alzheimer disease and frontotemporal dementias. Behavioral distinctions. Arch Neurol. 1996 Jul;53(7):687–690. doi: 10.1001/archneur.1996.00550070129021. [DOI] [PubMed] [Google Scholar]
  28. 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]
  29. Mega M. S., Cummings J. L., Fiorello T., Gornbein J. The spectrum of behavioral changes in Alzheimer's disease. Neurology. 1996 Jan;46(1):130–135. doi: 10.1212/wnl.46.1.130. [DOI] [PubMed] [Google Scholar]
  30. Merriam A. E., Aronson M. K., Gaston P., Wey S. L., Katz I. The psychiatric symptoms of Alzheimer's disease. J Am Geriatr Soc. 1988 Jan;36(1):7–12. doi: 10.1111/j.1532-5415.1988.tb03427.x. [DOI] [PubMed] [Google Scholar]
  31. 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]
  32. Miller B. L., Cummings J. L., Villanueva-Meyer J., Boone K., Mehringer C. M., Lesser I. M., Mena I. Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics. Neurology. 1991 Sep;41(9):1374–1382. doi: 10.1212/wnl.41.9.1374. [DOI] [PubMed] [Google Scholar]
  33. 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]
  34. Mummery C. J., Patterson K., Hodges J. R., Price C. J. Functional neuroanatomy of the semantic system: divisible by what? J Cogn Neurosci. 1998 Nov;10(6):766–777. doi: 10.1162/089892998563059. [DOI] [PubMed] [Google Scholar]
  35. Mummery C. J., Patterson K., Price C. J., Ashburner J., Frackowiak R. S., Hodges J. R. A voxel-based morphometry study of semantic dementia: relationship between temporal lobe atrophy and semantic memory. Ann Neurol. 2000 Jan;47(1):36–45. [PubMed] [Google Scholar]
  36. Mummery C. J., Patterson K., Wise R. J., Vandenberghe R., Vandenbergh R., Price C. J., Hodges J. R. Disrupted temporal lobe connections in semantic dementia. Brain. 1999 Jan;122(Pt 1):61–73. doi: 10.1093/brain/122.1.61. [DOI] [PubMed] [Google Scholar]
  37. Myers R. E., Swett C., Miller M. Loss of social group affinity following prefrontal lesions in free-ranging macaques. Brain Res. 1973 Dec 21;64:257–269. doi: 10.1016/0006-8993(73)90182-0. [DOI] [PubMed] [Google Scholar]
  38. 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]
  39. 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]
  40. Nelson H. E. A modified card sorting test sensitive to frontal lobe defects. Cortex. 1976 Dec;12(4):313–324. doi: 10.1016/s0010-9452(76)80035-4. [DOI] [PubMed] [Google Scholar]
  41. Pachana N. A., Boone K. B., Miller B. L., Cummings J. L., Berman N. Comparison of neuropsychological functioning in Alzheimer's disease and frontotemporal dementia. J Int Neuropsychol Soc. 1996 Nov;2(6):505–510. doi: 10.1017/s1355617700001673. [DOI] [PubMed] [Google Scholar]
  42. Rahman S., Sahakian B. J., Hodges J. R., Rogers R. D., Robbins T. W. Specific cognitive deficits in mild frontal variant frontotemporal dementia. Brain. 1999 Aug;122(Pt 8):1469–1493. doi: 10.1093/brain/122.8.1469. [DOI] [PubMed] [Google Scholar]
  43. Rogers R. D., Sahakian B. J., Hodges J. R., Polkey C. E., Kennard C., Robbins T. W. Dissociating executive mechanisms of task control following frontal lobe damage and Parkinson's disease. Brain. 1998 May;121(Pt 5):815–842. doi: 10.1093/brain/121.5.815. [DOI] [PubMed] [Google Scholar]
  44. Shallice T. Specific impairments of planning. Philos Trans R Soc Lond B Biol Sci. 1982 Jun 25;298(1089):199–209. doi: 10.1098/rstb.1982.0082. [DOI] [PubMed] [Google Scholar]
  45. Sinha D., Zemlan F. P., Nelson S., Bienenfeld D., Thienhaus O., Ramaswamy G., Hamilton S. A new scale for assessing behavioral agitation in dementia. Psychiatry Res. 1992 Jan;41(1):73–88. doi: 10.1016/0165-1781(92)90020-4. [DOI] [PubMed] [Google Scholar]
  46. Spillantini M. G., Bird T. D., Ghetti B. Frontotemporal dementia and Parkinsonism linked to chromosome 17: a new group of tauopathies. Brain Pathol. 1998 Apr;8(2):387–402. doi: 10.1111/j.1750-3639.1998.tb00162.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  47. Stone V. E., Baron-Cohen S., Knight R. T. Frontal lobe contributions to theory of mind. J Cogn Neurosci. 1998 Sep;10(5):640–656. doi: 10.1162/089892998562942. [DOI] [PubMed] [Google Scholar]
  48. Wragg R. E., Jeste D. V. Overview of depression and psychosis in Alzheimer's disease. Am J Psychiatry. 1989 May;146(5):577–587. doi: 10.1176/ajp.146.5.577. [DOI] [PubMed] [Google Scholar]

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