Login to MyKarger

New to MyKarger? Click here to sign up.

Login with Facebook

Forgot Password? Reset your password

Authors, Editors, Reviewers

For Manuscript Submission, Check or Review Login please go to Submission Websites List.

Submission Websites List

Institutional Login (Shibboleth)

For the academic login, please select your country in the dropdown list. You will be redirected to verify your credentials.

Table of Contents
Vol. 24, No. 5, 2007
Issue release date: October 2007
Section title: Original Research Article
Dement Geriatr Cogn Disord 2007;24:317–326

Clusters of Cognitive and Behavioral Disorders Clearly Distinguish Primary Progressive Aphasia from Frontal Lobe Dementia, and Alzheimer’s Disease

Marra C.a · Quaranta D.a · Zinno M.a · Misciagna S.b · Bizzarro A.a · Masullo C.a · Daniele A.a · Gainotti G.a
aNeuropsychology Service of the Catholic University of Rome, Policlinico Gemelli, and bFondazione ‘Don Carlo Gnocchi’, Rome, Italy
email Corresponding Author

Dr. Camillo Marra

Servizio di Neuropsicologia, Università Cattolica/Policlinico Gemelli

Largo A. Gemelli, 8

IT–00168 Roma (Italy)

Tel. +39 06 3015 4333, Fax +39 06 3550 1909, E-Mail cmarra@rm.unicatt.it


  1. Gustafson L: Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Arch Gerontol Geriatr 1987;6:209–223.
  2. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF: Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998;51:1546–1554.
  3. Hodges JR, Miller B: The classification, genetics and neuropathology of frontotemporal dementia. Introduction to the special topic papers. I. Neurocase 2001;7:31–35.
  4. Bathgate D, Snowden JS, Varma A, Blackshaw A, Neary D: Behaviour in frontotemporal dementia, Alzheimer’s disease and vascular dementia. Acta Neurol Scand 2001;103:367–378.
  5. Bozeat S, Gregory CA, Ralph MA, Hodges JR: Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer’s disease? J Neurol Neurosurg Psychiatry 2000;69:178–186.
  6. Snowden JS, Bathgate D, Varma A, Blackshaw A, Gibbons ZC, Neary D: Distinct behavioural profiles in frontotemporal dementia and semantic dementia. J Neurol Neurosurg Psychiatry 2001;70:323–332.
  7. Rosso SM, Donker Kaat L, Baks T, Joosse M, de Koning I, Pijnenburg Y, de Jong D, Dooijes D, Kamphorst W, Ravid R, Niermeijer MF, Verheij F, Kremer HP, Scheltens P, van Duijn CM, Heutink P, van Swieten JC: Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain 2003;126:2016–2022.
  8. Neary D, Snowden J, Mann D: Frontotemporal dementia. Lancet Neurol 2005;4:771–780.
  9. Cummings JL, Duchen LW: Klüver-Bucy syndrome in Pick disease: clinical and pathologic correlations. Neurology 1981;31:1415–1422.
  10. Snowden JS, Neary D, Mann DMA: Frontotemporal Lobar Degeneration: Frontotemporal Dementia, Progressive Aphasia, Semantic Dementia. Churchill Livingstone, New York, 1996.
  11. Mesulam MM: Primary progressive aphasia – differentiation from Alzheimer’s disease. Ann Neurol 1987;22:533–534.
  12. Mesulam MM: Primary progressive aphasia – a language-based dementia. N Engl J Med 2003;349:1535–1542.
  13. Mendez MF, Clark DG, Shapira JS, Cummings JL: Speech and language in progressive nonfluent aphasia compared with early Alzheimer’s disease. Neurology 2003;61:1108–1113.
  14. Le Rhun E, Richard F, Pasquier F: Natural history of primary progressive aphasia. Neurology 2005;65:887–891.
  15. Nestor PJ, Graham NL, Fryer TD, Williams GB, Patterson K, Hodges JR: Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula. Brain 2003;126:2406–2418.
  16. Mathuranath PS, Nestor PJ, Berrios GE, Rakowicz W, Hodges JR: A brief cognitive test battery to differentiate Alzheimer’s disease and frontotemporal dementia. Neurology 2000;55:1613–1620.
  17. Gregory CA, Orrell M, Sahakian B, Hodges JR: Can frontotemporal dementia and Alzheimer’s disease be differentiated using a brief battery of tests? Int J Geriatr Psychiatry 1997;12:375–383.
  18. Siri S, Benaglio I, Frigerio A, Binetti G, Cappa SF: A brief neuropsychological assessment for the differential diagnosis between frontotemporal dementia and Alzheimer’s disease. Eur J Neurol 2001;8:125–132.
  19. Lipton AM, Ohman KA, Womack KB, Hynan LS, Ninman ET, Lacritz LH: Subscores of the FAB differentiate frontotemporal lobar degeneration from AD. Neurology 2005;65:726–731.
  20. Kramer JH, Jurik J, Sha SJ, Rankin KP, Rosen HJ, Johnson JK, Miller BL: Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and Alzheimer disease. Cogn Behav Neurol 2003;16:211–218.
  21. Perri R, Koch G, Carlesimo GA, Serra L, Fadda L, Pasqualetti P, Pettenati C, Caltagirone C: Alzheimer’s disease and frontal variant of frontotemporal dementia – a very brief battery for cognitive and behavioural distinction. J Neurol 2005;252:1238–1244.
  22. Grossi D, Fragassi NA, Chiacchio L, Valoroso L, Tuccillo R, Perrotta C, Rapone P, Conchiglia G, Trojano L: Do visuospatial and constructional disturbances differentiate frontal variant of frontotemporal dementia and Alzheimer’s disease? an experimental study of a clinical belief. Int J Geriatr Psychiatry 2002;17:641–648.
  23. Thompson JC, Stopford CL, Snowden JS, Neary D: Qualitative neuropsychological performance characteristics in frontotemporal dementia and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 2005;76:920–927.
  24. Nyatsanza S, Shetty T, Gregory C, Lough S, Dawson K, Hodges JR: A study of stereotypic behaviours in Alzheimer’s disease and frontal and temporal variant frontotemporal dementia. J Neurol Neurosurg Psychiatry 2003;74:1398–1402.
  25. Ikeda M, Brown J, Holland AJ, Fukuhara R, Hodges JR: Changes in appetite, food preference, and eating habits in frontotemporal dementia and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 2002;73:371–376.
  26. Cummings JL, Mega M, Gray K, Rosenberg-Thompson S, Carusi DA, Gornbein J: The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology 1994;44:2308–2314.
  27. Levy ML, Miller BL, Cummings JL, Fairbanks LA, Craig A: Alzheimer disease and frontotemporal dementias. Behavioral distinctions. Arch Neurol 1996;53:687–690.
  28. Lindau M, Almkvist O, Kushi J, et al: First symptoms: frontotemporal dementia versus Alzheimer’s disease. Dement Geriat Cogn Disord 2000;11:286–293.
  29. McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan EM: 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;34:939–944.
  30. Rey A: Mémorisation d’une serie de 15 mots en 5 répétitions; in Rey A (ed): L’examen clinique en psychologie. Paris, Presses Universitaires de France, 1958.
  31. Lezak MD: Neuropsychological Assessment, ed 3. New York, Oxford University Press, 1995.
  32. Wechsler D: Wechsler Adult Intelligence Scale: WAIS-R Manual. New York, Psychological Corporation, 1981.
  33. Caltagirone C, Gainotti G, Masullo C, Miceli G: Validity of some neuropsychological tests in the assessment of mental deterioration. Acta Psychiatr Scand 1979;60:50–56.
  34. Villa G, Gainotti G, De Bonis C, Marra C: Double dissociation between temporal and spatial pattern processing in patients with frontal and parietal damage. Cortex 1990;26:399–407.
  35. Caffarra P, Vezzadini G, Dieci F, Zonato F, Venneri A: Una versione abbreviata del test di Stroop: dati normativi nella popolazione italiana. Nuova riv neurol 2002;12:111–115.
  36. Dubois B, Slachevsky A, Litvan I, Pillon B: The FAB: a Frontal Assessment Battery at bedside. Neurology 2000;55:1621–1626.
  37. Gainotti G, Marra C, Villa G: A double dissociation between accuracy and time of execution on attentional tasks in Alzheimer’s disease and multi-infarct dementia. Brain 2001;124:731–738.
  38. Raven JC: Progressive Matrices (1947): Sets A, Ab, B: Board and Book Forms. London, HK Lewis, 1949.
  39. Gainotti G, Miceli G, Caltagirone C: Constructional apraxia in left brain-damaged patients: a planning disorder? Cortex 1977;13:109–118.
  40. Lee KL, Harrell FE Jr, Tolley HD, Rosati RA: A comparison of test statistics for assessing the effects of concomitant variables in survival analysis. Biometrics 1983;39:341–350.
  41. Perry RJ, Hodges JR: Differentiating frontal and temporal variant frontotemporal dementia from Alzheimer’s disease. Neurology 2000;54:2277–2284.
  42. Pasquier F, Grymonprez L, Lebert F, Van der Linden M: Memory impairment differs in frontotemporal dementia and Alzheimer’s disease. Neurocase 2001;7:161–171.
  43. Glosser G, Gallo JL, Clark CM, Grossman M: Memory encoding and retrieval in frontotemporal dementia and Alzheimer’s disease. Neuropsychology 2002;16:190–196.
  44. Jenner C, Reali G, Puopolo M, Silveri MC: Can cognitive and behavioural disorders differentiate frontal variant-frontotemporal dementia from Alzheimer’s disease at early stages? Behav Neurol 2006;17:89–95.
  45. Snowden JS, Gibbons ZC, Blackshaw A, Doubleday E, Thompson J, Craufurd D, Foster J, Happe F, Neary D: Social cognition in frontotemporal dementia and Huntington’s disease. Neuropsychologia 2003;41:688–701.
  46. Kertesz A: Pick Complex: an integrative approach to frontotemporal dementia: primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy. Neurologist 2003;9:311–317.
  47. Knibb JA, Xuereb JH, Patterson K, Hodges JR: Clinical and pathological characterization of progressive aphasia. Ann Neurol 2006;59:156–165.
  48. Acciarri A, Masullo C, Bizzarro A, Valenza A, Quaranta D, Marra C, Tiziano FD, Brahe C, Seripa D, Matera MG, Fazio VM, Gainotti G, Daniele A: Apoe epsilon2-epsilon4 genotype is a possible risk factor for primary progressive aphasia. Ann Neurol 2006;59:436–437.
  49. Rosen HJ, Allison SC, Ogar JM, Amici S, Rose K, Dronkers N, Miller BL, Gorno-Tempini ML: Behavioral features in semantic dementia versus other forms of progressive aphasias. Neurology 2006;67:1752–1756.