Skip to main content

Advertisement

SpringerLink
Log in

The right temporal lobe variant of frontotemporal dementia

Cognitive and neuroanatomical profile of three patients

  • ORIGINAL COMMUNICATION
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

The right temporal variant of frontotemporal lobar degeneration (Rtv-FTLD) is a focal degenerative condition affecting predominantly the right temporal lobe. The aim of this study was to further characterize the profile of cognitive impairment and the neuroanatomical basis of Rtv-FTLD patients without behavioural disturbances. A group of three patients with this syndrome had a detailed neuropsychological assessment, along with Voxel-Based Morphometry (VBM) of their brain to determine location of cortical atrophy. VBM analyses showed a pattern of atrophy that was predominant in the right hemisphere and concerned primarily the right anterior temporal lobe region. Patients carried out a test of famous people in which their ability to recognize, name and provide semantic information about famous persons from their faces, their voices and their names was investigated. They all showed a severe defect in recognizing, naming and identifying famous people irrespective of modality. Therefore, their inability to recognize famous people resulted from a multimodal defect (semantic). These results highlight the semantic nature of the defect, and suggest that the anterior right temporal lobe may have a prominent role in processing person-based semantic knowledge. This study helps in further understanding the neuropsychological profile of patients with Rtv-FTLD.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Abbreviations

Rtv-FTLD::

Right temporal variant frontotemporal lobar degeneration

MMSE::

Mini-Mental State Examination

SD::

Semantic dementia

References

  1. 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 (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554

    CAS  PubMed  Google Scholar 

  2. Didic M, Ali Cherif A, Gambarelli D, Poncet M, Boudouresques J (1998) A permanent pure amnestic syndrome of insidious onset related to Alzheimer’s disease. Ann Neurol 43:526–530

    Article  CAS  PubMed  Google Scholar 

  3. Mesulam MM (1982) Slowly progressive aphasia without generalized dementia. Ann Neurol 11:592–598

    Article  CAS  PubMed  Google Scholar 

  4. Benson DF, Davis RJ, Snyder BD (1988) Posterior cortical atrophy. Arch Neurol 45:789–793

    CAS  PubMed  Google Scholar 

  5. De Renzi E (1986) Slowly progressive visual agnosia or apraxia without dementia. Cortex 22:171–180

    PubMed  Google Scholar 

  6. Snowden JS, Goulding PJ, Neary D (1989) Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 2:167–182

    Google Scholar 

  7. Hodges JR, Patterson K, Oxbury S, Funnell E (1992) Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain 115:1783–1806

    PubMed  Google Scholar 

  8. Tyrrell PJ, Warrington EK, Frackowiak RS, Rossor MN (1990) Progressive degeneration of the right temporal lobe studied with positron emission tomography. J Neurol Neurosurg Psychiatry 53:1046–1050

    Article  CAS  PubMed  Google Scholar 

  9. Evans JJ, Heggs AJ, Antoun N, Hodges JR (1995) Progressive prosopagnosia associated with selective right temporal lobe atrophy. A new syndrome? Brain 118:1–13

    PubMed  Google Scholar 

  10. Gentileschi V, Sperber S, Spinnler H (2001) Crossmodal agnosia for familiar people as a consequence of right infero-polar temporal atrophy. Cogn Neuropsychol 18:439–463

    Article  Google Scholar 

  11. Gentileschi V, Sperber S, Spinnler H (1999) Progressive defective recognition of familiar people. Neurocase 5:407–424

    Article  Google Scholar 

  12. Gainotti G, Barbier A, Marra C (2003) Slowly progressive defect in recognition of familiar people in a patient with right anterior temporal atrophy. Brain 126:792–803

    Article  PubMed  Google Scholar 

  13. Joubert S, Felician O, Barbeau E, Sontheimer A, Barton JJ, Ceccaldi M, Poncet M (2003) Impaired configurational processing in a case of progressive prosopagnosia associated with predominant right temporal lobe atrophy. Brain 126:2537–2550

    Article  PubMed  Google Scholar 

  14. Joubert S, Felician O, Barbeau E, Sontheimer A, Guedj E, Ceccaldi M, Poncet M (2004) Progressive prosopagnosia: clinical and neuroimaging results. Neurology 63:1962–1965

    CAS  PubMed  Google Scholar 

  15. Barbarotto R, Capitani E, Spinnler H, Trivelli C (1995) Slowly progressive semantic impairment with category specificity. Neurocase 1:107–119

    Article  Google Scholar 

  16. Barton JJ, Press DZ, Keenan JP, O’Connor M (2002) Lesions of the fusiform face area impair perception of facial configuration in prosopagnosia. Neurology 58:71–78

    PubMed  Google Scholar 

  17. Hanley JR, Young AW, Pearson NA (1989) Defective recognition of familiar people. Cogn Neuropsychol 6:179–210

    Google Scholar 

  18. Weschler D (1989) Echelle d’intelligence de Weschler pour Adultes, forme révisée. In: Les editions du Centre de Psychologie appliquee, Paris

  19. Weschler D, Weschler D (2001) Echelle clinique de memoire de Weschler MEM III (WMS-III). Les editions du Centre de Psychologie appliquee, Paris

    Google Scholar 

  20. Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198

    Article  CAS  PubMed  Google Scholar 

  21. Snodgrass JG, Vanderwart M (1980) A standardized set of 260 pictures: norms for name agreement, image agreement, familiarity, and visual complexity. J Exp Psychol [Hum Learn] 6:174–215

    Article  CAS  Google Scholar 

  22. Warrington EK, James M (1991) The visual object and space perception battery. Thames Valley Test Company, Bury St. Edmunds

    Google Scholar 

  23. Deloche G, Hannequin D (1997) Test de dénomination orale d’images: DO80. Les editions du Centre de Psychologie appliquée Paris

    Google Scholar 

  24. Bruce V, Young A (1986) Understanding face recognition. Br J Psychol 77:305–27

    PubMed  Google Scholar 

  25. Burton AM, Bruce V, Johnston RA (1990) Understanding face recognition with an interactive activation model. Br J Psychol l81:361–380

    Google Scholar 

  26. Snowden JS, Thompson JC, Neary D (2004) Knowledge of famous faces and names in semantic dementia. Brain 127:860–872

    Article  CAS  PubMed  Google Scholar 

  27. Damasio AR (1989) Time-locked multiregional retroactivation: a systems-level proposal for the neural substrates of recall and recognition. Cognition 33:25–62

    Article  CAS  PubMed  Google Scholar 

  28. Damasio AR (1990) Category-related recognition defects as a clue to the neural substrates of knowledge. Trends Neurosci 13:95–98

    Article  CAS  PubMed  Google Scholar 

  29. Damasio AR, Damasio H (1994) Cortical systems for retrieval of concrete knowledge: the convergence zone framework. In: Koch C (eds) Large-scale neuronal theories of the brain. Cambridge, MIT Press, pp 61–74

    Google Scholar 

  30. Kanwisher N, McDermott J, Chun MM (1997) The fusiform face area: a module in human extrastriate cortex specialized for face perception. J Neurosci 17:4302–4311

    CAS  PubMed  Google Scholar 

  31. Belin P, Zatorre RJ, Lafaille P, Ahad P, Pike B (2000) Voice-selective areas in human auditory cortex. Nature 403:309–312

    Article  CAS  PubMed  Google Scholar 

  32. Damasio H, Grabowski TJ, Tranel D, Hichwa RD, Damasio AR (1996) A neural basis for lexical retrieval. Nature 380:499–505

    Article  CAS  PubMed  Google Scholar 

  33. Thompson SA, Graham KS, Williams G, Patterson K, Kapur N, Hodges JR (2004) Dissociating person-specific from general semantic knowledge: roles of the left and right temporal lobes. Neuropsychologia 42:359–370

    Article  PubMed  Google Scholar 

  34. Thompson SA, Patterson K, Hodges JR (2003) Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. Neurology 61:1196–1203

    PubMed  Google Scholar 

  35. Edwards-Lee T, Miller BL, Benson DF, Cummings JL, Russell GL, Boone K, Mena I (1997) The temporal variant of frontotemporal dementia. Brain 120:1027–1040

    Article  PubMed  Google Scholar 

  36. Gorno-Tempini ML, Rankin KP, Woolley JD, Rosen HJ, Phengrasamy L, Miller BL (2004) Cognitive and behavioral profile in a case of right anterior temporal lobe neurodegeneration. Cortex 40:631–644

    PubMed  Google Scholar 

Download references

Acknowledgements

We are grateful CM, FC and FG who kindly contributed to this study and would like to thank them for their longstanding enthusiasm and cooperation. We are also grateful to Nathalie Walker for helpful comments.

Author information

Authors and Affiliations

  1. Centre de recherche de l’ Institut, universitaire de gériatrie, 4565, chemin Queen Mary, Montreal, Quebec, H3W 1W5, Canada

    Sven Joubert

  2. Service de Neurologie et de Neuropsychologie, AP-HM Timone, Marseille, France

    Sven Joubert, Olivier Felician, Emmanuel Barbeau, Marion Christophe, Mira Didic, Michel Poncet & Mathieu Ceccaldi

  3. Laboratoire de Neurobiologie intégrative et Adaptative, CNRS UMR 6149, Marseille, France

    Olivier Felician

  4. CERCO UMR-5549, CNRS-Université Paul Sabatier Toulouse 3 , France

    Emmanuel Barbeau

  5. Centre de Résonance Magnétique Biologique et Médicale (CRMBM), CNRS UMR 6612, Faculté de Médecine, Marseille, France

    Jean-Philippe Ranjeva

  6. Laboratoire de Neurophysiologie et de Neuropsychologie, INSERM U-751, Université de la Méditerranée, Marseille, France

    Mira Didic, Michel Poncet & Mathieu Ceccaldi

Authors
  1. Sven Joubert
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Olivier Felician
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Emmanuel Barbeau
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jean-Philippe Ranjeva
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Marion Christophe
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Mira Didic
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Michel Poncet
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mathieu Ceccaldi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sven Joubert.

Additional information

Received in revised form: 14 March 2006

Rights and permissions

Reprints and permissions

About this article

Cite this article

Joubert, S., Felician, O., Barbeau, E. et al. The right temporal lobe variant of frontotemporal dementia. J Neurol 253, 1447–1458 (2006). https://doi.org/10.1007/s00415-006-0232-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-006-0232-x

Keywords

Search

Navigation