Skip to main content

Advertisement

SpringerLink
Log in

The neuropsychological and neuroradiological correlates of slowly progressive visual agnosia

  • Case Report
  • Published:
Neurological Sciences Aims and scope Submit manuscript

Abstract

The case of a 64-year-old woman affected by slowly progressive visual agnosia is reported aiming to describe specific cognitive-brain relationships. Longitudinal clinical and neuropsychological assessment, combined with magnetic resonance imaging (MRI), spectroscopy, and positron emission tomography (PET) were used. Sequential neuropsychological evaluations performed during a period of 9 years since disease onset showed the appearance of apperceptive and associative visual agnosia, alexia without agraphia, agraphia, finger agnosia, and prosopoagnosia, but excluded dementia. MRI showed moderate diffuse cortical atrophy, with predominant atrophy in the left posterior cortical areas (temporal, parietal, and lateral occipital cortical gyri). 18FDG-PET showed marked bilateral posterior cortical hypometabolism; proton magnetic resonance spectroscopic imaging disclosed severe focal N-acetyl-aspartate depletion in the left temporoparietal and lateral occipital cortical areas. In conclusion, selective metabolic alterations and neuronal loss in the left temporoparietooccipital cortex may determine progressive visual agnosia in the absence of dementia.

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

References

  1. Lissauer H (1890) Ein fall von seelenblindhelt nebst einen beitrag zur theorie derselben. Arch Psychiatr 27:222–270

    Article  Google Scholar 

  2. Warrington EK, James M (1986) Visual object recognition in patients with right hemisphere lesion: axes or features? Perception 15:355–356

    Article  PubMed  CAS  Google Scholar 

  3. Warrington EK, James M (1988) Visual apperceptive agnosia: a clinico-anatomical study of three cases. Cortex 24:13–32

    PubMed  CAS  Google Scholar 

  4. Warrington EK (1975) The selective impairment of semantic memory. Q J Exp Psychol 27:635–657

    Article  PubMed  CAS  Google Scholar 

  5. Davidoff J, Wilson B (1985) A case of visual agnosia showing a disorder of pre-semantic visual classification. Cortex 21:121–134

    PubMed  CAS  Google Scholar 

  6. Feinberg TE, Schindler RJ, Ochoa E, Kwan PC, Farah JM (1994) Associative visual agnosia and alexia without prosopoagnosia. Cortex 30:395–412

    PubMed  CAS  Google Scholar 

  7. De Renzi E, Lucchelli F (1993) The fuzzy boundaries of apperceptive agnosia. Cortex 29:187–215

    PubMed  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  9. Sartori G, Job R, Miozzo M, Zago S, Marchiori G (1993) Category-specific form-knowledge in a patient with herpes simplex virus encephalitis. Neuropsychologia 2:280–299

    Google Scholar 

  10. Benson DF, Greenberg J (1969) Visual form agnosia. Arch Neurol 20:82–89

    PubMed  CAS  Google Scholar 

  11. Hécaen H, Albert M (1978) Human neuropsychology. Wiley, New York

    Google Scholar 

  12. Tang-Wai D, Mapstone M (2006) What are we seeing? Is posterior cortical atrophy just Alzheimer’s disease? Neurology 66:300–301

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  Google Scholar 

  15. Della Sala S, Spinnler H, Trivelli C (1996) Slowly progressive impairment of spatial exploration and visual perception. Neurocase 2:299–323

    Article  Google Scholar 

  16. Crystal HA, Horoupian DS, Katzman R, Jotkowitz S (1992) Biopsy-proved Alzheimer disease presenting as a right parietal lobe syndrome. Ann Neurol 12:186–188

    Article  Google Scholar 

  17. Rosenfeld M (1909) Die Partielle Grosshirnatrophie. J Psychol Neurol 14:115–130

    Google Scholar 

  18. Tang-Wai DF, Graff-Radford NR, Boeve BF et al (2004) Clinical, genetic, and neuropathological characteristics of posterior cortical atrophy. Neurology 63:1168–1174

    PubMed  CAS  Google Scholar 

  19. Kirshner HS, Lavin PJM (2006) Posterior cortical atrophy: a brief review. Curr Neurol Neurosci Rep 6:477–480

    Article  PubMed  Google Scholar 

  20. Luzzatti C, Willmes K, De Bleser R (1991) Aachener Aphasie Test. Versione italiana. Organizzazioni Speciali, Florence

    Google Scholar 

  21. Spinnler H, Tognoni G (1987) Standardizzazione e taratura italiana di test neuropsicologici. Ital J Neurol Sci 6(Suppl 8):1–120

    Google Scholar 

  22. Basso A, Capitani E, Laiacona M (1987) Raven’s coloured progressive matrices: normative data on 305 adult normal controls. Funct Neurol 2:189–194

    PubMed  CAS  Google Scholar 

  23. Weigl E (1927) On the psychology of so-called processes of abstraction. J Abnorm Soc Psychol 36:3–33

    Google Scholar 

  24. Novelli G, Papagno C, Capitani E, Laiacona M, Vallar G, Cappa SF (1986) Tre test clinici di ricerca e produzione lessicale. Taratura su soggetti normali. Arch Psicol Neurol Psichiatr 47:447–506

    Google Scholar 

  25. De Renzi E, Pieczuro E, Vignolo LA (1966) Oral apraxia and aphasia. Cortex 2:50–73

    Google Scholar 

  26. De Renzi E, Motti F, Nichelli P (1980) Imitating gestures. A quantitative approach to ideomotor apraxia. Arch Neurol 37:6–10

    PubMed  Google Scholar 

  27. Arrigoni C, De Renzi E (1964) Constructional apraxia and hemispheric locus of lesion. Cortex 1:170–197

    Google Scholar 

  28. Lezak M (1995) Neuropsychological assessment. Oxford University Press, New York

    Google Scholar 

  29. Novelli G, Papagno C, Capitani E, Laiacona M, Cappa SF, Vallar G (1986) Tre test clinici di memoria verbale a lungo termine. Taratura su soggetti normali. Arch Psicol Neurol Psichiatr 47:278–296

    Google Scholar 

  30. Riddoch MJ, Humphreys GW (1993) BORB: Birmingham Object Recognition Battery. Lawrence Erlbaum Associates, Hove

    Google Scholar 

  31. Benton AL, Varney NR, Hamsher K de S (1990) Test di orientamento di linee. Organizzazioni Speciali, Florence

  32. Benton AL, Hamsher K de S, Varney NR, Spreen O (1993) Test di riconoscimento di volti ignoti. Organizzazioni Speciali, Florence

  33. Trojano L, Grossi D (1992) Impaired drawing from memory in a visual agnosic patients. Brain Cogn 20:327–344

    Article  PubMed  CAS  Google Scholar 

  34. Riddoch MJ, Humpreys GW, Gannon T, Blott W, Jones V (1999) Memories are made of this: the effects of time on stored visual knowledge in a case of visual agnosia. Brain 122:537–559

    Article  PubMed  Google Scholar 

  35. Wilson BA, Davidoff J (1993) Partial recovery from visual object agnosia: a 10-year follow-up study. Cortex 29:529–542

    PubMed  CAS  Google Scholar 

  36. Magnani G, Bettoni L, Mazzucchi A (1982) Lesioni atrofiche bioccipitali di incerta natura all’origine di una agnosia visiva. Riv Neurol 52:137–148

    PubMed  CAS  Google Scholar 

  37. Mizuno M, Sartori G, Liccione D, Battelli L, Campo R (1996) Progressive visual agnosia with posterior cortical atrophy. Clin Neurol Neurosurg 98:176–178

    Article  PubMed  CAS  Google Scholar 

  38. Grunthal E (1926) Uber die Alzheimersche Krankheit: eine histopathologisch-klinische Studie. Z Ges Neurol Psychiatry 101:128–157

    Article  Google Scholar 

  39. Wakai M, Honda H, Takahashi A, Kato T, Ito K, Hamanaka T (1994) Unusual findings on PET study of a patient with posterior cortical atrophy. Acta Neurol Scand 89:458–461

    Article  PubMed  CAS  Google Scholar 

  40. Attig E, Jacquy J, Uytdenhoef P, Roland H (1993) Progressive focal degenerative disease of the posterior associative cortex. Can J Neurol Sci 20:154–157

    PubMed  CAS  Google Scholar 

  41. McMonagle P, Deering F, Berliner Y, Kertesz A (2006) The cognitive profile of posterior cortical atrophy. Neurology 66:331–338

    Article  PubMed  Google Scholar 

  42. Mendez MF, Tomsak RL, Remler B (1990) Disorders of the visual system in Alzheimer’s disease. J Clin Neuroophthalmol 10:62–69

    PubMed  CAS  Google Scholar 

  43. Hof PR, Vogt BA, Bouras C, Morrison JH (1997) Atypical form of Alzheimer’s disease with prominent posterior cortical atrophy: a review of lesion distribution and circuit disconnection in cortical visual pathways. Vision Res 37:3609–3625

    Article  PubMed  CAS  Google Scholar 

  44. Giannakopoulos P, Gold G, Duc M, Michel JP, Hof PR, Bouras C (1999) Neuroanatomic correlates of visual agnosia in Alzheimer’s disease: a clinico-pathologic study. Neurology 52:71–77

    PubMed  CAS  Google Scholar 

  45. Farah MJ (1984) The neurological basis of mental imagery: a componential analysis. Cognition 18:245–272

    Article  PubMed  CAS  Google Scholar 

  46. Renner JA, Burns JM, Hou CE et al (2004) Progressive posterior cortical dysfunction. A clinical pathologic series. Neurology 63:1175–1180

    PubMed  CAS  Google Scholar 

  47. Berthier ML, Leiguarda R, Stakstein SE, Sevlever G, Taratuto AL (1991) Alzheimer’s disease in a patient with posterior cortical atrophy. J Neurol Neurosurg Psychiatry 54:1110–1111

    Article  PubMed  CAS  Google Scholar 

  48. Hof PR, Archin N, Osmand AP, Dougherthy JK, Wells C, Bouras C, Morrison JK (1993) Posterior cortical atrophy in Alzheimer’s disease: analysis of a new case and re-evaluation of a historical report. Acta Neuropathol 86:215–223

    Article  PubMed  CAS  Google Scholar 

  49. Meyer A, Leigh D, Bagg CE (1954) A rare pre-senile dementia associated whit cortical blindness (Heidenhain’s syndrome). J Neurol Neurosurg Psychiatry 17:129–133

    Article  PubMed  CAS  Google Scholar 

  50. Victoroff J, Ross W, Benson DF, Verity MA, Vinters HV (1994) Posterior cortical atrophy. Neuropathologic correlations. Arch Neurol 51:269–274

    PubMed  CAS  Google Scholar 

  51. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192

    Article  PubMed  Google Scholar 

  52. Simmons M, Frondoza C, Coyle J (1991) Immunocytochemical localization of N-acetyl-aspartate with monoclonal antibodies. Neuroscience 45:37–45

    Article  PubMed  CAS  Google Scholar 

  53. Pfefferbaum A, Adalsteinsson E, Spielman D, Sullival EV, Lim KO (1999) In vivo brain concentrations of N-acetyl compounds, creatin and choline in Alzheimer disease. Arch Gen Psychiatry 56:185–192

    Article  PubMed  CAS  Google Scholar 

  54. Chawluk JB, Mesulam MM, Hurtig H et al (1986) Slowly progressive aphasia without generalised dementia: studies with positron emission tomography. Ann Neurol 19:68–74

    Article  PubMed  CAS  Google Scholar 

  55. Leger JM, Levasseur M, Benoit N et al (1991) Apraxie d’apparition lentement progressive: etude par IRM et tomographie a positions dans 4 cas. Rev Neurol 147:183–191

    PubMed  CAS  Google Scholar 

  56. Freedman L, Selchen DH, Black SE, Kaplan R, Garnett ES, Nahmias C (1991) Posterior cortical dementia with alexia: neurobehavioural, MRI, and PET findings. J Neurol Neurosurg Psychiatry 54:443–448

    Article  PubMed  CAS  Google Scholar 

  57. Nestor PJ, Caine D, Fryer TD et al (2003) The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer’s disease) with FDG-PET. J Neurol Neurosurg Psychiatry 74:1521–1529

    Article  PubMed  CAS  Google Scholar 

  58. Bauer RM, Rubens AB (1985) Agnosia. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 187–241

    Google Scholar 

  59. Friedman RB, Albert ML (1985) Alexia. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 49–73

    Google Scholar 

  60. Geschwind N (1965) Disconnection syndromes in animals and man. Brain 88:237–294

    Article  PubMed  CAS  Google Scholar 

  61. Vignolo LA (1972) Les deux niveaux de l’agnosie. In: Hécaen H (ed) Neuropsychologie de la Perception Visuelle. Masson, Paris, pp 222–240

    Google Scholar 

  62. Benton A (1985) Body schema disturbances: finger agnosia and right–left disorientation. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 116–121

    Google Scholar 

  63. De Renzi E (1990) Le agnosie. In: Denes G, Pizzamiglio L (eds) Manuale di neuropsicologia. Zanichelli, Bologna, pp 645–702

    Google Scholar 

  64. Benton A (1985) Visuoperceptual, visuospatial and visuoconstructive disorders. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 169–171

    Google Scholar 

  65. Benton A (1985) Visuoperceptual, visuospatial and visuoconstructive disorders. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 159–163

    Google Scholar 

  66. 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

    Article  PubMed  Google Scholar 

  67. Dricot L, Sorger B, Schiltz C, Goebel R, Rossion B (2008) Evidence for individual face discrimination in non-face selective areas of the visual cortex in acquired prosopagnosia. Behav Neurol 19:75–79

    PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  69. Joubert S, Felician O, Barbeau E, Ranjeva JP, Cristophe M, Didic M, Poncet M, Ceccaldi M (2006) The right temporal lobe variant of frontotemporal dementia: cognitive and neuroanatomical profile of three patients. J Neurol 253:1447–1458

    Article  PubMed  Google Scholar 

  70. Josephs KA, Whitwell JL, Vemuri P, Senjem ML, Boeve BF, Knopman DS, Smith GE, Ivnik RJ, Petersen RC, Jack CR Jr (2008) The anatomic correlate of prosopagnosia in semantic dementia. Neurology 71:1628–1633

    Article  PubMed  CAS  Google Scholar 

  71. Barton JJ (2008) Prosopagnosia associated with a left occipitotemporal lesion. Neuropsychologia 46:2214–2224

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neuropsychology Laboratory, Department of Clinical Neurosciences, “C. Besta” National Neurological Institute, Via Celoria 11, 20133, Milan, Italy

    Anna Rita Giovagnoli, Anna Aresi, Fabiola Reati & Alice Riva

  2. Division of Nuclear Medicine, S. Antonio Hospital, Gallarate, Italy

    Clara Gobbo

  3. Division of Neuroradiology, Department of Experimental and Diagnostic Research, “C. Besta” National Neurological Institute, Milan, Italy

    Alberto Bizzi

Authors
  1. Anna Rita Giovagnoli
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Anna Aresi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Fabiola Reati
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alice Riva
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Clara Gobbo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Alberto Bizzi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anna Rita Giovagnoli.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Giovagnoli, A.R., Aresi, A., Reati, F. et al. The neuropsychological and neuroradiological correlates of slowly progressive visual agnosia. Neurol Sci 30, 123–131 (2009). https://doi.org/10.1007/s10072-009-0019-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10072-009-0019-9

Keywords

Search

Navigation