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Neurological Sciences

, Volume 30, Issue 2, pp 123–131 | Cite as

The neuropsychological and neuroradiological correlates of slowly progressive visual agnosia

  • Anna Rita Giovagnoli
  • Anna Aresi
  • Fabiola Reati
  • Alice Riva
  • Clara Gobbo
  • Alberto Bizzi
Case Report

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.

Keywords

Slowly progressive visual agnosia Dementia Cognitive impairment Neuroimaging 

References

  1. 1.
    Lissauer H (1890) Ein fall von seelenblindhelt nebst einen beitrag zur theorie derselben. Arch Psychiatr 27:222–270CrossRefGoogle Scholar
  2. 2.
    Warrington EK, James M (1986) Visual object recognition in patients with right hemisphere lesion: axes or features? Perception 15:355–356PubMedCrossRefGoogle Scholar
  3. 3.
    Warrington EK, James M (1988) Visual apperceptive agnosia: a clinico-anatomical study of three cases. Cortex 24:13–32PubMedGoogle Scholar
  4. 4.
    Warrington EK (1975) The selective impairment of semantic memory. Q J Exp Psychol 27:635–657PubMedCrossRefGoogle Scholar
  5. 5.
    Davidoff J, Wilson B (1985) A case of visual agnosia showing a disorder of pre-semantic visual classification. Cortex 21:121–134PubMedGoogle Scholar
  6. 6.
    Feinberg TE, Schindler RJ, Ochoa E, Kwan PC, Farah JM (1994) Associative visual agnosia and alexia without prosopoagnosia. Cortex 30:395–412PubMedGoogle Scholar
  7. 7.
    De Renzi E, Lucchelli F (1993) The fuzzy boundaries of apperceptive agnosia. Cortex 29:187–215PubMedGoogle Scholar
  8. 8.
    Benson DF, Davis RJ, Snyder BD (1988) Posterior cortical atrophy. Arch Neurol 45:789–793PubMedGoogle Scholar
  9. 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 2:280–299Google Scholar
  10. 10.
    Benson DF, Greenberg J (1969) Visual form agnosia. Arch Neurol 20:82–89PubMedGoogle Scholar
  11. 11.
    Hécaen H, Albert M (1978) Human neuropsychology. Wiley, New YorkGoogle Scholar
  12. 12.
    Tang-Wai D, Mapstone M (2006) What are we seeing? Is posterior cortical atrophy just Alzheimer’s disease? Neurology 66:300–301PubMedCrossRefGoogle Scholar
  13. 13.
    Mesulam MM (1982) Slowly progressive aphasia without generalised dementia. Ann Neurol 11:592–598PubMedCrossRefGoogle Scholar
  14. 14.
    De Renzi E (1986) Slowly progressive visual agnosia or apraxia without dementia. Cortex 22:171–180PubMedGoogle Scholar
  15. 15.
    Della Sala S, Spinnler H, Trivelli C (1996) Slowly progressive impairment of spatial exploration and visual perception. Neurocase 2:299–323CrossRefGoogle Scholar
  16. 16.
    Crystal HA, Horoupian DS, Katzman R, Jotkowitz S (1992) Biopsy-proved Alzheimer disease presenting as a right parietal lobe syndrome. Ann Neurol 12:186–188CrossRefGoogle Scholar
  17. 17.
    Rosenfeld M (1909) Die Partielle Grosshirnatrophie. J Psychol Neurol 14:115–130Google Scholar
  18. 18.
    Tang-Wai DF, Graff-Radford NR, Boeve BF et al (2004) Clinical, genetic, and neuropathological characteristics of posterior cortical atrophy. Neurology 63:1168–1174PubMedGoogle Scholar
  19. 19.
    Kirshner HS, Lavin PJM (2006) Posterior cortical atrophy: a brief review. Curr Neurol Neurosci Rep 6:477–480PubMedCrossRefGoogle Scholar
  20. 20.
    Luzzatti C, Willmes K, De Bleser R (1991) Aachener Aphasie Test. Versione italiana. Organizzazioni Speciali, FlorenceGoogle Scholar
  21. 21.
    Spinnler H, Tognoni G (1987) Standardizzazione e taratura italiana di test neuropsicologici. Ital J Neurol Sci 6(Suppl 8):1–120Google Scholar
  22. 22.
    Basso A, Capitani E, Laiacona M (1987) Raven’s coloured progressive matrices: normative data on 305 adult normal controls. Funct Neurol 2:189–194PubMedGoogle Scholar
  23. 23.
    Weigl E (1927) On the psychology of so-called processes of abstraction. J Abnorm Soc Psychol 36:3–33Google Scholar
  24. 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 47:447–506Google Scholar
  25. 25.
    De Renzi E, Pieczuro E, Vignolo LA (1966) Oral apraxia and aphasia. Cortex 2:50–73Google Scholar
  26. 26.
    De Renzi E, Motti F, Nichelli P (1980) Imitating gestures. A quantitative approach to ideomotor apraxia. Arch Neurol 37:6–10PubMedGoogle Scholar
  27. 27.
    Arrigoni C, De Renzi E (1964) Constructional apraxia and hemispheric locus of lesion. Cortex 1:170–197Google Scholar
  28. 28.
    Lezak M (1995) Neuropsychological assessment. Oxford University Press, New YorkGoogle Scholar
  29. 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 47:278–296Google Scholar
  30. 30.
    Riddoch MJ, Humphreys GW (1993) BORB: Birmingham Object Recognition Battery. Lawrence Erlbaum Associates, HoveGoogle Scholar
  31. 31.
    Benton AL, Varney NR, Hamsher K de S (1990) Test di orientamento di linee. Organizzazioni Speciali, Florence Google Scholar
  32. 32.
    Benton AL, Hamsher K de S, Varney NR, Spreen O (1993) Test di riconoscimento di volti ignoti. Organizzazioni Speciali, Florence Google Scholar
  33. 33.
    Trojano L, Grossi D (1992) Impaired drawing from memory in a visual agnosic patients. Brain Cogn 20:327–344PubMedCrossRefGoogle Scholar
  34. 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 122:537–559PubMedCrossRefGoogle Scholar
  35. 35.
    Wilson BA, Davidoff J (1993) Partial recovery from visual object agnosia: a 10-year follow-up study. Cortex 29:529–542PubMedGoogle Scholar
  36. 36.
    Magnani G, Bettoni L, Mazzucchi A (1982) Lesioni atrofiche bioccipitali di incerta natura all’origine di una agnosia visiva. Riv Neurol 52:137–148PubMedGoogle Scholar
  37. 37.
    Mizuno M, Sartori G, Liccione D, Battelli L, Campo R (1996) Progressive visual agnosia with posterior cortical atrophy. Clin Neurol Neurosurg 98:176–178PubMedCrossRefGoogle Scholar
  38. 38.
    Grunthal E (1926) Uber die Alzheimersche Krankheit: eine histopathologisch-klinische Studie. Z Ges Neurol Psychiatry 101:128–157CrossRefGoogle Scholar
  39. 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 89:458–461PubMedCrossRefGoogle Scholar
  40. 40.
    Attig E, Jacquy J, Uytdenhoef P, Roland H (1993) Progressive focal degenerative disease of the posterior associative cortex. Can J Neurol Sci 20:154–157PubMedGoogle Scholar
  41. 41.
    McMonagle P, Deering F, Berliner Y, Kertesz A (2006) The cognitive profile of posterior cortical atrophy. Neurology 66:331–338PubMedCrossRefGoogle Scholar
  42. 42.
    Mendez MF, Tomsak RL, Remler B (1990) Disorders of the visual system in Alzheimer’s disease. J Clin Neuroophthalmol 10:62–69PubMedGoogle Scholar
  43. 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 37:3609–3625PubMedCrossRefGoogle Scholar
  44. 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 52:71–77PubMedGoogle Scholar
  45. 45.
    Farah MJ (1984) The neurological basis of mental imagery: a componential analysis. Cognition 18:245–272PubMedCrossRefGoogle Scholar
  46. 46.
    Renner JA, Burns JM, Hou CE et al (2004) Progressive posterior cortical dysfunction. A clinical pathologic series. Neurology 63:1175–1180PubMedGoogle Scholar
  47. 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 54:1110–1111PubMedCrossRefGoogle Scholar
  48. 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 86:215–223PubMedCrossRefGoogle Scholar
  49. 49.
    Meyer A, Leigh D, Bagg CE (1954) A rare pre-senile dementia associated whit cortical blindness (Heidenhain’s syndrome). J Neurol Neurosurg Psychiatry 17:129–133PubMedCrossRefGoogle Scholar
  50. 50.
    Victoroff J, Ross W, Benson DF, Verity MA, Vinters HV (1994) Posterior cortical atrophy. Neuropathologic correlations. Arch Neurol 51:269–274PubMedGoogle Scholar
  51. 51.
    Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192PubMedCrossRefGoogle Scholar
  52. 52.
    Simmons M, Frondoza C, Coyle J (1991) Immunocytochemical localization of N-acetyl-aspartate with monoclonal antibodies. Neuroscience 45:37–45PubMedCrossRefGoogle Scholar
  53. 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 56:185–192PubMedCrossRefGoogle Scholar
  54. 54.
    Chawluk JB, Mesulam MM, Hurtig H et al (1986) Slowly progressive aphasia without generalised dementia: studies with positron emission tomography. Ann Neurol 19:68–74PubMedCrossRefGoogle Scholar
  55. 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 147:183–191PubMedGoogle Scholar
  56. 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 54:443–448PubMedCrossRefGoogle Scholar
  57. 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 74:1521–1529PubMedCrossRefGoogle Scholar
  58. 58.
    Bauer RM, Rubens AB (1985) Agnosia. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 187–241Google Scholar
  59. 59.
    Friedman RB, Albert ML (1985) Alexia. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 49–73Google Scholar
  60. 60.
    Geschwind N (1965) Disconnection syndromes in animals and man. Brain 88:237–294PubMedCrossRefGoogle Scholar
  61. 61.
    Vignolo LA (1972) Les deux niveaux de l’agnosie. In: Hécaen H (ed) Neuropsychologie de la Perception Visuelle. Masson, Paris, pp 222–240Google Scholar
  62. 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 116–121Google Scholar
  63. 63.
    De Renzi E (1990) Le agnosie. In: Denes G, Pizzamiglio L (eds) Manuale di neuropsicologia. Zanichelli, Bologna, pp 645–702Google Scholar
  64. 64.
    Benton A (1985) Visuoperceptual, visuospatial and visuoconstructive disorders. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 169–171Google Scholar
  65. 65.
    Benton A (1985) Visuoperceptual, visuospatial and visuoconstructive disorders. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology. Oxford University Press, New York, pp 159–163Google Scholar
  66. 66.
    Evans JJ, Heggs AJ, Antoun N, Hodges JR (1995) Progressive prosopagnosia associated with selective right temporal lobe atrophy. A new syndrome? Brain 118:1–13PubMedCrossRefGoogle Scholar
  67. 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 19:75–79PubMedGoogle Scholar
  68. 68.
    Snowden JS, Thompson JC, Neary D (2004) Knowledge of famous faces and names in semantic dementia. Brain 127:860–872PubMedCrossRefGoogle Scholar
  69. 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 253:1447–1458PubMedCrossRefGoogle Scholar
  70. 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 71:1628–1633PubMedCrossRefGoogle Scholar
  71. 71.
    Barton JJ (2008) Prosopagnosia associated with a left occipitotemporal lesion. Neuropsychologia 46:2214–2224PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Anna Rita Giovagnoli
    • 1
  • Anna Aresi
    • 1
  • Fabiola Reati
    • 1
  • Alice Riva
    • 1
  • Clara Gobbo
    • 2
  • Alberto Bizzi
    • 3
  1. 1.Neuropsychology Laboratory, Department of Clinical Neurosciences“C. Besta” National Neurological InstituteMilanItaly
  2. 2.Division of Nuclear MedicineS. Antonio HospitalGallarateItaly
  3. 3.Division of Neuroradiology, Department of Experimental and Diagnostic Research“C. Besta” National Neurological InstituteMilanItaly

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