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Posterior cortical atrophy: variant of Alzheimer’s disease?

A case series with PET findings

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Abstract

Nine patients with posterior cortical atrophy (PCA), a rare degenerative brain disease of unclear etiology and nosology, were followed over a mean time of 7.4 years. The mean age at onset was low (56.2 years). At onset, eight patients had visuo-spatial and eight had memory impairment. A minority showed early signs of occipital lobe involvement with visual agnosia or hemianopia. Eight patients developed dementia after a mean course of five years. 18F-FDG-PET data of six patients were analysed with statistical parametric mapping. They showed hypometabolism centred on the lateral and medial parietal associative cortex, with variable involvement of the adjacent temporal and occipital associative cortex. A minority showed involvement of the frontal lobes, possibly related to deafferenting of areas related to the control of eye movements. Atrophy and hypometabolism were markedly asymmetric in a subset of cases. Autopsy was performed in one patient. Presenile onset, location, and asymmetry of atrophy suggest that PCA represents a biologically separable variant of Alzheimer’s disease.

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References

  1. Aharon-Perez J, Israel O, Peretz A (1999) Posterior cortical atrophy variants of Alzheimer’s disease. Dementi Geriatr Cogn Disord 10:483–487

    Google Scholar 

  2. Azouvi P, Bergego C, Robel L, Marlier N, Durand I, Held JP, Bussel B (1993) Slowly progressive apraxia: two case studies. J Neurol 240:347–350

    Google Scholar 

  3. Benson DF,Davis RJ, Snyder BD (1985) Posterior cortical atrophy. Arch Neurol 45:789–793

    Google Scholar 

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

    Google Scholar 

  5. Bokde ALW, Pietrini P, Ibanez V, Furey ML, Alexander GE, Graff-Redford NR, Rapoport SI, Schapiro MB, Horwitz B (2001) The effect of brain atrophy on cerebral hypometabolism in the visual variant of Alzheimer’s Disease. Arch Neurol 58:480–486

    Google Scholar 

  6. Cambier J, Masson M, Dairou R, Henin D (1981) Étude anatomo-clinique d’une forme pariétale de maladie de Pick. Rev Neurol (Paris) 137:33–38

    Google Scholar 

  7. Delay J, Neveu P, Desclaux P (1944) La forme pariéto-occipitale de la maladie de Pick. Etude de l’agnosie visuelle. Rev Neurol (Paris) 76:264–265

    Google Scholar 

  8. Filoteo JV, Friedrich FJ, Rabbel C, Stricker JL (2000) Visual perception without awareness in a patient with posterior cortical atrophy: impaired explicit but not implicit processing of global information. J Int Neuropsychol Soc 8:461–472

    Google Scholar 

  9. 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

    Google Scholar 

  10. Fukui T, Sugita K, Kawamura M, Shiota J, Nakano I (1996) Primary progressive apraxia in Pick’s Disease: a clinicopathological study. Neurology 47:467–473

    Google Scholar 

  11. Galton CJ, Patterson K, Xuereb JH, Hodges JR (2000) Atypical and typical presentations of Alzheimer’s Disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 123:484–498

    Article  PubMed  Google Scholar 

  12. Goethals M, Santens P (2001) Posterior cortical atrophy. Two case reports and review of the literature. Clin Neurol Neurosurg 103:115–119

    Google Scholar 

  13. Helmer C, Joly P, Letenneur L, Commenges D, Dartigues JF (2001) Mortality with dementia: results from a French prospective community-based cohort. Am J Epidemiol 154:642–648

    Google Scholar 

  14. Herholz K (1995) FDG PET and differential diagnosis of dementia. Alzheimer Dis Assoc Disord 9:6–16

    CAS  Google Scholar 

  15. Hof PR, Bouras C, Constantinidis J, Morrison JH (1989) Balint’s syndrome in Alzheimer’s Disease: specific disruption of the occipito-parietal visual pathway. Brain Res 493:368–375

    Google Scholar 

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

    Google Scholar 

  17. Jacquet MF, Boucquey D, Theaux R, Smidts M, Callewaert A, Brucher JM, Kevers L (1990) L’atrophie corticale postérieure: variante anatomoclinique de la maladie d’Alzheimer. Acta Neurol Belg 90:265–373

    Google Scholar 

  18. Jakob H (1960) Zur pathologischen Anatomie der Pick’schen Krankheit. Vergleichende Untersuchungen über Ausdehnung und Schwerpunkte der Atrophie. Archiv Psychiatrie Nervenkrankheiten 201:269–297

    Google Scholar 

  19. Kaida K, Takeda K, Nagata N, Kamakura K (1998) Alzheimer’s disease with asymmetric parietal lobe atrophy: a case report. J Neurol Sci 160:96–99

    Google Scholar 

  20. Kramer JH, Miller BL (2000) Alzheimer’s disease and its focal variants. Sem Neurol 20:447–454

    Google Scholar 

  21. Levine DN, Lee JM, Fisher CM (1993) The visual variant of Alzheimer’s Disease: a clinico-pathological case study. Neurology 43:305–313

    Google Scholar 

  22. Lüers T, Spatz H (1957) Pick’sche Krankheit (Progressive umschriebene Großhirnatrophie). In: Lubarsch O, Henke F, Rössle R (eds) Handbuch der speziellen pathologischen Anatomie und Histologie, Vol. 13: Nervensystem. Berlin, Göttingen, Heidelberg: Springer, 1957, pp 614–715

  23. Mackenzie Ross SJ, Graham N, Stuart-Green L, Prins M, Xuareb J, Patterson K,Hodges JR (1996) Progressive biparietal atrophy: an atypical presentation of Alzheimer’s disease. J Neurol Neurosurg Psychiatry 61:388–395

    Google Scholar 

  24. Mendez MF, Ghajarania M, Perryman KM (2002) Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer’s disease. Dement Geriatr Cogn Disord 14:33–40

    Google Scholar 

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

    Google Scholar 

  26. Nestor PJ, Caine D, Fryer TD, Clarke J, Hodges JR (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

    Google Scholar 

  27. Neumann MA (1949) Pick’s Disease. J Neuropathol Exp Neurol 8:255–282

    Google Scholar 

  28. Pantel J, de Bleser R, Schwarz M, Weiller C (1995) Rapid progressive form of posterior cerebral dysfunction in a 34 year old man—another case of Haidenhein’s Syndrome? Neurocase 1:319–329

    Google Scholar 

  29. Pantel J, Schröder J (1996) “Posterior cortical atrophy”—ein neues Demenzsyndrom oder Sonderform des Morbus Alzheimer? Fortschr Neurol Psychiatr 64:492–508

    Google Scholar 

  30. Schmidtke K, Hüll M (2002) Neuropsychological differentiation of Small Vessel Disease, Alzheimer’s Disease and Mixed Dementia. J Neurol Sci 203–204(C):17–22

    Google Scholar 

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

    Google Scholar 

  32. 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

    Google Scholar 

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Authors and Affiliations

  1. Department of Psychiatry and Psychotherapy, University Hospital Freiburg, Hauptstr. 5, 79104, Freiburg, Germany

    Michael Hüll

  2. Department of Nuclear Medicine, University Hospital Freiburg, Hugstetter Str. 55, 79106, Freiburg, Germany

    Jochen Talazko

  3. Center for Geriatric Medicine and Gerontology Freiburg, Lehener Str. 88, 79106, Freiburg, Germany

    Klaus Schmidtke &  Michael Hüll

Authors
  1. Klaus Schmidtke
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  2. Michael Hüll
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  3. Jochen Talazko
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Correspondence to Klaus Schmidtke.

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Schmidtke, K., Hüll, M. & Talazko, J. Posterior cortical atrophy: variant of Alzheimer’s disease?. J Neurol 252, 27–35 (2005). https://doi.org/10.1007/s00415-005-0594-5

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  • DOI: https://doi.org/10.1007/s00415-005-0594-5

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