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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Aharon-Perez J, Israel O, Peretz A (1999) Posterior cortical atrophy variants of Alzheimer’s disease. Dementi Geriatr Cogn Disord 10:483–487
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
Benson DF,Davis RJ, Snyder BD (1985) Posterior cortical atrophy. Arch Neurol 45:789–793
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
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
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
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
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
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
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
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
Goethals M, Santens P (2001) Posterior cortical atrophy. Two case reports and review of the literature. Clin Neurol Neurosurg 103:115–119
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
Herholz K (1995) FDG PET and differential diagnosis of dementia. Alzheimer Dis Assoc Disord 9:6–16
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
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
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
Jakob H (1960) Zur pathologischen Anatomie der Pick’schen Krankheit. Vergleichende Untersuchungen über Ausdehnung und Schwerpunkte der Atrophie. Archiv Psychiatrie Nervenkrankheiten 201:269–297
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
Kramer JH, Miller BL (2000) Alzheimer’s disease and its focal variants. Sem Neurol 20:447–454
Levine DN, Lee JM, Fisher CM (1993) The visual variant of Alzheimer’s Disease: a clinico-pathological case study. Neurology 43:305–313
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
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
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
Mizuno M, Sartori G, Liccione D, Battelli L, Campo R (1996) Progressive visual agnosia with posterior cortical atrophy. Clin Neurol Neurosurg 98:176–178
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
Neumann MA (1949) Pick’s Disease. J Neuropathol Exp Neurol 8:255–282
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
Pantel J, Schröder J (1996) “Posterior cortical atrophy”—ein neues Demenzsyndrom oder Sonderform des Morbus Alzheimer? Fortschr Neurol Psychiatr 64:492–508
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
Victoroff J, Ross GW, Benson DF, Verity MA, Vinters HV (1994) Posterior cortical atrophy.Neuropathological correlations. Arch Neurol 51:269–274
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
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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