A Case of Alzheimer s Disease Manifesting as Posterior Cortical Atrophy

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1 A Case of Alzheimer s Disease Manifesting as Posterior Cortical Atrophy Seong Hye Choi, M.D.*, Duk L. Na, M.D., Choong-Kun Ha, M.D.*, Yeon-Lim Suh, M.D.** Department of Neurology and Pathology,** Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Neurology, College of Medicine, Inha University* A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory disturbance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F 18 -FDG-PET disclosed bilateral occipitotemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neuropathological findings consistent with Alzheimer s disease. J Korean Neurol Assoc 17(6):886~890, 1999 Key Words : Posterior cortical atrophy, Alzheimer s disease, Dementia, Balint syndrome Duk L. Na, M.D. 886 Copyright 1999 by the Korean Neurological Association

*, Duk L. Na, M.D., Choong-Kun Ha, M.D.*, Yeon-Lim Suh, M.D.** Department of Neurology and Pathology,** Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Neurology,

2 J Korean Neurol Assoc / Volume 17 / November,

3 A B Figure 1. T1-axial (A) and -sagittal (B) MR images show mild diffuse brain atrophy. Figure 2. The F 18 -FDG brain PET shows marked hypometabolism of occipitotemporoparietal cortex bilaterally, more pronounced on the right than on the left. 888 J Korean Neurol Assoc / Volume 17 / November, 1999

4 A B Figure 3. Neuritic plaques. ( A ) A neuritic plaque (arrow) in a section of temporal cortex stained with the Bodian silver technique. The plaque shows both neuritic and amyloid core components (400). ( B ) Note both core and the neuritic halo of the plaque (arrow) stained with PAS (400). ( C ) I m m u n o s t a i n i n g the specimen with anti-glial fibrillary acidic protein reveals astrocytes within the numerous plaques (arrows) and at the periphery of the numerous plaques (4 0 ). C Figure 4. Bodian silver-stained section of right temporal cortex reveals several flame-shaped neurofibrillary tangles (arrows) in viable neurons (4 0 0 ). J Korean Neurol Assoc / Volume 17 / November,

( C ) I m m u n o s t a i n i n g the specimen with anti-glial fibrillary acidic protein reveals astrocytes within the numerous plaques (arrows) and at the periphery of the

5 Posterior cortical atrophy. Neuropathologic correlations. Arch Neurol 1994 ; 51 : Mackenzie Ross SJ, Graham N, Stuart-Green L, et al. Progressive biparietal atrophy: an atypical presentation of Alzheimer s disease. J Neurol Neurosurg Psychiatry 1996 ; 61 : Hof PR, Archin N, Osmand AP, et al. Posterior cortical atrophy in Alzheimer s disease: analysis of a new case and re-evaluation of a historical report. Acta Neuropathol 1993 ; 86 : Hof PR, Vogt BA, Bouras C, Morrison JH. 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 1997 ; 37 : Benson DF, Davis RJ, Snyder BD. Posterior cortical atrophy. Arch Neurol 1988;45: Berthier ML, Leiguarda R, Starkstein SE, Sevlever G, Taratuto AL. Alzheimer s disease in a patient with posterior cortical atrophy. J Neurol Neurosurg Psychiatry 1991;54: Levine DN, Lee JM, Fisher CM, The visual variant of Alzheimer s disease: a clinicopathologic case study. N e u - rology 1993;43: Victoroff J, Ross GW, Benson DF, Verity MA, Vinters HV. 08.,,. K - MMSE ; 15 : ,. Boston Godwin-Austen RB. A case of visual disorientation. J Neurol Neurosurg Psychiatry 1965;28: ,,,. Balint ;15: Mirra SS, Heyman A, McKeel D, et al. The Consortium to establish a registry for Alzheimer s disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer s disease. Neurology 1991;41: Black SE. Focal cortical atrophy syndromes. Brain Cogn 1996;31: Zeki S, Shipp S. The functional logic of cortical connections. Nature 1988;335: Haxby JV, Grady CL, Horwitz B, et al. Dissociation of object and spatial visual processing pathways in human extrastriate cortex. Proc Natl Acad Sci USA ; 8 8 : Freedman L, Selchen DH, Black SE, Kaplan R, Garnett ES, Nahmias C. Posterior cortical dementia with alexia: neurobehavioural, MRI, and PET findings. J Neurol Neurosurg Psychiatry 1991;54: Beversdorf DQ, Heilman KM. Progressive ventral posterior cortical degeneration presenting as alexia for music and words. Neurology 1998;50: Wakai M, Honda H, Takahashi A, Kato T, Ito K, Hamanaka T. Unusual findings on PET study of a patient with posterior cortical atrophy. Acta Neurol Scand ; 8 9 : Ala TA, Frey II WH. Posterior cortical atrophy: neuropathological correlations. Arch Neurol 1996;53: J Korean Neurol Assoc / Volume 17 / November, 1999

Posterior cortical atrophy in Alzheimer s disease: analysis of a new case and re-evaluation of a historical report. Acta Neuropathol 1993 ; 86 : 215-223. 07. Hof PR, Vogt BA, Bouras C, Morrison JH.

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