Research Article
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Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features

Year 2024, Volume: 41 Issue: 1, 40 - 46, 29.03.2024

Abstract

Posterior cortical atrophy (PCA) represents a rare variant of degenerative dementia, predominantly marked by visuospatial deficits and cognitive impairment. The diagnostic complexity consist of atypical symptoms at onset and non-specific neuroimaging findings. In this retrospective case study, we investigated clinical, neuropsychological, and imaging aspects in five cases with pure PCA. 754 patients who presented to the dementia outpatient clinic of Çukurova University Faculty of Medicine Neurology Department between 2013-2022 were retrospectively analyzed, and 5 cases diagnosed with PCA were included in the study. Mini-Mental State Examination (MMSE), clock drawing test (CDT), cookie thief test, simultanognosy tests (Arcimboldo pictures, Navon letters), and imaging findings of the cases were documented. The average age of the subjects was 60.6 years, with an onset typically occurring at a presenile stage. Simultanagnosia was a consistent finding in all patients, with some exhibiting additional visuospatial deficits. Mini-Mental State Examination (MMSE) scores averaged 20.20, and clock drawing test results indicated impairment. Notably, 40% of the patients displayed depressive symptoms, often alongside visual complaints as a common presentation. Crucially, all patients retained speech fluency. Our findings underscore the significance of employing neurocognitive assessments, particularly the clock drawing test, when evaluating patients with complaints related to visuospatial and cognitive functions. Early diagnosis and intervention are pivotal for effectively managing PCA.

References

  • Cerami C, Crespi C, Della Rosa PA, Dodich A, Marcone A, Magnani G et al. Brain changes within the visuo-spatial attentional network in posterior cortical atrophy. J Alzheimers Dis. 2015;43(2):385-95.
  • Torso M, Ahmed S, Butler C, Zamboni G, Jenkinson M, Chance S. Cortical diffusivity investigation in posterior cortical atrophy and typical Alzheimer's disease. J Neurol. 2021 Jan;268(1):227-239.
  • Esteves S, Ramirez Romero DA, Torralva T, Martínez Cuitiño M, Herndon S, Couto B et al. Posterior cortical atrophy: a single case cognitive and radiological follow-up. Neurocase. 2018 Feb;24(1):16-30.
  • Solyga VM, Western E, Solheim H, Hassel B, Kerty E. Posterior kortikal atrofi [Posterior cortical atrophy]. Tidsskr Nor Laegeforen. 2015 Jun 2;135(10):949-52.
  • DE Brito MH, Zampieri PF, Coutinho AM, Brucki SMD. Brain [18F]FDG PET-CT imaging in posterior cortical atrophy. Arq Neuropsiquiatr. 2019 Oct 24;77(10):752-753.
  • Whitwell JL, Graff-Radford J, Singh TD, Drubach DA, Senjem ML, Spychalla AJ et al. 18F-FDG PET in Posterior Cortical Atrophy and Dementia with Lewy Bodies. J Nucl Med. 2017 Apr;58(4):632-638.
  • Holden SK, Bettcher BM, Pelak VS. Update on posterior cortical atrophy. Curr Opin Neurol. 2020 Feb;33(1):68-73.
  • North C, Desai R, Saunders R, Suárez-González A, Bamiou D, Costafreda SG et al. Neuropsychological deficits in Posterior Cortical Atrophy and typical Alzheimer's disease: A meta-analytic review. Cortex. 2021 Oct;143:223-236.
  • Neitzel J, Ortner M, Haupt M, Redel P, Grimmer T, Yakushev I et al. Neuro-cognitive mechanisms of simultanagnosia in patients with posterior cortical atrophy. Brain. 2016 Dec;139(Pt 12):3267-3280.
  • Crutch SJ, Schott JM, Rabinovici GD, Murray M, Snowden JS, van der Flier WM et al. Alzheimer's Association ISTAART Atypical Alzheimer's Disease and Associated Syndromes Professional Interest Area. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017 Aug;13(8):870-884.
  • Keskinoglu P, Ucku R, Yener G, Yaka E, Kurt P, Tunca Z. Reliability and validity of revised Turkish version of Mini Mental State Examination (rMMSE-T) in community-dwelling educated and uneducated elderly. Int J Geriatr Psychiatry. 2009 Nov;24(11):1242-50.
  • Manos PJ, Wu R. The ten point clock test: a quick screen and grading method for cognitive impairment in medical and surgical patients. Int J Psychiatry Med. 1994;24(3):229-44.
  • Nyffeler T, Pflugshaupt T, Hofer H, Baas U, Gutbrod K, von Wartburg R, Hess CW, Müri RM. Oculomotor behaviour in simultanagnosia: a longitudinal case study. Neuropsychologia. 2005;43(11):1591-7.
  • Kubon J, Sokolov AN, Popp R, Fallgatter AJ, Pavlova MA. Face Tuning in Depression. Cereb Cortex. 2021 Mar 31;31(5):2574-2585.
  • Navon D.Forest before the trees:the precedence of global features in visual perception. Cogn Psychol 1977; 9(3):353-83
  • Saleh C, Seidl U, Stegentritt K, Schumacher F, Fehrenbach RA. Posteriores kortikales Atrophie-Plus-Syndrom. Eine Kasuistik [Posterior cortical atrophy-plus syndrome. A case report]. Fortschr Neurol Psychiatr. 2020 Aug;88(8):528-531.
  • Mukku SSR, Chintala H, Nagaraj C, Mangalore S, Sivakumar PT, Varghese M. Posterior cortical atrophy variant of Alzheimer's dementia-A case report. Asian J Psychiatr. 2018 Jun;35:109-112.
  • Giunta M, Libri I, Premi E, Brattini C, Paghera B, Archetti S et al. Clinical and radiological features of posterior cortical atrophy (PCA) in a GRN mutation carrier: a case report. Eur J Neurol. 2021 Jan;28(1):344-348.
  • Shebani Z, Nestor PJ, Pulvermüller F. What's "up"? Impaired Spatial Preposition Processing in Posterior Cortical Atrophy. Front Hum Neurosci. 2021 Dec 1;15:731104.
  • Colakoglu BD, Kurt P, Kaya GC, Yener G. A Distinct dementia syndrome: posterior cortical atrophy: clinical and cognitive assesment, and brain spect findings Archives of Neuropsychiatry, 2010;47(3):201-207.
  • Tang-Wai DF, Graff-Radford NR, Boeve BF, Dickson DW, Parisi JE, Crook R et al. Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy. Neurology. 2004 Oct 12;63(7):1168-74.
  • Wong B, Lucente DE, MacLean J, Padmanabhan J, Quimby M, Brandt KD et al. Diagnostic evaluation and monitoring of patients with posterior cortical atrophy. Neurodegener Dis Manag. 2019 Aug;9(4):217-239.
  • Mendez MF, Ghajarania M, Perryman KM. Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer's disease. Dement Geriatr Cogn Disord. 2002;14(1):33-40.
  • Suárez-González A, Crutch SJ, Franco-Macías E, Gil-Néciga E. Neuropsychiatric Symptoms in Posterior Cortical Atrophy and Alzheimer Disease. J Geriatr Psychiatry Neurol. 2016 Mar;29(2):65-71.
  • Aharon-Peretz J, Israel O, Goldsher D, Peretz A. Posterior cortical atrophy variants of Alzheimer's disease. Dement Geriatr Cogn Disord. 1999 Nov-Dec;10(6):483-7.
  • Schmidtke K, Hüll M, Talazko J. Posterior cortical atrophy: variant of Alzheimer's disease? A case series with PET findings. J Neurol. 2005 Jan;252(1):27-35.
  • Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Tang-Wai DF, Drubach DA et al. Visual hallucinations in posterior cortical atrophy. Arch Neurol. 2006 Oct;63(10):1427-32.
  • Yerstein O, Parand L, Liang LJ, Isaac A, Mendez MF. Benson's Disease or Posterior Cortical Atrophy, Revisited. J Alzheimers Dis. 2021;82(2):493-502.
  • Singh TD, Josephs KA, Machulda MM, Drubach DA, Apostolova LG, Lowe VJ, Whitwell JL. Clinical, FDG and amyloid PET imaging in posterior cortical atrophy. J Neurol. 2015 Jun;262(6):1483-92
  • Agosta F, Mandic-Stojmenovic G, Canu E, Stojkovic T, Imperiale F, Caso F, Stefanova E, Copetti M, Kostic VS, Filippi M. Functional and structural brain networks in posterior cortical atrophy: A two-centre multiparametric MRI study. Neuroimage Clin. 2018 Jun 12;19:901-910.
  • Wang XD, Lu H, Shi Z, Cai L, Liu S, Liu S, Han T, Wang Y, Zhou Y, Wang X, Gao S, Ji Y. A Pilot Study on Clinical and Neuroimaging Characteristics of Chinese Posterior Cortical Atrophy: Comparison with Typical Alzheimer's Disease. PLoS One. 2015 Aug 12;10(8):e0134956
  • Nestor PJ, Caine D, Fryer TD, Clarke J, Hodges JR. The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer's disease) with FDG-PET. J Neurol Neurosurg Psychiatry. 2003 Nov;74(11):1521-9.
  • Koch G, Stefani A, Panella M, Giordano A, Schillaci O, Marfia GA. Posterior cortical atrophy with unilateral occipito-temporal degeneration. J Neurol. 2004 Dec;251(12):1530-1.
  • Mendez MF, Monserratt LH, Liang LJ, Chavez D, Jimenez EE, Maurer JJ et al. Neuropsychological Similarities and Differences Between Amnestic Alzheimer's Disease and its Non-Amnestic Variants. J Alzheimers Dis. 2019;69(3):849-855.
  • Yousaf T, Dervenoulas G, Valkimadi PE, Politis M. Neuroimaging in Lewy body dementia. J Neurol. 2019 Jan;266(1):1-26.
Year 2024, Volume: 41 Issue: 1, 40 - 46, 29.03.2024

Abstract

References

  • Cerami C, Crespi C, Della Rosa PA, Dodich A, Marcone A, Magnani G et al. Brain changes within the visuo-spatial attentional network in posterior cortical atrophy. J Alzheimers Dis. 2015;43(2):385-95.
  • Torso M, Ahmed S, Butler C, Zamboni G, Jenkinson M, Chance S. Cortical diffusivity investigation in posterior cortical atrophy and typical Alzheimer's disease. J Neurol. 2021 Jan;268(1):227-239.
  • Esteves S, Ramirez Romero DA, Torralva T, Martínez Cuitiño M, Herndon S, Couto B et al. Posterior cortical atrophy: a single case cognitive and radiological follow-up. Neurocase. 2018 Feb;24(1):16-30.
  • Solyga VM, Western E, Solheim H, Hassel B, Kerty E. Posterior kortikal atrofi [Posterior cortical atrophy]. Tidsskr Nor Laegeforen. 2015 Jun 2;135(10):949-52.
  • DE Brito MH, Zampieri PF, Coutinho AM, Brucki SMD. Brain [18F]FDG PET-CT imaging in posterior cortical atrophy. Arq Neuropsiquiatr. 2019 Oct 24;77(10):752-753.
  • Whitwell JL, Graff-Radford J, Singh TD, Drubach DA, Senjem ML, Spychalla AJ et al. 18F-FDG PET in Posterior Cortical Atrophy and Dementia with Lewy Bodies. J Nucl Med. 2017 Apr;58(4):632-638.
  • Holden SK, Bettcher BM, Pelak VS. Update on posterior cortical atrophy. Curr Opin Neurol. 2020 Feb;33(1):68-73.
  • North C, Desai R, Saunders R, Suárez-González A, Bamiou D, Costafreda SG et al. Neuropsychological deficits in Posterior Cortical Atrophy and typical Alzheimer's disease: A meta-analytic review. Cortex. 2021 Oct;143:223-236.
  • Neitzel J, Ortner M, Haupt M, Redel P, Grimmer T, Yakushev I et al. Neuro-cognitive mechanisms of simultanagnosia in patients with posterior cortical atrophy. Brain. 2016 Dec;139(Pt 12):3267-3280.
  • Crutch SJ, Schott JM, Rabinovici GD, Murray M, Snowden JS, van der Flier WM et al. Alzheimer's Association ISTAART Atypical Alzheimer's Disease and Associated Syndromes Professional Interest Area. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017 Aug;13(8):870-884.
  • Keskinoglu P, Ucku R, Yener G, Yaka E, Kurt P, Tunca Z. Reliability and validity of revised Turkish version of Mini Mental State Examination (rMMSE-T) in community-dwelling educated and uneducated elderly. Int J Geriatr Psychiatry. 2009 Nov;24(11):1242-50.
  • Manos PJ, Wu R. The ten point clock test: a quick screen and grading method for cognitive impairment in medical and surgical patients. Int J Psychiatry Med. 1994;24(3):229-44.
  • Nyffeler T, Pflugshaupt T, Hofer H, Baas U, Gutbrod K, von Wartburg R, Hess CW, Müri RM. Oculomotor behaviour in simultanagnosia: a longitudinal case study. Neuropsychologia. 2005;43(11):1591-7.
  • Kubon J, Sokolov AN, Popp R, Fallgatter AJ, Pavlova MA. Face Tuning in Depression. Cereb Cortex. 2021 Mar 31;31(5):2574-2585.
  • Navon D.Forest before the trees:the precedence of global features in visual perception. Cogn Psychol 1977; 9(3):353-83
  • Saleh C, Seidl U, Stegentritt K, Schumacher F, Fehrenbach RA. Posteriores kortikales Atrophie-Plus-Syndrom. Eine Kasuistik [Posterior cortical atrophy-plus syndrome. A case report]. Fortschr Neurol Psychiatr. 2020 Aug;88(8):528-531.
  • Mukku SSR, Chintala H, Nagaraj C, Mangalore S, Sivakumar PT, Varghese M. Posterior cortical atrophy variant of Alzheimer's dementia-A case report. Asian J Psychiatr. 2018 Jun;35:109-112.
  • Giunta M, Libri I, Premi E, Brattini C, Paghera B, Archetti S et al. Clinical and radiological features of posterior cortical atrophy (PCA) in a GRN mutation carrier: a case report. Eur J Neurol. 2021 Jan;28(1):344-348.
  • Shebani Z, Nestor PJ, Pulvermüller F. What's "up"? Impaired Spatial Preposition Processing in Posterior Cortical Atrophy. Front Hum Neurosci. 2021 Dec 1;15:731104.
  • Colakoglu BD, Kurt P, Kaya GC, Yener G. A Distinct dementia syndrome: posterior cortical atrophy: clinical and cognitive assesment, and brain spect findings Archives of Neuropsychiatry, 2010;47(3):201-207.
  • Tang-Wai DF, Graff-Radford NR, Boeve BF, Dickson DW, Parisi JE, Crook R et al. Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy. Neurology. 2004 Oct 12;63(7):1168-74.
  • Wong B, Lucente DE, MacLean J, Padmanabhan J, Quimby M, Brandt KD et al. Diagnostic evaluation and monitoring of patients with posterior cortical atrophy. Neurodegener Dis Manag. 2019 Aug;9(4):217-239.
  • Mendez MF, Ghajarania M, Perryman KM. Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer's disease. Dement Geriatr Cogn Disord. 2002;14(1):33-40.
  • Suárez-González A, Crutch SJ, Franco-Macías E, Gil-Néciga E. Neuropsychiatric Symptoms in Posterior Cortical Atrophy and Alzheimer Disease. J Geriatr Psychiatry Neurol. 2016 Mar;29(2):65-71.
  • Aharon-Peretz J, Israel O, Goldsher D, Peretz A. Posterior cortical atrophy variants of Alzheimer's disease. Dement Geriatr Cogn Disord. 1999 Nov-Dec;10(6):483-7.
  • Schmidtke K, Hüll M, Talazko J. Posterior cortical atrophy: variant of Alzheimer's disease? A case series with PET findings. J Neurol. 2005 Jan;252(1):27-35.
  • Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Tang-Wai DF, Drubach DA et al. Visual hallucinations in posterior cortical atrophy. Arch Neurol. 2006 Oct;63(10):1427-32.
  • Yerstein O, Parand L, Liang LJ, Isaac A, Mendez MF. Benson's Disease or Posterior Cortical Atrophy, Revisited. J Alzheimers Dis. 2021;82(2):493-502.
  • Singh TD, Josephs KA, Machulda MM, Drubach DA, Apostolova LG, Lowe VJ, Whitwell JL. Clinical, FDG and amyloid PET imaging in posterior cortical atrophy. J Neurol. 2015 Jun;262(6):1483-92
  • Agosta F, Mandic-Stojmenovic G, Canu E, Stojkovic T, Imperiale F, Caso F, Stefanova E, Copetti M, Kostic VS, Filippi M. Functional and structural brain networks in posterior cortical atrophy: A two-centre multiparametric MRI study. Neuroimage Clin. 2018 Jun 12;19:901-910.
  • Wang XD, Lu H, Shi Z, Cai L, Liu S, Liu S, Han T, Wang Y, Zhou Y, Wang X, Gao S, Ji Y. A Pilot Study on Clinical and Neuroimaging Characteristics of Chinese Posterior Cortical Atrophy: Comparison with Typical Alzheimer's Disease. PLoS One. 2015 Aug 12;10(8):e0134956
  • Nestor PJ, Caine D, Fryer TD, Clarke J, Hodges JR. The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer's disease) with FDG-PET. J Neurol Neurosurg Psychiatry. 2003 Nov;74(11):1521-9.
  • Koch G, Stefani A, Panella M, Giordano A, Schillaci O, Marfia GA. Posterior cortical atrophy with unilateral occipito-temporal degeneration. J Neurol. 2004 Dec;251(12):1530-1.
  • Mendez MF, Monserratt LH, Liang LJ, Chavez D, Jimenez EE, Maurer JJ et al. Neuropsychological Similarities and Differences Between Amnestic Alzheimer's Disease and its Non-Amnestic Variants. J Alzheimers Dis. 2019;69(3):849-855.
  • Yousaf T, Dervenoulas G, Valkimadi PE, Politis M. Neuroimaging in Lewy body dementia. J Neurol. 2019 Jan;266(1):1-26.
There are 35 citations in total.

Details

Primary Language English
Subjects Neurology and Neuromuscular Diseases
Journal Section Research Article
Authors

Zeynep Selcan Şanlı 0000-0001-6098-9920

Ahmet Evlıce 0000-0002-4451-9475

Publication Date March 29, 2024
Submission Date August 31, 2023
Acceptance Date March 18, 2024
Published in Issue Year 2024 Volume: 41 Issue: 1

Cite

APA Şanlı, Z. S., & Evlıce, A. (2024). Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features. Journal of Experimental and Clinical Medicine, 41(1), 40-46.
AMA Şanlı ZS, Evlıce A. Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features. J. Exp. Clin. Med. March 2024;41(1):40-46.
Chicago Şanlı, Zeynep Selcan, and Ahmet Evlıce. “Posterior Cortical Atrophy: Clinical, Neuroimaging and Neuropsychological Features”. Journal of Experimental and Clinical Medicine 41, no. 1 (March 2024): 40-46.
EndNote Şanlı ZS, Evlıce A (March 1, 2024) Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features. Journal of Experimental and Clinical Medicine 41 1 40–46.
IEEE Z. S. Şanlı and A. Evlıce, “Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features”, J. Exp. Clin. Med., vol. 41, no. 1, pp. 40–46, 2024.
ISNAD Şanlı, Zeynep Selcan - Evlıce, Ahmet. “Posterior Cortical Atrophy: Clinical, Neuroimaging and Neuropsychological Features”. Journal of Experimental and Clinical Medicine 41/1 (March 2024), 40-46.
JAMA Şanlı ZS, Evlıce A. Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features. J. Exp. Clin. Med. 2024;41:40–46.
MLA Şanlı, Zeynep Selcan and Ahmet Evlıce. “Posterior Cortical Atrophy: Clinical, Neuroimaging and Neuropsychological Features”. Journal of Experimental and Clinical Medicine, vol. 41, no. 1, 2024, pp. 40-46.
Vancouver Şanlı ZS, Evlıce A. Posterior cortical atrophy: Clinical, neuroimaging and neuropsychological features. J. Exp. Clin. Med. 2024;41(1):40-6.