Highlights
- •‘Non-visual’ PCA patients present with posterior dysfunction without visual symptoms.
- •‘Non-visual’ PCA patients are less impaired than visual PCA in visuospatial/perceptual tasks.
- •Patients with ‘non-visual’ PCA and visual PCA show similar severity of apraxia.
- •Patients with ‘non-visual’ PCA show greater impairment in posterior functions than tAD.
- •Absence of clinically significant visual impairment should not exclude a diagnosis of PCA.
Abstract
Background
Early and progressive disabling visual impairment is a core feature for the diagnosis
of posterior cortical atrophy (PCA). However, some individuals that fulfil criteria
over time might initially present with an onset of prominent posterior dysfunction
other than visuoperceptual.
Methods
The clinical profile of five patients with a predominantly ‘non-visual’ posterior
presentation (PCA2) was investigated and compared with sixteen individuals with visually
predominant PCA (PCA1) and eighteen with typical amnestic Alzheimer disease (tAD).
Results
PCA2 patients showed significantly better performance than PCA1 in one visuospatial
task and were free of Balint's syndrome and visual agnosia. Compared to tAD, PCA2
showed trends towards significantly lower performance in visuoperceptual tasks, more
severe apraxia and more symptoms of Gerstmann's syndrome.
Conclusions
Our sample of PCA2 patients did not present with clinically prominent visual symptoms
but did show visual dysfunction on formal neuropsychological assessment (less pronounced
than in PCA1 but more than in tAD) in addition to other posterior deficits. Broadening
the definition of PCA to encompass individuals presenting with prominent ‘non-visual’
posterior dysfunction should be potentially considered in clinical and research contexts.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Clinical, genetic and neuropathologic characteristics of posterior cortical atrophy.Neurology. 2004; 63: 1168-1174
- Posterior cortical atrophy.Arch. Neurol. 1988; 45: 789-793
- Posterior cortical atrophy variants of Alzheimer's disease.Dement. Geriatr. Cogn. Disord. 1999; 10: 483-487
- Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer's disease.Dement. Geriatr. Cogn. Disord. 2002; 14: 33-40
- Posterior cortical atrophy.Lancet Neurol. 2012; 11: 170-178
- The diagnosis of dementia due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's disease.Alzheimers Dement. 2011; 7: 263-269
- Advancing research diagnostic criteria for Alzheimer's disease: the IWG-2 criteria.The Lancet Neurology. 2014; 13: 614-629
- Progressive posterior cortical dysfunction.Neurology. 2006; 63: 1175-1180
- Corticobasal degeneration: a pathologically distinct 4R tauopathy.Nat Rev Neurol. 2011; 7: 263-272
- Cortical thickness and voxel-based morphometry in posterior cortical atrophy and typical Alzheimer's disease.Neurobiol. Aging. 2011; 32: 1466-1476
- The cognitive profile of posterior cortical atrophy.Neurology. 2006; 66: 331-338
Kas A, Cruz de Souza, L, Samri D, Bartolomeo P, Lacomblez L, Kalafat M, et al. Neural correlates of cognitive impairment in posterior cortical atrophy. Brain 2011;134:1464–1478.
- Slowly progressive apraxia in Alzheimer's disease.Journal of Neurology, Neurosurgery and Psychiatry. 1995; 59: 312-315
- Progressive biparietal atrophy: an atypical presentation of Alzheimer's disease.Journal of Neurology Neurosurgery and Psychiatry. 1996; 61: 388-395
- Atypical and typical presentations of Alzheimer's disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases.Brain. 2000; 123: 484-498
- Biparietal variant ofAlzheimer's disease: a rare presentation of a common disease.BMJ Case Rep. 2015 Jan; 5https://doi.org/10.1136/bcr-2014-207011
- Nonamnestic presentations of early-onset Alzheimer's disease.Am J Alzheimer Dis Other Demen. 2012; 27: 413-420
- Minimental state. A practical method for grading the cognitive state of patients for the clinician.J. Psychiatr. Res. 1975; 12: 189-198
- Programa integrado de exploración neuropsicológica.Masson, Manual. Barcelona1990
- Wechsler Memory Scale – Revised (WMS-R).Psychological Corporation, New York1987
- Boston Diagnostic Aphasia Examination (BDAE-3).Lippincott, Williams and Wilkins, Philadelphia2000
- The Visual Object and Space Perception Battery.Thames Valley Test Co, Bury St Edmunds1991
- Posterior cortical atrophy: clinical presentation and cognitive deficits compared to Alzheimer's disease.Behav. Neurol. 2005; 16: 15-23
- Functional dissociation of saccade and hand reaching control with bilateral lesions of the medial wall of the intraparietal sulcus: implications for optic ataxia.NeuroImage. 2007; 36: T69-T76
- Visual assessment of medial temporal lobe atrophy on magnetic resonance imaging: inter observer reliability.J. Neurol. 1995; 242: 557-560
- Visual assessment of posterior atrophy development of a MRI rating scale.Eur. Radiol. 2011; 21: 2618-2625
- Analysis of apolipoprotein E genotypes by the amplification refractory mutation system.Clin. Chem. 1991; 37 (Feb): 241-244
- Progressive biparietal atrophy: an atypical presentation of Alzheimer's disease.Journal of Neurology Neurosurgery and Psychiatry. 1996; 61: 388-395
- Posterior cerebral atrophy in the absence of medial temporal lobe atrophy in pathologically confirmed Alzheimer's disease.Neurobiology of Aging. 2012; 33: 627e1-627e12
- Apolipoprotein e genotype modifies the phenotype of Alzheimer disease.Archives of Neurology. 2006; 63: 155-156
- Cognitive phenotypes in Alzheimer's disease and genetic risk.Cortex. 2007; 43: 835-845
- Early-onset versus late-onset Alzheimer's disease: the case of the missing APOE ɛ4 allele.Lancet Neurol. 2011; 10: 280-288
- Clinical syndromes associated with posterior atrophy early age at onset AD spectrum.Neurology. 2009; 73: 1571-1578
Article info
Publication history
Published online: May 12, 2016
Accepted:
May 11,
2016
Received in revised form:
April 13,
2016
Received:
December 14,
2015
Identification
Copyright
© 2016 Elsevier B.V. All rights reserved.