Progressive posterior cortical dysfunction

Renner, John A.; Burns, Jeffrey M.; Hou, Craig E.; McKeel, Daniel W.; Storandt, Martha; Morris, John C.

doi:10.1212/01.wnl.0000140290.80962.bf

Cited by 299 publications

(247 citation statements)

References 25 publications

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“…In fact, NFT density in the visual association cortex has been shown to be greater in PCA than in typical AD [24]. These findings correlate with the visuospatial and visual perceptual deficits encountered in our patients with PCA, and the fact that patients with PCA typically perform worse on tests of visuospatial function than typical AD subjects [43].…”

Section: Discussionsupporting

confidence: 76%

“…The clinical features of these 38 PCA patients were typical of other described cases and case series of PCA [4,19,39,43,48]. In all our patients the clinical features were characterized by predominant visuospatial, visuoperceptual deficits, and visual agnosia, as well as problems with reading and writing and environmental disorientation.…”

Section: Discussionsupporting

confidence: 71%

“…These included hallucinations, parkinsonism, REM sleep behavior disorder and myoclonic jerks, however these features are not novel and have been reported in a previous clinical series on PCA [48]. It is not unexpected to find these features in PCA since PCA is a syndrome that may result from multiple different pathologies [43].…”

Section: Discussionmentioning

confidence: 78%

“…In fact the typical AD subjects had significantly greater atrophy of the left medial temporal lobe (particularly the hippocampus) and inferior and middle temporal gyri than the PCA patients, reflecting the deficits in episodic memory that are found in patients with typical AD. This is perhaps unsurprising since patients were excluded from a diagnosis of PCA if they had significant and early episodic memory impairment, and AD patients perform worse on tests of episodic memory than PCA patients [39,43].…”

Section: Discussionmentioning

confidence: 99%

“…Visual assessments of MRI have been reported in cases of PCA and have typically shown a pattern of bilateral posterior cerebral atrophy, predominantly affecting the occipital, parietal, and the temporal lobes [1,4,21,24,32,39,41,43]. However, a number of recent automated image analysis techniques have been applied to study patterns of cerebral atrophy on MRI.…”

Section: Introductionmentioning

confidence: 99%

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Imaging correlates of posterior cortical atrophy

Whitwell

Jack

Kantarci

et al. 2007

Neurobiology of Aging

182

139

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The aim of this study was to compare patterns of cerebral atrophy on MRI, and neurochemistry on magnetic resonance spectroscopy (MRS), in subjects with posterior cortical atrophy (PCA) and typical Alzheimer's disease (AD). Voxel-based morphometry was used to assess grey matter atrophy in 38 subjects with PCA, 38 subjects with typical AD, and 38 controls. Clinical data was assessed in all PCA subjects. Single-voxel 1 H MRS located in the posterior cingulate was analyzed in a subset of subjects with PCA, typical AD, and control subjects. PCA showed a pattern of atrophy affecting occipital, parietal and posterior temporal lobes, compared to controls. The pattern was bilateral, but more severe on the right. Subjects with PCA showed greater atrophy in the right visual association cortex than subjects with typical AD, whereas those with AD showed greater atrophy in the left hippocampus than those with PCA. 1 H MRS suggested loss of neuronal integrity and glial activation in subjects with PCA and AD. The differing patterns of atrophy on MRI suggest that PCA should be considered a distinct entity from typical AD.

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Section: Discussionsupporting

confidence: 76%

Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Imaging correlates of posterior cortical atrophy

Whitwell

Jack

Kantarci

et al. 2007

Neurobiology of Aging

182

139

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Visual Hallucinations in Posterior Cortical Atrophy

Josephs¹,

Whitwell²,

Boeve³

et al. 2006

Arch Neurol

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Background: Visual hallucinations have been reported to occur in up to 25% of patients who meet the criteria for posterior cortical atrophy (PCA). It is not known, however, whether patients who meet the criteria for PCA and have hallucinations are different from those who meet the criteria and do not have hallucinations.Objective: To compare the clinical and imaging features of patients with PCA with and without wellformed visual hallucinations.Design: Case-control study.Setting: Tertiary care medical center.Patients: Fifty-nine patients fulfilling the criteria for PCA were retrospectively identified and divided into 2 groups based on the presence (n = 13) or absence (n = 46) of visual hallucinations.Main Outcome Measures: Statistically significant clinical differences and imaging differences using voxelbased morphometry between the 2 groups.

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Posterior Cortical Atrophy as an Extreme Phenotype ofGRNMutations

et al. 2015

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Figure 2), which is rather different from PCA with AD pathologic features. In the latter process, the damage remains relatively centered on the posterior lobes, even in the late stage. 1,15 Conclusions This study enlarges the mutational spectrum of PCA to GRN mutations and provides evidence that GRN analyses could be indicated in PCA, particularly when the damage progresses to the anterior cerebral regions and a family history of dementia is present. The link between PCA and genetic forms of FTLD is supported by the recent identification of a MAPT mutation in 1 case of PCA. 16 Analyses of genes in FTLD in patient cohorts will be necessary to better evaluate their genetic contribution to PCA. Finally, this study underlines a possible continuum in degenerative dementias and highlights the limits of actual nosologic boundaries. Clarifying these boundaries by identifying factors driving phenotypic heterogeneity will have important implications for the definition of new diagnosis criteria of degenerative dementias and recruitment for clinical trials in the future. ARTICLE INFORMATION

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Progressive posterior cortical dysfunction

Abstract: AD was the most frequent cause of PPCD in this series, although non-Alzheimer's dementing disorders also should be considered.

Cited by 299 publications

References 25 publications

Imaging correlates of posterior cortical atrophy

Imaging correlates of posterior cortical atrophy

Visual Hallucinations in Posterior Cortical Atrophy

Posterior Cortical Atrophy as an Extreme Phenotype ofGRNMutations

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