Clinical Characteristics of Dementia Associated with Argyrophilic Grain Disease

Steuerwald, Gertrud M.; Baumann, Thomas; Taylor, Kirsten I.; Mittag, Michael; Adams, Heiner; Tolnay, Markus; Monsch, Andreas U.

doi:10.1159/000107085

Cited by 14 publications

(7 citation statements)

References 30 publications

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“…Amnesia is its most common initial symptom (Ferrer, Santpere, & van Leeuwen, 2008), whereas other cognitive functions are relatively spared. However AGD usually appears in very old patients (in our sample only three patients were above 75); in addition, our TLD patients lack the behavioral disturbances (irritability agitation, delusions, and apathy) frequently reported in dementia associated with AGD (Steuerwald et al, 2007;Togo et al, 2005).…”

Section: Discussionmentioning

confidence: 48%

Probable Alzheimer's Disease Patients Presenting as “Focal Temporal Lobe Dysfunction” Show a Slow Rate of Cognitive Decline

Marra

Villa

Quaranta

et al. 2011

J Int Neuropsychol Soc

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Several authors have recently shown that anterograde amnesia is often associated with semantic memory impairment in amnesic MCI patients. Similarly, after the MCI condition, some patients who convert to Alzheimer's disease (AD) show the classic onset (cAD) characterized by the impairment of memory and executive functions, whereas other AD patients show isolated defects of episodic and semantic memory without deficits in other cognitive domains. The latter have been considered an AD variant characterized by 'focal Temporal Lobe Dysfunction' (TLD). The aim of the present study was to assess the differences in disease progression between cAD and TLD. For this purpose a continuous series of newly diagnosed probable AD patients presenting as cAD (n = 30) and TLD (n = 25), matched for severity, and 65 healthy controls underwent a comprehensive neuropsychological evaluation at baseline; TLD and cAD were re-evaluated at a 24-month follow-up. At follow-up, TLD patients showed no significant worsening of cognitive functions, whereas cAD subjects displayed a significant worsening in all explored cognitive domains. In conclusion, our results confirm that probable AD presenting as TLD represents a specific onset of AD characterized by a slower rate of progression.

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Section: Discussionmentioning

confidence: 48%

Probable Alzheimer's Disease Patients Presenting as “Focal Temporal Lobe Dysfunction” Show a Slow Rate of Cognitive Decline

Marra

Villa

Quaranta

et al. 2011

J Int Neuropsychol Soc

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“…The reason we included the study of AGs in our study design is that other researchers have reported an overrepresentation of AG pathology in TDP‐43[+] cases . We note that in our autopsy cohort , as in others , AGs were not associated with cognitive impairment independent of other pathologies, whereas AGs are associated with dementia in other cohorts . Thus, there may be a discrepancy between the diseases underlying AG pathology seen in various populations and/or autopsy cohorts.…”

Section: Discussionmentioning

confidence: 61%

Overlapping but distinct TDP‐43 and tau pathologic patterns in aged hippocampi

et al. 2017

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Intracellular proteinaceous aggregates (inclusion bodies) are almost always detectable at autopsy in brains of elderly individuals. Inclusion bodies composed of TDP-43 and tau proteins often coexist in the same brain, and each of these pathologic biomarkers is associated independently with cognitive impairment. However, uncertainties remain about how the presence and neuroanatomical distribution of inclusion bodies correlate with underlying diseases including Alzheimer's disease (AD). To address this knowledge gap, we analyzed data from the University of Kentucky AD Center autopsy series (n = 247); none of the brains had frontotemporal lobar degeneration. A specific question for this study was whether neurofibrillary tangle (NFT) pathology outside of the Braak NFT staging scheme is characteristic of brains with TDP-43 pathology but lacking AD, that is those with cerebral age-related TDP-43 with sclerosis (CARTS). We also tested whether TDP-43 pathology is associated with comorbid AD pathology, and whether argyrophilic grains are relatively likely to be present in cases with, vs. without, TDP-43 pathology. Consistent with prior studies, hippocampal TDP-43 pathology was associated with advanced AD - Braak NFT stages V/VI. However, argyrophilic grain pathology was not more common in cases with TDP-43 pathology in this data set. In brains with CARTS (TDP-43[+]/AD[-] cases), there were more NFTs in dentate granule neurons than were seen in TDP-43[-]/AD[-] cases. These dentate granule cell NFTs could provide a proxy indicator of CARTS pathology in cases lacking substantial AD pathology. Immunofluorescent experiments in a subsample of cases found that, in both advanced AD and CARTS, approximately 1% of dentate granule neurons were PHF-1 immunopositive, whereas ∼25% of TDP-43 positive cells showed colocalized PHF-1 immunoreactivity. We conclude that NFTs in hippocampal dentate granule neurons are often present in CARTS, and TDP-43 pathology may be secondary to or occurring in parallel with tauopathy.

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“…9 In addition, it is difficult to differentiate between FTD or DLB and early-stage AD. 10 Previous reports have shown that the clinical features of AGD 11,12 and NFTPD 13,14 are indistinguishable from those of AD. In some cases, these diseases are characterized by gradually progressing dementia with prominent memory disturbances and considerable preservation of other cognitive functions.…”

Section: Discussionmentioning

confidence: 99%

“…Amyloid-b (Ab) protein is a major component of these senile plaques. Klunk et al reported that 11 C-labeled Pittsburgh compound B ([ 11 C]-PiB) has a high affinity for Ab, and [ 11 C]-PiB has been used as an amyloidimaging compound since their report was published. 1 11 Clabeled Pittsburgh compound B-positron emission tomography (PET) has shown amyloid deposition in patients with AD and in some patients with mild cognitive impairment (MCI).…”

Section: Introductionmentioning

confidence: 99%

Pittsburgh Compound B-Negative Dementia—A Possibility of Misdiagnosis of Patients With Non-Alzheimer Disease-Type Dementia as Having AD

Shimada

Ataka

Takeuchi

et al. 2011

J Geriatr Psychiatry Neurol

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Amyloid imaging has been used to detect amyloid deposition in the brain. We performed Pittsburgh compound B (PiB)-positron emission tomography on 63 patients with dementia having cognitive decline or memory disturbance. In addition, we measured the patients' apolipoprotein E4 (apo E4) status and cerebrospinal fluid (CSF) levels of amyloid-β (Aβ)1-42, tau, and P-tau. Finally, the patients were diagnosed as having probable Alzheimer disease (AD) on the basis of their neuropsychological findings and because they met the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association criteria. Among the patients diagnosed with probable AD, 10 patients were PiB negative. The CSF levels of P-tau and tau in PiB-negative patients were significantly lower than those in the PiB-positive patients. In addition, the CSF levels of Aβ1-42 in the PiB-negative patients were significantly higher than those in the PiB-positive patients. None of the PiB-negative patients were apo E4 carriers. These results suggest that the PiB-negative patient group included not only AD patients but also non-AD-type dementia patients. However, our finding is based on a relatively small number of patients and therefore should be replicated in a larger cohort. In addition, it will be necessary to categorize these participants by longitudinal follow-up and postmortem pathological examinations.

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Clinical Characteristics of Dementia Associated with Argyrophilic Grain Disease

Cited by 14 publications

References 30 publications

Probable Alzheimer's Disease Patients Presenting as “Focal Temporal Lobe Dysfunction” Show a Slow Rate of Cognitive Decline

Probable Alzheimer's Disease Patients Presenting as “Focal Temporal Lobe Dysfunction” Show a Slow Rate of Cognitive Decline

Overlapping but distinct TDP‐43 and tau pathologic patterns in aged hippocampi

Pittsburgh Compound B-Negative Dementia—A Possibility of Misdiagnosis of Patients With Non-Alzheimer Disease-Type Dementia as Having AD

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