Pick-body-like inclusions in corticobasal degeneration differ from Pick bodies in Pick's disease

2002 ◽  
Vol 103 (2) ◽  
pp. 115-118 ◽  
Author(s):  
Kenji Ikeda ◽  
Tetsuaki Arai ◽  
Kuniaki Tsuchiya ◽  
Haruhiko Akiyama
Keyword(s):  
Corticobasal Degeneration ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Pick Bodies ◽  
Pick Body

scholarly journals Progress in Clinical Neurosciences: Frontotemporal Dementia-Pick's Disease

2006 ◽  
Vol 33 (2) ◽  
pp. 141-148 ◽  
Author(s):  
Andrew Kertesz
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Frontotemporal Dementia ◽  
Unsolved Problem ◽  
Corticobasal Degeneration ◽  
Primary Progressive Aphasia ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Diagnostic Features ◽  
Pick Bodies ◽  
Lateral Sclerosis

ABSTRACT:Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.

Frontotemporal dementias

2012 ◽  
pp. 344-350
Author(s):  
Lars Gustafson ◽  
Arne Brun
Keyword(s):  
Frontal Lobe ◽  
Current Knowledge ◽  
Corticobasal Degeneration ◽  
Clinical Syndrome ◽  
Cortical Atrophy ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Pick Bodies ◽  
Temporal Lobes

Nosological classification of organic dementia is based on current knowledge and theories of aetiology, including genetics, clinical picture, the pathological substrate, and the predominant location of brain damage. This chapter is concerned with dementia syndromes caused by a degenerative disease primarily affecting the frontal and temporal lobes, named frontal-lobe dementia or frontotemporal dementia (FTD). The terminology should be viewed from a historical perspective. The relationship between localized cortical atrophy in dementia and symptoms of aphasia was first reported by Pick in 1892. The pathological account of this lobar degeneration by Alzheimer in 1911 described ‘ballooned’ neurones (Pick cells) and argentophilic globes (Pick bodies), and the clinicopathological entity was named Pick's disease. In the 1980s, attention was drawn to a larger group of frontal-lobe dementias associated with frontotemporal cortical degeneration. The Lund–Manchester consensus of 1994 delineated the prototypical clinical syndrome of FTD with three neuropathological constituents, frontal lobe degeneration of non-Alzheimer type (FLD), (alternatively designated ‘dementia lacking distinctive histology’), Pick's disease, and motor neurone disease (MND) with dementia (FTD-MND). The 1998 consensus on clinical diagnostic criteria for frontotemporal lobar degeneration (FTLD) encompassed two additional dementia syndromes; progressive non-fluent aphasia (PA), and semantic dementia. Corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) have also been associated with FTLD. A changing clinical classification is shown in Fig. 4.1.3.1. The addition of important genetic and histochemical characteristics has further added to the complex classification of FTD and FTLD with a risk of developing numerous and partly competing definitions. FTLD may be further subclassified into forms positive or negative for tau and ubiquitin. The ubiquitinated form will be referred to as FTD-U, which is synonymous to FLTD-U.

scholarly journals Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases

Brain ◽  
2020 ◽  
Vol 143 (8) ◽  
pp. 2398-2405 ◽  
Author(s):  
Shinsuke Ishigaki ◽  
Yuichi Riku ◽  
Yusuke Fujioka ◽  
Kuniyuki Endo ◽  
Nobuyuki Iwade ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Alzheimer Disease ◽  
Progressive Supranuclear Palsy ◽  
Corticobasal Degeneration ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Subsequent Increase ◽  
Fused In Sarcoma ◽  
Wide Range

Abstract Fused in sarcoma (FUS) is genetically and clinicopathologically linked to frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). We have previously reported that intranuclear interactions of FUS and splicing factor, proline- and glutamine-rich (SFPQ) contribute to neuronal homeostasis. Disruption of the FUS-SFPQ interaction leads to an increase in the ratio of 4-repeat tau (4R-tau)/3-repeat tau (3R-tau), which manifests in FTLD-like phenotypes in mice. Here, we examined FUS-SFPQ interactions in 142 autopsied individuals with FUS-related ALS/FTLD (ALS/FTLD-FUS), TDP-43-related ALS/FTLD (ALS/FTLD-TDP), progressive supranuclear palsy, corticobasal degeneration, Alzheimer’s disease, or Pick’s disease as well as controls. Immunofluorescent imaging showed impaired intranuclear co-localization of FUS and SFPQ in neurons of ALS/FTLD-FUS, ALS/FTLD-TDP, progressive supranuclear palsy and corticobasal degeneration cases, but not in Alzheimer’s disease or Pick’s disease cases. Immunoprecipitation analyses of FUS and SFPQ revealed reduced interactions between the two proteins in ALS/FTLD-TDP and progressive supranuclear palsy cases, but not in those with Alzheimer disease. Furthermore, the ratio of 4R/3R-tau was elevated in cases with ALS/FTLD-TDP and progressive supranuclear palsy, but was largely unaffected in cases with Alzheimer disease. We concluded that impaired interactions between intranuclear FUS and SFPQ and the subsequent increase in the ratio of 4R/3R-tau constitute a common pathogenesis pathway in FTLD spectrum diseases.

Clustering of Pick bodies in the dentate gyrus in Pick's disease

2000 ◽  
Vol 20 (3) ◽  
pp. 170-175 ◽  
Author(s):  
RA Armstrong ◽  
NJ Cairns ◽  
PL Lantos
Keyword(s):  
Dentate Gyrus ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Pick Bodies

Coexistence of Pick bodies and atypical Lewy bodies in the locus ceruleus neurons of Pick's disease

1995 ◽  
Vol 90 (1) ◽  
pp. 93-100
Author(s):  
S. Takauchi ◽  
S. Yamauchi ◽  
Y. Morimura ◽  
K. Ohara ◽  
Y. Morita ◽  
...  
Keyword(s):  
Lewy Bodies ◽  
Locus Ceruleus ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Pick Bodies

Distribution of basal ganglia lesions in Pick's disease with Pick bodies: A topographic neuropathological study of eight autopsy cases

1999 ◽  
Vol 19 (4) ◽  
pp. 370-379 ◽  
Author(s):  
Kuniaki Tsuchiya ◽  
Kunimasa Arima ◽  
Toshiya Fukui ◽  
Toshihiko Kuroiwa ◽  
Chie Haga ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Pick Bodies

An autopsy case of incipient Pick's disease: Immunohistochemical profile of early-stage Pick body formation

2014 ◽  
pp. n/a-n/a ◽  
Author(s):  
Yasuo Miki ◽  
Fumiaki Mori ◽  
Kunikazu Tanji ◽  
Hidekachi Kurotaki ◽  
Akiyoshi Kakita ◽  
...  
Keyword(s):  
Early Stage ◽  
Pick's Disease ◽  
Pick’S Disease ◽  
Autopsy Case ◽  
Body Formation ◽  
Pick Body ◽  
Immunohistochemical Profile

scholarly journals Novel tau filament fold in corticobasal degeneration, a four-repeat tauopathy

2019 ◽  
Author(s):  
Wenjuan Zhang ◽  
Airi Tarutani ◽  
Kathy L. Newell ◽  
Alexey G. Murzin ◽  
Tomoyasu Matsubara ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Chronic Traumatic Encephalopathy ◽  
Association Studies ◽  
Corticobasal Degeneration ◽  
Pick's Disease ◽  
Genome Wide Association Studies ◽  
Pick’S Disease ◽  
Tau Isoforms ◽  
Cognitive Disturbances

Corticobasal degeneration (CBD) is a neurodegenerative tauopathy that is characterised by motor and cognitive disturbances (1–3). A higher frequency of the H1 haplotype of MAPT, the tau gene, is present in cases of CBD than in controls (4, 5) and genome-wide association studies have identified additional risk factors (6). By histology, astrocytic plaques are diagnostic of CBD (7, 8), as are detergent-insoluble tau fragments of 37 kDa by SDS-PAGE (9). Like progressive supranuclear palsy (PSP), globular glial tauopathy (GGT) and argyrophilic grain disease (AGD) (10), CBD is characterised by abundant filamentous tau inclusions that are made of isoforms with four microtubule-binding repeats (4R) (11–15). This distinguishes 4R tauopathies from Pick’s disease, filaments of which are made of three-repeat (3R) tau isoforms, and from Alzheimer’s disease and chronic traumatic encephalopathy (CTE), where both 3R and 4R tau isoforms are found in the filaments (16). Here we report the structures of tau filaments extracted from the brains of three individuals with CBD using electron cryo-microscopy (cryo-EM). They were identical between cases, but distinct from those of Alzheimer’s disease, Pick’s disease and CTE (17–19). The core of CBD filaments comprises residues K274-E380 of tau, spanning the last residue of R1, the whole of R2, R3 and R4, as well as 12 amino acids after R4. It adopts a novel four-layered fold, which encloses a large non-proteinaceous density. The latter is surrounded by the side chains of lysine residues 290 and 294 from R2 and 370 from the sequence after R4. CBD is the first 4R tauopathy with filaments of known structure.

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