Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions

@article{Yokota2009ClinicopathologicalCO,
  title={Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions},
  author={Osamu Yokota and Kuniaki Tsuchiya and Tetsuaki Arai and Saburo Yagishita and Osamu Matsubara and Akihide Mochizuki and Akira Tamaoka and Mitsuru Kawamura and Hidetoshi Yoshida and Seishi Terada and Hideki Ishizu and Shigetoshi Kuroda and Haruhiko Akiyama},
  journal={Acta Neuropathologica},
  year={2009},
  volume={117},
  pages={429-444}
}
Although frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions (FTLD-TDP) and Pick’s disease are common pathological substrates in sporadic FTLD, clinical differentiation of these diseases is difficult. We performed a retrospective review of medical records and semiquantitative examination of neuronal loss of 20 sporadic FTLD-TDP and 19 Pick’s disease cases. Semantic dementia as the first syndrome developed only in FTLD-TDP patients. Impaired speech output in the early… Expand
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TLDR
Clinical data useful for clinical differentiation of pathological subtypes of FTLD besides AD with atypical cerebral atrophy will be essential in the future because adequate clinical data to infer pathologies are not available. Expand
Clinicopathological characteristics of FTLD-TDP showing corticospinal tract degeneration but lacking lower motor neuron loss
TLDR
It is clarified thatFTLD-MND-P cases have characteristic clinicopathological features distinct from those of FTLD- MND-A, and TDP-43 positive neuronal cytoplasmic inclusions were absent or rare in the LMNs, while TDP+ round structures were frequently identified in the neuropil of the spinal cord anterior horn in some cases. Expand
Clinical phenotypes in autopsy-confirmed Pick disease
TLDR
The pathologic course of the disease in FTLD cases with Pick bodies is not uniform and disease duration can be estimated based on early clinical features, having relevance as treatment options, which are likely to be pathology specific, are developed. Expand
Pick's disease.
TLDR
In this chapter, recent findings regarding the distinct clinical and histopathological features of these pathological disease entities are presented including the discussion on the possibility of future antemortem diagnosis of patients with the disease. Expand
Clinicopathological study of diffuse neurofibrillary tangles with calcification With special reference to TDP-43 proteinopathy and alpha-synucleinopathy
TLDR
The molecular basis of DNTC was clarified by immunohistochemically examining the appearance and distribution of accumulated alpha-synuclein (aSyn) and TAR DNA-binding protein of 43kDa (TDP-43) in the brains of 10 Japanese autopsy cases and there was a significant correlation between some selected items of NPI-Q scores and the severity of the limbic TDP- 43 pathology. Expand
Chapter 12 – Pick’s Disease: The Evolution of Theory and Knowledge in Neurodegenerative Tauopathies
TLDR
Clinical identification remains challenging due to overlapping, varied clinical phenotypes, and Clinicians and researchers alike are encouraged to consider the increasingly recognized roles of cooccurring pathologies in diagnosis and treatment innovations. Expand
TDP-43 pathology in primary lateral sclerosis
TLDR
The extremely minor involvement of LMN, even after very long disease duration in some cases, suggests that PLS is a distinct form of MND in which LMN are spared or protected. Expand
Frontotemporal lobar degeneration proteinopathies have disparate microscopic patterns of white and grey matter pathology
TLDR
FTLD-Tau and FT LD-TDP proteinopathies have distinct severity and regional distribution of WM and GM pathology, which may impact their clinical presentation, with overall greater severity of WM pathology as a distinguishing feature of tauopathies. Expand
Frontotemporal lobar degeneration with motor neuron disease showing severe and circumscribed atrophy of anterior temporal lobes
TLDR
Typical features of FTLD-MND in clinical course and TDP-43 pathology with unusual severity and distribution of cerebral atrophy are shown, suggesting a unique manifestation ofFTLD- MND. Expand
シンポジウム19―4 認知症研究の新しい視点 TDP-43陽性封入体をともなう孤発性FTLDの臨床病理学的特徴
TLDR
It is suggested that the early impairment of semantic memory and asymmetric motor disturbances in sporadic FTLD patients predict FTLD-TDP rather than Pick's disease, while initial behavioral symptoms or non-fluent aphasia without subsequent asymmetricMotor disturbances predict Pick’s disease rather than FTLD -TDP. Expand
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