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

  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},
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… 
Clinicopathological characteristics of FTLD-TDP showing corticospinal tract degeneration but lacking lower motor neuron loss
Clinical phenotypes in autopsy-confirmed Pick disease
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.
Pick's disease.
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.
TDP-43 pathology in primary lateral sclerosis
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.
Dopamine Transporter Imaging for Frontotemporal Lobar Degeneration With Motor Neuron Disease
Dopamine transporter single photon emission computed tomography revealed abnormal findings in patients with FTLD-MND, which may manifest even before the onset of MND symptoms.
Frontotemporal lobar degeneration proteinopathies have disparate microscopic patterns of white and grey matter pathology
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.
Frontotemporal lobar degeneration with motor neuron disease showing severe and circumscribed atrophy of anterior temporal lobes
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
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.
Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
A pathological continuity between FTLD-TDP and ALS is supported at the level of the LMN system, and lower motor neuron loss and TDP-43-positive skeinlike inclusions were observed in all pathological subtypes.


TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD
The highly consistent clinical and neuropathological phenotype supports the concept that TDP-43-negative FTLD-U should be considered as a new clinicopathological FTLD entity.
Frontotemporal lobar degeneration: clinical and pathological relationships
The findings demonstrate predictable relationships between clinical phenotype and both topographical distribution of brain atrophy and immunohistochemical characteristics and emphasise the importance of refined delineation of both clinical and pathological phenotype in furthering understanding of FTLD and its molecular substrate.
Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions
Three cases of FTLD with ubiquitin- and p62-positive neuronal cytoplasmic inclusions confirmed the existence of TDP-43-negative FTLD-U and extend the clinical and pathological spectrum of this disorder.
Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy.
FTLD-U or FTLD-MND should be considered in the differential diagnosis of progressive frontal dementia with an akinetic rigid syndrome and supranuclear gaze palsy or Steele-Richardson-Olszewski disease.
Neuropathologic Features of Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions With Progranulin Gene (PGRN) Mutations
The results suggest that frontotemporal lobar degeneration with ubiquitin-positive inclusions due to PGRN mutations has several characteristic features, including ubiquitIn-immunoreactive neuritic pathology in superficial cortical layers and neuronal intranuclear inclusions, while there is no histopathologic feature or combination of features that is pathognomonic.
Neuropathological discrepancy between Japanese Pick’s disease without Pick bodies and frontal lobe degeneration type of frontotemporal dementia proposed by Lund and Manchester Group
  • K. Ikeda
  • Psychology
    Neuropathology : official journal of the Japanese Society of Neuropathology
  • 2000
This work investigated cases of Japanese Pick’s disease without Pick bodies (PB), the majority of which are thought to correspond to FLD type, in order to clarify whether the nature of the degeneration in these cases could be distinguishable from that in Japanese Pick's disease with PB, which corresponds approximately to the Pick type of the Lund and Manchester group.
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia.
A clinico-pathological investigation of two FTLD families with PGRN mutations is reported, for the first time, and provides compelling evidence for the link between FTD and PNFA, while raising the possibility of identifiable clinical differences between FTLD patients with MAPT and P GRN mutations.
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration
The aim of this study was to improve the neuropathologic recognition and provide criteria for the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal lobar degeneration (FTLD) and incorporate up-to-date neuropathology in the light of recent immunohistochemical, biochemical, and genetic advances.
Epidemiology of Frontotemporal Lobar Degeneration
E Epidemiologic studies, both community-based and hospital-based, demonstrate that FTLD is a more common cause of early-onset dementia than previously recognized.