Amyotrophic lateral sclerosis with TDP-43 abnormalities exhibiting globular glial tau inclusions in frontotemporal lobes and pallido-nigral system

Kaoru Yagita, Naokazu Sasagasako, Sachiko Koyama, Hideko Noguchi, Hiroyuki Honda

研究成果: ジャーナルへの寄稿学術誌査読

抄録

Here we present the autopsy case of an 80-year-old woman with a 9-year history of motor neuron disease and atypical Parkinsonism. Her initial symptom was gait disturbance, and she subsequently developed limb weakness and Parkinsonism without response to levodopa. Her motor symptoms progressed to bulbar palsy, and she died of respiratory failure. Postmortem examination revealed characteristic findings of amyotrophic lateral sclerosis (ALS), including motor neuronal loss with astrogliosis, corticospinal tract degeneration, and TAR DNA-binding protein of 43 kDa abnormalities, including nuclear loss and skein-like inclusions. In contrast, severe tau pathological changes were seen in the frontotemporal lobes and pallido-nigral system. Tau pathologies affected not only neuronal components, such as neurofibrillary tangles and neuropil threads, but also glial cells (astrocytes and oligodendrocytes). Some glial tau pathologies exhibited peculiar round accumulations, reminiscent of globular glial inclusions (GGIs) in globular glial tauopathy. This unique autopsy case demonstrates that ALS with TDP-43 could be comorbid with globular glial tau inclusions and indicates that common pathological mechanisms exist among ALS and GGI formation.

本文言語英語
ジャーナルNeuropathology
DOI
出版ステータス印刷中 - 2022
外部発表はい

!!!All Science Journal Classification (ASJC) codes

  • 病理学および法医学
  • 臨床神経学

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