Yonsei Med J.  2020 Aug;61(8):731-735. 10.3349/ymj.2020.61.8.731.

An Autopsy-Proven Case of Limbic-Predominant Age-Related TDP-43 Encephalopathy

• Cho SH
• Choi SM
• Kim BC
• Song WY
• Kim HS
• Lee KH

• KMID: 2504733
• DOI: http://doi.org/10.3349/ymj.2020.61.8.731

Abstract

Limbic-predominant age-related TDP-43 encephalopathy (LATE) is a recently established neurodegenerative disease entity. LATE neuropathological change (LATE-NC) is characterized by a TDP-43 proteinopathy that mainly involves the amygdala and medial temporal structures, with or without hippocampal sclerosis. LATE-NC is typically observed in individuals aged 80 years or older and manifests clinically as amnestic memory decline. Herein, we report a case of LATE diagnosed by brain autopsy in an 82-year-old male who had an 11-year history of memory impairment. Pathological examination revealed high Alzheimer disease neuropathological changes, as well as amygdala-predominant Lewy body pathology. In addition, immunohistochemistry for TDP-43 revealed neuronal and glial cytoplasmic inclusions in the dentate gyrus of the hippocampus, amygdala, and inferior temporal cortex. Increasing awareness of the newly defined entity LATE will enhance our understanding of the neurodegenerative processes that occur in the oldest individuals.

Keyword

TDP-43 proteinopathy; limbic system; Alzheimer disease; Lewy body disease; autopsy