Postmortem investigations on pathological and diagnostic biomarkers of frontotemporal lobar degeneration

2016 Fiscal Year Final Research Report

• Project/Area Number: 15K19483
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Neurology
• Research Institution: Nagoya University
• Principal Investigator: Riku Yuichi 名古屋大学, 医学部附属病院, 医員 (50748382)
• Project Period (FY): 2015-04-01 – 2017-03-31
• Keywords: 前頭側頭葉変性症 / TDP-43 / 線条体 / 剖検脳

Outline of Final Research Achievements

We analyzed pathological changes in the striatal efferent system of 59 consecutively autopsied patients with sporadic FTLD-TDP or ALS-TDP. The axon terminals of striatal efferent neurons were immunohistochemically assessed in the substantia nigra pars reticulata (SNr) and globus pallidus (GP). All of the FTLD-TDP patients exhibited a marked depletion of axon terminals, irrespective of disease duration. In particular, losses of substance-P-positive projections to the SNr and internal segment of GP were consistently severe. Similar findings were also observed in 69.0% of the ALS-TDP patients, although the severity was much less than that in the FTLD-TDP patients. The accumulation of phosphorylated TDP-43 was observed in the striatal efferent neurons, efferent tracts, or their axon terminals in the SNr and GP in both groups. Thus, striatal efferent projections are essentially and commonly involved in the TDP-43-related FTLD/ALS disease spectrum.

Free Research Field

神経病理学