Clinical and neuropathological classification of frontotemporal lobar degeneration :A review

Koichi Okamoto, M.D.

Department of Neurology, Gunma University Graduate School of Medicine

Frontotemporal lobar degeneration (FTLD) is the third most common cause of cortical dementia, following Alzheimer disease and dementia with Lewy body. Clinical and neuropathological criteria of FTLD were reviewed. Ubiquitin positive and tau negative neuronal inclusions were also reviewed. We investigated the relationship between TDP-43 immunoreactivities and fragmentation of the Golgi apparatus using anti-polyclonal trans-Golgi-network-46 antibodies in ALS spinal cords. Almost all anterior horn cells with abnormal TDP-43 immunoreactivities showed fragmentation of Golgi apparatus, which suggest neurons with abnormal TDP-43 immunoreactivities are associated with dysfunction of the secretory pathway in motor neurons. We recently produced polyclonal antibodies against phsophorylated C-terminal 12 amino acid of TDP-43 (p409/410 and p409), and those antibodies detected only abnormal aggregates of TDP-43.

Address correspondence to Dr. Koichi Okamoto, Department of Neurology, Gunma University Graduate School of Medicine (3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan)