[Neuropathological similarities and differences between frontotemporal lobar degeneration with ubiquitin inclusions and amyotrophic lateral sclerosis with dementia].
Findings of clinical, neuropathological and biochemical studies have supported the idea that frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) are part of a neurological disease spectrum. This concept is now further strengthened by the recent discovery of a 43-kDa transactivating responsive sequence DNA-binding protein (TDP-43) as a key component of the underlying neuropathology of FTLD-U, ALS with dementia (ALS-D) and ALS. Here we describe the clinicopathological features of selected autopsy cases belonging to this disease spectrum, and discuss the neuropathological similarities and differences between FTLD-U and ALS-D, with special reference to the morphology, distribution and density of ubiquitin/TDP-43-positive abnormal structures, along with a review of the literature.
Tan
Chun-Feng
CF
Department of Pathology, Brain Research Institute, Niigata University, 1-757 Asahimachi, Chuo-ku, Niigata 951-8585, Japan.
Toyoshima
Yasuko
Y
Kakita
Akiyoshi
A
Takahashi
Hitoshi
H
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D001921:Brain / Q000473:pathology; D057174:Frontotemporal Lobar Degeneration / Q000473:pathology*; D006801:Humans; D002479:Inclusion Bodies / Q000737:chemistry*; D008297:Male; D008875:Middle Aged; D025801:Ubiquitin / Q000032:analysis*
D025801:Ubiquitin