title: Comparison of phosphorylated TDP-43-positive inclusions in oculomotor neurons in patients with non-ALS and ALS disorders.
abstract: TDP-43 has been identified as a major component of the pathological inclusions in most forms of frontotemporal lobar degeneration with ubiquitin-positive inclusions and in amyotrophic lateral sclerosis (ALS). In the present study, paraffin sections of the midbrain in 112 patients with various non-ALS disorders and 27 patients with sporadic ALS were immunostained with antibody against phosphorylated TDP-43 (pTDP-43). pTDP-43-positive inclusions in oculomotor neurons were detected in 18 of 112 patients with non-ALS disorders (16.1%). The appearance of the inclusions showed fine filamentous structures rather than the skein-like inclusions seen in the anterior horn cells of ALS spinal cords. The incidence was increased in the age range of 80-89 years old (10/37 cases; 27.0%), in which 6 of 10 cases demonstrated AD pathology in the temporal lobes. Twenty-seven ALS patients were examined and the findings were compared with those of non-ALS patients. There were 13 cases demonstrating pTDP-43-positive inclusions (48.1%) which showed stronger immunoreactivities in ALS cases. This is the first report demonstrating fine filamentous pTDP-43-positive inclusions in oculomotor neurons in non-ALS disorders. Although the mechanisms underlying pTDP-43 in oculomotor neurons are currently unknown, its detection is of interest, and the expression may occur not only in ALS but also during the aging process.
authors.affiliation: Department of Neurology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan. mizunoy@med.gunma-u.ac.jp
authors.forename: Yuji
authors.initials: Y
authors.lastname: Mizuno
authors.forename: Yukio
authors.initials: Y
authors.lastname: Fujita
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Takatama
authors.forename: Koichi
authors.initials: K
authors.lastname: Okamoto
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism* / Q000473:pathology*; D004268:DNA-Binding Proteins / Q000096:biosynthesis*; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology*; D009802:Oculomotor Nerve / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology; D010766:Phosphorylation / Q000502:physiology; D055815:Young Adult
chemical_list: D004268:DNA-Binding Proteins