Relationship between Bunina bodies and TDP-43 inclusions in spinal anterior horn in amyotrophic lateral sclerosis

• Published in: Neuropathology and applied neurobiology Vol. 36; no. 4; pp. 345 - 352
• Main Authors: Mori, F., Tanji, K., Miki, Y., Kakita, A., Takahashi, H., Wakabayashi, K.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.06.2010; Blackwell
• Subjects: Aged; Aged, 80 and over; amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - metabolism; Amyotrophic Lateral Sclerosis - pathology; Biological and medical sciences; Bunina body; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; DNA-Binding Proteins - metabolism; DNA-Binding Proteins - ultrastructure; Female; Humans; Immunohistochemistry; Inclusion Bodies - metabolism; Inclusion Bodies - pathology; Inclusion Bodies - ultrastructure; Lumbar Vertebrae; Male; Medical sciences; Microscopy, Immunoelectron; Middle Aged; Neurology; Neurons - metabolism; Neurons - pathology; Neurons - ultrastructure; skein-like inclusion; Spinal Cord - metabolism; Spinal Cord - pathology; Spinal Cord - ultrastructure; TDP-43; ultrastructure; Spinal cord; Nervous system diseases; Inclusion; skein-like inclusion; Central nervous system; Amyotrophic lateral sclerosis; Anterior spinal horn; Bunina body; Ultrastructure; TDP-43; Central nervous system disease; Degenerative disease; Inclusion body; Spinal cord disease
• ISSN: 0305-1846; 1365-2990
• DOI: 10.1111/j.1365-2990.2010.01081.x

F. Mori, K. Tanji, Y. Miki, A. Kakita, H. Takahashi and K. Wakabayashi (2010) Neuropathology and Applied Neurobiology36, 345–352
Relationship between Bunina bodies and TDP‐43 inclusions in spinal anterior horn in amyotrophic lateral sclerosis Aims: Amyotrophic lateral sclerosis (ALS) is characterized by upper and lower motor neurone involvement with Bunina bodies (BBs) and TDP‐43 inclusions. To elucidate the relationship between BBs and TDP‐43 inclusions, we examined the spinal cord from 18 patients with ALS. Methods: Five serial sections from lumbar cord were first stained with haematoxylin and eosin to detect BBs and subsequently immunostained with anti‐TDP‐43 antibody. Immunoelectron microscopy was performed on vibratome sections from two cases of ALS. Results: BBs were found in 15 out of 18 cases. TDP‐43 inclusions were found in all the cases. The average incidence of anterior horn cells with BBs and TDP‐43 inclusions relative to the total number of neurones was 17.1% and 46.4%, respectively. The concurrence of both inclusions in the same neurones was found in 15 cases. The incidence of co‐localization of BBs and TDP‐43 inclusions was 15.7% of total neurones. The frequency of TDP‐43 inclusions was significantly higher in neurones with BBs than in those without. Ultrastructurally, TDP‐43‐immunoreactive filamentous structures were intermingled with early‐stage BBs, but not associated with advanced‐stage BBs. Conclusion: These findings suggest that there is a close relationship in the occurrence between BBs and TDP‐43 inclusions.