An autopsy case of early‐stage amyotrophic lateral sclerosis with TDP‐43 immunoreactive neuronal, but not glial, inclusions

• Published in: Neuropathology Vol. 39; no. 3; pp. 224 - 230
• Main Authors: Kon, Tomoya, Mori, Fumiaki, Oyama, Yoshinobu, Tanji, Kunikazu, Kimura, Tamaki, Takahashi, Shirushi, Wakabayashi, Koichi
• Format: Journal Article
• Language: English
• Published: Melbourne John Wiley & Sons Australia, Ltd 01-06-2019; Wiley Subscription Services, Inc
• Subjects: Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - pathology; Autopsy; Brain stem; Bunina bodies; Cortex (motor); Degeneration; DNA-Binding Proteins - analysis; Frontotemporal dementia; Glial cells; glial cytoplasmic inclusion; Humans; Inclusion bodies; Male; Middle Aged; motor neuron disease; Motor neurone disease; Motor Neurons - chemistry; Motor Neurons - pathology; Motor nuclei; Neuroglia - chemistry; Neuroglia - pathology; neuronal cytoplasmic inclusion; Neuronal-glial interactions; Spinal cord; Suicide; TDP‐43; motor neuron disease; amyotrophic lateral sclerosis; TDP-43; glial cytoplasmic inclusion; neuronal cytoplasmic inclusion
• ISSN: 0919-6544; 1440-1789
• DOI: 10.1111/neup.12554

Phosphorylated transactivation response DNA‐binding protein 43 kDa (p‐TDP‐43)‐immunoreactive neuronal and glial cytoplasmic inclusions are a histopathological hallmark of sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP‐43. We report an autopsy case of lower motor neuron‐predominant ALS in a 47‐year‐old Japanese man who committed suicide 5 months after onset. Histopathologically, neuronal loss was restricted to the anterior horn of the spinal cord, and no obvious neuronal loss was noted in the motor cortex or brainstem motor nuclei. Bunina bodies were found in the spinal anterior horn cells and the facial and hypoglossal nuclei. Immunohistochemically, p‐TDP‐43‐immunoreactive neuronal, but not glial, cytoplasmic inclusions were frequently found in the spinal anterior horn and facial and hypoglossal nuclei, and rarely in the motor cortex. We considered the present case to be an example of lower motor neuron‐predominant ALS. p‐TDP‐43‐immunoreactive aggregates in neurons, but not in glial cells, may be an early‐stage pathology of ALS.