An Autopsy Case of Amiotrophic Lateral Sclerosis Characterized by Upper Motor Neuron Degeneration and Progressive Pseudobulbar Palsy Raita Kikuchi 1 , Kenji Ishihara 2 , Kiyomi Nagumo 1 , Junichi Sioda 3 , Mitsuru Kawamura 4 , Mari Yoshida 5 1Department of Neurology, Ushioda General Hospital 2Department of Internal Medicine, Ushioda General Hospital 3Ushioda Home Clinic 4Okusawa Hospital & Clinics 5Institute for Medical Science of Aging Aichi Medical University Keyword: 偽性球麻痺 , 上位運動ニューロン , 前頭側頭葉変性症 , 筋萎縮性側索硬化症 , TDP-43 , amyotrophic lateral sclerosis , dementia , pseudobulbar palsy , anosognosia pp.75-80
Published Date 2019/1/1
DOI https://doi.org/10.11477/mf.1416201219
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This is a case report of autopsy findings for a male Japanese patient who presented with progressive gait disturbance and dysarthria. Neurological examination at the age of 61 years revealed pseudobulbar palsy and upper motor neuron disorder. The patient appeared unaware of his illness. Electrophysiological examination showed lower neuron damage. The patient was diagnosed with amyotrophic lateral sclerosis characterized by lower motor neuron damage. He died of pneumonia 2 years and 3 months after diagnosis. Neuropathological examination demonstrated severe degeneration of the upper neurons and mild degeneration of the lower neurons. Immunohistological examination indicated transactivation-responsive DNA-binding protein-43-positive pathology in the frontal and temporal lobes, amygdala, corpus striatum, and spinal cord. We believe the neuropathological findings correlate well with the clinical features. Furthermore, we also discuss the lesions involved in the patient's dementia.

(Received March 28, 2017; Accepted September 19, 2018; Published November 1, 2018)

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