Progressive Respiratory Failure in an Autopsy Case of Amyotrophic Lateral Sclerosis With Cardiac and Pulmonary Amyloidosis

ABSTRACT A 76‐year‐old woman died of progressive respiratory failure 10 months after amyotrophic lateral sclerosis (ALS) onset. At autopsy, a neuropathological diagnosis of ALS was made. Only mild neurogenic changes appeared in the diaphragm. Transthyretin amyloid deposits were observed in the heart and lungs and diffusely located in the alveolar parenchyma, indicating diffuse alveolar‐septal pulmonary amyloidosis, which could impair gas exchange. Pulmonary amyloidosis may contribute to respiratory failure, potentially acting synergistically with respiratory muscle paralysis due to ALS and cardiac failure caused by cardiac amyloidosis.

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Journal
Neurology and Clinical Neuroscience
Published
2025-04-17
DOI
https://doi.org/10.1111/ncn3.70014
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
Field-Weighted Citation Impact
0.00

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article

Progressive Respiratory Failure in an Autopsy Case of Amyotrophic Lateral Sclerosis With Cardiac and Pulmonary Amyloidosis

Masahiko Tomiyama, Tomoya Kon, Takashi Nakamura, Chieko Suzuki et al.
Neurology and Clinical Neuroscience
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Progressive Respiratory Failure in an Autopsy Case of Amyotrophic Lateral Sclerosis With Cardiac and Pulmonary Amyloidosis

Masahiko Tomiyama, Tomoya Kon, Takashi Nakamura, Chieko Suzuki, Haruo Nishijima, Fumiaki Mori, Koichi Wakabayashi, Shintaro Goto
article en

Abstract

ABSTRACT A 76‐year‐old woman died of progressive respiratory failure 10 months after amyotrophic lateral sclerosis (ALS) onset. At autopsy, a neuropathological diagnosis of ALS was made. Only mild neurogenic changes appeared in the diaphragm. Transthyretin amyloid deposits were observed in the heart and lungs and diffusely located in the alveolar parenchyma, indicating diffuse alveolar‐septal pulmonary amyloidosis, which could impair gas exchange. Pulmonary amyloidosis may contribute to respiratory failure, potentially acting synergistically with respiratory muscle paralysis due to ALS and cardiac failure caused by cardiac amyloidosis.

Neurology and Clinical NeuroscienceVol. 13(4)
Hirosaki University (JP)
Japan Society for the Promotion of Science
Openalex Percentile: Top 89%
Amyloidosis: Diagnosis, Treatment, Outcomes
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Progressive Respiratory Failure in an Autopsy Case of Amyotrophic Lateral Sclerosis With Cardiac and Pulmonary Amyloidosis — Masahiko Tomiyama, Tomoya Kon, et al. · Neurology and Clinical Neuroscience (2025) | TGRS Research Map | TGRS