Key result
Autopsy of a patient with fatal post-polio syndrome demonstrated motoneuron loss and glial scars throughout the spinal cord without ALS-specific inclusions, confirming PPS as the cause of progressive muscle weakness and respiratory failure.
Case Report (n=1)
No
This case report demonstrates that post-polio syndrome can rarely be fatal due to rapidly progressive dyspnea and dysphagia, with autopsy showing widespread motoneuron loss without ALS-specific inclusions.
Alerts clinicians to rare fatal PPS; supports PPS-ALS distinction but leaves open larger series.
We report the case of a 72-year-old man who had contracted acute paralytic poliomyelitis in his childhood. Thereafter, he had suffered from paresis involving the left lower limb, with no relapse or progression of the disease. He began noticing slowly progressive muscle weakness and atrophy in the upper and lower extremities in his 60s. At the age of 72, muscle weakness developed rapidly, and he demonstrated dyspnea on exertion and dysphagia. He died after about 14 years from the onset of muscle weakness symptoms. Autopsy findings demonstrated motoneuron loss and glial scars not only in the plaque-like lesions in the anterior horns, which were sequelae of old poliomyelitis, but also throughout the spine. No Bunina bodies, TDP-43, and ubiquitin inclusions were found. Post-polio syndrome is rarely fatal due to rapid progressive dyspnea and dysphagia. Thus, the pathological findings in the patient are considered to be related to the development of muscle weakness.
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Oki et al. (2015) conducted a case report in Post-polio syndrome (n=1). Post-polio syndrome was evaluated on Pathological findings at autopsy. Autopsy of a patient with fatal post-polio syndrome demonstrated motoneuron loss and glial scars throughout the spinal cord without ALS-specific inclusions, confirming PPS as the cause of progressive muscle weakness and respiratory failure.
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