A subgroup of multiple system atrophy with rapid decline in vital capacity.

INTRODUCTION

Patients with neurodegenerative disorders resulting in progressive dysphagia often require gastrostomy. Pulmonary function tests (PFTs) are crucial in presurgical evaluation; however, reports on pulmonary function in multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) are limited.

MATERIALS AND

METHODS

This single-center, retrospective study analyzed the PFT records from patients with MSA and PSP admitted between January 2012 and October 2023.

RESULTS

Data from 104 PFTs in 70 patients with MSA (21 MSA-P, 49 MSA-C) and 29 PFTs in 22 patients with PSP, showed a weak correlation between the percentage of measured vital capacity to the predicted vital capacity (%VC) and disease duration (MSA: -0.34 and PSP: -0.19). The variation in %VC decline was greater in MSA than in PSP, with a rapid progression (%VC < 65 % within 5 years of disease duration) in seven patients with MSA (6 MSA-P and 1 MSA-C).

CONCLUSION

A decline in %VC with disease duration was observed in both MSA and PSP, with greater variation observed in MSA. Notably, a rapid decline in %VC was predominantly observed in patients with MSA-P during the early courses.