ArticleBrain sciences2024
Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy.
Article in Brain sciences, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Critical Kennedy's disease with Guillain-Barré syndrome and respiratory failure: A case report.Medicine · 2026Article
- Clinical Significance of Plasma Sphingosine-1-Phosphate Decline in Progression of Spinal and Bulbar Muscular Atrophy.Journal of clinical neurology (Seoul, Korea) · 2026Article
- The Impact of Surgical Correction of Neuromuscular Scoliosis on Respiratory Muscle Function in Individuals with Spinal Muscular Atrophy-Preliminary Report.Journal of clinical medicine · 2026Article
- Neuromuscular junction transcriptome analysis of spinal and bulbar muscular atrophy mice implicates sarcomere gene expression and calcium flux dysregulation in disease pathogenesis.Human molecular genetics · 2025Article
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Authors and funding
8 authors.
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Abstract
BACKGROUND/
objectivesThe primary life-threatening complication in spinal-bulbar muscular atrophy (SBMA) is ventilatory failure. The present study analyzes the longitudinal patterns of respiratory function tests over a follow-up of 11 years.
methodsWe collected data from 9 genetically confirmed SBMA patients. Spirometric measurements [maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), and forced vital capacity (FVC)], serum biochemical indices and SBMA functional rating scale (SBMAFRS) were collected every 6 months for 11 years. An average time curve was utilized to assess the changes in both pulmonary tests and serum biochemical indices of the patients. One-way repeated-measures ANOVA was applied to assess statistical differences. The Spearman's rank correlation coefficient was utilized to evaluate the correlations between the respiratory function tests and serum biochemical and clinical indices.
resultsA progressive decrease was observed in the respiratory function tests; the slope of the linear regression was significantly non-zero (
conclusionsThis longitudinal study shows a progressive decline of spirometric data throughout life in SBMA patients. The decline appears to be related to clinical deterioration and muscle denervation. Spirometric measures relative to maximal strength of the respiratory muscles (MIP and MEP) may have a better predictive value for pulmonary and muscular decline than FVC.
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