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Dyspnea in Patients with Stiff-Person Syndrome.

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eMediNexus    14 August 2019

A new study published in The American Journal of Medical Sciences aimed to analyze the occurrence of dyspnea inpatients with Stiff-person syndrome (SPS) – a rare autoimmune disorder characterized by progressively worsening stiffness and spasm of thoracic and proximal-limb musculature. This prospective study included 17 patients from a university-based neurology clinic. Dyspnea was assessed using vertical visual analog scales (VAS), the University of California San Diego Shortness of Breath Questionnaire (UCSD-SOBQ) and dyspnea "descriptors". Standardized assessments of SPS severity were performed by an experienced neurologist. Forced vital capacity (FVC) spirometric analysis was performed on all patients.It was noted that 17 patients complained of dyspnea—including dyspnea at rest, on exertion and sleep disturbance. A restrictive pattern was the most common abnormality noted on spirometry. While FVCand forced expiratory volume in 1-second (FEV1) percent predicted, correlated with dyspnea measured by VAS over the preceding 2 weeks. On the other hand, pulmonary function did not correlate with UCSB-SOBQ or standardized measures of SPS severity.Hence, it was inferred that dyspnea in SPS is common and occurs at rest with exertion and disturbs sleep. The finding of restrictive physiology and correlation between pulmonary function variables and dyspnea support the hypothesis that thoracic cage constriction by rigidity and/or spasm of the muscles of the trunk causes or contributes to the sensation of dyspnea. However, the possibility of diaphragmatic involvement requires further study.

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