Successful management of dyspnea in a patient with stiff person syndrome with noninvasive positive-pressure ventilation: A case report

Abstract

Approximately half of patients with stiff person syndrome experienced dyspnea due to the involvement of the respiratory muscles. If these muscles are severely affected, respiratory failure can occur. Here, we describe a patient with stiff person syndrome whose nighttime dyspnea was successfully controlled by noninvasive positive-pressure ventilation (NIPPV). A 38-year-old female patient visited the emergency room due to nighttime dyspnea. Her O2 saturation was 93%, and PaCO2 increased to 53.4 mmHg on arterial blood gas analysis. No specific lesion was found on the chest radiograph. We considered that her dyspnea was caused by respiratory muscle stiffness following SPS. For the management of nighttime hypercapnia, we applied NIPPV with volume-targeted assist-control ventilation mode using an oronasal mask interface during sleep. After such treatment, PaCO2 was normalized, and nighttime dyspnea disappeared. We think that NIPPV would be a useful therapeutic option for managing dyspnea induced by stiff person syndrome.