Multiple system atrophy (MSA) is a adult onset, neurodegenerative disease which includes autonomic dysfunction, cerebellar syndrome, and parkinsonism. The incidence rate is about 0.6 cases per 100,000 persons per year. Patients with MSA are clinically classified into cerebellar (MSA-C) and parkinsonism (MSA-P) subtypes. In MSA the motor symptoms affecting respiratory system are common. such as stridor (42%), sleep disordered breathing (37%), respiratory insufficiency, rarely Acute Respiratory Distress Syndrome (ARDS).
A 55-year-old man diagnosed as multiple system atrophy with mixed subtype (Figure 1.) (MSA-P&C) visited our emergency department with fever and dyspnea. Similar episode occurred a week ago, impression of acute pharyngitis have resolved with PO prednisolone 20 mg for 3 days. At the arrival, sPO2 79-80% and fever up to 39.8°C was admitted to the intensive care unit. He was emergently cannulated at the bedside for venovenous extracorporeal membrane oxygenation (VV-ECMO) and percutaneous tracheostomy was performed for refractory hypoxemia. In order to exclude organic problems bronchoscopy was performed and there was no endobronchial lesion. After stabilization of the respiratory status, the patient was transferred to Department of Physical Medicine & Rehabilitation for further evaluation and management. Video Fluoroscopy Swallowing Study (VFSS) and Pulmonary Function Test (Figure 2.) (PFT) were performed and revealed aspiration in drinking liquid 5cc and penetration in drinking cup of fluid thickner level 3, and also severe obstructive pattern and severe restrictive pattern by spirometric parameters with Forced Vital Capacity (FVC) = 49%, Forced Expired Volume in first second (FEV1) = 45%, Tiffeneau index (FEV1/FVC) = 67%. Swallowing and pulmonary rehabilitation was done for 2 week. Inspiratory resistance training, inspiratory threshold training, and isocapneic hyperventilation was done for strengthening inspiration muscle and accessory muscle. Also pulsed lip breathing and diaphragmatic breathing education was done. Follow up VFSS and PFT (Figure 3.) were performed which shown no penetration and aspiration in liquid cup drinking and mild restrictive pattern with FVC = 78%, FEV1 = 85%, FEV1/FVC = 73%.
According to a review of literatures, respiratory failure or dysfunction is one of multiple system atrophy's clinical feature. This case suggests that acute respiratory distress syndrome may occasionally arise MSA. Unexplained respiratory failure, bilateral vocal cord paralysis, stridor could be considered in the manifestation of MSA. When MSA is diagnosed, we should focus on respiration as it may be the cause of death in most patient. In this case, pulmonary rehabilitation might improved patient’s respiratory function. Also it could be helpful for other patient who is suffering from MSA. |
