Won-A Choi

Purpose: The purpose of this study is to investigate how respiratory muscle strength correlates to cough capacity in patients with respiratory muscle weakness. Materials and Methods: Forty-five patients with amyotrophic lateral sclerosis (ALS), 43 with cervical spinal cord injury (SCI), and 42 with Duchenne muscular dystrophy (DMD) were recruited. Pulmonary function tests including forced vital capacity (FVC) and respiratory muscle strength (maximal expiratory pressure, MEP;maximal inspiratory p

Purpose: To assess the ability of a mechanical in-exsufflator (MI-E), either alone or in combination with manual thrust, to augment cough in patients with neuromuscular disease (NMD) and respiratory muscle dysfunction. Materials and Methods: For this randomized crossover single-center controlled trial, patients with noninvasive ventilator-dependentNMD were recruited. The primary outcome was peak cough flow (PCF), which was measured in each patient after a cough that was unassisted, manually assi

Purpose: Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are similar genetic disorders whose patternsof mutation and disease phenotypes might be expected to show differences among different countries. We analyzed multiplexligation-dependent probe amplification (MLPA) data in a large number of Korean patients with DMD/BMD. Materials and Methods: We obtained 130 positive MLPA results (86 DMD, 27 BMD, and 17 female carriers) from 272 candidates (237 clinically suspected patien

Purpose: One obstacle in early diagnosis of amyotrophic lateral sclerosis (ALS) is its vague initial presentation, which is generallyclassified into limb- and bulbar-dominant types and may be mistaken for other musculoskeletal conditions. We analyzed clinicaldata from patients in relation to their initial presentation and prognosis from symptom onset to diagnosis. Materials and Methods: We retrospectively analyzed the medical records of patients with ALS who were admitted for pulmonaryrehabilita

Objective: To investigate the real condition of pulmonary rehabilitation for patients with advanced neuromuscular diseases (NMDs) on mechanical ventilation in Korea. Method: In order to estimate current state of pulmonary rehabilitative management, chart review and pulmonary function evaluation were conducted in a total of 267 NMD patients who had applied mechanical home ventilator in our center from March 2001 to December 2008. Results: Total 267 patients were included: 95 with Duchenne muscula

Purpose: To survey the use of invasive and noninvasive home mechanical ventilation(HMV) methods in South Korea from the perspective of physical medicine and rehabilitation (PM&R). Materials and Methods: For 413 users of HMV, retrospectivereviews of PM&R interventions and survey of HMV methods employed from Mar 2000 to Dec 2009. Results: Of the 413 users, the majority of whom with progressive neuromuscular disorders (NMDs) (n=358), 284 patients initially used noninvasive mechanical ventilation (N

Objective: To verify the safety and clinical utility of noninvasive respiratory management as an alternative method of invasive respiratory management for the patients with cervical spinal cord injury (CSCI) who often present with ventilatory insufficiency (due to inspiratory muscle paralysis) or difficulty in removing airway secretions (because of expiratory muscle weakness). Method: Nineteen patients with CSCI (male: 15, female: 4, mean age: 45.6) were recruited. All of the patients were in ne

Duchenne muscular dystrophy (DMD) is a severe X-linked recessive disorder characterized by rapid progression of muscle wasting and weakness. Long term noninvasive intermittent positive pressure ventilation (NIPPV) is an important treatment for neuromuscular patients with chronic respiratory failure. NIPPV is easy to administer and life-threatening complications are rare. We report two cases of pneumothorax associated with long term NIPPV in DMD. Given the increasing utilization of chronic NIPPV,

Since 2001, financial support has been provided for all patients with neuromuscular disease (NMD) who require ventilatory support due to the paralysis of respiratory muscles in Korea. The purpose of this study was to identify ventilator usage status and appropriateness in these patients. We included 992 subjects with rare and incurable NMD registered for ventilator rental fee support. From 21 February 2011 to 17 January 2013, ventilator usage information, regular follow-up observation, and sympt

Purpose: To investigate the effect of regular monitoring of pulmonary function and ventilatory status on the initiation of non-invasiveventilation (NIV) between patients who were routinely monitored before receiving NIV and those who were not. Materials and Methods: This retrospective cohort study included subjects with Duchenne muscular dystrophy (DMD) who first receivedNIV between 2010 and 2019. The subjects were assigned to either the regular-follow-up (REG) group or the non-REG group,accordi

Objective To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis.Methods Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected. Survival was a