Clinical Implications of Routine Monitoring of Pulmonary Function and Ventilation in Patients with Duchenne Muscular Dystrophy

Purpose@#To investigate the effect of regular monitoring of pulmonary function and ventilatory status on the initiation of non-invasive ventilation (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 received NIV between 2010 and 2019. The subjects were assigned to either the regular-follow-up (REG) group or the non-REG group, according to their follow-up status, before initiating NIV. We compared the number of emergent cases, the results of nocturnal ventilatory monitoring, and the pulmonary function of each group at initial ventilatory support. @*Results@#In total, 73 subjects were enrolled in the REG group and 47 subjects in the non-REG group. There were significantly more emergency cases due to respiratory insufficiency in the non-REG group (12/47, 25.5%) than in the REG group (3/73, 4.1%). At the time of initial ventilatory support, hypoventilatory symptoms were more common and relatively severe in the non-REG group (37/47, 78.7%) than in the REG group (18/73, 24.7%). The average age at initial ventilatory support of the non-REG group was 2.15 years older than that of the subjects in the REG group. Moreover, subjects who were not regularly monitored exhibited greater deterioration in pulmonary function compared to those who were regularly followed up. @*Conclusion@#Regular evaluation of pulmonary function and ventilatory status before the onset of ventilatory insufficiency is crucial to reduce the risk of patients with DMD requiring emergency care due to ventilatory insufficiency.

Complication of the Cervical Thrust Technique in a Patient with Athetoid Cerebral Palsy: A Case Report

Manual therapy has been widely practiced in almost all countries worldwide. With its potential complications, there are contraindications for thrust technique. A 56-year-old woman diagnosed with athetoid cerebral palsy visited a local clinic due to a tingling sensation in right hand that lasted for a year and aggravated to both upper extremities. The patient underwent three sessions of cervical manual therapy including thrust technique. No evaluation was performed. The patient immediately felt weakness in all extremities after performing cervical thrust technique. Magnetic resonance imaging showed atlantoaxial instability and cervical myelopathy. Approximately one-third of adults with cerebral palsy reported chronic musculoskeletal pain and they often experience neck pain. Particularly in athetoid cerebral palsy, malalignment or instability of the cervical spine is prevalent and often results in myelopathy. Therefore, musculoskeletal evaluation is necessary to identify cervical instability in case of cervical thrust technique, and it should be performed by relevant medical professionals.

Effect of Ultrasound-Guided Perineural Injection with Dextrose for Direct Traumatic Injury of Median Nerve

Ultrasound (US)-guided hydrodissection (HD) is a widely applied therapeutic method to release the entrapped peripheral nerve. However, this therapy has only been studied for the nerve entrapments such as carpal tunnel syndrome, and there are no reports of its effect on direct nerve injuries with incomplete axonal damage. Here, we report a case of direct traumatic injury of a median nerve with incomplete axonal injury in a 28-year-old man. He presented hypoesthesia and weakness along with the median nerve territory of the left hand after a laceration wound of the wrist. The patient underwent a surgical procedure, but did not experience prominent improvement for the next six months. Symptoms improved after we performed the US-guided HD with dextrose. We propose this procedure as one of the new treatment methods for direct axonal injury of nerves including the median nerve.

Ultrasound Guided Thoracic Paravertebral Space Block for Chronic Intractable Upper Back Pain

There are some cases of myofascial pain syndrome (MPS) with chronic upper back pain that does not respond to dry needling or trigger point injection, well-known treatments for MPS. A 67-year-old female developed a stabbing upper back pain with trigger point at left T7∼8 levels 10 years ago. She complained of the pain with Numeral Rating Scale (NRS) 8 points. Myofascial release technique and trigger point injection had no effect. Under ultrasound guidance 20 ml of 1% lidocaine was injected into thoracic paravertebral space. Immediately, the pain was reduced to NRS 4 points. One week later, the second block was performed in the same way as the first, and the pain was reduced to NRS 2 points. The stabbing pain disappeared, and oral opioids were discontinued. Ultrasound guided thoracic paravertebral space block is an effective and safe treatment for refractory MPS with chronic upper back pain.

Incidence and Reappraisal of Known Risk Factors Associated With Carpal Tunnel Syndrome: A Nationwide, 11-Year, Population-Based Study in South Korea

Background@#and PurposePrevious studies have revealed various risk factors for carpal tunnel syndrome (CTS), but few large-scale studies have been conducted. We used data from the 11-year, longitudinal, nationwide population-based National Health Insurance Service–National Health Screening cohort to identify the actual risk factors for CTS. @*Methods@#We collected patients with CTS newly diagnosed using electrodiagnostic studies while excluding radiculopathy, plexopathy, or polyneuropathy, which can be confused with CTS. The crude and standardized incidence rates of CTS were calculated. Univariate and multivariate Cox analyses and the incidence of CTS were used to identify the risk factors for newly diagnosed CTS. @*Results@#The standardized incidence was 130.8/100,000 person-years based on the World Health Organization World Standard Population as a reference. Multivariate Cox analysis identified that the risk factors for CTS were being middle-aged, female, and obese, and having rheumatoid arthritis and Raynaud's syndrome, whereas gout and hypothyroidism were not risk factors. Diabetes and end-stage renal disease did not show a significant hazard ratio, although it is implicit that the durations of these diseases affect the development of CTS. @*Conclusions@#This study calculated the incidence of CTS and reappraised the associated risk factors found in previous studies. This information will be helpful for determining the pathophysiology of CTS, and hence aid the establishment of effective new public health policies.

Ultrasonography-Guided Multifidus Cervicis Plane Block as a New Approach for the Treatment of Chronic Axial Neck Pain

Multifidus cervicis plane block has been effectively used to provide analgesia during and after cervical spine surgery, but not for any other purpose. Here, we report three cases of chronic axial neck pain unresponsive to medical treatment. We performed multifidus cervicis plane block bilaterally, which lowered numerical rating scale (NRS) score within 2 weeks. Thus, multifidus cervicis plane block is one of the novel options that may be used for chronic axial neck pain.

Incidence and Reappraisal of Known Risk Factors Associated With Carpal Tunnel Syndrome: A Nationwide, 11-Year, Population-Based Study in South Korea

Background@#and PurposePrevious studies have revealed various risk factors for carpal tunnel syndrome (CTS), but few large-scale studies have been conducted. We used data from the 11-year, longitudinal, nationwide population-based National Health Insurance Service–National Health Screening cohort to identify the actual risk factors for CTS. @*Methods@#We collected patients with CTS newly diagnosed using electrodiagnostic studies while excluding radiculopathy, plexopathy, or polyneuropathy, which can be confused with CTS. The crude and standardized incidence rates of CTS were calculated. Univariate and multivariate Cox analyses and the incidence of CTS were used to identify the risk factors for newly diagnosed CTS. @*Results@#The standardized incidence was 130.8/100,000 person-years based on the World Health Organization World Standard Population as a reference. Multivariate Cox analysis identified that the risk factors for CTS were being middle-aged, female, and obese, and having rheumatoid arthritis and Raynaud's syndrome, whereas gout and hypothyroidism were not risk factors. Diabetes and end-stage renal disease did not show a significant hazard ratio, although it is implicit that the durations of these diseases affect the development of CTS. @*Conclusions@#This study calculated the incidence of CTS and reappraised the associated risk factors found in previous studies. This information will be helpful for determining the pathophysiology of CTS, and hence aid the establishment of effective new public health policies.

Ultrasonography-Guided Multifidus Cervicis Plane Block as a New Approach for the Treatment of Chronic Axial Neck Pain

Multifidus cervicis plane block has been effectively used to provide analgesia during and after cervical spine surgery, but not for any other purpose. Here, we report three cases of chronic axial neck pain unresponsive to medical treatment. We performed multifidus cervicis plane block bilaterally, which lowered numerical rating scale (NRS) score within 2 weeks. Thus, multifidus cervicis plane block is one of the novel options that may be used for chronic axial neck pain.

Precise Pulmonary Function Evaluation and Management of a Patient With Freeman-Sheldon Syndrome Associated With Recurrent Pneumonia and Chronic Respiratory Insufficiency

Freeman-Sheldon syndrome (FSS) is a rare distal arthrogryposis syndrome. There are few reports on the respiratory insufficiency of FSS. Additionally, there is no detailed information on pulmonary functional evaluation. A 17-year-old male patient with FSS developed respiratory failure, leading him to be admitted to hospital several times for evaluation and treatment. Of those times he was admitted, two were due to pneumonia. His pulmonary functions were indicative of a restrictive lung disease potentially caused by severe scoliosis. After a non-invasive ventilatorwas applied correctly to the patient, pulmonary hypertension was normalized. His pulmonary function has been maintained for 13 years. Since receiving proper respiratory care, which includes assisted coughing methods, the patient has not developed pneumonia. It is important to properly evaluate the pulmonary function of patients who have FSS and scoliosis to eliminate the risk of long-term respiratory complications.

Clinical Usefulness of the Korean Developmental Screening Test (K-DST) for Developmental Delays

OBJECTIVE: To evaluate the clinical usefulness of the Korean Developmental Screening Test (K-DST) via comparison with Korean Ages and Stages Questionnaire (K-ASQ) for the diagnosis of developmental delay in pediatric patients. METHODS: The K-DST and K-ASQ were used to screen pediatric patients who visited the hospital for evaluation and diagnosis of delayed development. Korean Bayley Scales of Infant Development-II (K-BSID-II) or Korean Wechsler Preschool and Primary Scale of Intelligence III (K-WPPSI-III) were used for the standardized assessment. Moreover, the final clinical diagnosis was confirmed by three expert physicians (rehabilitation doctor, psychiatrist, and neurologist). The sensitivity and specificity of each screening tool for the final diagnosis were investigated and correlated with standardized assessments. RESULTS: A total of 145 pediatric consultations were conducted, which included 123 developmental disorders (40 autism spectrum disorders, 46 global developmental delay/intellectual disability, and 37 developmental language disorders) and another 22 that were not associated with any such disorders. The sensitivity and specificity of K-DST based on the final clinical diagnosis were 82.9% and 90.9%, respectively, which were not significantly different from that of K-ASQ (83.7% and 77.3%). Both K-DST and K-ASQ showed good correlation with K-BSID-II and K-WPPSI-III. No significant difference was found between the K-DST and K-ASQ measures. CONCLUSION: K-DST is an excellent screening tool and is expected to replace K-ASQ with high validity.

Management of Ventilatory Insufficiency in Neuromuscular Patients Using Mechanical Ventilator Supported by the Korean Government

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 symptoms of chronic hypoventilation were surveyed by phone. Home visits were conducted for patients judged by an expert medical team to require medical examination. Abnormal ventilatory status was assessed by respiratory evaluation. Chronic respiratory insufficiency symptoms were reported by 169 of 992 subjects (17%), while 565 subjects (57%) did not receive regular respiratory evaluation. Ventilatory status was abnormal in 102 of 343 home-visit subjects (29.7%). Although 556 subjects (56%) reported 24-hour ventilator use, only 458 (46%) had an oxygen saturation monitoring device, and 305 (31%) performed an airstacking exercise. A management system that integrates ventilator usage monitoring, counselling and advice, and home visits for patients who receive ventilator support could improve the efficiency of the ventilator support project.

Comparison of Pulmonary Functions at Onset of Ventilatory Insufficiency in Patients With Amyotrophic Lateral Sclerosis, Duchenne Muscular Dystrophy, and Myotonic Muscular Dystrophy

OBJECTIVE: To evaluate pulmonary functions of patients with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and myotonic muscular dystrophy (MMD) at the onset of ventilatory insufficiency. METHODS: This retrospective study included ALS, DMD, and MMD patients with regular outpatient clinic follow-up in the Department of Rehabilitation Medicine at Gangnam Severance Hospital before the application of non-invasive positive pressure ventilation (NIPPV). The patients were enrolled from August 2001 to March 2014. If patients experienced ventilatory insufficiency, they were treated with NIPPV, and their pulmonary functions were subsequently measured. RESULTS: Ninety-four DMD patients, 41 ALS patients, and 21 MMD patients were included in the study. The mean SpO2 was lower in the MMD group than in the other two groups. The mean forced vital capacity (FVC) in the supine position was approximately low to mid 20% on average in DMD and ALS patients, whereas it was 10% higher in MMD patients. ALS patients showed a significantly lower FVC in the supine position than in the sitting position. Maximal insufflation capacity, unassisted peak cough flow, maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP) were significantly higher in MMD group than in the other groups. MEP was significantly the lowest in DMD patients, followed by in ALS, and MMD patients, in order. CONCLUSION: Disease-specific values of pulmonary function, including FVC, MEP, and MIP, can be accurately used to assess the onset of ventilatory insufficiency in patients with ALS, DMD, and MMD.