Evaluation of Current Resources Available for Community-Based Cardiac Rehabilitation in Korea:A Nationwide Survey Study

Background@#In Korea, the actual distribution of cardiac rehabilitation (CR) to the clinical field is insufficient due to the many barriers for cardiovascular patients to participate in CR. Community-based CR is a useful alternative to overcome these obstacles. Through a nationwide survey, we investigated the possibility of regional medical and public health management institutes which can be in charge of community-based CR in Korea. @*Methods@#The questionnaires on recognition of CR and current available resources in health-related institutions were developed with reference to the CR evaluation tools of York University and the International Council of Cardiovascular Prevention and Rehabilitation.The questionnaires were sent to regional public and private medical institutions and public health management institutions. @*Results@#In total, 2,267 questionnaires were sent to 1,186 institutions. There were 241 and 242 responses from 173 and 179 regional private and public medical institutions, respectively. And a total of 244 responses were gathered from 180 public health management institutions. Although many institutions were equipped with the necessary facilities for exercise training, there were few patient-monitoring systems during exercise. Most institutions were aware of the need for CR, but were burdened with the cost of establishing personnel and facilities to operate CR. @*Conclusion@#Most regional medical, and public health management institutions in Korea are unprepared for the implementation of community-based CR programs. To encourage the utilization of such, there should be efforts to establish a national consensus.

Two Korean siblings with autosomal recessive spinocerebellar ataxia 20 caused by homozygous variants in SNX14

Autosomal recessive spinocerebellar ataxia 20 (SCAR20; OMIM #616354) is a recently described disorder that is characterized by ataxia, intellectual disability, cerebellar atrophy, macrocephaly, coarse face, and absent speech. It is caused by lossof-function mutations in SNX14. To date, all cases with homozygous pathogenic variants have been identified in consanguineous families. This report describes the first Korean cases of SCAR20 family caused by homozygous variants in SNX14. Two siblings were referred to our clinic because of severe global developmental delay. They presented similar facial features, including a high forehead, long philtrum, thick lips, telecanthus, depressed nasal bridge, and broad base of the nose. Because the older sibling was unable to walk and newly developed ataxia, repeated brain magnetic resonance imaging (MRI) was performed at the age of 4 years, revealing progressive cerebellar atrophy compared with MRI performed at the age of 2 years.The younger sibling’s MRI revealed a normal cerebellum at the age of 2 years. Whole-exome sequencing was performed, and homozygous variants, such as c.2746-2A>G, were identified in SNX14 from the older sibling. Sanger sequencing confirmed homozygous SNX14 variants in the two siblings as well as a heterozygous variant in both parents. This report extends our knowledge of the phenotypic and mutational spectrum of SCAR20. We also highlight the importance of deep phenotyping for the diagnosis of SCAR20 in individuals with developmental delay, ataxia, cerebellar atrophy, and distinct facial features.

A Vanishing White Matter Disease Case with a Homozygous Point Mutation in the EIF2B2 Gene Assessed by the Whole-Exome Sequencing

A 30-year-old female patient presented with a progressive gait disturbance, who had been previously diagnosed for cataract and ovarian failure. Brain magnetic resonance imaging showed a high signal intensity of white matter in fluid attenuated inversion recovery and low signal intensity in brain volume imaging, suggesting demyelinating leukodystrophy. Genetic analysis confirmed the pathogenic homozygous mutations c.245T>A in the EIF2B2 gene, which is associated with vanishing white matter disease.

Do Cardiac Rehabilitation Affect Clinical Prognoses Such as Recurrence, Readmission, Revascularization, and Mortality After AMI?: Systematic Review and Meta-Analysis

Objective@#We conducted a systematic review and meta-analysis to analyze the effects of cardiac rehabilitation (CR) on post-discharge prognoses of patients with acute myocardial infarction (AMI). @*Methods@#A literature search was conducted through four international medical and two Korean databases. Primary outcomes for the effectiveness of CR included all-cause mortality, cardiovascular mortality, recurrence, revascularization, major adverse cardiovascular event, major adverse cardiocerebrovascular event, and readmission. We summarized and analyzed results of studies about CR for AMI, including not only randomized controlled trials (RCTs) but also non-RCTs. We calculated the effect size separately by the study type. @*Results@#Fourteen articles were finally selected. Of these, two articles were RCTs, while 12 were non-RCTs. In RCTs, the overall mortality rate was lower in the group that participated in CR than that in the conventional care group by 28% (relative risk=0.72; 95% confidence interval, 0.34–1.57). Among non-RCTs, CR participation significantly decreased the overall risk of mortality. Moreover, the rates of recurrence and major adverse cardiovascular events were lower in the group that participated in CR compared to those in the non-CR group. @*Conclusion@#The meta-analysis shows that CR reduces the risk of re-hospitalization and all-cause mortality after AMI, compared to no participation in CR. This outcome was seen in RCTs as well as in non-RCTs. More studies are necessary for concrete conclusions about the beneficial effects of CR after AMI in various settings.

Association between clinical risk factors and severity of dysphagia after extubation based on a videofluoroscopic swallowing study

Background/Aims@#This study aimed to evaluate the correlation between clinical risk factors of post-extubation dysphagia (PED) and the severity of impaired pharyngeal swallowing function assessed via videofluoroscopic swallowing studies (VFSSs). @*Methods@#This study was a retrospective review of medical records. Of 116 patients who were admitted to the intensive care unit and underwent VFSS, 32 who had non-neurologic disorders and experienced prolonged intubation (for more than 48 hours) were diagnosed with PED. The severity of PED was evaluated by using a functional dysphagia scale (FDS) and a penetration aspiration scale (PAS), on the basis of VFSS. @*Results@#The Simplified Acute Physiology Score 3 and total FDS score were positively correlated (r = 0.40, p = 0.02). Intubation duration was positively correlated with total PAS and FDS scores (r = 0.62, p < 0.001; r = 0.65, p < 0.001, respectively). The amounts of residue in the valleculae (RV) and pyriform sinuses (RP) were associated with intubation duration (r = 0.58, p < 0.001; r = 0.57, p < 0.001, respectively). Multivariate regression analysis revealed that intubation duration was significantly associated with the total FDS score, RV and RP subscales of the FDS, and total PAS score. @*Conclusions@#The severity of impaired swallowing function, particularly the amount of residue in the pharyngeal recesses assessed via VFSS, was strongly associated with both severity of medical illness and intubation duration. Intubation duration could be a prognostic factor for assessing impaired swallowing function on the basis of VFSS.

Clinical Practice Guideline for Cardiac Rehabilitation in Korea

BACKGROUND@#Though clinical practice guidelines (CPGs) for cardiac rehabilitation (CR) are an effective and widely used treatment method worldwide, they are as yet not widely accepted in Korea. Given that cardiovascular disease is the second leading cause of death in Korea, it is urgent that CR programs be developed. In 2008, the Government of Korea implemented CR programs at 11 university hospitals as part of its Regional Cardio-Cerebrovascular Center Project, and 3 additional medical facilities will be added in 2019. In addition, owing to the promotion of CR nationwide and the introduction of CR insurance benefits, 40 medical institutions nationwide have begun CR programs even as a growing number of medical institutions are preparing to offer CR. The purpose of this research was to develop evidence-based CPGs to support CR implementation in Korea.@*METHODS@#This study is based on an analysis of CPGs elsewhere in the world, an extensive literature search, a systematic analysis of multiple randomized control trials, and a CPG management, development, and assessment committee comprised of 33 authors—primarily rehabilitation specialists, cardiologists, and thoracic surgeons in 21 university hospitals and 2 general hospitals. Twelve consultants, primarily rehabilitation, sports medicine, and preventive medicine specialists, CPG experts, nurses, physical therapists, clinical nutritionists, and library and information experts participated in the research and development of these CPGs. After the draft guidelines were developed, 3 rounds of public hearings were held with staff members from relevant academic societies and stakeholders, after which the guidelines were further reviewed and modified.@*RESULTS@#CR involves a more cost-effective use of healthcare resources relative to that of general treatments, and the exercise component of CR lowers cardiovascular mortality and readmission rates, regardless of the type of coronary heart disease and type and setting of CR.@*CONCLUSION@#Individualized CR programs should be considered together with various factors, including differences in heart function and lifestyle, and doing so will boost participation and adherence with the CR program, ultimately meeting the final goals of the program, namely reducing the recurrence of myocardial infarction and mortality rates.

Clinical Practice Guideline for Cardiac Rehabilitation in Korea Online only

OBJECTIVE: Though clinical practice guidelines (CPGs) for cardiac rehabilitation (CR) are an effective and widely used treatment method worldwide, they are as yet not widely accepted in Korea. Given that cardiovascular disease is the second leading cause of death in Korea, it is urgent that CR programs be developed. In 2008, the Government of Korea implemented CR programs at 11 university hospitals as part of its Regional Cardio-Cerebrovascular Center Project, and three additional medical facilities will be added in 2019. In addition, owing to the promotion of CR nationwide and the introduction of CR insurance benefits, 40 medical institutions nationwide have begun CR programs even as a growing number of medical institutions are preparing to offer CR. The purpose of this research was to develop evidence-based CPGs to support CR implementation in Korea. METHODS: This study is based on an analysis of CPGs elsewhere in the world, an extensive literature search, a systematic analysis of multiple randomized control trials, and a CPG management, development, and assessment committee comprised of 33 authors—primarily rehabilitation specialists, cardiologists, and thoracic surgeons in 21 university hospitals and two general hospitals. Twelve consultants, primarily rehabilitation, sports medicine, and preventive medicine specialists, CPG experts, nurses, physical therapists, clinical nutritionists, and library and information experts participated in the research and development of these CPGs. After the draft guidelines were developed, three rounds of public hearings were held with staff members from relevant academic societies and stakeholders, after which the guidelines were further reviewed and modified. PRINCIPAL CONCLUSIONS: CR involves a more cost-effective use of healthcare resources relative to that of general treatments, and the exercise component of CR lowers cardiovascular mortality and readmission rates, regardless of the type of coronary heart disease and type and setting of CR. Individualized CR programs should be considered together with various factors, including differences in heart function and lifestyle, and doing so will boost participation and adherence with the CR program, ultimately meeting the final goals of the program, namely reducing the recurrence of myocardial infarction and mortality rates.

Clinical Practice Guideline for Cardiac Rehabilitation in Korea / 대한흉부외과학회지

BACKGROUND: Though clinical practice guidelines (CPGs) for cardiac rehabilitation (CR) are an effective and widely used treatment method worldwide, they are as yet not widely accepted in Korea. Given that cardiovascular disease is the second leading cause of death in Korea, it is urgent that CR programs be developed. In 2008, the Government of Korea implemented CR programs at 11 university hospitals as part of its Regional Cardio-Cerebrovascular Center Project, and 3 additional medical facilities will be added in 2019. In addition, owing to the promotion of CR nationwide and the introduction of CR insurance benefits, 40 medical institutions nationwide have begun CR programs even as a growing number of medical institutions are preparing to offer CR. The purpose of this research was to develop evidence-based CPGs to support CR implementation in Korea. METHODS: This study is based on an analysis of CPGs elsewhere in the world, an extensive literature search, a systematic analysis of multiple randomized control trials, and a CPG management, development, and assessment committee comprised of 33 authors—primarily rehabilitation specialists, cardiologists, and thoracic surgeons in 21 university hospitals and 2 general hospitals. Twelve consultants, primarily rehabilitation, sports medicine, and preventive medicine specialists, CPG experts, nurses, physical therapists, clinical nutritionists, and library and information experts participated in the research and development of these CPGs. After the draft guidelines were developed, 3 rounds of public hearings were held with staff members from relevant academic societies and stakeholders, after which the guidelines were further reviewed and modified. RESULTS: CR involves a more cost-effective use of healthcare resources relative to that of general treatments, and the exercise component of CR lowers cardiovascular mortality and readmission rates, regardless of the type of coronary heart disease and type and setting of CR. CONCLUSION: Individualized CR programs should be considered together with various factors, including differences in heart function and lifestyle, and doing so will boost participation and adherence with the CR program, ultimately meeting the final goals of the program, namely reducing the recurrence of myocardial infarction and mortality rates.

Clinical Practice Guideline for Cardiac Rehabilitation in Korea: Recommendations for Cardiac Rehabilitation and Secondary Prevention after Acute Coronary Syndrome

Though clinical practice guidelines (CPGs) for cardiac rehabilitation (CR) are an effective and widely used treatment method worldwide, they are as yet not widely accepted in Korea. Given that cardiovascular (CV) disease is the second leading cause of death in Korea, it is urgent that CR programs be developed. In 2008, the Government of Korea implemented CR programs at 11 university hospitals as part of its Regional Cardio-Cerebrovascular Center Project, and 3 additional medical facilities will be added in 2019. In addition, owing to the promotion of CR nationwide and the introduction of CR insurance benefits, 40 medical institutions nationwide have begun CR programs even as a growing number of medical institutions are preparing to offer CR. The purpose of this research was to develop evidence-based CPGs to support CR implementation in Korea. This study is based on an analysis of CPGs elsewhere in the world, an extensive literature search, a systematic analysis of multiple randomized control trials, and a CPG management, development, and assessment committee comprised of thirty-three authors—primarily rehabilitation specialists, cardiologists, and thoracic surgeons in 21 university hospitals and 2 general hospitals. Twelve consultants, primarily rehabilitation, sports medicine, and preventive medicine specialists, CPG experts, nurses, physical therapists, clinical nutritionists, and library and information experts participated in the research and development of these CPGs. After the draft guidelines were developed, 3 rounds of public hearings were held with staff members from relevant academic societies and stakeholders, after which the guidelines were further reviewed and modified. CR involves a more cost-effective use of healthcare resources relative to that of general treatments, and the exercise component of CR lowers CV mortality and readmission rates, regardless of the type of coronary heart disease and type and setting of CR. Individualized CR programs should be considered together with various factors, including differences in heart function and lifestyle, and doing so will boost participation and adherence with the CR program, ultimately meeting the final goals of the program, namely reducing the recurrence of myocardial infarction and mortality rates.

Clinical Practice Guideline for Cardiac Rehabilitation in Korea: Recommendations for Cardiac Rehabilitation and Secondary Prevention after Acute Coronary Syndrome

Though clinical practice guidelines (CPGs) for cardiac rehabilitation (CR) are an effective and widely used treatment method worldwide, they are as yet not widely accepted in Korea. Given that cardiovascular (CV) disease is the second leading cause of death in Korea, it is urgent that CR programs be developed. In 2008, the Government of Korea implemented CR programs at 11 university hospitals as part of its Regional Cardio-Cerebrovascular Center Project, and 3 additional medical facilities will be added in 2019. In addition, owing to the promotion of CR nationwide and the introduction of CR insurance benefits, 40 medical institutions nationwide have begun CR programs even as a growing number of medical institutions are preparing to offer CR. The purpose of this research was to develop evidence-based CPGs to support CR implementation in Korea. This study is based on an analysis of CPGs elsewhere in the world, an extensive literature search, a systematic analysis of multiple randomized control trials, and a CPG management, development, and assessment committee comprised of thirty-three authors—primarily rehabilitation specialists, cardiologists, and thoracic surgeons in 21 university hospitals and 2 general hospitals. Twelve consultants, primarily rehabilitation, sports medicine, and preventive medicine specialists, CPG experts, nurses, physical therapists, clinical nutritionists, and library and information experts participated in the research and development of these CPGs. After the draft guidelines were developed, 3 rounds of public hearings were held with staff members from relevant academic societies and stakeholders, after which the guidelines were further reviewed and modified. CR involves a more cost-effective use of healthcare resources relative to that of general treatments, and the exercise component of CR lowers CV mortality and readmission rates, regardless of the type of coronary heart disease and type and setting of CR. Individualized CR programs should be considered together with various factors, including differences in heart function and lifestyle, and doing so will boost participation and adherence with the CR program, ultimately meeting the final goals of the program, namely reducing the recurrence of myocardial infarction and mortality rates.

Hemiplegic Shoulder Pain in Shoulder Subluxation after Stroke: Associated with Range of Motion Limitation

We grouped hemiplegic patients with shoulder subluxation according to the presence of hemiplegic shoulder pain (HSP) and aimed to compare the difference in clinical and magnetic resonance (MR) arthrography findings between 2 groups in the effort to find factors possibly related to HSP. We performed a retrospective, cross-sectional analysis of all eligible inpatients treated at one university rehabilitation hospital. Clinical findings including passive range of motion (ROM), motor power, and spasticity and MR arthrography findings in the affected shoulder were compared between the 2 groups. Of 59 patients who met the criteria, 21 (35.6%) and 38 (64.4%) were classified as HSP group and no-HSP group, respectively. Limitation of ROM in flexion and rotator cuff atrophy significantly related with HSP (R² = 0.449, p < 0.05). Limitation of ROM in flexion and rotator cuff atrophy in MR arthrography was 1.1 and 6.4 times more likely associated with HSP, respectively. Additionally, spasticity in flexion correlated with limitation of ROM in flexion (p = 0.049), external rotation (p = 0.034), and with total limitation of ROM (p = 0.033). A significant correlation was evident between HSP and ROM of the shoulder. However, HSP did not correlate with MR arthrography findings, except rotator cuff atrophy.

Precise Muscle Selection Using Dynamic Polyelectromyography for Treatment of Post-stroke Dystonia: A Case Report

Dystonia has a wide range of causes, but treatment of dystonia is limited to minimizing the symptoms as there is yet no successful treatment for its cause. One of the optimal treatment methods for dystonia is chemodenervation using botulinum toxin type A (BTX-A), alcohol injection, etc., but its success depends on how precisely the dystonic muscle is selected. Here, we reported a successful experience in a 49-year-old post-stroke female patient who showed paroxysmal repetitive contractions involving the right leg, which may be of dystonic nature. BTX-A and alcohol were injected into the muscles which were identified by dynamic polyelectromyography. After injection, the dystonic muscle spasm, cramping pain, and the range of motion of the affected lower limb improved markedly, and she was able to walk independently indoors. In such a case, dynamic polyelectromyography may be a useful method for selecting the dominant dystonic muscles.

Precise Muscle Selection Using Dynamic Polyelectromyography for Treatment of Post-stroke Dystonia: A Case Report

Dystonia has a wide range of causes, but treatment of dystonia is limited to minimizing the symptoms as there is yet no successful treatment for its cause. One of the optimal treatment methods for dystonia is chemodenervation using botulinum toxin type A (BTX-A), alcohol injection, etc., but its success depends on how precisely the dystonic muscle is selected. Here, we reported a successful experience in a 49-year-old post-stroke female patient who showed paroxysmal repetitive contractions involving the right leg, which may be of dystonic nature. BTX-A and alcohol were injected into the muscles which were identified by dynamic polyelectromyography. After injection, the dystonic muscle spasm, cramping pain, and the range of motion of the affected lower limb improved markedly, and she was able to walk independently indoors. In such a case, dynamic polyelectromyography may be a useful method for selecting the dominant dystonic muscles.

A Long-Term Follow-up of Pontine Hemorrhage With Hearing Loss

A pontine intracranial hemorrhage (ICH) evokes several neurological symptoms, due to the various nuclei and nerve fibers; however, hearing loss from a pontine ICH is rare. We have experienced a non-traumatic pontine ICH patient, with hearing loss. A 43-year-old male patient had a massive pontine hemorrhage; his brain magnetic resonance imaging revealed the hemorrhage on the bilateral dorsal pons, with the involvement of the trapezoid body. Also, profound hearing loss on the pure-tone audiogram and abnormal brainstem auditory evoked potential were noticed. Fifty-two months of long-term follow-up did not reveal any definite improvement on the patient's hearing ability.

Two-Year Outcomes of Deep Brain Stimulation in Adults With Cerebral Palsy

OBJECTIVE: To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS. METHODS: Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment. RESULTS: On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction. CONCLUSION: Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.

Two-Year Outcomes of Deep Brain Stimulation in Adults With Cerebral Palsy

OBJECTIVE: To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS. METHODS: Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment. RESULTS: On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction. CONCLUSION: Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.

Factors Affecting Bone Mineral Density in Adults with Cerebral Palsy

OBJECTIVE: To clarify factors affecting bone mineral density (BMD) in adults with cerebral palsy (CP). METHOD: Thirty-five patients with CP participated in this study. Demographic data including gender, age, body mass index (BMI), subtype according to neuromotor type and topographical distribution, ambulatory function, and functional independence measure (FIM) were investigated. The BMD of the lumbar spine and femur were measured using Dual-energy X-ray absorptiometry, and the factors affecting BMD were analyzed. RESULTS: The BMD had no significant association with factors such as gender, age, and subtype in adults with CP. However, BMI was significantly correlated with the BMD of lumbar spine and femur (p<0.05). The FIM score was also positively correlated with the BMD of femur (p<0.05). Moreover, CP patients with higher ambulatory function had significantly higher BMD of femur (p<0.05). CONCLUSION: These findings suggest that BMI and functional levels such as FIM and ambulatory function can affect BMD in adults with CP. The results might be used as basic data, suggesting the importance of treatment including weight bearing exercise and gait training in adults with CP.

Dopa-responsive Dystonia Misdiagnosed as Cerebral Palsy and Hereditary Spastic Paraplegia 2 Cases: Two cases report

Dystonia is a movement disorder caused by involuntary, sustained muscle contractions, frequently resulting in twitching and repetitive movements or abnormal postures. Dopa-responsive dystonia (DRD) is characterized by early childhood onset, marked diurnal fluctuation of symptoms and dramatic response to levodopa. The aim of this report is to present the two cases of DRD misdiagnosed respectively as cerebral palsy and hereditary spastic paraplegia. Proper understanding of this disease entity and its treatment options are necessary for comprehensive rehabilitative management of DRD.