Early Neurodevelopmental Assessments of Neonates Discharged From the Neonatal Intensive Care Unit: A Physiatrist’s Perspective

The survival rate of children admitted in the neonatal intensive care unit (NICU) after birth is on the increase; hence, proper evaluation and care of their neurodevelopment has become an important issue. Neurodevelopmental assessments of individual domains regarding motor, language, cognition, and sensory perception are crucial in planning prompt interventions for neonates requiring immediate support and rehabilitation treatment. These assessments are essential for identifying areas of weakness and designing targeted interventions to improve future functional outcomes and the quality of lives for both the infants and their families. However, initial stratification of risk to select those who are in danger of neurodevelopmental disorders is also important in terms of cost-effectiveness. Efficient and robust functional evaluations to recognize early signs of developmental disorders will help NICU graduates receive interventions and enhance functional capabilities if needed. Several age-dependent, domain-specific neurodevelopmental assessment tools are available; therefore, this review summarizes the characteristics of these tools and aims to develop multidimensional, standardized, and regular follow-up plans for NICU graduates in Korea.

Update of Guidelines: International Dysphagia Diet Standardization Initiative 2019 / 대한연하장애학회지

The need for globally recognized terminology for dysphagia diet has been continuously proposed. Established in 2012, the International Dysphagia Diet Standardization Initiative (IDDSI) Board developed a framework for the dysphagia diet that can commonly be applied in clinical settings and by the caregivers, for all age groups, patients, food industry partners, and bridging all ethnicities. The application of common expressions will ensure patient safety during transfer between medical institutions, by reducing the confusion resulting from diverse terminologies used, and by minimizing unnecessary re-testing. This review introduces the recently updated IDDSI, and discusses and describes the test methods for each level.

Successful Management of Secondary Achalasia in a Patient with Myotonic Dystrophy / 대한연하장애학회지

Myotonic dystrophy (MD) is a systemic disease that affects any level of the gastrointestinal tract. Due to its rare occurrence, achalasia could be misdiagnosed due to symptoms being similar to gastroesophageal reflux (GERD). Moreover, there is no known appropriate treatment for achalasia in patients with MD. A 58-year-old man was diagnosed with type 1 MD. He complained of dyspepsia and chest pain, which showed no improvement even after administering GERD medication. Secondary achalasia was subsequently diagnosed after examining the lower esophageal sphincter by performing a video fluoroscopic-swallowing study (VFSS) and high-resolution manometry. Thereafter, the patient was successfully treated with balloon dilatation, and showed significant improvements in the symptoms. The patient remained well for 1 year. We report a rare case of secondary achalasia in an MD patient. Our study validates that VFSS might be useful for the early diagnosis of achalasia, and esophageal intervention (such as balloon dilatation) should be considered on confirmation of the affliction.

Cardiac and Pulmonary Management Status of Duchenne Muscular Dystrophy in South Korea Based on Data From the National Health Insurance Database

Background@#and Purpose The purpose of this study was to determine the cardiac and pulmonary management status of patients with Duchenne muscular dystrophy (DMD) in South Korea based on the Korean National Health Insurance database. @*Methods@#This study used data of patients with code G71.0 in the National Health Information database, and also those with the special case of code V012. Cardiac function was assessed based on whether echocardiography and 24-h electrocardiography were performed, as well as the frequency of these investigations. Furthermore, information on the use of angiotensin-converting enzyme inhibitors (ACEi), angiotensin II receptor blockers (ARB), and beta blockers (BB) in the drug benefit list was checked. Medical charge records were also checked regarding the prescription of respiratory functional assessments and treatments. @*Results@#The diagnostic criteria were met by 479 patients, with 41% of these patients receiving a cardiac evaluation, 29.8% being prescribed ACEi at 14.4±3.7 years of age, 59.5% undergoing pulmonary function tests, and 42.1% received pulmonary rehabilitation. The age at receiving ventilator support was 19.4±2.7 years. The frequency of cardiac and respiratory function tests increased with age, but the interval between tests was longer than the recent DMD care recommendations. The trend of taking ACEi, ARB, and BB for cardiac management in South Korea did not change during the study analysis period. @*Conclusions@#The findings of this study will contribute to recognizing the current status and the importance of applying an anticipatory approach to cardiopulmonary function in DMD patients.

An Autopsy Confirmed Case of Amyotrophic Lateral Sclerosis with TDP Pathology

The phosphorylated 43-kDa transactive response DNA-binding protein (TDP-43) was identified as a major disease protein in sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. We present a case with progressive muscle weakness who was diagnosed with sporadic ALS. On postmortem examination, TDP-43 immunoreactive neuronal cytoplasmic inclusions were noted in motor cortex, hippocampus and anterior horns of spinal cord, which was compatible with ALS-TDP, stage 4. This is the first documented autopsy-confirmed ALS case with ALS-TDP pathology in Korea.

Safety and Effectiveness of Intra-articular Injection on the Ipsilateral Adhesive Capsulitis after Breast Cancer Surgery

Objective@#To confirm the safety of Intra-articular (IA) injection on the ipsilateral adhesive capsulitis (AC) after breast cancer surgery. @*Methods@#Between January 2017 and May 2020, we retrospectively studied 29 patients after breast cancer surgery who underwent IA injection in the glenohumeral joint for AC in aseptic procedure. @*Results@#There were no side effects or complications such as lymphedema or cellulitis in the patients. There was a significant improvement in pain score and range of motion (ROM) at the 1st, 3rd, and 6th months visits compared to the baseline (p<0.05). The presence or absence of axillary lymph node dissection and radiation therapy had no significant difference in improvement of ROM. But, in rotator cuff syndrome (RCS) group, there was a significant difference in improvement of shoulder IR in patients without RCS. @*Conclusion@#IA Injection on the ipsilateral AC after breast cancer surgery was safe and even effective to improve pain and shoulder ROM. Ipsilateral IA injection can be a good treatment for breast cancer surgery patients suffering from AC.

Post-stroke Hyperglycemia in Non-diabetic Ischemic Stroke is Related With Worse Functional Outcome: A Cohort Study

Objective@#To investigate long-term and serial functional outcomes in ischemic stroke patients without diabetes with post-stroke hyperglycemia. @*Methods@#The Korean Stroke Cohort for Functioning and Rehabilitation (KOSCO) is a large, multi-center, prospective cohort study of stroke patients admitted to participating hospitals in nine areas of Korea. From KOSCO, ischemic stroke patients without diabetes were recruited and divided into two groups: patients without diabetes without (n=779) and with post-stroke hyperglycemia (n=223). Post-stroke hyperglycemia was defined as a glucose level >8 mmol/L. Functional assessments were performed 7 days and 3, 6, and 12 months after stroke onset. @*Results@#There were no significant differences in baseline characteristics between the groups, except in the age of onset and smoking. Analysis of the linear correlation between the initial National Institutes of Health Stroke Scale (NIHSS) score and glucose level showed no significant difference. Among our functional assessments, NIHSS, Fugl-Meyer Assessment (affected side), Functional Ambulatory Category, modified Rankin Scale, and Korean Mini-Mental State Examination (K-MMSE) showed statistically significant improvements in each group. All functional improvements except K-MMSE were significantly higher in patients without post-stroke hyperglycemia at 7 days and 3, 6, and 12 months. @*Conclusion@#The glucose level of ischemic stroke patients without diabetes had no significant correlation with the initial NIHSS score. The long-term effects of stress hyperglycemia showed worse functional outcomes in ischemic stroke patients without diabetes with post-stroke hyperglycemia.

The relationship between lymphedema severity and awareness of lymphedema surgery

Background@#During the early stages of lymphedema, active physiologic surgical treatment can be applied. However, lymphedema patients often have limited knowledge and misconceptions regarding lymphedema and surgical treatment. We analyzed the correlations between lymphedema severity and surgical technique according to patients’ awareness of surgical treatment for secondary upper extremity lymphedema (UEL). @*Methods@#Patients with UEL diagnosed between December 2017 and December 2019 were retrospectively evaluated. At the time of their presentation to our hospital for the treatment of lymphedema, they were administered a questionnaire about lymphedema and lymphedema surgery. Based on the results, patients were classified as being aware or unaware of surgical treatment. Lymphedema severity was classified according to the arm dermal backflow (ADB) stage and the MD Anderson Cancer Center (MDACC) stage based on indocyanine green lymphography conducted at presentation. Surgical techniques were compared between the two groups. @*Results@#Patients who were aware of surgical treatment had significantly lower initial ADB and MDACC stages (P<0.05) and more frequently underwent physiologic procedures than excisional procedures (P=0.003). @*Conclusions@#If patients are actively educated regarding surgical treatment of lymphedema, physiologic procedures may be performed during the early stages of UEL.

The relationship between lymphedema severity and awareness of lymphedema surgery

Background@#During the early stages of lymphedema, active physiologic surgical treatment can be applied. However, lymphedema patients often have limited knowledge and misconceptions regarding lymphedema and surgical treatment. We analyzed the correlations between lymphedema severity and surgical technique according to patients’ awareness of surgical treatment for secondary upper extremity lymphedema (UEL). @*Methods@#Patients with UEL diagnosed between December 2017 and December 2019 were retrospectively evaluated. At the time of their presentation to our hospital for the treatment of lymphedema, they were administered a questionnaire about lymphedema and lymphedema surgery. Based on the results, patients were classified as being aware or unaware of surgical treatment. Lymphedema severity was classified according to the arm dermal backflow (ADB) stage and the MD Anderson Cancer Center (MDACC) stage based on indocyanine green lymphography conducted at presentation. Surgical techniques were compared between the two groups. @*Results@#Patients who were aware of surgical treatment had significantly lower initial ADB and MDACC stages (P<0.05) and more frequently underwent physiologic procedures than excisional procedures (P=0.003). @*Conclusions@#If patients are actively educated regarding surgical treatment of lymphedema, physiologic procedures may be performed during the early stages of UEL.

Concordance between Oropharyngeal Flora and Peristomal Wound Culture Bacteria Following Percutaneous Endoscopic Gastrostomy: A Prospective Cohort Study / 대한연하장애학회지

Objective@#To examine the concordance between the oropharyngeal flora and peristomal wound culture bacteria following percutaneous endoscopic gastrostomy (PEG) placement. @*Methods@#Cultures of oropharyngeal flora before PEG placement and peristomal wound cultures after PEG placement were examined prospectively in patients who underwent PEG between June 1, 2017, and June 1, 2019, at a local rehabilitation center. Microorganisms in the oropharyngeal flora and peristomal wound culture were identified, and the relationship between them was examined. The risk factors that may increase the PEG infection rate were also examined. @*Results@#The predominant bacterium identified in the oropharyngeal flora and peristomal wounds in patients who required PEG was Pseudomonas aeruginosa. The concordance between the oropharyngeal flora and peristomal wound culture bacteria in patients with PEG infections was confirmed. Different oropharyngeal flora was observed in patients with a prolonged hospital stay compared to that of healthy adults. The concordance between the oropharyngeal flora and peristomal wounds was also identified with a high prevalence of gram-negative bacilli. There was no potential risk factor, such as comorbidities or length of hospitalization of patients associated with PEG infections. @*Conclusion@#Because the oropharyngeal flora showed concordance with the bacteria found in the PEG stoma site, management of the oropharyngeal flora before PEG insertion should be considered for patients with dysphagia.

Comparison of Neuropathological Characteristics between Multiple System Atrophy Cerebellar Type and Parkinsonian Type

Background@#Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterized by various combinations of parkinsonism, cerebellar ataxia, autonomic dysfunction and pyramidal signs. Two clinical subtypes are recognized: MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P). The aim of this study was to compare pathological features between MSA-C and MSA-P. @*Methods@#Two autopsy confirmed cases with MSA were included from the Pusan National University Hospital Brain Bank. Case 1 had been clinically diagnosed as MSA-C and case 2 as MSA-P. The severity of neuronal loss and gliosis as well as the glial and neuronal cytoplasmic inclusions were semiquantitatively assessed in both striatonigral and olivopontocerebellar regions. Based on the grading system, pathological phenotypes of MSA were classified as striatonigral degeneration (SND) predominant (SND type), olivopontocerebellar degeneration (OPC) predominant (OPC type), or equivalent SND and OPC pathology (SND=OPC type). @*Results@#Both cases showed widespread and abundant α-synuclein positive glial cytoplasmic inclusions in association with neurodegenerative changes in striatonigral or olivopontocerebellar structures, leading to the primary pathological diagnosis of MSA. Primary age-related tauopathy was incidentally found but Lewy bodies were not in both cases. The pathological phenotypes of MSA were MSA-OPC type in case 1 and MSA-SND=OPC type in case 2. @*Conclusions@#Our data suggest that clinical phenotypes of MSA reflect the pathological characteristics.

What is the Adequate Cuff Volume for Tracheostomy Tube? A Pilot Cadaver Study

Objective@#To determine the patterns of tracheostomy cuff pressure changes with various air inflation amounts in different types of tracheostomy tubes to obtain basic data for appropriately managing longterm tracheostomy. @*Methods@#We performed tracheostomy on a 46-year-old male cadaver. Three types of tracheostomy tubes (single-cuffed, double-cuffed, and adjustable flange), divided into 8 different subtypes based on internal tube diameters and cuff diameters, were inserted into the cadaver. Air was inflated into the cuff, and starting with 1 mL air, the cuff pressure was subsequently measured using a manometer. @*Results@#For the 7.5 mm/14 mm tracheostomy tube, cuff inflation with 3 mL of air yielded a cuff pressure within the recommended range of 20–30 cmH2O. The 7.5 mm/24 mm tracheostomy tube showed adequate cuff pressure at 5 mL of air inflation. Similar values were observed for the 8.0 mm/16 mm and 8.0 mm/27 mm tubes. Double-cuffed tracheostomy cuff pressures (7.5 mm/20 mm and 8.0 mm/20 mm tubes) at 3 mL air inflation had cuff pressures of 18–20 cmH2O at both the proximal and distal sites. For the adjustable flange tracheostomy tube, cuff pressure at 6 mL of cuff air inflation was within the recommended range. Maximal cuff pressure was achieved at inflation with almost 14 mL of air, unlike other tube types. @*Conclusion@#Various types of tracheostomy tubes showed different cuff pressures after inflation. These values might aid in developing guidelines For patients who undergo tracheostomy and are discharged home without cuff pressure manometers, this standard might be helpful to develop guidelines.

Correlation of Swallowing Function With Bilateral Diaphragmatic Movement in Hemiplegic Stroke Patients

OBJECTIVE: To investigate difference in bilateral diaphragm movement of patients with tracheal aspiration according to post stroke residue severity and determine correlations of Penetration-Aspiration Scale (PAS), residue scale, and bilateral diaphragm movement. METHODS: A total of 47 patients diagnosed with hemiplegic stroke were enrolled in this study. PAS, severity of valleculae, and pyriform sinus retention during videofluoroscopic swallowing study (VFSS) were assessed. Bilateral fluoroscopic diaphragm movements during spontaneous breathing and forced breathing were measured. RESULTS: Patients with tracheal aspiration (PAS≥6) had significantly (p=0.035) lower ipsilateral diaphragm movement during spontaneous breathing. Post-swallow residue severity showed statistically significant (p=0.028) difference in patients with ipsilateral diaphragm movement during forced breathing. In linear regression analysis, PAS showed weak correlations with ipsilateral spontaneous diaphragm movement (r=0.397, p=0.006), ipsilateral forced diaphragm movement (r=0.384, p=0.008), and contralateral forced diaphragm movement (r=0.323, p=0.027). Weak correlation was also observed between post swallow residue severity and ipsilateral diaphragm movement during spontaneous breathing (r=0.331, p=0.023) and forced breathing (r=0.343, p=0.018). CONCLUSION: We confirmed the relationship between swallowing function and bilateral diaphragm movement in this study. The severity of dysphagia after hemiplegic stroke was correlated with bilateral diaphragm movement. Further longitudinal studies are needed to assess the effect of breathing exercise on post-stroke dysphagia.

Correlations between Aspiration and Pharyngeal Residue Scale Scores for Fiberoptic Endoscopic Evaluation and Videofluoroscopy

PURPOSE: To examine correlations among rating scales and findings suggestive of tracheal aspiration and pharyngeal residue between fiberoptic endoscopic evaluation of swallowing (FEES) and videofluoroscopic swallowing study (VFSS) in dysphagia patients. MATERIALS AND METHODS: We studied patients referred to our hospital for dysphagia assessment. Three raters judged the residue severity and laryngeal penetration or tracheal aspiration of FEES and VFSS. The raters applied the Penetration-Aspiration Scale (PAS) for tracheal aspiration and pixel-based circumscribed area ratio and Yale Pharyngeal Residue Severity for post-swallow residue in VFSS and FEES, respectively. Anatomy-physiologic findings during FEES associated with tracheal aspiration were also analyzed. RESULTS: A total of 178 participants were enrolled in our study. In correlation analysis, PAS (r=0.74), vallecula retention (r=0.76), and pyriform sinus retention (r=0.78) showed strong positive correlations between FEES and VFSS. Intra-rater agreement between VFSS and FEES was good for PAS (κ=0.65) and vallecula (κ=0.65) and pyriform sinus retention (κ=0.69). Among 72 patients who showed subglottic shelf residue, a suspected finding of aspiration, in FEES, 68 had concomitant tracheal aspiration during VFSS. Both vocal fold hypomobility and glottic gap during phonation were significantly associated with findings suggestive of tracheal aspiration during FEES (p<0.05). CONCLUSION: Quantitative and reliable aspiration and post swallow residue rating scales showed strong positive correlations and good agreement between VFSS and FEES.

Secondary Lymphedema After Intestinal Tuberculosis: A Case Report

Lymphedema, a chronic disease that lowers patients' quality of life, is categorized as primary or secondary. Secondary lymphedema can be improved by treating the underlying cause. However, in many cases, efforts are not made to identify the primary cause of lymphedema and treatment is targeted at the edema itself, resulting in misdiagnosis. Here, we describe the case of a 61-year-old man with right leg edema of unknown cause that had persisted for 3 years. Intestinal tuberculosis was confirmed during a re-evaluation of the cause, and his symptoms improved after anti-tuberculous treatment. This improvement was assessed qualitatively by indocyanine green lymphography before and after treatment, as well as by observation of the clinical symptoms. Lower extremity lymphedema caused by intestinal tuberculosis is extremely rare, and this case reveals the importance of continuing to identify the causes of resistant pathologies.

Duodenoduodenal Intussusception due to a Migrated Percutaneous Radiologic Gastrostomy Tube: A Case Report / 대한연하장애학회지

Patients with dysphagia often have difficulty in supplying adequate nutrition orally, and thus they often use gastrostomy for nutrition support. If the nutrition affects the deterioration of the disease, as in amyotrophic lateral sclerosis, the majority of patients will have a gastrostomy tube for proper nutrition. To prevent complications from gastrostomy tubes, it is important to educate the caregiver or patient about how to properly manage it. If these patients opt for home care because of financial or cultural reasons, it will be difficult for their healthcare team to observe them closely, leading to complications due to lax tube management. In this case, appropriate management education becomes more important. This paper reports an extremely rare case of duodenal intussusception caused by a migrated percutaneous radiologic gastrostomy (PRG) tube in a patient with amyotrophic lateral sclerosis (ALS) using simultaneous oral and enteral nutrition. The patient was treated successfully with urgent tube removal using the air reduction maneuver. This case highlights the importance of gastrostomy management.

Early Stage Lymphedema in Breast Cancer Patient Detected by Indocyanine Green Lymphography but not by Lymphoscintigraphy: A Case Report

Secondary upper extremity lymphedema after lymph node dissection in breast cancer patients is a major complication affecting their quality of life. As lymphatic function is compromised before symptoms develop in most cases, early diagnosis and intervention before symptomatic manifestation is known to prevent severe progression of lymphedema. In this case report, we present a patient with secondary lymphedema, whose disease could be diagnosed at an early stage using indocyanine green (ICG) lymphography. The patient had mild edema of the left upper extremity that regressed with elevation, had normal lymph vessel distribution without dermal backflow pattern in lymphoscintigraphy. Her symptom improved after early prescription of complex decongestive physiotherapy. We conclude that ICG lymphography could be an effective diagnostic tool in addition to other imaging methods, for early diagnosis and intervention of lymphedema.

Motor and Sensory Function as a Predictor of Respiratory Function Associated With Ventilator Weaning After High Cervical Cord Injury

OBJECTIVE: To analyze the respiratory function of high cervical cord injury according to ventilator dependence and to examine the correlations between diaphragm movement found on fluoroscopy and sensory and motor functions. METHODS: A total of 67 patients with high cervical spinal cord injury (SCI), admitted to our hospital were enrolled in the study. One rehabilitation physician performed sensory and motor examinations on all patients while each patient was in the supine position on the American Spinal Injury Association (ASIA) standard. In addition, fluoroscopic diaphragm movement studies and bedside spirometry were performed. RESULTS: Bedside spirometry and diaphragm fluoroscopic tests were analyzed according to ventilator dependence. Forced vital capacity and maximal inspiratory pressure were significantly higher in the ventilator weaned group. Natural breathing during the fluoroscopic diaphragm examinations and ventilator weaning showed statistical significance with the movement on the right, while deep breathing showed statistical significance with the movement on both sides. Deep breathing movement has correlation with the C5 key muscle. Diaphragm movement has correlation with right C3 and bilateral C4 sensory functions. CONCLUSION: The present expansion study showed that, through simple bedside physical examinations, rehabilitation physicians could relatively easily predict diaphragm movement and respiratory function recovery, which showed significance with ventilator weaning in patients with high cervical SCI.

Successful Management of Acquired Tracheomalacia of Patients With Amyotrophic Lateral Sclerosis: A Report of Three Cases

Tracheomalacia is characterized by weakness of the tracheal walls and supporting cartilage. It results in dynamic compression of the airway, where the cross-sectional area of the trachea is reduced by expiratory compression. Acquired tracheomalacia results from complications associated with the use of endotracheal or tracheostomy tubes. In this report, we present three cases of patients with amyotrophic lateral sclerosis (ALS) successfully treated for tracheomalacia, including one case where the patient underwent surgery for combined tracheoesophageal fistula. We discuss the appropriate management strategies for tracheomalacia in patients with ALS. Through these case reports, we note the results of ALS patients who will have tracheostomy, and who are therefore at risk of sustaining a long term high cuff pressure, this study provides an evaluation for tracheomalacia and therapeutic management which should be considered for improving patient care outcomes.

Amount of Weight-Bearing During Tilt Table Inclination, With Neutral and Unilateral Knee Flexion Postures

OBJECTIVE: To analyze the amount of weight-bearing during tilt table increments, with a review of neutral and unilateral knee flexion postures. METHODS: There were 17 healthy participants enrolled in this study. The subjects were tilted from 10° to 90°, and their body weight was measured at each 10° increment. In the first test, both plantar pressures, with the subjects in neutral posture, were recorded. During the second and third tests, the angle of inclination was thus recorded and increased, with the subjects in unilateral knee flexion posture; flexion was maintained at 25° by attaching a cylindrical support to the tilt table at the level of the popliteal fossa. RESULTS: The study was divided into two types of postures: neutral and unilateral knee flexion. The percentage of body weight (%BW) between each leg during neutral posture was noted as not being statistically significant. The %BW of one side during tilt table inclination was significantly different between the two postures at 10° to 80° (p < 0.05). The weight during unilateral knee flexion posture was lower as analyzed, regardless of tilt table inclination compared with that in neutral posture. We note that fifty percent of the ratio of %BW was noted at 33.12° and 38.76° in neutral and flexion postures, respectively. CONCLUSION: The unilateral knee flexion could induce the effect of decreased body weight compared with non-flexion side. The results of this study will help in setting a safe and quantitative percentage of weight-bearing on the lower extremity during tilt training.