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.

Clinical Practice Guidelines for Oropharyngeal Dysphagia / 대한연하장애학회지

Objective@#Dysphagia is a common clinical condition characterized by difficulty in swallowing. It is sub-classified into oropharyngeal dysphagia, which refers to problems in the mouth and pharynx, and esophageal dysphagia, which refers to problems in the esophageal body and esophagogastric junction. Dysphagia can have a significant negative impact one’s physical health and quality of life as its severity increases. Therefore, proper assessment and management of dysphagia are critical for improving swallowing function and preventing complications. Thus a guideline was developed to provide evidence-based recommendations for assessment and management in patients with dysphagia. @*Methods@#Nineteen key questions on dysphagia were developed. These questions dealt with various aspects of problems related to dysphagia, including assessment, management, and complications. A literature search for relevant articles was conducted using Pubmed, Embase, the Cochrane Library, and one domestic database of KoreaMed, until April 2021. The level of evidence and recommendation grade were established according to the Grading of Recommendation Assessment, Development and Evaluation methodology. @*Results@#Early screening and assessment of videofluoroscopic swallowing were recommended for assessing the presence of dysphagia. Therapeutic methods, such as tongue and pharyngeal muscle strengthening exercises and neuromuscular electrical stimulation with swallowing therapy, were effective in improving swallowing function and quality of life in patients with dysphagia. Nutritional intervention and an oral care program were also recommended. @*Conclusion@#This guideline presents recommendations for the assessment and management of patients with oropharyngeal dysphagia, including rehabilitative strategies.

Perceived Physical and Mental Health and Healthy Eating Habits During the COVID-19 Pandemic in Korea

Background@#The coronavirus disease 2019 (COVID-19) pandemic has disrupted the lives of people around the world since 2020. This study aims to reveal perceived impact of the coronavirus pandemic on physical and mental health and eating behaviors among people with disabilities and without disabilities in South Korea, as compared to other countries. @*Methods@#A secondary analysis of a prospective cross-sectional study which was conducted with a web-based global survey. @*Results@#Among the 3,550 responses from 65 countries, 2,621 responses with nation information were set as full data, 189 for South Korea and 2,432 for other countries. In Korea, there was no significant difference in healthy lifestyle behaviors between people with and without disabilities before the COVID-19 pandemic. Perceived physical and mental health and changes in eating habits during the COVID-19 pandemic showed no significant difference between people with and without disabilities in Korea. There were significant differences in physical health and dietary habits, but no differences in its effect on mental health between people living in Korea and other countries in both people with and without disabilities groups. In other words, more than 60% of people in all groups (disability vs. non-disability, Korea vs. non-Korea) reported worse mental health than before the COVID-19 pandemic. @*Conclusion@#In Korea and other countries, mental health showed a tendency to deteriorate regardless of the presence or absence of disability during the COVID-19 pandemic. In terms of healthy eating habits, Koreans were relatively less affected than people from other countries.

Hemoptysis in Quadriplegia with Atrial Fibrillation Who Was Taking Edoxaban: a Case Report

The direct-acting oral anticoagulants (DOACs) would be the standard treatment for the prevention of stroke and thromboembolism in nonvalvular atrial fibrillation patients. The adverse effects of greatest concern are bleeding especially major bleeding. We present a case of a patient with a history of nonvalvular atrial fibrillation and pacemaker, who developed severe anemia after massive hemoptysis while taking DOAC; however, he has continued taking DOAC. Through this case, we have summarized the current management of major bleeding associated with anticoagulation and discuss the optimal regimen for restarting of anticoagulation therapy.

Correlation Between Duration From Injury and Bone Mineral Density in Individuals With Spinal Cord Injury

Objective@#To investigate the correlation between bone mineral density (BMD) and duration of injury in individuals with spinal cord injury (SCI). @*Methods@#Patients with SCI who visited the outpatient department between January 2009 and January 2019 were enrolled. Patients’ most recent dual energy X-ray absorptiometry images were reviewed. According to the 2007 International Society for Clinical Densitometry guidelines, vertebrae with a local structural change were excluded when deriving spine BMD. If one or no vertebra is suitable for evaluation, spine BMD was judged as “improper for assessment”. Correlation analysis was performed between duration from injury and BMD Z-scores of the hip and spine. @*Results@#Among 83 individuals with SCI, the spines of 44 were judged as improper for assessment. The correlation analysis showed a significant negative relationship between the duration from injury and femur neck BMD (r=-0.40, p<0.01) and total proximal femur BMD (r=-0.39, p<0.01). However, no significant correlation was found between the duration from injury and spine BMD Z-score. @*Conclusion@#The duration of SCI correlated with hip BMD, but not with spine BMD. Further, more than half of the individuals with SCI could not undergo spinal assessment due to local structural changes. Therefore, spine BMD measurement is not an appropriate method for predicting future fracture risk in those with SCI.

Determinant of Quality of Life in Patients with Chronic Cerebral Infarct

This study investigated how physical and cognitive function and psychological factors affected the health-related quality of life (HRQoL, hereafter HQ) of stroke patients in South Korea. The study enrolled 32 right-handed subjects with chronic cerebral infarction with disability and preserved cognitive function (Mini-Mental State Examination ≥ 20). Physical disability was assessed using the modified Rankin Scale (mRS) and Korean modified Barthel Index (KMBI). Quality of life was measured using the World Health Organization Quality of Life-Abbreviated form (WHOQOL-BREF, hereafter WB) and the 36-Item Short-form Health Survey (SF-36) in face-to-face interviews. Psychological distress was investigated using the Beck Depression Inventory Scale-II. The associations of each domain of WB and SF-36 were investigated using Pearson correlation analyses. Physical disability was negatively correlated with HQ in the SF-36. The physical function and bodily pain scales of the SF-36 were negatively correlated with physical disability. The general health domain of the SF-36 was negatively correlated with psychological scores. Emotional status was associated with physical health, social relationships, and general health in HQ. In summary, the severity of physical disability was associated with the patient's general and physical health and body pain. These findings suggest the importance of psychological, cognitive, and physiological interventions for improving the quality of life of patients after cerebral infarction.

Determinant of Quality of Life in Patients with Chronic Cerebral Infarct

This study investigated how physical and cognitive function and psychological factors affected the health-related quality of life (HRQoL, hereafter HQ) of stroke patients in South Korea. The study enrolled 32 right-handed subjects with chronic cerebral infarction with disability and preserved cognitive function (Mini-Mental State Examination ≥ 20). Physical disability was assessed using the modified Rankin Scale (mRS) and Korean modified Barthel Index (KMBI). Quality of life was measured using the World Health Organization Quality of Life-Abbreviated form (WHOQOL-BREF, hereafter WB) and the 36-Item Short-form Health Survey (SF-36) in face-to-face interviews. Psychological distress was investigated using the Beck Depression Inventory Scale-II. The associations of each domain of WB and SF-36 were investigated using Pearson correlation analyses. Physical disability was negatively correlated with HQ in the SF-36. The physical function and bodily pain scales of the SF-36 were negatively correlated with physical disability. The general health domain of the SF-36 was negatively correlated with psychological scores. Emotional status was associated with physical health, social relationships, and general health in HQ. In summary, the severity of physical disability was associated with the patient's general and physical health and body pain. These findings suggest the importance of psychological, cognitive, and physiological interventions for improving the quality of life of patients after cerebral infarction.

Determinant of Quality of Life in Patients with Chronic Cerebral Infarct

This study investigated how physical and cognitive function and psychological factors affected the health-related quality of life (HRQoL, hereafter HQ) of stroke patients in South Korea. The study enrolled 32 right-handed subjects with chronic cerebral infarction with disability and preserved cognitive function (Mini-Mental State Examination ≥ 20). Physical disability was assessed using the modified Rankin Scale (mRS) and Korean modified Barthel Index (KMBI). Quality of life was measured using the World Health Organization Quality of Life-Abbreviated form (WHOQOL-BREF, hereafter WB) and the 36-Item Short-form Health Survey (SF-36) in face-to-face interviews. Psychological distress was investigated using the Beck Depression Inventory Scale-II. The associations of each domain of WB and SF-36 were investigated using Pearson correlation analyses. Physical disability was negatively correlated with HQ in the SF-36. The physical function and bodily pain scales of the SF-36 were negatively correlated with physical disability. The general health domain of the SF-36 was negatively correlated with psychological scores. Emotional status was associated with physical health, social relationships, and general health in HQ. In summary, the severity of physical disability was associated with the patient's general and physical health and body pain. These findings suggest the importance of psychological, cognitive, and physiological interventions for improving the quality of life of patients after cerebral infarction.

The Role of Regular Physical Therapy on Spasticity in Children With Cerebral Palsy

OBJECTIVE: To investigate the effect of physical therapy (PT) intervention on spasticity in patients with cerebral palsy (CP), and to assess the degree of deterioration of spasticity when regular PT is interrupted in those patients. METHODS: We recruited 35 children with spastic CP who visited our hospital for PT, and whose Modified Tardieu Scale (MTS) scores were serially recorded including before and after a 10-day public holiday time frame period. The outcome measures were the angle of range of motion (ROM) of dorsiflexion of the ankle joint (R1 and R2) in the knee flexion and extension positions as assessed using the MTS. RESULTS: The range of dorsiflexion of the ankle joint (R1 and R2) after the holiday period was significantly decreased as compared with that measured ROM noted before the holiday period, regardless of the knee position, age, or gross motor function. The dynamic component of the MTS (R2–R1) showed a slight decrease in the knee flexion position. CONCLUSION: Interruption of regular PT aggravated spasticity and decreased ankle joint ROM in children with spastic CP. Our findings suggest that regular PT in the care continuum for children with CP is crucial for the maintenance of ROM in the spastic ankle joints.

A Man with Dysphagia after Cervical Esophagogastrostomy: A Case Report / 대한연하장애학회지

Dysphagia caused by a functional gastric outlet obstruction is rare and the natural course of a functional gastric outlet obstruction is unclear. This paper reports a patient with dysphagia and a functional gastric outlet obstruction after a cervical esophagogastrostomy. A 62-year-old man with a history of esophageal cancer and cervical esophagogastrostomy presented with dysphagia and discomfort during meals. A videofluoroscopic swallowing study (VFSS) with barium revealed a mild increase in the post-swallow remnants and delayed emptying of the gastric outlet, reflecting a functional gastric outlet obstruction at the diaphragm. One month later, VFSS revealed a worsened functional gastric outlet obstruction at the diaphragm. A delayed chest X-ray showed worsened delayed emptying of the gastric outlet. A cervical esophagogastrostomy for early esophageal cancer can cause functional gastric outlet obstruction, presenting as dysphagia and discomfort during meals. Physicians should be concerned about dysphagia and the functional changes in the esophagus in patients with a cervical esophagogastrostomy.

The Changes for Strength of Oropharyngeal Muscles in Patients with Dementia and Dysphagia

Although dysphagia is an important health problem and one of the determinants of quality of life in patients with dementia, the neurophysiologic changes of dysphagia in dementia have not been fully uncovered, yet. we investigated the changes of strength of tongue lip and pharyngeal muscles in patients with dementia. This study included 30 subjects with dementia. In all subjects, clinical assessments consisted of the Iowa Oral Performance Instrument (IOPI) for tongue and lip, surface electromyography (sEMG) with Vital stim plus on suprahyoid muscles, the Mini-Mental State Examination, and Clinical Dementia Rating (CDR) scales. All subjects were classified into 3 groups according to severity; CDR 1, 2, and 3. There was no difference between IOPI and sEMG among all 3 groups. The values of tongue and lip IOPI from all 3 dementia groups were significantly lower than values of control. The comparisons for values of tongue and lip IOPI among 3 dementia group were not different from each other. The sEMG of suprahyoid muscles were not different between all 3 dementia groups and control. These findings are supportive of dysphagia therapy for oral phase would be beneficial for the restoration of swallowing function in patients with dementia and dysphagia.

Delayed Extensive White Matter Injury Caused by a Subdural Hemorrhage and Role of Corticospinal Tract Integrity

A subdural hemorrhage (SDH) is a common disorder with usually good prognosis. Most SDHs resolve with or without with minimal sequelae. We present a case report of a patient with SDH, who had delayed extensive white matter injury with disruptions of corticospinal tracts (CSTs) by diffusion tensor imaging (DTI) and showed abysmal prognosis, despite long-term rehabilitation. A 62-year-old man with an SDH underwent burr hole trephination for hematoma removal. Within 7 days, the hemorrhage diminished. At 12 weeks after the onset, the patient's weakness did not improve, and a follow-up magnetic resonance imaging revealed extensive leukomalacia, especially in the white matter. The DTI for CST revealed severe injury of CST integrity. He did not re-gain muscle strength and functional independence, despite 3 months of inpatient rehabilitation. This case describes SDH with delayed extensive white matter injury and exceptional poor prognosis and urges caution in that the SDH may induce very variable functional recovery. Besides, DTI for CST would be useful in predicting the long-term functional prognosis in extensive white matter injury.

A Man Presenting with Sudden Weakness and Pain of the Right Hand, by Non-Small Cell Lung Cancer with Brain Metastases

Unexplained pain and weakness, i.e., without obvious predisposing factors, are often encountered by physiatrists and efforts should be made to determine the cause. A 63-year-old male presented with radiating pain in his right arm and mild weakness of the right hand. An electrodiagnostic examination revealed distal symmetric sensory polyneuropathy in the upper and lower extremities, and denervation potentials in the forearm muscles, which were inconsistent with the cervical spine MRI images and symptoms. A predisposing undiscovered disease was revealed, i.e., squamous cell carcinoma in the lung; brain metastasis affecting the left primary motor cortex was also detected. Therefore, we concluded that the pain and weakness were related to paraneoplastic syndrome and brain metastases of the hand knob. The observed denervation potentials were characterized as trans-synaptic changes in the brain metastasis. This case highlights the importance of unexplainable focal pain and weakness in the increasing prevalence of cancer.

Subtle Dysphagia as an Initial Presentation of Hidden Malignancy: A Report of 2 Cases / 대한연하장애학회지

Subtle dysfphagia, which is increased post-swallowing remnants, is a frequent finding in the elderly with various etiologies. These changes in swallowing are frequently overlooked by physicians. On the other hand, subtle changes evident on a videofluoroscopic swallowing study (VFSS) may suggest hidden disease. Therefore, clinicians should evaluate incidental dysphagia. Case 1: A 65-year-old man with no relevant medical history, presented with dysphagia and residual sensation during meals. VFSS showed moderate post-swallowing remnants in the vallecular fossa and pyriformis sinus. Further examination revealed prostate cancer with multiple bone metastases including the skull. Case 2: A 60-year-old man complained of residual sensation after swallowing, which started 2 months ago. He had a history of lung cancer. Pharyngeal residue was observed on VFSS. A brain metastasis was observed on MRI. Post-swallowing residue is often neglected or overlooked by clinicians who regard them as the features of aging. The present cases show that mild dysphagia with increased post-swallowing remnants may be an initial presentation of a hidden malignancy with metastasis. Physicians should consider unexplained dysphagia or tongue atrophy as possible initial presentations of hidden malignancies.

Sinking Skin Flap Syndrome or Syndrome of the Trephined: A Report of Two Cases

Decompressive craniectomy (DC) is commonly performed in patients with intracranial hypertension or brain edema due to traumatic brain injury. Infrequently, neurologic deteriorations accompanied by sunken scalp may occur after DC. We report two patients with traumatic subdural hemorrhage who had neurologic deteriorations accompanied by sunken scalp after DC. Neurologic function improved dramatically in both patients after cranioplasty. Monitoring for neurologic deterioration after craniectomy is advised. For patients showing neurologic deficit with a sunken scalp, early cranioplasty should be considered.

Central Hypoventilation Syndrome in Posterior Circulation Stroke Treated by Respiratory Rehabilitation: a Case Report

Central hypoventilation syndrome is a rare and fatal condition resulting from various central nervous system disorders that is characterized by a failure of automatic breathing. We report a case of central hypoventilation syndrome following posterior circulation stroke whose pulmonary function was improved by respiratory rehabilitation. A 59-year-old woman with a history of hemorrhagic stroke of the bilateral cerebellum was hospitalized due to pneumonia. A portable ventilator was applied via tracheostomy, recurrent episodes of apnea and hypercapnia impeded weaning. A respiratory rehabilitation program including chest wall range of motion exercise, air stacking exercise, neuromuscular electrical stimulation (NMES) on abdominal muscles, upper extremity ergometer, locomotor training, high-frequency chest wall oscillator, mechanical insufflation, and exsufflation was employed, as spirometry showed a severe restrictive pattern. A spontaneous breathing trial was started, and a portable ventilator was applied for 8 hours, only during nighttime, to prevent sudden apneic event. After 4 weeks of treatment, follow-up spirometry showed much improved respiratory parameters. This case suggests that respiratory rehabilitation can improve pulmonary function parameters and quality of life in central hypoventilation syndrome.

Chromosomal Microarray Analysis as a First-Tier Clinical Diagnostic Test in Patients With Developmental Delay/Intellectual Disability, Autism Spectrum Disorders, and Multiple Congenital Anomalies: A Prospective Multicenter Study in Korea

BACKGROUND: To validate the clinical application of chromosomal microarray analysis (CMA) as a first-tier clinical diagnostic test and to determine the impact of CMA results on patient clinical management, we conducted a multicenter prospective study in Korean patients diagnosed as having developmental delay/intellectual disability (DD/ID), autism spectrum disorders (ASD), and multiple congenital anomalies (MCA). METHODS: We performed both CMA and G-banding cytogenetics as the first-tier tests in 617 patients. To determine whether the CMA results directly influenced treatment recommendations, the referring clinicians were asked to complete a 39-item questionnaire for each patient separately after receiving the CMA results. RESULTS: A total of 122 patients (19.8%) had abnormal CMA results, with either pathogenic variants (N=65) or variants of possible significance (VPS, N=57). Thirty-five well-known diseases were detected: 16p11.2 microdeletion syndrome was the most common, followed by Prader-Willi syndrome, 15q11-q13 duplication, Down syndrome, and Duchenne muscular dystrophy. Variants of unknown significance (VUS) were discovered in 51 patients (8.3%). VUS of genes putatively associated with developmental disorders were found in five patients: IMMP2L deletion, PTCH1 duplication, and ATRNL1 deletion. CMA results influenced clinical management, such as imaging studies, specialist referral, and laboratory testing in 71.4% of patients overall, and in 86.0%, 83.3%, 75.0%, and 67.3% of patients with VPS, pathogenic variants, VUS, and benign variants, respectively. CONCLUSIONS: Clinical application of CMA as a first-tier test improves diagnostic yields and the quality of clinical management in patients with DD/ID, ASD, and MCA.

Cervical Spine Malformations Associated With a 5q34-5q35.2 Micro-interstitial Deletion: A Case Report

We report a female proband carrying a de novo 5q34-q35.2 deletion breakpoint, and review the unique skeletal phenotype and possible genotype related to this mutation. The patient presented with a persistent head tilt and limited head rotation. Non-contrast-enhanced three-dimensional computed tomography of the cervical spine revealed several malformations including a bone cleft in the right pars interarticularis, a bone defect in both C5 lamina and the transverse foramen at C2–C3, agenesis of the right articular process of C5, bony fusion of C4–C5, and subluxation of the craniocervical joints. Several deformities of the cervical spine seen in this patient have not been associated with the 5q deletion. A review of 5q-related mutations suggests that abnormalities associated with MSX2 gene might cause cervical spine abnormalities.

The clinical manifestations and outcomes of neuralgic amyotrophy

Background & Objective: Although the clinical manifestations and outcomes of neuralgic amyotrophy have been previously described, some controversies remain. Thus, we evaluated clinical manifestations and outcomes of patients with neuralgic amyotrophy. Methods: We evaluated the clinical and electrodiagnostic data, and the outcomes, of 32 patients with neuralgic amyotrophy.Of the 32 patients, 26 were followed-up for one year after onset of the disease.Results:The initial symptoms were pain (50.0%), pain with weakness (21.9%), other sensory symptoms without weakness (6.3%), and painless weakness or atrophy (21.9%). The commonly involved nerves were the median (75.0%), radial (68.8%), suprascapular (50.0%), ulnar (50.0%), axillary (46.9%), and musculocutaneous (40.6%) nerves. The initial symptoms were not associated with nerve involvement. Of all patients, 59% recovered fully, 16% had residual mild weakness without functional disability, and 6% experienced persistent severe weakness and were unable to return to work. Some patients were not evaluated because they were lost to follow-up. Conclusions: Painless weakness as an initial symptom of neuralgic amyotrophy may be more common than previously noted. Of all patients, 75% enjoyed favorable outcomes by one year after disease onset. These results will be useful when planning treatment strategies and will deepen our understanding of prognosis of neuralgic amyotrophy.

Pontine Necrosis Related with Radiation Therapy, Complicated with Spontaneous Hemorrhage

The brain necrosis induced by radiation therapy (RT) is an uncommon pathology of brain. A case of spontaneous hemorrhage at necrotic brain is also rare. A 52-year-old man who had nasopharyngeal carcinoma and had been treated with RT, presented with gait disturbance, dizziness, ataxia, dysarthria, and dysphagia. Magnetic resonance imaging (MRI) demonstrated progressed radiation necrosis of pons, and spontaneous hemorrhage at the site of necrosis. The hematoma was diminished by conservative treatment. However, the patient’s neurologic symptoms did not recover. Two years later, spontaneous bleeding recurred at necrotic brain. His neurologic symptoms worsened. One year later, his neurologic symptoms were more progressed. He showed severe dysphagia, profound weakness and respiratory failure. This case provides the description of relapsed spontaneous hemorrhage and medullary dysfunction caused by pontine necrosis and progressed post-radiation injury, complicated with hemorrhage, and urges caution in that the necrotic brain tissue may be vulnerable to bleeding.