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1.
Sci Rep ; 14(1): 12909, 2024 06 05.
Article in English | MEDLINE | ID: mdl-38839858

ABSTRACT

Patients with brain tumors require extensive and prolonged rehabilitation efforts as they suffer from lesion-induced motor weakness as well as treatment-related side effects, often leading to a significant decline in function. Protein supplements have shown positive effects on promoting muscle strength and physical performance in various tumor etiologies. However, reports on their effects specifically in brain tumor patients remain scarce. This study aims to investigate the feasibility and efficacy of protein supplements in enhancing rehabilitative outcomes via muscle strengthening and functional gain in brain tumor patients with neurological demise. Sixty brain tumor patients were randomly assigned to either a protein supplement or a control group, receiving either protein supplements or a placebo for 6 weeks, in conjunction with conventional rehabilitation therapy. Assessments before and after the intervention included laboratory tests, anthropometric measures using bioimpedance analysis, and functional assessments, which included the MMSE, the modified Barthel Index, the Beck Depression Inventory, the Brief Fatigue Inventory, the Timed Up and Go test, the 6-min walk test, the isokinetic quadriceps muscle strength test, and the handgrip power. After the intervention, the levels of serum hemoglobin, protein, albumin, and C-reactive protein were improved in both groups, however, the change was significant only in the protein group. The muscle strength was enhanced in both groups, however, the significant increase in pinch grasp power was only noted in the protein group (P < 0.05). The distance on 6MWT was also significantly extended at follow-up in the protein group (P < 0.01). In the subgroup analysis according to nutritional status, the moderate malnutrition group showed greater augmentation of muscle mass than those with adequate nutrition (P < 0.05). Interestingly, the amelioration of malnutrition was observed only the in protein group. This study using protein supplements to promote the rehabilitative potential of brain tumor patients revealed a significant effect on improving hemodynamic nutritional indices, muscle power reimbursement, and functional improvement, especially in malnourished patients. The safety and feasibility of protein supplements in brain tumor patients were affirmative in this study. Further studies with more patients may help confirm the secondary functional gain resulting from increased muscle power.Trial registration: This study was retrospectively registered in the Clinical Research Information Service, CRIS no. KCT0009113 on Jan 12, 2024.


Subject(s)
Brain Neoplasms , Muscle Strength , Muscle, Skeletal , Nutritional Support , Humans , Male , Female , Middle Aged , Brain Neoplasms/rehabilitation , Muscle, Skeletal/physiopathology , Nutritional Support/methods , Dietary Supplements , Adult , Treatment Outcome , Dietary Proteins/administration & dosage , Aged , Hand Strength
2.
Stem Cell Res Ther ; 14(1): 362, 2023 12 12.
Article in English | MEDLINE | ID: mdl-38087394

ABSTRACT

BACKGROUND: This 1-year open-label extension study aimed to identify the persistent synergistic effects of allogeneic umbilical cord blood (UCB) cells and erythropoietin (EPO) in children with cerebral palsy (CP) for up to 2 years. METHODS: This open-label extension study followed children with CP who were enrolled in the previous randomized, double blind, placebo-controlled trial. The following groups from the first trial were maintained: (A) UCB + EPO, (B) UCB, (C) EPO, and (D) only placebo, and all the participants had continued active rehabilitation. This extended study started 3 months after termination of the first trial, which had a 1-year follow-up duration. All subjects received single additional UCB intravenous infusion at the extension baseline regardless of their initial allocation. Outcome measures were the gross motor performance measure (GMPM), gross motor function measure-66 (GMFM-66), and Bayley scales of infant development-II (BSID-II), which were followed at 3, 6, and 12 months after the extension baseline. Changes in the outcome scores from the baseline values of the previous trial and this study were analysed. RESULTS: Sixty-nine children (4.29 ± 1.28 years, M:F = 34:35) were included in this study. Each group showed improvements in the outcome measures at 12 months after additional UCB infusion compared to the baseline scores, except for GMFM and GMPM in Group C which were elevated at 3 and 6 months post-therapy. Total subject analyses did not show significant differences in the outcome measures between the four different groups at 3, 6 and 12 months after additional UCB therapy. However, patients with severe dysfunction, whose GMFCS levels were IV and V, revealed a larger improvement of the GMPM score in Group A than in Group D (Ps < 0.05) from the baseline value of the previous trial. The changes in BSID-II mental scale scores were positively correlated with the number of administered total nucleated cells per unit body weight during this one-year extension study period (r = 0.536, P = 0.001). CONCLUSIONS: These results suggest that when administering UCB to treat patients with CP, combination therapy with EPO is more effective, and the effect might last as long as 2 years, especially in patients with severe impairments. TRIAL REGISTRATION: CHA Bundang Medical Center IRB, No. 2015-06-093, approved on July 29, 2015, ( https://www.e-irb.com:3443/devlpg/nlpgS200.jsp ), ClinicalTrials.gov, NCT03130816, retrospectively registered on April 27, 2017 ( https://clinicaltrials.gov/ct2/show/NCT03130816?term=NCT03130816&draw=2&rank=1 ).


Subject(s)
Cerebral Palsy , Erythropoietin , Child , Infant , Humans , Cerebral Palsy/drug therapy , Fetal Blood , Erythropoietin/therapeutic use , Blood Cells , Combined Modality Therapy , Treatment Outcome
3.
Int J Urol ; 30(1): 100-106, 2023 01.
Article in English | MEDLINE | ID: mdl-36305675

ABSTRACT

OBJECTIVE: Renal dysfunction is a major cause of morbidity in patients with spinal cord injury (SCI). A 24-h urine creatinine (Cr) clearance (24-h urine CCr) is cost-effective and easy to implement compared to renal scintigraphy in the evaluation of renal function. This study aimed to verify the feasibility of 24-h urine CCr in the SCI population by assessing the correlation with effective renal plasma flow (ERPF) on renal scintigraphy. METHODS: Data from 245 SCI patients (189 males, mean age: 50.2 years) were used in this retrospective review. Clinical characteristics, 24-h urine CCr, serum Cr, comorbidities, and body composition analyses were assessed for correlation with laboratory parameters including renal scintigraphy. Strong predictors of ERPF were determined by multivariate linear regression analysis. Areas under receiver-operating characteristic curves were calculated to evaluate the discriminating power of 24-h urine CCr to predict ERPF <250 ml/min. RESULTS: Spinal cord injury patients showed tubular dysfunction despite normal serum Cr and 24-h urine CCr. There was a significant correlation between 24-h urine CCr and ERPF, and 24-h urine CCr was one of the strongest predictors for ERPF (area under the curve 0.72, 95% CI 0.64-0.80, p < 0.000) among other parameters such as age, appendicular lean mass index, and body mass index. 24-h urine CCr was an independent predictor of ERPF in subacute (R2 = 0.497, p < 0.001) and chronic SCI patients (R2 = 0.664, p < 0.0001). The optimized 24-h urine CCr cut-off was 139.4 ml/min/1.72 m2 for predicting decreased ERPF <250 ml/min (sensitivity 67.6% and specificity 64.0%). CONCLUSION: 24-h urine CCr is a sensitive indicator for renal function deterioration of SCI patients. Further longitudinal studies with larger numbers of SCI patients are needed to confirm the feasibility of 24-h urine CCr for monitoring this population.


Subject(s)
Spinal Cord Injuries , Male , Humans , Middle Aged , Creatinine , Feasibility Studies , Kidney Function Tests , Kidney/diagnostic imaging , Kidney/physiology , Glomerular Filtration Rate
4.
J Clin Med ; 11(17)2022 Aug 24.
Article in English | MEDLINE | ID: mdl-36078901

ABSTRACT

Cachexia and low muscle mass in motor-complete tetraplegia are associated with poor outcomes. This study aimed to document anthropometric, body composition, and nutritional indices in patients and to assess the effect of a comprehensive rehabilitation nutrition program in cachexia and low muscle mass. For 34 motor-complete tetraplegia in the subacute phase, a comprehensive rehabilitation nutrition program was provided for 8 to 9 weeks. Risk of malnutrition, anthropometric and body composition indices, as well as laboratory tests were assessed upon admission and at discharge. A body mass index of less than 20.2 kg/m2 was used as the cut-off value for obesity. Upon admission, 73.5% of patients were classified as obese, half were at risk of malnutrition, and 29.4% were compatible with cachexia. Compared to the premorbid state, the non-obese group showed greater weight reduction (p < 0.01) and higher prevalence of low muscle mass (p = 0.004) than the obese group. Disease duration was different between groups (p < 0.01). After rehabilitation, malnutrition risk, protein levels, and hemodynamic results improved in both groups (p < 0.05). A significant time × group interaction was observed for muscle mass, skeletal muscle mass, and appendicular lean mass index (p < 0.05). Muscle mass was maintained while fat components increased in both groups. Functional improvement was negatively correlated with an increase in fat components (p < 0.05). A personalized rehabilitation nutrition intervention improved the nutritional status, body composition, and functional outcomes in motor-complete tetraplegia. The increase in muscle mass was associated with functional gain; healthy weight gain or maintenance may improve the level of independence. Further studies to endorse this beneficial evidence of rehabilitation nutrition in the maintenance of muscle component are needed.

5.
BMC Geriatr ; 22(1): 606, 2022 07 21.
Article in English | MEDLINE | ID: mdl-35864452

ABSTRACT

BACKGROUND: The geriatric population and advanced knee osteoarthritis are rapidly increasing in Korea, and the socioeconomic burden of total knee arthroplasty (TKA) is increasing. This study aimed to analyze the demographic, clinical and socioeconomic characteristics of patients who underwent TKA and to differentiate the factors affecting participation in inpatient-intensive rehabilitation programs after TKA in the Jeju regional rheumatoid and degenerative arthritis center established by the government. METHODS: This retrospective cohort study included 845 patients (735 females; 72.0 ± 5.8 years) diagnosed with primary osteoarthritis (OA) of the knee who underwent elective unilateral primary TKA between January 2013 and June 2016. Demographic, clinical, and socioeconomic characteristics, including age, body mass index, obesity, length of stay, OA severity, underlying disease, education level, occupation, and location of residence were reviewed. Patients were allocated to the TKA-only group (home discharge) and to the TKA + rehab group (participation in post-TKA rehabilitation). The variables were analyzed and compared before and after the establishment of the center and according to participation in intensive rehabilitation. RESULTS: Patients who underwent TKA were mostly female, in the 60 s, and had a high prevalence of comorbidities and obesity. After the rehabilitation center's establishment, the intensive post-TKA participation increased profoundly from 3% to 59.2%. Participants after the center establishment had lower mean BMI and a higher proportion of K-L grade 4 compared to those before the center establishment. The location of residence was the only factor differentiating the participation in the intensive rehabilitation. CONCLUSION: The regional rheumatoid and degenerative arthritis center was appropriate to satisfy the high unmet need for participating in the intensive rehabilitation after TKA and to execute the qualified integrated post-TKA care system. Policy support should ensure the early rehabilitation and a qualified integrated care system and prepare for the increased burden of revision. Future longitudinal studies should be conducted to assess the long-term effect of the integrated post-TKA rehabilitation program on functional outcomes and patient survivorship free from revision.


Subject(s)
Arthroplasty, Replacement, Knee , Delivery of Health Care, Integrated , Osteoarthritis, Knee , Aged , Arthroplasty, Replacement, Knee/rehabilitation , Female , Humans , Male , Obesity , Osteoarthritis, Knee/epidemiology , Osteoarthritis, Knee/surgery , Retrospective Studies , Treatment Outcome
6.
Front Neurol ; 13: 838302, 2022.
Article in English | MEDLINE | ID: mdl-35720059

ABSTRACT

Introduction: Taxanes are associated with a distal sensory neuropathy, significantly affecting cancer survivor quality of life. However, chemotherapy-induced peripheral neuropathy (CIPN) assessments are primarily based on clinical symptoms rather than objective neurophysiologic findings. Therefore, we investigated neurophysiologic changes in symptomatic subjects, comparing them with symptom severity. Materials and Methods: Medical charts of 111 subjects who were referred for CIPN diagnosis after chemotherapy for breast or ovarian cancer between May 1, 2016, and December 31, 2019, were retrospectively reviewed. Demographics, anthropometric parameters, and Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) pain scale data were collected. The nerve conduction study (NCS) results, including sensory nerve action potentials recorded from sural nerves, were analyzed relative to clinical symptoms. To optimize follow-up (FU) NCS diagnostic sensitivity, relative references of FU sural amplitude reductions to >30% and an absolute reference <10 µV were used. Results: Eighty-eight female patients met the criteria, and 20 underwent FU NCS. Baseline and FU sural amplitudes showed significant positive correlation with respective LANSS scores (p < 0.01). FU sural amplitude was significantly lower than the initial result (p < 0.05). The FU LANSS score was not different from the initial score. Initial NCS sensitivity and specificity for clinically suspected CIPN diagnoses with LANSS were 69.7 and 47.3%, respectively. All 20 subjects with FU evaluation were clinically compatible with CIPN (LANSS >12) at initial and FU assessments. Among them, only six (30.0%) had abnormal sural amplitudes (<10µV for ≤50 s, <3 µV for 60 s, <1 µV for 70 s) in the initial NCS. In the FU NCS, sural amplitude became abnormal in five additional subjects. Between the initial and FU NCS, sural amplitude was reduced by > 30% in eight subjects (40.0%). NCS sensitivity increased to 65.0% when including either abnormal sural amplitudes or a > 30% reduction in sural amplitude in FU studies. Conclusions: Although clinical symptoms and NCS results were positively correlated, a single NCS point had limited value for suspected CIPN electrophysiological diagnoses. Serial NCS during chemotherapy might help assess the degree of chemotherapy-induced nerve damage, attain evidence of CIPN prior to symptom aggravation, and monitor the progression of CIPN. Further study is needed to find specific relative references for variable patient factors to increase the sensitivity of electrophysiological studies of clinically suspected CIPN.

7.
Stem Cell Res Ther ; 11(1): 509, 2020 11 27.
Article in English | MEDLINE | ID: mdl-33246489

ABSTRACT

BACKGROUND: Concomitant administration of allogeneic umbilical cord blood (UCB) infusion and erythropoietin (EPO) showed therapeutic efficacy in children with cerebral palsy (CP). However, no clinical studies have investigated the effects of UCB and EPO combination therapy using a 2 × 2 four-arm factorial blinded design with four arms. This randomized placebo-controlled trial aimed to identify the synergistic and individual efficacies of UCB cell and EPO for the treatment of CP. METHODS: Children diagnosed with CP were randomly segregated into four groups: (A) UCB+EPO, (B) UCB+placebo EPO, (C) placebo UCB+EPO, and (D) placebo UCB+placebo EPO. Based on the UCB unit selection criteria of matching for ≥ 4/6 of human leukocyte antigen (HLA)-A, -B, and DRB1 and total nucleated cell (TNC) number of ≥ 3 × 107/kg, allogeneic UCB was intravenously infused and 500 IU/kg human recombinant EPO was administered six times. Functional measurements, brain imaging studies, and electroencephalography were performed from baseline until 12 months post-treatment. Furthermore, adverse events were closely monitored. RESULTS: Eighty-eight of 92 children enrolled (3.05 ± 1.22 years) completed the study. Change in gross motor performance measure (GMPM) was greater in group A than in group D at 1 month (△2.30 vs. △0.71, P = 0.025) and 12 months (△6.85 vs. △2.34, P = 0.018) post-treatment. GMPM change ratios were calculated to adjust motor function at the baseline. Group A showed a larger improvement in the GMPM change ratio at 1 month and 12 months post-treatment than group D. At 12 months post-treatment, the GMPM change ratios were in the order of groups A, B, C, and D. These results indicate synergistic effect of UCB and EPO combination better than each single therapy. In diffusion tensor imaging, the change ratio of fractional anisotropy at spinothalamic radiation was higher in group A than group D in subgroup of age ≥ 3 years. Additionally, higher TNC and more HLA-matched UCB units led to better gross motor outcomes in group A. Adverse events remained unchanged upon UCB or EPO administration. CONCLUSIONS: These results indicate that the efficacy of allogeneic UCB cell could be potentiated by EPO for neurological recovery in children with CP without harmful effects. TRIAL REGISTRATION: ClinicalTrials.gov, NCT01991145 , registered 25 November 2013.


Subject(s)
Cerebral Palsy , Erythropoietin , Cell- and Tissue-Based Therapy , Child , Child, Preschool , Diffusion Tensor Imaging , Fetal Blood , Humans
8.
Sci Rep ; 10(1): 4446, 2020 03 10.
Article in English | MEDLINE | ID: mdl-32157146

ABSTRACT

In a clinical trial of cerebral palsy, the level of plasma interleukin-8 (IL-8) was increased, correlated with motor improvement, after human umbilical cord blood mononuclear cell (hUCBC) infusion. This study aimed to elucidate the role of IL-8 in the therapeutic effects of hUCBCs in a mouse model of hypoxic-ischaemic brain injury (HI). In P7 HI mouse brains, hUCBC administration at day 7 after HI upregulated the gene expression of Cxcl2, the mouse IL-8 homologue and increased the expression of its receptor, CXCR2. hUCBC administration restored the sequential downstream signalling axis of p-p38/p-MAPKAPK2, NFκB, and angiogenic factors, which were downregulated by HI. An in vitro assay revealed the downregulation of the angiogenic pathway by CXCR2 knockdown and p38 inhibition. In vivo p38 inhibition prior to hUCBC administration in HI mouse brains produced identical results. Behavioural outcomes revealed a therapeutic effect (ps < 0.01) of hUCBC or IL-8 administration, which was correlated with decreases in infarct size and angiogenic findings in the striatum. In conclusion, the response of the host to hUCBC administration in mice upregulated Cxcl2, which led to the activation of the IL-8-mediated p-p38 signalling pathway. The upregulation of the downstream pathway and angiogenic growth factors via NFκB can be inferred to be the potential therapeutic mechanism of hUCBCs.


Subject(s)
Brain Injuries/therapy , Cord Blood Stem Cell Transplantation/methods , Human Umbilical Vein Endothelial Cells/cytology , Hypoxia-Ischemia, Brain/therapy , Interleukin-8/metabolism , Neovascularization, Physiologic , Animals , Animals, Newborn , Brain Injuries/metabolism , Brain Injuries/pathology , Cells, Cultured , Disease Models, Animal , Humans , Hypoxia-Ischemia, Brain/metabolism , Hypoxia-Ischemia, Brain/pathology , Intercellular Signaling Peptides and Proteins/metabolism , Leukocytes, Mononuclear/cytology , Mice
9.
Front Oncol ; 10: 111, 2020.
Article in English | MEDLINE | ID: mdl-32117760

ABSTRACT

Background: Cancer treatment-related lymphedema (CTRL) affects patients physically, psychologically and emotionally, and remains a significant quality of life issue among patients with cancer. Reliable methods to measure changes in lymphedema are required for early detection, acute intensive treatment, and long-term management. Here, we evaluated the use of bioimpedance analysis (BIA) as a tool to measure lymphedema before and after treatment. Patients and Methods: Patients with CTRL who were admitted to a secondary university hospital between October 2017 and July 2018 for complex decongestive therapy (CDT) were eligible for this prospective cohort study. Circumferential measure (CM) and BIA were used to evaluate lymphedema at admission (initial) and before discharge (follow-up, FU). Volume was calculated from the CM using the truncated cone formula. The inter-limb ratios (ILRs) of the circumference, volume, and impedance were also calculated as the unaffected limb to affected limb. Each parameter before and after treatment and correlations between parameters also were analyzed. Results: A total of 29 patients (12 upper- and 17 lower-extremity CTRL) completed were included in this analysis. Absolute value and the ILRs of circumference, volume or impedance, and extracellular water/total body water (ECW/TBW) were significantly improved at FU (p < 0.01, p < 0.05). The initial and FU absolute values, ILRs, ECW/TBW correlated significantly with each other (p < 0.01, p < 0.05). The cutoff values of ECW/TBW for moderate and severe degree of CTRL were 0.3855 and 0.3955, respectively. The changes of ILRs between initial and FU assessments were significantly different among three groups according to lymphedema severity (p < 0.01, p < 0.05). Conclusions: BIA data correlates significantly with clinical measurement, and therefore can be a practical tool in monitoring outcome measure after lymphedema treatment. In addition, BIA is more sensitive to subtle changes in lymphedema, and therefore can be useful for the long-term maintenance of lymphedema.

10.
Front Neurol ; 10: 1264, 2019.
Article in English | MEDLINE | ID: mdl-31866926

ABSTRACT

Objectives: The purposes of this study are to determine whether there is a correlation between the Mann Assessment of Swallowing Ability (MASA) and modified MASA (mMASA) according to various cognitive status and to investigate whether the cognitive status of patients with brain damage affects the prediction of aspiration using the MASA. Methods: We retrospectively assessed 146 dysphagic patients with brain lesion due to various causes. Dysphagia was assessed using the MASA and mMASA. According to the videofluoroscopic swallowing study results, patients were divided into two groups: aspirators and non-aspirators. Patients were classified into four groups according to cognitive function according to the Korean version of Mini-mental State Examination scores: normal (>24), mild (21-24), moderate (10-20), and severe (<10) cognitive impairment. The correlation between the MASA and mMASA scores according to cognitive function were analyzed. The sensitivity, specificity, and positive and negative predictive values of the MASA scores for predicting aspiration were assessed. Results: The MASA and mMASA scores showed a significant positive correlation in all cognition groups. In patients with more severe cognitive impairment MASA scores had high sensitivity and low specificity for prediction of aspiration. On the other hand, the MASA scores had low sensitivity and high specificity for prediction of aspiration in the normal and mild cognitive impairment groups. Conclusions: The MASA and mMASA scores correlated with each other in patients with various levels of cognitive function. Interestingly, this study results demonstrated that patients with good cognitive function may have false negative results of MASA screening due to low sensitivity. Thus, when interpreting the MASA results, the impact of cognitive status should be taken into consideration.

11.
Sci Rep ; 9(1): 17091, 2019 11 19.
Article in English | MEDLINE | ID: mdl-31745108

ABSTRACT

Cervical and upper thoracic spinal cord injury causes impairments in respiratory muscle performance, leading to variable degrees of pulmonary dysfunction and rendering deep breathing difficult for affected individuals. In this retrospective study, we investigated the effects of self-directed respiratory muscle training in this context by assessing pulmonary function relative to spinal cord injury characteristics. A total of 104 spinal cord injury patients (tetraplegia/paraplegia; 65/39, acute/subacute/chronic; 14/42/48) were admitted for short-term (4-8 weeks) in-patient clinical rehabilitation. Initial evaluation revealed a compromised pulmonary function with a percentage of predicted value of 62.0 and 57.5 in forced vital capacity in supine and forced vital capacity in sitting positions, respectively. Tetraplegic patients had more compromised pulmonary function compared with paraplegic patients. At follow-up evaluation, the percentage of predicted value of forced vital capacity in supine and sitting position improved overall on average by 11.7% and 12.7%, respectively. The peak cough flow improved by 22.7%. All assessed pulmonary function parameters improved significantly in all subgroups, with the greatest improvements found in patients with tetraplegia and subacute spinal cord injury. Therefore, short-term self-directed respiratory muscle training should be incorporated into all spinal cord injury rehabilitation regimens, especially for patients with tetraplegia and subacute spinal cord injury, as well as those with chronic spinal cord injury.


Subject(s)
Breathing Exercises/methods , Lung/physiopathology , Respiratory Muscles/physiopathology , Spinal Cord Injuries/rehabilitation , Female , Humans , Male , Middle Aged , Prognosis , Respiratory Function Tests , Retrospective Studies , Vital Capacity
12.
BMC Med Genet ; 19(1): 152, 2018 08 29.
Article in English | MEDLINE | ID: mdl-30157790

ABSTRACT

BACKGROUND: Sjogren-Larsson syndrome is a hereditary neurocutaneous syndrome that is non-progressive in nature. Although neuroregression has been reported in seizure-prone preschool children requiring anti-epileptic treatment, teenage-onset dystonia precipitating neurodegeneration without any immediate causal events has yet to be reported. CASE PRESENTATION: We describe a young woman with spastic diplegia and intellectual disability who began to show progressive neurological deterioration from 12 years of age, with the onset of dystonia and tremor. She was initially diagnosed with spastic cerebral palsy and periventricular leukomalacia based on brain magnetic resonance imaging. Follow-up brain imaging from 13 years of age did not reveal apparent changes, though abnormal electroencephalographic findings occurred in parallel with her decline in motor function. By 19 years of age, she had developed dysphagia and became completely dependent on others for most activities of daily living. Ultimately, whole-exome sequencing revealed a heterozygous compound mutation in the ALDH3A2 gene that corresponds to Sjogren-Larsson syndrome: an exon 9 deletion (1291-1292delAA) from the mother and an exon 5 splicing mutation (798 + 1delG) from the father. Neuroregression has been reported in preschool children after seizures requiring treatment, though our patient did not experience any immediate causal events. This report summarizes the clinical, radiologic, and electrophysiological findings observed over a decade concurrent with neurological deterioration after the onset of dystonia and tremor at the age of developmental ceiling in Sjogren-Larsson syndrome. CONCLUSIONS: In addition to the influence of additive variants or other environmental factors, accumulation of metabolites due to defective fatty aldehyde dehydrogenase is a potential pathomechanism of neurodegeneration in this patient. Neurological deterioration may be a presentation that is unnoticed in Sjogren-Larsson syndrome due to the rarity of the disease. This report highlights a unique clinical feature of Sjogren-Larsson syndrome with progressive neurodegeneration associated with dystonia and tremor.


Subject(s)
Neurodegenerative Diseases/genetics , Sjogren-Larsson Syndrome/genetics , Adolescent , Aldehyde Oxidoreductases/genetics , Cerebral Palsy/genetics , Female , Follow-Up Studies , Humans , Intellectual Disability/genetics
14.
Ann Rehabil Med ; 41(6): 944-950, 2017 Dec.
Article in English | MEDLINE | ID: mdl-29354570

ABSTRACT

OBJECTIVE: To investigate the serum vitamin D level and its determinant factors in stroke patients. METHODS: Fifty-one stroke patients who had documented serum level of 25-hydroxyvitamin D(25(OH)D) were included. Patients were divided into subacute (n=23) and chronic groups (n=28). The mean levels of 25(OH)D of the two groups were compared. Correlations between each 25(OH)D level and post-stroke duration were also analyzed. To assess other possible influencing factors, patients were subdivided by ambulation ability and feeding methods for comparison of 25(OH)D level. RESULTS: The mean level of 25(OH)D was significantly lower in the chronic group than in the subacute group (12.3 vs. 16.3 ng/mL; p<0.05). The serum 25(OH)D level decreased according to the duration after stroke (r=-0.52, p=0.01). Patients with a history of total parenteral nutrition had lower 25(OH)D levels than subjects who had enteral nutrition in the subacute group (7.3 vs. 18.8 ng/mL; p<0.01). However, the levels of 25(OH)D were not different between the oral feeding and tube feeding groups. Among the chronic group subjects, patients who could walk without assistance had higher 25(OH)D levels than non-ambulatory patients (ambulatory vs. non-ambulatory group; 18.3 vs. 11.3 ng/mL; p<0.05). CONCLUSION: After stroke onset, serum vitamin D level decreases with time regardless of feeding methods, and total parenteral nutrition may aggravate its deficiency. In terms of long-term care, non-ambulatory patients might be at a higher risk of vitamin D deficiency. Supplementation of vitamin D should be considered especially for stroke patients who are non-ambulatory and on total parenteral nutrition.

15.
Ann Rehabil Med ; 40(4): 725-33, 2016 Aug.
Article in English | MEDLINE | ID: mdl-27606280

ABSTRACT

OBJECTIVE: To investigate the energy expenditure (EE) of Korean young adults based on activities refined to a deskbound lifestyle. METHODS: Sixty-four healthy office workers aged between 25 and 46 years participated in this study. EE was expressed as metabolic equivalent of task (MET). Participants were evaluated in terms of their EE during physical activities of sleeping (n=22), typing (n=37), folding laundry (n=34), dishwashing (n=32), studying (n=18), mopping (n=35), walking (n=33), stair climbing (n=23), and running (n=29). Volume of oxygen consumption was measured by indirect calorimetry K4b(2) (COSMED). The results were compared to the established Compendium MET. RESULTS: The MET of activities were: sleeping, 1.24±0.43; typing, 1.35±0.25; folding laundry, 1.58±0.51; dishwashing, 2.20±0.51; studying, 2.11±0.90; mopping, 2.72±0.69; walking at 4 km/hr, 3.48±0.65; stair climbing of five stories, 6.18±1.08; and running at 8 km/hr, 7.57±0.57. The values of typing and mopping were similar to those in the Compendium, whereas those of sleeping, folding laundry, dishwashing, studying, walking, stair climbing and running were different. CONCLUSION: To our knowledge, this estimation of EE in MET during activities of daily living is the first data of young adults in Korea. These data could be used as a reference to modify the guidelines of physical activities for the age group examined in this study.

16.
Ann Rehabil Med ; 40(2): 318-25, 2016 Apr.
Article in English | MEDLINE | ID: mdl-27152283

ABSTRACT

OBJECTIVE: To compare quantitative muscle activation between erect and slouched sitting postures in the muscles around the scapula, and to investigate the correlation between the angle of thoracic kyphosis and the alteration of muscle activity depending on two different sitting postures. METHODS: Ten healthy males participated in the study. Unilateral surface electromyography (SEMG) was performed for serratus anterior, middle trapezius (MT), and lower trapezius (LT), which are scapular stabilizer muscles, as well as latissimus dorsi. Participants elevated their shoulders for 3 seconds up to 90° abduction in the scapular plane, tilting 30° anterior in the coronal plane. They were told to hold the position for 10 seconds and voluntary isometric contractions were recorded by SEMG. These movement procedures were conducted for three times each for erect and slouched sitting postures and data were averaged. RESULTS: Activities of MT and LT increased significantly more in the slouched sitting posture than in the erect one. There was no significant correlation between kyphotic angle and the area under curve of each muscle. CONCLUSION: Because MT and LT are known as prime movers of scapular rotation, the findings of this study support the notion that slouched sitting posture affects scapular movement. Such scapular dyskinesis during arm elevation leads to scapular stabilizers becoming overactive, and is relevant to muscle fatigue. Thus, slouched sitting posture could be one of the risk factors involved in musculoskeletal pain around scapulae.

17.
J Child Neurol ; 31(10): 1227-34, 2016 09.
Article in English | MEDLINE | ID: mdl-27233796

ABSTRACT

This study was conducted to assess the safety and efficacy of recombinant human erythropoietin in young children with cerebral palsy aged between 6 months and 3 years. All participants received subcutaneous recombinant human erythropoietin and 8 weeks of rehabilitation therapy. Adverse events, changes of vital signs, and hematologic tests were monitored up to 8 weeks postinjection. Functional measures of development at 4 and 8 weeks postinjection were compared with baseline values, and improvements were compared with those of an age-matched historical control group. Nine participants completed the trial from June 2012 to February 2015. No adverse events were related to recombinant human erythropoietin. Erythropoiesis was noted, although within normal range. Functional improvements were observed in all participants (P < .05) and increases in motor function were higher in recombinant human erythropoietin group than the control group. Accordingly, recombinant human erythropoietin administration was safe without any significant adverse events and improved the functional outcomes in young children with cerebral palsy.


Subject(s)
Cerebral Palsy/drug therapy , Erythropoietin/therapeutic use , Neuromuscular Agents/therapeutic use , Cerebral Palsy/rehabilitation , Child, Preschool , Erythropoietin/adverse effects , Female , Humans , Infant , Injections, Subcutaneous , Male , Neuromuscular Agents/adverse effects , Recombinant Proteins/adverse effects , Recombinant Proteins/therapeutic use , Treatment Outcome
18.
Am J Phys Med Rehabil ; 89(2): 147-52, 2010 Feb.
Article in English | MEDLINE | ID: mdl-19730356

ABSTRACT

Cinical experience has shown us that some infants with congenital muscular torticollis have a cough reflex while stretching the sternocleidomastoid muscle. The objective of this study is to present a case series with the maneuver inducing the cough reflex and facial color change and to provide the possible mechanism underlying this phenomenon. This is a case series from a prospective cohort. Among 290 children with congenital muscular torticollis who came to a single torticollis clinic from January to December 2008, the children who showed cough reflex were consecutively enrolled. Twenty-four infants (8.28%) showed the cough reflex. The age of first presentation with congenital muscular torticollis was 37.65 +/- 19.60 days old. They showed 57.5 +/- 7.3 degrees of the passive cervical rotation to the congenital muscular torticollis side at the initial visit. The mean thickness of the sternocleidomastoid muscle in those with cough reflex was 13.79 +/- 1.96 mm at the side of congenital muscular torticollis and 5.43 +/- 0.85 mm on the contralateral side. The cough reflex disappeared, and 90 degrees of passive cervical rotation to the congenital muscular torticollis side were regained with stretching exercises and/or surgical release in all 24 children. One of the possible mechanisms for this cough reflex is surmised to be the mechanical irritation of the internal branch of the superior laryngeal nerve during the maneuver, which is one of the branches of the vagus nerve and is responsible for the sensation of the mucous membrane of the larynx. 8.28% of the infants with congenital muscular torticollis showed positive sign of cough reflex and had at least double or more thickness of the sternocleidomastoid muscle compared with that of unaffected sternocleidomastoid muscle and, at the same time, had 60 degrees or less of passive cervical rotation toward the affected side. To the best of our literature review, this laryngeal cough reflex is a new finding that has never been described before. One of the possible mechanisms for this cough reflex is surmised to be the mechanical irritation of the internal branch of the superior laryngeal nerve during the maneuver, which is one of the branches of the vagus nerve, acting as the afferent nerve of laryngeal cough reflex.


Subject(s)
Cough/physiopathology , Laryngeal Nerves/physiology , Muscle Stretching Exercises , Torticollis/physiopathology , Torticollis/therapy , Cohort Studies , Cough/complications , Cough/therapy , Female , Humans , Infant , Infant, Newborn , Male , Muscle, Skeletal/diagnostic imaging , Muscular Diseases/congenital , Muscular Diseases/diagnostic imaging , Range of Motion, Articular , Torticollis/congenital , Ultrasonography
19.
Arch Phys Med Rehabil ; 90(8): 1332-9, 2009 Aug.
Article in English | MEDLINE | ID: mdl-19651267

ABSTRACT

OBJECTIVES: To investigate (1) the relation between needle diameter and treatment efficacy of myofascial pain syndrome and (2) the relation between needle diameter and pain intensity during injection. DESIGN: Randomized controlled trial. SETTING: University-affiliated tertiary-care hospital. PARTICIPANTS: Volunteers (N=77) with myofascial pain syndrome affecting upper- and middle-trapezius muscles with at least 3 months' duration of pain. INTERVENTION: Participants were randomly assigned to receive trigger point injections on 1 side of the trapezius with a 21-, 23-, or 25-gauge needle. After a 1-time injection, participants were followed up for 14 days. Participants and the assessor were blinded for group assignment. MAIN OUTCOME MEASURES: Treatment efficacy was measured with the visual analog scale (VAS; at pretreatment, and posttreatment on days 1, 4, 7, 14) for neck and upper-back pain, the Neck Disability Index (NDI; at pretreatment, and posttreatment on days 7, 14), and the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36, at pretreatment and posttreatment on days 7, 14) for health-related quality of life. Pain intensity during injection was evaluated immediately after injection with VAS. RESULTS: VAS scores for posttreatment on days 4, 7, and 14 decreased significantly compared with pretreatment scores in all groups; NDI scores on days 7 and 14 decreased significantly compared with pretreatment scores in all groups; SF-36 scores on days 7 and 14 decreased significantly compared with pretreatment scores in the 21- and 23-gauge needle groups; and SF-36 score on day 14 showed significant difference between the 21- and 25-gauge needle groups. For pain intensity during injection, VAS scores indicated no significant difference between the 3 groups. CONCLUSIONS: No difference between the needle types was observed in terms of VAS or NDI, or in terms of pain intensity felt by patients during injection. In terms of SF-36 scores, injections with 21- or 23-gauge needles were found to be more effective. However, a well-controlled investigation is needed to explore the effect of needle thickness on health-related quality of life.


Subject(s)
Analgesics/administration & dosage , Injections, Intramuscular/instrumentation , Myofascial Pain Syndromes/drug therapy , Neck Pain/drug therapy , Needles , Adult , Aged , Analysis of Variance , Equipment Design , Female , Humans , Male , Middle Aged , Neck Muscles , Pain Measurement , Quality of Life , Surveys and Questionnaires , Treatment Outcome
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