Hirayama disease is a slowly progressing benign motor neuron disease that affects the distal upper limb. A 29-year-old man visited the hospital with a 1-year history of weakened left proximal upper limb. He was diagnosed with Hirayama disease 9 years ago, while there was no further progression of the muscle weakness afterward. Atrophy and weakness was detected in proximal upper limb muscles. Magnetic resonance imaging and somatosensory evoked potentials were normal. Needle electromyography showed abnormal findings in proximal upper limb muscles. Our patient had Hirayama disease involving the proximal portion through secondary progression. Clinical manifestation and accurate electromyography may be useful for diagnosis. Rare cases with progression patterns as described here are helpful and have clinical meaning for clinicians.
Adult ; Atrophy ; Diagnosis ; Electromyography ; Evoked Potentials, Somatosensory ; Humans ; Magnetic Resonance Imaging ; Motor Neuron Disease ; Muscle Weakness ; Muscles ; Needles ; Upper Extremity

OBJECTIVE: To obtain reference values, to suggest optimal recording and stimulation site for radial motor nerve conduction study (RmNCS), and to analyze the correlation among RmNCS parameters, demographics and ultrasonography (US) findings. METHODS: A total of 55 volunteers participated in this study. We hypothesized that ‘lateral edge of spiral groove (A)’ was the optimal stimulation site, and the ‘largest cross-sectional area (CSA) of extensor indicis proprius (EIP) muscle (B)’ was the optimal recording site. The surface distance between ‘A’ and the lateral epicondyle of the humerus divided by upper arm length, was named the spiral groove ratio. The surface distance between ‘B’ and the ulnar styloid process divided by forearm length, was named the EIP ratio. Using US, we identified these sites, and further conducted RmNCS. RESULTS: Data was collected from 100 arms of the 55 volunteers. Mean amplitude and latency were 5.7±1.1 mV and 5.7±0.5 ms, respectively, at the spiral groove, and velocity between elbow and spiral groove was 73.7±7.0 m/s. RmNCS parameters correlated significantly with height, weight, arm length, and CSA of the EIP muscle. Spiral groove ratio and EIP ratio were 0.338±0.03 and 0.201±0.03, respectively; both values were almost the same, regardless of age, sex and handedness. CONCLUSION: We established a reference value and standardized method of RmNCS using US. Optimal RmNCS can be conducted by placing the recording electrode 20% (about one-fifth) of forearm length from the ulnar styloid process, and stimulating at 34% (about one-third) of the humeral length from the lateral epicondyle.
Adult* ; Arm ; Demography ; Elbow ; Electrodes ; Electromyography ; Forearm ; Functional Laterality ; Humans ; Humerus ; Methods ; Neural Conduction* ; Radial Nerve ; Reference Values ; Ultrasonography* ; Volunteers

Objective: To compare the relationship of the Bayley Scales of Infant and Toddler Development 3rd Edition (K-BSID-III) language score and the Sequenced Language Scale for Infant (SELSI) score and evaluate the sensitivity and specificity of K-BSID-III language score and optimal cutoff value with receiver operator characteristic (ROC) curve analysis in infants and toddlers with delayed language development. Methods: A total of 104 children with suspected language developmental delay were included in this retrospective study. Subjects were tested using the K-BSID-III and SELSI and subdivided into several groups according to the severity of language scores. ROC curve analysis was performed to assess K-BSID-III for delayed language development. Results: Receptive and expressive language subscales of the K-BSID-III showed markedly significant correlation with the SELSI scores (p<0.001). ROC analysis showed an area under the curve of 0.877 (p<0.001) in SELSI receptive score and 0.935 (p<0.001) in SELSI expressive score. The optimal cutoff value where sensitivity of 85% and specificity of 81% were achieved with the K-BSID-III receptive score was 1.50 (between average and low average) in the SELSI receptive score. The optimal cutoff value where sensitivity of 96% and specificity of 82% were achieved with the K-BSID-III expressive score was also 1.50 in the SELSI expressive score. Conclusion In this study, the correlations between K-BSID-III and SELSI language scores were statistically significant. However, the interpretation should be considered carefully in low average group due to tendency of underestimation of delayed language development.

Objective: To investigate the association between vitamin E and handgrip strength (HGS) with multiple factors. Methods: A total of 1,814 participants were included (822 men and 981 women) from the Korean subjects of the 7th Korea National Health and Nutrition Examination Survey in 2018. Data were analyzed using multiple logistic regression to determine the correlation between vitamin E and HGS with potential confounding factors. Results: In the multiple logistic regression model, only the young age group (19–40 years) of men showed a positive relationship between vitamin E and HGS. However, in older age groups (41–80 years) of men and all age groups of women, there was no statistically significant result. After adjusting for confounding factors, young men showed higher vitamin E levels and higher HGS. Conversely, women and older age groups did not show significant results after adjusting for confounding factors. Conclusion In this study, the serum vitamin E level had a positive effect on HGS in young men (<40 years). Further research is needed on this topic regarding vitamin E intake and other objective measures.

Objective: To investigate the association between vitamin E and handgrip strength (HGS) with multiple factors. Methods: A total of 1,814 participants were included (822 men and 981 women) from the Korean subjects of the 7th Korea National Health and Nutrition Examination Survey in 2018. Data were analyzed using multiple logistic regression to determine the correlation between vitamin E and HGS with potential confounding factors. Results: In the multiple logistic regression model, only the young age group (19–40 years) of men showed a positive relationship between vitamin E and HGS. However, in older age groups (41–80 years) of men and all age groups of women, there was no statistically significant result. After adjusting for confounding factors, young men showed higher vitamin E levels and higher HGS. Conversely, women and older age groups did not show significant results after adjusting for confounding factors. Conclusion In this study, the serum vitamin E level had a positive effect on HGS in young men (<40 years). Further research is needed on this topic regarding vitamin E intake and other objective measures.

Background: Several noninvasive tools are available for the assessment of nonalcoholic fatty liver disease (NAFLD) including clinical and blood biomarkers, transient elastography (TE), and magnetic resonance imaging (MRI) techniques, such as proton density fat fraction (MRI-PDFF) and magnetic resonance elastography (MRE). In the present study, we aimed to evaluate whether magnetic resonance (MR)-based examinations better discriminate the pathophysiologic features and fibrosis progression in NAFLD than other noninvasive methods. Methods: A total of 133 subjects (31 healthy volunteers and 102 patients with NAFLD) were subjected to clinical and noninvasive NAFLD evaluation, with additional liver biopsy in some patients (n=54). Results: MRI-PDFF correlated far better with hepatic fat measured by MR spectroscopy (r=0.978, P<0.001) than with the TE controlled attenuation parameter (CAP) (r=0.727, P<0.001). In addition, MRI-PDFF showed stronger correlations with various pathophysiologic parameters for cellular injury, glucose and lipid metabolism, and inflammation, than the TE-CAP. The MRI-PDFF and TE-CAP cutoff levels associated with abnormal elevation of serum alanine aminotransferase were 9.9% and 270 dB/m, respectively. The MRE liver stiffness measurement (LSM) showed stronger correlations with liver enzymes, platelets, complement component 3, several clinical fibrosis scores, and the enhanced liver fibrosis (ELF) score than the TE-LSM. In an analysis of only biopsied patients, MRE performed better in discriminating advanced fibrosis with a cutoff value of 3.9 kPa than the TE (cutoff 8.1 kPa) and ELF test (cutoff 9.2 kPa). Conclusion Our results suggest that MRI-based assessment of NAFLD is the best non-invasive tool that captures the histologic, pathophysiologic and metabolic features of the disease.

Background: Several noninvasive tools are available for the assessment of nonalcoholic fatty liver disease (NAFLD) including clinical and blood biomarkers, transient elastography (TE), and magnetic resonance imaging (MRI) techniques, such as proton density fat fraction (MRI-PDFF) and magnetic resonance elastography (MRE). In the present study, we aimed to evaluate whether magnetic resonance (MR)-based examinations better discriminate the pathophysiologic features and fibrosis progression in NAFLD than other noninvasive methods. Methods: A total of 133 subjects (31 healthy volunteers and 102 patients with NAFLD) were subjected to clinical and noninvasive NAFLD evaluation, with additional liver biopsy in some patients (n=54). Results: MRI-PDFF correlated far better with hepatic fat measured by MR spectroscopy (r=0.978, P<0.001) than with the TE controlled attenuation parameter (CAP) (r=0.727, P<0.001). In addition, MRI-PDFF showed stronger correlations with various pathophysiologic parameters for cellular injury, glucose and lipid metabolism, and inflammation, than the TE-CAP. The MRI-PDFF and TE-CAP cutoff levels associated with abnormal elevation of serum alanine aminotransferase were 9.9% and 270 dB/m, respectively. The MRE liver stiffness measurement (LSM) showed stronger correlations with liver enzymes, platelets, complement component 3, several clinical fibrosis scores, and the enhanced liver fibrosis (ELF) score than the TE-LSM. In an analysis of only biopsied patients, MRE performed better in discriminating advanced fibrosis with a cutoff value of 3.9 kPa than the TE (cutoff 8.1 kPa) and ELF test (cutoff 9.2 kPa). Conclusion Our results suggest that MRI-based assessment of NAFLD is the best non-invasive tool that captures the histologic, pathophysiologic and metabolic features of the disease.