Search

Abstract: Background: Although autonomic dysfunction is common in patients with Parkinson’s disease (PD), few data are available regarding its pattern and quantitative severity with increasing Hoehn and Yahr (H&Y) stage. We conducted autonomic function tests to quantify autonomic dysfunction in PD patients and to elucidate its possible relationship with disease progression. Methods: We performed autonomic function tests including Valsalva ratio, heart rate response to deep breathing, quantitative sudomotor axon reflex test, and head-up tilt test in 66 patients with PD. We compared clinical characteristics and results of autonomic function tests between stages, and correlated the proportion of abnormal patients in each test with their H&Y stage. In addition, logistic regression analyses were conducted to examine the contribution of increasing H&Y stage to impairments of each domain of the autonomic nervous system. Results: We found that PD patients with higher disease stage tended to have impairments in cardiovagal and sudomotor domains of the autonomic nervous system. Cardiovagal function was the domain most influenced by disease progression. Our findings also demonstrated that the pattern of sudomotor impairment in PD was similar to that in patients with peripheral autonomic neuropathy. Conclusions: Our study demonstrates that autonomic dysfunction is not only common in early stage PD but it increases in severity with increasing disease stage. Given that the patterns of sudomotor impairments in PD are similar to those in peripheral neuropathy, our data support a previous hypothesis that pathophysiology of PD involves both the central and peripheral nervous systems. [Copyright &y& Elsevier]

Introduction The aims of this study were to identify factors affecting the measurement of nerve cross-sectional area (CSA) and to establish normal reference values for nerve ultrasonography of the upper extremity. Methods Ninety-seven age-matched, healthy individuals were enrolled. Ultrasonographic tests were performed by tracing nerves from the axilla to the distal arm. CSA was measured at the following locations: median nerve (carpal tunnel inlet, forearm, antecubital fossa, midhumerus); ulnar nerve (wrist, 2 cm proximal to the wrist, forearm, cubital tunnel outlet and inlet, midhumerus); radial nerve (spiral groove, antecubital fossa); and musculocutaneous nerve (proximal humerus). Results The CSA reference values differed between the measured points along the path of each nerve. Nerve CSA correlated significantly with weight, body mass index, height, and gender; however, the CSA ratio (distal/proximal) did not correlate with demographic factors. Conclusions The normal reference values may be helpful in diagnosis of pathologies involving these nerves. Muscle Nerve 47: 864-871, 2013 [ABSTRACT FROM AUTHOR]

Introduction: The aim of this study was to determine normal reference values for cross-sectional area (CSA) and the correlation between demographic factors and CSA in the cervical roots and brachial plexus trunks using ultrasonography. Methods: Ninety-five age-matched healthy individuals were studied. Ultrasonographic tests were performed via nerve tracing from the cervical root to the brachial plexus trunk. The CSA of each nerve was measured in the C5-8 ventral roots and brachial plexus (trunk level). Results: Normal values of each cervical root were: C5, 5.66 ± 1.02 mm2; C6, 8.98 ± 1.65 mm2; C7, 10.43 ± 1.86 mm2; and C8, 10.76 ± 2.02 mm2. Values for the brachial plexus were: upper trunk, 16.70 ± 2.88 mm2; middle trunk, 14.01 ± 2.70 mm2; and lower trunk, 13.75 ± 2.57 mm2. The side-to-side discrepancy was 11.91 ± 11.11%. Body mass index (BMI) and height correlated frequently with nerve CSA. Conclusions: These reference values may be helpful in investigating pathologies involving the cervical area. Muscle Nerve 46: 711-716, 2012 [ABSTRACT FROM AUTHOR]

Abstract: We performed this study to evaluate whether or not the cutaneous silent period (CSP) is a useful metric to identify small-fiber neuropathy in diabetic patients. The CSP was measured from the abductor pollicis brevis muscle in 30 healthy controls and 110 diabetic patients, who in turn were divided into 3 subgroups (patients with large-fiber neuropathy, patients with small-fiber neuropathy, and asymptomatic patients). The measured CSP and clinical characteristics were compared among the groups. The power of the CSP in discriminating patients from controls and any correlation with other clinical variables were analyzed. Each patient subgroup had a significantly delayed CSP latency compared to the controls. The latency of patients with large-fiber neuropathy was also significantly prolonged compared to the other subgroups of patients. The CSP latency was the only variable to discriminate patients. The latency showed a significant correlation with the late responses in nerve conduction studies. Thus, the CSP latency may be a useful tool in evaluating small neural fiber function in diabetic patients. [Copyright &y& Elsevier]

Application of high-resolution ultrasonography in neuromuscular disorders has been rapidly expanding. Traditionally, clinical assessment of patients with the neuromuscular disorders starts with obtaining the history, clinical examination and is followed by an electrodiagnostic test, which is a golden standard to assess the functional status of the peripheral neuromuscular system. Nowadays, in addition to the assessment of neuromuscular function using the traditional electrodiagnostic test, evaluation of the morphology of involved nerves and muscles using US is performed in patients with a wide spectrum of neuromuscular disorders. To get a better diagnosis, we need to increase our understanding of physiology and morphology of peripheral nerve and muscle as assessed by electrodiagnostic test and US. In this lecture, we will discuss about ultrasound images of nerves and muscles in upper extremity according to the cross-sectional anatomic knowledge. In the extremities, nerves frequently run with vessels and small arteries and can mimic nerve fascicles. Cross-sectional anatomy is a key to locate target structure during ultrasound. 3-dimensional spatial relationship of nerve, muscle, and vessels has specific pattern. The anatomic knowledge will help you to be an expert in NM US. [ABSTRACT FROM AUTHOR]

Postcraniectomy orthostatic headache is a rare, but disabling, condition which can often interfere with patient rehabilitation. Previously, postsurgical CSF leaks have generally been managed with surgical repair. We present the case of a 32-year-old man with postcraniectomy orthostatic headache which was successively managed with repeated lumbar epidural blood patches. [ABSTRACT FROM AUTHOR]

Background Context: Lumbar discography has been widely used for evaluating discogenic low back pain (LBP). Comparison of pain responses from suspected symptomatic discs with pain responses from asymptomatic negative discs is routine. However, the ability of discography to distinguish asymptomatic morphologically abnormal discs from those that are symptomatic has been understudied. In addition, the discographic characteristics of negative discs in patients with chronic discogenic LBP have not been reported. Criteria for negative morphologically abnormal discs may be valuable for excluding discs from further treatment and examination. Purpose: To determine if discography can distinguish asymptomatic discs among morphologically abnormal discs in patients with suspected chronic discogenic LBP and establish the standard characteristics of negative discs. Study Design/setting: Prospective, experimental with control group. Patient Sample: Fifty-five discs from a control group of 16 healthy volunteers without current back pain (11 men, 5 women, 32-61 years of age, mean age: 47 years) and 282 discs from a patient group of 90 LBP patients (59 men, 31 women, 20-70 years of age, mean age: 44.7 years) were recruited. Methods: Discography was performed using a pressure-controlled manometric technique with an injection rate of 0.05 mL/s and a 3.5 mL restricted total volume. Concordance was rated as none/unfamiliar, or familiar. Pain was rated via a 0-10 numerical rating scale (NRS). The pressure and volume at which pain was evoked and NRS pain responses at 15, 30, and 50 psi were recorded. Annular disruption grade was rated during the procedure by computed tomography discography and fluoroscopic imaging. Negative discogram required no pain described by the participant as "familiar," with no pain responses >or=6/10 NRS at pressures Results: Among 55 asymptomatic control group discs, 32 (58.2%) exhibited Grade 3 annular tear. All discs in the asymptomatic control group satisfied negative response criteria. Among 282 patient group discs, 199 (70.6%) exhibited Grade 3 annular tear. Of 199 discs with Grade 3 annular tears, 104 (52.3%) satisfied negative response criteria and were categorized as the Neg-D group. The other 95 discs were categorized as a Pos-D group. Patients showed significantly lower pain tolerance relative to control subjects (p<.05). The control and Neg-D groups showed similar pressures and volumes at which pain was initially evoked. Mean control group pain scores were 0.47 NRS at 15 psi and 1.58 NRS at 50 psi. Mean Neg-D group pain scores were 0.11 NRS at 15 psi and 1.1 NRS at 50 psi. Discographic findings for the Pos-D group were significantly different from those of the control and Neg-D groups (p<.001). Conclusions: Pain tolerance was significantly lower in patients relative to asymptomatic subjects. Negative patient discs and asymptomatic subject discs showed similar characteristics. Pressure-controlled manometric discography using strict criteria may distinguish asymptomatic discs among morphologically abnormal discs with Grade 3 annular tears in patients with suspected chronic discogenic LBP. [ABSTRACT FROM AUTHOR]

Abstract: The affect of muscle length and voluntary contraction upon compound muscle action potentials (CMAPs) in subjects with carpal tunnel syndrome (CTS) has been evaluated. Twenty-five hands in a CTS patient group and 29 hands in a normal subject control group were studied. The CMAPs from the abductor pollicis brevis induced by median nerve stimulation at the wrist were obtained for five thumb positions: neutral, abduction for shortening with and without contraction, and adduction for lengthening with and without contraction. Upon muscle shortening with relaxation, CMAP duration decreased in both groups, whereas waveform amplitude increased in the control group and showed no significant change in the CTS group. Muscle shortening with contraction afforded decreased CMAP duration and increased CMAP amplitude in both groups. Upon muscle lengthening with relaxation, both groups showed a reduction in CMAP amplitude and an increase in CMAP duration. Upon lengthening with contraction, CMAP duration decreased in the control group; in contrast, the CTS group showed further amplitude reduction and the waveform duration returned to the neutral value. These results demonstrate that, in patients with CTS, physiologic CMAP summations by muscle shortening or contraction may be less effective, whereas decreases in amplitude and increases in duration may be accentuated by lengthening and contraction. [Copyright &y& Elsevier]

Objective Methods Results Interpretation Conventionally, MRI aids in differentiating acute unilateral peripheral vestibulopathy/vestibular neuritis (AUPV/VN) from mimickers. Meanwhile, the diagnostic utility of MRIs dedicated to the inner ear remains to be elucidated for diagnosing AUPV/VN.We prospectively recruited 53 patients with AUPV/VN (mean age ± SD = 60 ± 15 years, 29 men). Initial MRIs were performed with a standard protocol, and an additional axial 3D‐fluid‐attenuated inversion recovery (3D‐FLAIR) sequence was obtained 4 h after intravenous injection of gadoterate meglumine. Abnormal enhancement was defined as a signal intensity that exceeded the mean + 2SD value on the healthy side. The findings of neurotologic evaluation and MRIs were compared.Overall, the inter‐rater agreement for gadolinium enhancement was 0.886 (Cohen's kappa coefficient). Enhancement was observed in 26 patients (49%), most frequently in the vestibule (n = 20), followed by the anterior (n = 12), horizontal (HC, n = 8), posterior canal (n = 5), and superior (n = 3) and inferior (n = 1) vestibular nerves. In multivariable logistic regression analysis, the enhancement was associated with decreased HC gain in video head‐impulse tests (p = 0.036), increased interaural difference in ocular vestibular‐evoked myogenic potentials (p = 0.001), and a longer onset‐to‐MRI time span (p = 0.024). The sensitivity and specificity were 92.3% and 81.5%, respectively, with an area under the curve of 0.90 for predicting gadolinium enhancement.Robust gadolinium enhancement was observed on 4‐hour‐delayed 3D‐FLAIR images in nearly half of the patients with AUPV/VN, with a good correlation with the results of neurotologic evaluation. The positivity may be determined by the extent of vestibular deficit, timing of imaging acquisition, and possibly by the underlying etiology causing AUPV/VN. MRIs may aid in delineating the involved structures in AUPV/VN. [ABSTRACT FROM AUTHOR]

The cubital tunnel is the most common site of ulnar nerve entrapment. Previous ultrasound studies have demonstrated enlargement of the ulnar nerve in cubital tunnel syndrome but did not report on the cubital tunnel itself. Twenty-two individuals with cubital tunnel syndrome were evaluated with nerve conduction studies and ultrasound. The ultrasound measurement that most strongly correlated with conduction velocity was the ratio of ulnar nerve to cubital tunnel cross-sectional area with the elbow flexed. Measurement of this ratio may improve the diagnostic accuracy of ultrasound in cubital tunnel syndrome, although further investigation is needed. [ABSTRACT FROM AUTHOR]

Purpose: The specific characteristics of autonomic involvement in patients with early Parkinson's disease (PD) are unclear. This study aimed to evaluate the characteristics of autonomic dysfunction in drug-naïve patients with early-stage PD without orthostatic hypotension (OH) by analyzing Valsalva maneuver (VM) parameters. Methods: We retrospectively analyzed drug-naïve patients without orthostatic hypotension (n = 61) and controls (n = 20). The patients were subcategorized into early PD (n = 35) and mid-PD (n = 26) groups on the basis of the Hoehn and Yahr staging. VM parameters, including changes in systolic blood pressure at late phase 2 (∆SBPVM2), ∆HRVM3, Valsalva ratio (VR), pressure recovery time, adrenergic baroreflex sensitivity, and vagal baroreflex sensitivity, were assessed. Results: In the early PD group, ∆SBPVM2, a marker of sympathetic function, was significantly lower compared with that in controls (risk ratio = 0.95, P = 0.027). Receiver operating characteristic (ROC) curve analysis showed an optimal cut-off value of −10 mmHg for ∆SBPVM2 [P = 0.002, area under the curve (AUC): 0.737]. VR exhibited an inverse relationship with Unified Parkinson's Disease Rating Scale Part 3 scores in the multivariable regression analysis (VR: P = 0.038, β = −28.61), whereas age showed a positive relationship (age: P = 0.027, β = 0.35). Conclusion: The ∆BPVM2 parameter of the VM may help detect autonomic nervous system involvement in early-PD without OH. Our results suggest that sympathetic dysfunction is an early manifestation of autonomic dysfunction in patients with PD. [ABSTRACT FROM AUTHOR]

The results of video head impulse tests (video-HITs) may be confounded by data artifacts of various origins, including pupil size and eyelid obstruction of the pupil. This study aimed to determine the effect of these factors on the results of video-HITs. We simulated ptosis by adopting pharmacological dilatation of the pupil in 21 healthy participants (11 women; age 24–58 years). Each participant underwent video-HITs before and after pupillary dilatation using 0.5% tropicamide. We assessed the changes in the vestibulo-ocular reflex (VOR) gain, corrective saccade amplitude, and frequency of eyelid flicks. After pupillary dilatation, the VOR gain decreased for both right (RAC; 1.12 ± 0.12 vs. 1.01 ± 0.16, p = 0.011) and left anterior canals (LACs; 1.15 ± 0.13 vs. 0.96 ± 0.14, p < 0.001), and right posterior canal (RPC, 1.10 ± 0.13 vs. 0.98 ± 0.09, p = 0.001). The corrective saccade amplitudes also decreased significantly for all four vertical canals. The frequency of eyelid flicks, however, did not change. The changes of VOR gain were positively correlated with the lid excursion in RPC (r = 0.629, p = 0.002) and LPC (r = 0.549, p = 0.010). Our study indicates that eyelid position and pupil size should be considered when interpreting the results of video-HITs, especially for the vertical canals. Pupils should be shrunk in a very well-lit room, and artifacts should be prevented by taping or lifting the eyelids as required during video-HITs. [ABSTRACT FROM AUTHOR]

Purpose: To delineate the association between otolith function and changes in mean orthostatic blood pressure (BP) and heart rate (HR) in patients with postural orthostatic tachycardia syndrome (POTS). Methods: Forty-nine patients with POTS were prospectively recruited. We analyzed the results of ocular vestibular-evoked myogenic potentials (oVEMPs) and cervical vestibular-evoked myogenic potentials (cVEMPs), as well as head-up tilt table tests using a Finometer. The oVEMP and cVEMP responses were obtained using tapping stimuli and 110 dB tone-burst sounds, respectively. We measured maximal changes in 5-s averaged systolic BP (SBP), diastolic BP (DBP), and heart rate (HR) within 15 s and during 10 min after tilting. We compared the results with those of 20 age- and sex-matched healthy participants. Results: The n1–p1 amplitude of oVEMPs was larger in patients with POTS than in healthy participants (p = 0.001), whereas the n1 latency (p = 0.280) and interaural difference (p = 0.199) did not differ between the two. The n1–p1 amplitude was a positive predictor for POTS (odds ratio 1.07, 95% confidence interval 1.01–1.13, p = 0.025). Body weight (p = 0.007) and n1–p1 amplitude of oVEMP (p = 0.019) were positive predictors for ΔSBP15s in POTS, whereas aging was a negative predictor (p = 0.005). These findings were not observed in healthy participants. Conclusions: Augmented utricular inputs may be associated with a relative predominance of sympathetic over vagal control of BP and HR, especially for an early response during orthostasis in patients with POTS. Overt sympathoexcitation due to exaggerated utricular input and lack of readaptation may be associated with the pathomechanism of POTS. [ABSTRACT FROM AUTHOR]

Discusses a case in which clinically diagnosed tuberculous meningitis was associated with thoracolumbosacral polyradiculitis and myelopathy. Lumbar puncture procedure performed; Clinical neurophysiologic testing performed; Diagnosis.

The need for developing a simple and effective assessment tool for muscle mass has been increasing in a rapidly aging society. This study aimed to evaluate the feasibility of the surface electromyography (sEMG) parameters for estimating muscle mass. Overall, 212 healthy volunteers participated in this study. Maximal voluntary contraction (MVC) strength and root mean square (RMS) values of motor unit potentials from surface electrodes on each muscle (biceps brachii, triceps brachii, biceps femoris, rectus femoris) during isometric exercises of elbow flexion (EF), elbow extension (EE), knee flexion (KF), knee extension (KE) were acquired. New variables (MeanRMS, MaxRMS, and RatioRMS) were calculated from RMS values according to each exercise. Bioimpedance analysis (BIA) was performed to determine the segmental lean mass (SLM), segmental fat mass (SFM), and appendicular skeletal muscle mass (ASM). Muscle thicknesses were measured using ultrasonography (US). sEMG parameters showed positive correlations with MVC strength, SLM, ASM, and muscle thickness measured by US, but showed negative correlations with SFM. An equation was developed for ASM: ASM = −26.04 + 20.345 × Height + 0.178 × weight − 2.065 × (1, if female; 0, if male) + 0.327 × RatioRMS(KF) + 0.965 × MeanRMS(EE) (SEE = 1.167, adjusted R2 = 0.934). sEMG parameters in controlled conditions may represent overall muscle strength and muscle mass in healthy individuals. [ABSTRACT FROM AUTHOR]

Background: We aimed to evaluate the diagnostic accuracy of enzyme-linked immunosorbent assay (ELISA) for anti-muscle specific tyrosine kinase (MuSK) antibody (Ab) in a large cohort of anti-acetylcholine receptor (AChR) Ab-negative generalized myasthenia gravis (MG), and also to investigate clinical contexts for the diagnosis of MuSK MG. Methods: A retrospective study of 160 patients with a clinical suspicion of AChR Ab-negative generalized MG was performed. The serum samples were tested for anti-clustered AChR Ab by cell-based assay (CBA), anti-MuSK Ab by ELISA, CBA and/or radioimmunoprecipitation assay (RIPA). Clinical data were compared between anti-MuSK Ab-positive MG and double seronegative (AChR and MuSK) MG groups. Results: After excluding non-MG and clustered AChR Ab-positive patients, we identified 89 patients as a cohort of AChR Ab-negative generalized MG. Anti-MuSK Ab was positive by ELISA in 22 (24.7%) patients. While CBA identified five additional anti-MuSK Ab-positive patients, the results of ELISA were mostly consistent with CBA and RIPA with Cohen's kappa of 0.80 and 0.90, respectively (p < 0.001). The most frequent differential diagnosis was motor neuron disease particularly of bulbar onset which showed remarkably overlapping clinical and electrophysiological features with MuSK MG at presentation. Conclusion: While confirming the highest sensitivity of CBA for detecting anti-MuSK Ab, our results highlight the clinical pitfalls in making a diagnosis of MuSK MG and may support a diagnostic utility of MuSK-ELISA in clinical practice. [ABSTRACT FROM AUTHOR]

The measurement of skeletal muscle mass is essential for the diagnosis of sarcopenia. Muscle ultrasonography has emerged as a useful tool for evaluating sarcopenia because it can be used to assess muscle quality and quantity. This study investigated whether muscle ultrasonography is effective for estimating appendicular skeletal muscle mass (ASM) and screening for sarcopenia. This study prospectively enrolled 212 healthy volunteers aged 40–80 years. ASM was measured using the bioelectrical impedance analysis. Muscle thickness (MT) and echo-intensity (EI) were measured in four muscles (biceps brachii, BB; triceps brachii, TB; rectus femoris, RF; biceps femoris, BF) on the dominant hand. A hold-out cross-validation method was used to develop and validate the ASM prediction equation. In the model development group, the ASM prediction equations were deduced as follows: estimated ASM for men (kg) = 0.167 × weight (kg) + 0.228 × height (cm) + 0.143 × MT of BF (mm)– 0.822 × EI to MT ratio of BB– 28.187 (R2 = 0.830) and estimated ASM for women (kg) = 0.115 × weight + 0.215 × height (cm) + 0.139 × MT of RF–0.638 × EI to MT ratio of BB– 23.502 (R2 = 0.859). In the cross-validation group, the estimated ASM did not significantly differ from the measured ASM in both men (p = 0.775; intraclass correlation coefficient [ICC] = 0.948) and women (p = 0.516; ICC = 0.973). In addition, multiple logistic regression analysis revealed that the ratios of EI to MT in the BF and RF muscles in men and MT in the BB muscle in women could be valuable parameters for sarcopenia screening. Therefore, our study suggests that muscle ultrasound could be an effective tool for estimating ASM and screening sarcopenia. [ABSTRACT FROM AUTHOR]

Ophthalmoplegia is the diagnostic hallmark of anti-GQ1b antibody syndrome. This study aimed to define the patterns of acute comitant strabismus in patients with anti-GQ1b antibody syndromes. We retrospectively analyzed the ocular motor findings in 84 patients with anti-GQ1b antibody-associated ophthalmoplegia during the acute phases. Of the 84 patients, 11 (13%) showed acute comitant strabismus. Compared to those without, patients with acute comitant strabismus frequently showed abnormal ocular motor findings that included gaze-evoked (n = 8), spontaneous (n = 4) and positional nystagmus (n = 4), saccadic hypermetria (n = 3), head-shaking nystagmus (n = 2), pulse-step mismatch (n = 1), and impaired visual cancellation of the vestibulo-ocular reflex (n = 1, p < 0.001). On the contrary, iridoplegia (p = 0.029) and ptosis (p = 0.001) were more commonly observed in patients with paralytic (incomitant) strabismus than in those with acute comitant strabismus. Comitant strabismus can manifest during the acute phase of anti-GQ1b antibody syndromes in association with other central ocular motor abnormalities. These findings implicate that the cerebellum and/or brainstem can be the primary target of the anti-GQ1b antibodies. [ABSTRACT FROM AUTHOR]

Purpose: To delineate the association between otolithic dysfunction and orthostatic hypotension (OH). Methods: We retrospectively reviewed the medical records of 382 patients who presented with orthostatic dizziness at a tertiary dizziness center between July 2017 and December 2021. Patients were included for analyses when they had completed ocular (oVEMP) and/or cervical vestibular-evoked myogenic potentials (cVEMP), and head-up tilt table test with a Finometer (n = 155). We compared the results between the patients with OH (n = 38) and those with NOI (normal head-up tilt table test despite orthostatic intolerance, n = 117). Results: Thirty-eight patients with OH were further categorized as either classic (n = 30), delayed (n = 7), or initial (n = 1) types. Multivariable logistic regression showed that OH was associated with high baseline systolic BP (p = 0.046), presence of heart failure (p = 0.016), and unilateral oVEMP abnormalities (p = 0.016). n1 latency of oVEMP were negatively correlated with the maximal changes of systolic blood pressure (BP) in 15 s (Δ SBP15s, p = 0.013), 3 min (Δ SBP3min, p = 0.005) and 10 min (Δ SBP10min, p = 0.002). In contrast, the n1-p1 amplitude was positively correlated with Δ SBP15s (p = 0.029). Meanwhile, p13 latency of cVEMP was negatively correlated with Δ SBP10min (p = 0.018). Conclusions: Our study provides evidence of utricular dysfunction related to OH. [ABSTRACT FROM AUTHOR]

Three-dimensional (3-D) analysis of intraepidermal nerve fibers (IENFs) is conducted to advance assessment methods for peripheral neuropathies and pruritic skin disorders. The skin-clearing technique was proven to be a reliable method for 3-D imaging of IENFs. Nonetheless, it still requires further improvement in the imaging process. The aim of this study was to standardize the 3-D evaluation method of IENFs and to suggest promising 3-D biomarkers for clinical application. A total of nine healthy individuals were prospectively enrolled. The newly adopted suction blister method was combined with the tissue-clearing technique. The detailed structure of the IENFs was reconstructed and quantified using the neuron tracing software. The suction blister method showed improved linear integrity of IENFs compared with those obtained from the previously used salt-split skin test. The 3-D parameters most significantly related to natural aging were the convex hull two-dimensional perimeter and the total length (both p = 0.020). The meaningful correlations were followed by total volume (p = 0.025), ends (p = 0.026), convex hull 3-D surface, and complexity (both p = 0.030). Thus, the 3-D parameters could be utilized as possible biomarkers to identify ambiguous pathologies of peripheral neuropathies and pruritic skin disorders. [ABSTRACT FROM AUTHOR]

Three‐dimensional (3‐D) analysis of human epidermal melanocytes is required for deeper understanding of melanocytic disorders. The purpose of this study was to standardize 3‐D imaging and quantification for the evaluation of epidermal melanocytes. The epidermal specimen was obtained using the suction blister method from a patient with melanocytic nevus on the forearm skin. Cutaneous ACT‐PRESTO, the tissue‐clearing and labeling technique, was subsequently performed. With the 3‐D image analysis program, morphological reconstruction and quantification of selected perilesional and melanocytic nevus areas were possible. The region of melanocytic nevus showed higher numbers of total melanocytic dendrites and similar numbers of cell bodies compared with perilesional area. In addition, the mean area and volume of cell bodies increased in the melanocytic nevus area compared with the results in the perilesional area. The 3‐D evaluation method of human epidermal melanocytes can be applied to investigate novel pathologies related to hyper‐ or hypo‐pigmentary disorders. [ABSTRACT FROM AUTHOR]

In central as well as peripheral vestibular lesions, right-beating horizontal nystagmus is almost always associated with clockwise (top poles of the eyes beating to the right ear) torsional nystagmus when observed and vice versa (concordant nystagmus). This study aimed to determine the etiologies and mechanisms of horizontal and torsional nystagmus beating in the opposite directions (discordant nystagmus). We reviewed the medical records of 16 consecutive patients with discordant horizontal–torsional nystagmus who had been evaluated at the dizziness clinics of Seoul National University Bundang Hospital (n = 11, from March 2003 to March 2021) and Korea University Medical Center (n = 5, from March 2019 to March 2021). The underlying etiologies included inferior vestibular neuritis (n = 7), Meniere's disease (n = 4), internuclear ophthalmoplegia (n = 3), medullary hemorrhage (n = 1), and normal pressure hydrocephalus (n = 1). The torsional nystagmus decreased during the gaze in the same direction (for instance, during rightward gaze in clockwise nystagmus) and increased during the gaze in the opposite direction. Head-impulse tests (HITs) were positive for the ipsilesional posterior canal (PC) in all 11 patients with unilateral peripheral vestibulopathy and two of the three patients with unilateral central vestibulopathy. Discordant horizontal–torsional nystagmus may be observed in peripheral as well as central lesions. Given the findings of HITs and modulation of spontaneous nystagmus during lateral gazes, discordant horizontal–torsional nystagmus may be ascribed to selective damage of the excitatory or inhibitory pathway from the PC that innervates the ipsilateral superior oblique and contralateral inferior rectus muscles. [ABSTRACT FROM AUTHOR]

Step length is a critical hallmark of health status. However, few studies have investigated the modifiable factors that may affect step length. An exploratory, cross-sectional study was performed to evaluate the surface electromyography (sEMG) and body impedance analysis (BIA) parameters, combined with individual demographic data, to predict the individual step length using the GAITRite® system. Healthy participants aged 40–80 years were prospectively recruited, and three models were built to predict individual step length. The first model was the best-fit model (R2 = 0.244, p < 0.001); the root mean square (RMS) values at maximal knee flexion and height were included as significant variables. The second model used all candidate variables, except sEMG variables, and revealed that age, height, and body fat mass (BFM) were significant variables for predicting the average step length (R2 = 0.198, p < 0.001). The third model, which was used to predict step length without sEMG and BIA, showed that only age and height remained significant (R2 = 0.158, p < 0.001). This study revealed that the RMS value at maximal strength knee flexion, height, age, and BFM are important predictors for individual step length, and possibly suggesting that strengthening knee flexor function and reducing BFM may help improve step length. [ABSTRACT FROM AUTHOR]

Introduction. Diagnosis of carpal tunnel syndrome (CTS) is based on clinical findings and electrodiagnostic tests (EDT). However, EDT has high false negative rates, and the results often do not reflect the severity of symptoms. The aim of this study is to investigate the value of shear wave elastography (SWE) and high definition (HD) color in diagnosis and evaluation of symptom severity in CTS. Methods. The study prospectively enrolled 39 wrists from 23 patients with EDT-confirmed CTS, 15 wrists from 12 patients who have CTS symptoms without EDT abnormality and 28 wrists from 14 healthy volunteers. Cross-sectional area (CSA) and longitudinal diameter of median nerve were measured. SWE of median nerve at the wrist and midforearm were performed, and the ratio between them was calculated. SWE of abductor pollicis brevis (APB) and abductor digiti minimi (ADM) were also measured and the ratio was obtained. HD color was conducted in the longitudinal plane at carpal tunnel. Results. The CSA of the median nerve at the carpal inlet and longitudinal diameter were significantly larger for the EDT-confirmed CTS group compared to control group (13.21[11.28,17.23] vs 7.98[6.53,8.94]; p < 0.001, 2.77[2.41,3.08] vs 2.07[1.79,2.24]; p < 0.001). It also had significantly higher median nerve elasticity ratio (2.59[1.77,4.09] vs 1.61[1.32,1.94]; p = 0.007). HD color showed significantly higher grades in the former group (p = 0.004). In ROC analysis, a 1.97 cut-off value on SWE ratio revealed sensitivity, specificity, PPV, NPV of 74.4%, 78.6%, 82.9%, 68.8%, respectively (AUC: 0.796[0.687,0.905]). Inter-rater reliability was excellent for SWE measurements (ICC: 0.908(0.761,0.967)). There was no significant correlation of CSA, elasticity and HD color grade with clinical symptom scores. In comparison between the group of CTS patients without EDT abnormality and control group, the former had significantly larger CSA in carpal inlet and elasticity ratio (9.61[7.21,11.55] vs 7.98[6.53,8.94]; p = 0.011, 2.59[0.94,4.65] vs 1.61[1.32,1.94]; p = 0.009). Conclusion. SWE and HD color are helpful supportive tools in diagnosing CTS, especially in patients who have typical CTS symptoms but normal results in EDT. [ABSTRACT FROM AUTHOR]

• Falls were not associated with cVEMP or oVEMP abnormalities. • Falls were associated with higher MDS-UPDRS-III scores. • cVEMP and oVEMP amplitudes were not associated with disease duration or disease severity. [ABSTRACT FROM AUTHOR]

The integrity of the vestibulo-ocular reflex (VOR) remains to be delineated in patients with parkinsonism. We aimed to define the findings of the VOR using head-impulse tests (HITs) and their differential diagnostic value in patients with Parkinson's disease (PD) and multiple system atrophy (MSA). From December 2019 to January 2021, 30 patients with PD and 23 patients with MSA (17 with cerebellar-type MSA and 6 with parkinsonian-type MSA) had a video-oculographic recording of HITs at two university hospitals in South Korea. Reversed (p = 0.034) and perverted (p = 0.015) catch-up saccades were more frequently observed in MSA than in PD during HITs. The gain difference between the ACs and the PCs were larger in MSA than in PD (p = 0.031), and positively correlated with the disease duration in patients with MSA (Spearman's coefficient = 0.512, p = 0.012). Multivariate logistic regression analysis showed that reversed (p = 0.044) and perverted (p = 0.039) catch-up saccades were more frequently associated with MSA than with PD during HITs. In conclusion, HITs aid in differentiation of MSA from PD, and may serve as a surrogate marker for the clinical decline. [ABSTRACT FROM AUTHOR]

Background: A single cycle (two repeated treatments) with intrathecal autologous bone marrow-derived mesenchymal stem cells (BM-MSCs, 26-day interval) showed safety and provided therapeutic benefit lasting 6 months in patients with ALS but did not demonstrate long-term efficacy. This phase III clinical trial (ALSUMMIT) protocol was developed to evaluate the long-term efficacy and safety of the combined protocol of single-cycle intrathecal therapy and three additional booster injections of BM-MSC (Lenzumestrocel) treatment in patients with ALS.Methods: ALSUMMIT is a multicentre, randomized, double-blind, parallel-group, sham procedure-controlled, phase III trial for ALS. The 115 subjects will be randomized (1:2:2) into three groups: (1) study Group 1 (single-cycle, two repeated injections with 26-day interval), (2) study Group 2 (single-cycle + three additional booster injections at 4, 7, and 10 months), and (3) the control group. Participants who have an intermediate rate of disease progression will be included in this trial to reduce clinical heterogeneity. The primary endpoint will be evaluated by combined assessment of function and survival (CAFS), also known as joint rank scores (JRS), at 6 months (study Group 1 vs. control) and 12 months (study Group 2 vs. control) after the first Lenzumestrocel or placebo administration. Safety assessment will be performed throughout the study period. Additionally, after the 56-week main study, a long-term follow-up observational study will be conducted to evaluate the long-term efficacy and safety up to 36 months.Discussion: Lenzumestrocel is the orphan cell therapy product for ALS conditionally approved by the South Korea Ministry of Food and Drug Safety (MFDS). This ALSUMMIT protocol was developed for the adoption of enrichment enrolment, add-on design, and consideration of ethical issues for the placebo group.Trial Registration: ClinicalTrials.gov NCT04745299 . Registered on Feb 9, 2021. Clinical Research Information Service (CRIS) KCT0005954 . Registered on Mar 4, 2021. [ABSTRACT FROM AUTHOR]

• Shear wave elastography (SWE) and high-definition (HD) color provide useful supportive information for the evaluation of carpal tunnel syndrome (CTS). • SWE discriminated CTS severity, and detected CTS patients with normal nerve conduction study results. • 50.12-kPa for median nerve (MN) stiffness at the wrist and 1.91 for the MN stiffness ratio are cutoff values for CTS diagnosis. The diagnostic values of measuring median nerve (MN) stiffness and vascularity with shear wave elastography (SWE) and high-definition (HD) color were investigated in carpal tunnel syndrome (CTS). Seventy patients (123 wrists) with CTS and thirty-five healthy volunteers (70 wrists) were enrolled. Based on nerve conduction studies (NCS), the patients were subdivided into NCS-negative, mild-to-moderate, and severe CTS groups. MN and abductor pollicis brevis (APB) SWE and MN HD color were performed on a longitudinal plane. The mild-to-moderate and severe CTS groups showed increased MN stiffness at the wrist and MN stiffness ratio (wrist-to forearm) compared with the control (p < 0.001). The NCS-negative CTS group showed increased MN stiffness at the wrist (p = 0.022) and MN stiffness ratio (p = 0.032) compared with the control. The severe CTS group showed increased MN stiffness at the wrist compared with the mild-to-moderate CTS group (p = 0.034). The cutoff-values in diagnosing NCS-confirmed CTS were 50.12 kPa for MN stiffness at the wrist, 1.91 for MN stiffness ratio, and grade 1 for HD color. SWE and HD color are good supportive tools in diagnosing and assessing severity in CTS. SWE and HD color demonstrated that MN in CTS was associated with increased stiffness and hypervascularity. [ABSTRACT FROM AUTHOR]

Abstract: Backgrounds: It has been reported that patients with restless legs syndrome (RLS) may have cognitive deficit. The authors performed EEG and ERP analysis during daytime to identify electrophysiologic relations with cognitive dysfunction in unmedicated RLS patients. Methods: Seventeen drug naive RLS patients (53.7±9.6years) and 13 age-matched healthy controls participated in the present study. EEG was recorded during the waking–resting state and during a visual oddball task. RLS severities were determined using the International RLS Severity Scale. Stanford sleepiness scale (SSS) and bothersomeness visual analog scale (VAS) scores were determined immediately after ERP sessions. EEG power spectra and P300 amplitude and latency were compared for patients and controls. Clinical variables were correlated with P300 findings. Results: Waking–resting EEG showed that RLS patients had significantly higher beta activity in frontocentral regions than controls. SSS scores were not different in the two groups. But the bothersomeness VAS scores of RLS patients were significantly higher than those of controls. Furthermore, P300 latency was significantly longer in patients, and patients had significantly lower P300 amplitudes in frontal and central locations. In addition, P300 latency was found to be significantly correlated with bothersomeness during the ERP test, whereas P300 amplitude showed no such tendency. Conclusions: Our study supports the notion that RLS patients have an underlying cognitive dysfunction. Significant correlations found between P300 latency and bothersomeness, a lack of sleepiness during the ERP test, and increased beta activity in resting state EEGs suggest that a combination of inattention and cortical dysfunction underlie cognitive dysfunction in RLS. [Copyright &y& Elsevier]

Abstract: Objectives: The aims of this study were to obtain data on the frequency with which Korean patients with autoimmune myasthenia gravis (MG) present solely with ocular disturbances and progress to develop generalized disease and to identify the prognostic factors associated with secondary generalization. Methods: We conducted a retrospective multicenter survey in which a total of 376 adult patients who were newly diagnosed with MG from 2000 through 2005 were reviewed for analysis. Patients with ocular MG at the time of symptom presentation (n =202, 53.7%) were divided into two subgroups according to their prognosis: the patients whose disease remained ocular throughout the follow-ups were placed in the OMG-R group, and the patients who progressed to develop generalized disease were placed in the OMG-G group. Clinical characteristics and laboratory findings were compared between the two subgroups. Results: Secondary generalization developed in 47 (23.3%) of the 202 study subjects, mostly within the first 6 months after symptom presentation, while the disease remained ocular throughout the follow-up duration (median 11.8 months) in the remaining 155 patients (76.7%). AChR antibody, abnormal repetitive nerve stimulation tests (RNST) and thymoma were more frequently observed in the patients in the OMG-G group than in those in the OMG-R group (p <0.01 in all). In seropositive cases, the titers of AChR antibody were also significantly higher in the OMG-G group than in the OMG-R group (median, 3.8 nM vs. 6.4 nM; p <0.05). Cox proportional hazards regression analyses showed that early oral prednisolone treatment significantly reduced the risk of secondary generalization (HR, 0.24; 95% CI, 0.11–0.56), whereas abnormal AChR antibody (HR, 5.34; 95% CI, 1.60–17.8) and thymoma (HR, 2.32; 95% CI, 1.21–4.45) were predictive of the development of secondary generalization. Conclusions: Our findings suggest that several factors, including the AChR antibody, thymoma, early corticosteroid treatment, and possibly latent neuromuscular abnormality revealed by RNST, may have an impact on the risk of developing generalized disease in Korean patients presenting with ocular myasthenia. [Copyright &y& Elsevier]

Abstract: Objective: To investigate the pathophysiological relationship between RLS and small fiber neuropathy using the cutaneous silent period (CSP), which is a spinal reflex mediated by Aδ cutaneous afferents and is useful for the evaluation of small-diameter nerve function. Methods: The CSP was measured from the extensor digitorum brevis in 157 patients with RLS and 60 healthy controls. The CSP measurement was repeated in the RLS patients after dopamine agonist treatment for one month. The RLS rating scale for clinical severity was used to evaluate each patient before and after treatment. The measured CSP variables were compared between the patient group and the control group. In addition, the possible correlation between the CSP variables and the RLS rating scale score related to treatment was analyzed. Results: The mean CSP latencies did not differ between the RLS patients and the healthy controls; however, the mean CSP duration was significantly longer in the RLS patients than in the controls, and this prolonged CSP duration improved to the level of the control subjects after dopamine agonist treatment (p =0.003). The mean RLS rating scale score also significantly decreased after medication (p =0.000). However, the changes in the CSP variables did not correlate with the decrement in the RLS rating scale score. Conclusions: Although our results do not support the role of Aδ fiber dysfunction in RLS, the observed change in CSP duration may be useful as a clinical measure of the improvement with dopamine agonist treatment in patients with RLS. Significance: Further study is needed to elucidate the exact mechanism involved in the prolonged CSP duration in response to treatment. [Copyright &y& Elsevier]

The article presents a case study of a 49-year-old man presented with a 1-yr history of relapsing and remitting sensory deficits of the legs and his sensory loss and paresthesia started at his right toe and subsequently spread to the opposite side 6 mos later. Topics icnlude the symptoms initially resolved 3 mos before presentation, the physical examination revealed decreased sensation in all sensory modalities below the umbilicus, and the bilateral lower limbs showed slight proximal weakness.

Postural instability is a cardinal symptom of Parkinson's disease (PD), which suggests the vestibular system may be affected in PD. This study aimed to determine whether vestibular dysfunction is associated with the risk of falls in PD. We prospectively recruited patients with de-novo PD at a tertiary medical center between December 2019 and March 2023. During initial assessment, each patient was queried about falls within the preceding year. All patients underwent evaluation of video head-impulse tests (video-HITs), motion analysis, mini-mental state examination (MMSE), and Montreal Cognitive Assessment (MOCA). We determined whether head impulse gain of the vestibulo-ocular reflex (VOR) was associated with clinical severity of PD or risk of falls. Overall, 133 patients (mean age ± SD = 68 ± 10, 59 men) were recruited. The median Movement Disorder Society-Unified Parkinson's Disease Rating Scale motor part (MDS-UPDRS-III) was 23 (interquartile range = 16–31), and 81 patients (61 %) scored 2 or less on the Hoehn and Yahr scale. Fallers were older (p = 0.001), had longer disease duration (p = 0.001), slower gait velocity (p = 0.009), higher MDS-UPDRS-III (p < 0.001) and H&Y scale (p < 0.001), lower MMSE (p = 0.018) and MOCA scores (p = 0.001) than non-fallers. Multiple logistic regression showed that MDS-UPDRS-III had a positive association with falling (p = 0.004). Falling was not associated with VOR gain (p = 0.405). The VOR gain for each semicircular canal showed no correlation with the MDS-UPDRS-III or disease duration. The semicircular canal function, as determined by video-HITs, is relatively spared and has little effect on the risk of falls in patients with mild-to-moderate PD. • Falling is a major risk for immobilization and consequent poor quality of life in patients with Parkinson's disease (PD). • The canal function is relatively preserved and has little effect on the risk of falls in patients with mild-to-moderate PD. • The vestibulo-ocular reflex gain showed no correlation with the MDS-UPDRS-III or disease duration. [ABSTRACT FROM AUTHOR]

A clinical scale fully dedicated to evaluating ocular motor abnormalities is required for now. We investigated the utility of a recently developed Scale for Ocular motor Disorders in Ataxia (SODA) in patients with multiple system atrophy (MSA). We prospectively assessed SODA in consecutive patients with MSA between August 2021 and August 2023 at the Korea University Medical Center. The results of the clinical exam-based SODA were compared with those measured using video-oculography (VOG-guided SODA). We also compared the findings with other established clinical scales targeting patients with MSA, including the Unified Multiple System Atrophy Rating Scale (UMSARS) I-II, Movement Disorder Society-Unified Parkinson’s Disease Rating Scale motor part (UPDRS-III), Scale for Assessment of Rating of Ataxia (SARA), Composite Autonomic Symptom Score-31 (COMPASS-31), and Composite Autonomic Severity Score (CASS). Twenty patients were enrolled in our study (17 with cerebellar-type MSA and three with Parkinson-type MSA). Scores ranged from 1 to 14 (median [interquartile range (IQR)] = 8 [5−10]). Among the subscales, saccades had a median score of 2.5 (IQR = 1–3), followed by ocular pursuit (1 [0–1]), nystagmus (1 [0–2]), saccadic intrusions (1 [0–1]), vestibulo-ocular reflex (VOR) (0.5 [0–1]), ocular alignment (0 [0–1]), and VOR cancellation (1 [0–1]). The clinical-exam-based SODA (p = 0.020) and VOG-guided SODA (p = 0.034) positively correlated with disease duration. No correlation was found between clinical exam-based SODA and other scales. Skew deviation, gaze-evoked nystagmus, VOR cancellation, and smooth pursuit had the highest precision among the items. Ocular misalignment and spontaneous and positional nystagmus were frequently false positive and were poorly detected with clinical exam-based SODA. Six patients with repeated evaluation exhibited higher scores, along with deterioration documented on other clinical scales. The SODA can reliably predict neurodegeneration as an additional clinical surrogate in MSA. [ABSTRACT FROM AUTHOR]

Background: Neuromyelitis optica spectrum disorder (NMOSD) targets astrocytes and elevates the levels of astrocyte-injury markers during attacks. FAM19A5, involved in reactive gliosis, is secreted by reactive astrocytes following central nervous system (CNS) damage. Objective: To investigate the significance of serum FAM19A5 in patients with NMOSD. Methods: We collected clinical data and sera of 199 patients from 11 hospitals over 21 months. FAM19A5 levels were compared among three groups: NMOSD with positive anti-aquaporin-4 antibody (NMOSD-AQP4), other CNS demyelinating disease, and healthy controls. Results: The median serum FAM19A5 level was higher in the NMOSD-AQP4 (4.90 ng/mL (3.95, 5.79)) than in the other CNS demyelinating (2.35 ng/mL (1.83, 4.07), p < 0.001) or healthy control (1.02 ng/mL (0.92, 1.14), p < 0.001) groups. There were significant differences in the median serum FAM19A5 levels between the attack and remission periods (5.89 ng/mL (5.18, 6.98); 4.40 ng/mL (2.72, 5.13), p < 0.001) in the NMOSD-AQP4 group. Sampling during an attack (p < 0.001) and number of past attacks (p = 0.010) were independently associated with increased serum FAM19A5. Conclusion: Serum FAM19A5 was higher in patients with NMOSD-AQP4 and correlated with clinical characteristics. Thus, serum FAM19A5 may be a novel clinical biomarker for NMOSD-AQP4. [ABSTRACT FROM AUTHOR]

Abstract: There are few reports of brachial plexopathy following the onset of a herpes zoster skin rash. Moreover, the MRI findings of zoster-induced brachial plexopathy have rarely been described. In the present study, we describe two cases of zoster brachial plexopathy and their MRI findings. MRI of the brachial plexus demonstrated T2 hyperintensity and contrast enhancement in the part of the brachial plexus that was compatible with both the clinical symptoms and the electrophysiological findings. Especially, MR imaging reflected the functional impairments more accurately than electrophysiological studies in the acute phase, during which MRI showed more extensive inflammatory involvement of the brachial plexus. MRI findings in the present cases suggest that, in addition to electrophysiological studies, MRI of the brachial plexus could provide valuable information for evaluating the location and extent of lesions and for understanding the pathophysiological mechanisms of zoster brachial plexopathy. [Copyright &y& Elsevier]

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. However, no reliable biomarkers have been identified to represent the clinical status. This study aimed to investigate whether diffusion tensor imaging (DTI) findings are useful imaging biomarkers to indicate the clinical status of ALS patients. Ninety-six probable or definite ALS cases and 47 age- and sex-matched, normal controls were enrolled. Demographic and clinical data were collected at the time of DTI. DTI data were acquired using a 3-Tesla magnetic resonance imaging scanner and analysed by voxel-wise statistical analyses for fractional anisotropy, axial diffusivity, radial diffusivity, mean diffusivity, and mode of anisotropy. Compared with the healthy control group, the ALS group had significant differences in DTI scalars in the diffuse tracts of the brain, which was predominant in the corticospinal tract at the brainstem and cerebellar peduncle area. Furthermore, the DTI values correlated with the ALS functional rating scale-revised (ALSFRS-R) scores and the delta ALSFRS-R score representing the rate of disease progression. The subgroup analysis revealed a more severe and widespread brain degeneration was observed in rapidly progressive ALS. Therefore, our results suggest that DTI findings are useful as imaging biomarkers for evaluating the clinical severity and rate of disease progression in ALS. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the validity of the revised 2017 McDonald criteria for multiple sclerosis (MS) compared with the 2010 McDonald criteria to predict conversion to clinically definite multiple sclerosis (CDMS) in patients with clinically isolated syndrome (CIS). Methods: A total of 163 patients from seven referral hospitals in Korea, who experienced a first clinical event suggestive of MS between 2006 and 2017, were enrolled. Patients were stratified into two groups according to outcome at the last visit: CDMS converters who experienced a second clinical event and non-converters. Results: Of the 163 patients with a mean follow-up of 63 months, 60% converted to CDMS. The sensitivity, specificity, positive and negative predictive values and accuracy were, respectively, 88.8%, 43.1%, 70.2%, 71.8% and 70.6% for the 2017 McDonald criteria and 53.1%, 69.2%, 72.2%, 49.5% and 59.5% for the 2010 McDonald criteria. After exclusion of 82 patients who received disease-modifying agents before the second attack, the specificity of the 2017 and 2010 McDonald criteria increased to 85.0% and 95.0%, but sensitivity decreased to 83.6% and 47.5%, respectively. Conclusion: The 2017 McDonald criteria afforded higher sensitivity and accuracy but lower specificity compared with the 2010 McDonald criteria for prediction of conversion to CDMS in Korean CIS patients. [ABSTRACT FROM AUTHOR]

Several studies used automated segmentation of hippocampal subfield (ASHS) for detailed measurements of anatomic subregions of the hippocampus, especially in the field of neurodegenerative disorders. In this study, we investigated the hippocampal subfield volume of patients with early-stage nondementia PD compared with normal healthy subjects using the ASHS method. A total of 32 subjects were enrolled in this study (sixteen patients with drug naive nondementia PD and sixteen healthy controls). All subjects were scanned with a 1.5 tesla MRI. The volumes of the seven subfields were calculated separately, and then, the whole hippocampal volume was calculated by the summing of CA1, CA2-3, CA4-DG, subiculum, presubiculum, and fimbria, excluding the hippocampal fissure. There were significant diagnosis-by-hemisphere interactive effects on the total hippocampal volume (F = 5.197; p=0.031) and the subfield volume of CA2-3 (F = 7.586; p=0.010) and CA4-DG (F = 7.403; p=0.011). The volumes of CA2-3 (F = 19.911; p<0.001), CA4-DG (F = 20.273; p<0.001), and total hippocampus (F = 10.573; p=0.005) in the left hemisphere were reduced compared to the right hemisphere. We suggest that the hippocampal volume asymmetry, especially in CA4-DG and CA2-3, could be observed in drug-naïve PD patients even in the early stage of the disease. [ABSTRACT FROM AUTHOR]

Highlights • Experts routinely use ultrasound to evaluate both common and rare disorders of nerve and muscle. • Accumulated evidence now puts diagnostic neuromuscular ultrasound on par with EMG and NCS. • Medical centers must acquire ultrasound equipment to keep electrodiagnostic laboratories current. Abstract Over the last two decades, dozens of applications have emerged for ultrasonography in neuromuscular disorders. We wanted to measure its impact on practice in laboratories where the technique is in frequent use. After identifying experts in neuromuscular ultrasound and electrodiagnosis, we assessed their use of ultrasonography for different indications and their expectations for its future evolution. We then identified the earliest papers to provide convincing evidence of the utility of ultrasound for particular indications and analyzed the relationship of their date of publication with expert usage. We found that experts use ultrasonography often for inflammatory, hereditary, traumatic, compressive and neoplastic neuropathies, and somewhat less often for neuronopathies and myopathies. Usage significantly correlated with the timing of key publications in the field. We review these findings and the extensive evidence supporting the value of neuromuscular ultrasound. Advancement of the field of clinical neurophysiology depends on widespread translation of these findings. [ABSTRACT FROM AUTHOR]

Objectives: We compared validity of 2010 McDonald and newly proposed 2016 Magnetic Resonance Imaging in Multiple Sclerosis (MAGNIMS) criteria for dissemination in space (DIS) in predicting the conversion to clinically definite multiple sclerosis (CDMS) in patients with clinically isolated syndrome (CIS). Methods: Between 2006 and 2016, we enrolled 170 patients who had a first clinical event suggestive of multiple sclerosis (MS) from seven referral hospitals in Korea. Patients were classified into two groups based on the main outcome at the last follow-up: CDMS converters, who experienced a second attack, and non-converters. Results: Of 170 patients with mean follow-up duration of 54 months, 51% converted to CDMS. The sensitivity, specificity, accuracy, and positive and negative predictive values of 2010 McDonald criteria were 70.9%, 63.1%, 67.1%, 66.3%, and 67.9%, and those for 2016 MAGNIMS criteria were 88.4%, 46.4%, 67.7%, 62.8%, and 79.6%, respectively. When we excluded 80 patients who underwent disease-modifying therapy before the second clinical event, the specificity increased to 92.3% and 84.6%, but the sensitivity decreased to 58.8% and 82.4% for 2010 McDonald and 2016 MAGNIMS criteria, respectively. Conclusion: 2016 MAGNIMS magnetic resonance imaging (MRI) criteria for DIS showed higher sensitivity but lower specificity than 2010 McDonald criteria in predicting conversion to CDMS in CIS patients. [ABSTRACT FROM AUTHOR]

Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA). We also included 8 patients with ocular MG, 3 with Lambert-Eaton myasthenic syndrome, 5 with motor neuron disease, and 9 with other diagnoses as comparators for the serological testing. Antibodies were identified in 25/62 (40.3%) patients: 7 had antibodies to clustered AChR, 17 to MuSK, and 2 to LRP4. Three patients were double seropositive: 1 for MuSK and LRP4, and 2 for MuSK and clustered AChR. The patients with MuSK antibodies were mostly female (88.2%) and characterized by predominantly bulbar involvement (70%) and frequent myasthenic crises (58.3%). The patients with antibodies to clustered AChR, including 2 with ocular MG, tended to have a mild phenotype and good prognosis. [ABSTRACT FROM AUTHOR]

The present study concerns the three-dimensional CFD analysis of hydrogen explosions in APR1400 containment. Initial conditions for combustion simulations are obtained by a cost-effective coupling with a lumped parameter code for preceding mixture distribution analysis. Three severe accident scenarios are examined: Small-Break Loss-Of-Coolant Accident (SB-LOCA) and Station Black-Out (SBO) with activated as well as deactivated three-way valve. Only in the last case, thermal ignition of the hydrogen-air-steam mixture could be triggered. Flame propagation in the containment and particularly in the In-containment Refueling Water Storage Tank (IRWST) is investigated in detail. Additional generic scenarios demonstrate the methods ability to reproduce strong Flame Acceleration (FA) and even the hazardous Deflagration-to-Detonation Transition (DDT). [ABSTRACT FROM AUTHOR]

Introduction Several clinical studies using tacrolimus revealed reasonable therapeutic mechanisms and efficacy in patients with myasthenia gravis (MG). However, long-period studies in a large number of patients with MG are limited; therefore, the aim of this study was to investigate the therapeutic efficacies and safety of tacrolimus in patients with MG during a 12-month follow-up period. Methods Tacrolimus was administered to 150 patients with MG who were recruited based on the inclusion criteria. Fifteen medical centers in Korea participated in this study. The efficacy of tacrolimus was assessed using MG composite scales (MGCS) and the prednisolone-sparing effect. And the adverse drug reactions (ADRs) of tacrolimus were monitored in each patient from the beginning of tacrolimus treatment to the end of the follow-up period. Results After starting tacrolimus, the 32 patients were affected by ADRs, and consequentially 134 patients of the enrolled patients were followed up for 12 months. They showed that the mean prednisolone dosage significantly decreased (6.1 ± 7.6 mg/day), compared to that in the baseline (11.3 ± 9.5 mg/day), and MGCS significantly improved after 12 months of tacrolimus treatment, compared to that at the baseline. Conclusions Our study showed that tacrolimus would be an effective immunosuppressant as an initial therapeutic agent in patients with MG; in addition, it showed tolerable safety profiles during the 12-month follow-up evaluation. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 39-year old female who was admitted to the neurology department due to recurrent genital ulcers, oral ulcers, and pain on her left sole. Neurologic examination reveals weakness in left toe's flexion, extension, and abduction while ankle magnetic resonance imaging (MRI) shows diffuse swelling and left tibial nerve enhancement. It states that the case suggests Behcet's disease (BD) characterized by recurrent tibial neuropathy.

The pathogenesis of fibromyalgia (FM) has not been clearly elucidated, but central sensitization, which plays an important role in the development of neuropathic pain, is considered to be the main mechanism. The cutaneous silent period (CSP), which is a spinal reflex mediated by A-delta cutaneous afferents, is useful for the evaluation of sensorimotor integration at the spinal and supraspinal levels. To understand the pathophysiology of FM, we compared CSP patterns between patients with FM and normal healthy subjects. Twenty-four patients with FM diagnosed in accordance with the 1990 American College of Rheumatology classification system and 24 age- and sex-matched healthy volunteers were recruited. The CSP was measured from the abductor pollicis brevis muscle. Demographic data, number of tender points, and visual analog scale and FM impact questionnaire scores were collected. The measured CSP and clinical parameters of the patient and control groups were compared. In addition, possible correlations between the CSP parameters and the other clinical characteristics were analyzed. Mean CSP latencies did not differ between patients (55.50 ± 10.97 ms) and healthy controls (60.23 ± 11.87 ms; p = 0.158), although the mean CSP duration was significantly longer in patients (73.75 ± 15.67 ms) than in controls (63.50 ± 14.05 ms; p = 0.021). CSP variables did not correlate with any clinical variables. The significantly longer CSP duration in FM patients suggests central dysregulation at the spinal and supraspinal levels, rather than peripheral small fiber dysfunction. [ABSTRACT FROM AUTHOR]

Although the clinical presentation of myocarditis is very diverse, ranging from mild dyspnea to hemodynamic collapse, myocarditis accompanied with extracardiac myositis is extremely rare. We report a single case of fulminant myocarditis associated with orbital myositis and diaphragmatic paralysis in a 40-year-old man, which was successfully managed by immunosuppressive therapy with steroid. [ABSTRACT FROM AUTHOR]

ABSTRACT Introduction: Cross-sectional area (CSA) reference values of lower extremity nerves using ultrasonography have only been reported in a few studies and have been limited to white populations. Methods: For this study, 94 healthy Korean volunteers were recruited for measurement of the CSA at 7 sites of lower extremity nerves. The side-to-side difference in CSA was calculated for each nerve, and reference ranges were derived. External validity evaluation for the reference values was performed with 10 newly recruited volunteers at a different institution. Results: Nerve CSA was correlated significantly with body mass index, weight, and height; however, the absolute value of the side-to-side difference had no significant correlation with demographic factors. The external validity was adequate for all sites, ranging from 80% to 100%. Conclusions: The lower extremity nerve CSA values obtained in this study may provide normal reference values for the Asian population. Muscle Nerve 50: 564-570, 2014 [ABSTRACT FROM AUTHOR]