Dexmedetomidine for reducing succinylcholine-induced myalgia in patients undergoing electroconvulsive therapy: A randomised controlled trial.

Background and Aim: Electroconvulsive therapy (ECT) is an effective intervention for psychiatric patients. Succinylcholine is considered the drug of choice for muscle relaxation for ECT. Significant adverse effects of succinylcholine include fasciculation and myalgia. Dexmedetomidine is a highly selective α-2 adrenergic agonist. This study aims to determine the efficacy of a low dose of dexmedetomidine in reducing succinylcholine-induced myalgia in patients receiving ECT. Methods: This randomised controlled trial was conducted on 100 patients, aged 18-65 years, undergoing ECT, who were randomly allocated into two groups with an allocation ratio of 1:1. Group D received intravenous (IV) dexmedetomidine 0.25 µg/kg, and Group C received IV normal saline (0.9%). Patients' self-reported myalgia scores were measured after 60 min of the procedure. Fasciculations were noted after IV succinylcholine administration. Heart rate (HR) and mean blood pressure (MBP) were measured at baseline, after infusion (5 min) and after ECT (0, 2.5, 5, 10, 15, 30 min). Continuous data were analysed using a Student's t-test for two-group comparisons, a mixed model analysis of variance for group comparisons and various time point analyses. Categorical data were analysed using the Chi-square/Fisher's exact test. Results: There were no differences between the groups regarding demographics. Myalgia and fasciculations were less in Group D than in Group C (P < 0.001). MBP and HR changes were comparable (P > 0.05). Conclusion: A low dose of dexmedetomidine (0.25 µg/kg) effectively reduces myalgia and fasciculations due to succinylcholine in patients undergoing electroconvulsive therapy.

Pregabalina preoperatoria previene las fasciculaciones y mialgias inducidas por succinilcolina: metaanálisis de ensayos aleatorizados / Preoperative pregabalin prevents succinylcholine-induced fasciculations and myalgias: A meta-analysis of randomized trials

La succinilcolina es el bloqueador neuromuscular de referencia para la inducción de secuencia rápida. Sin embargo, su uso se asocia a fasciculaciones y mialgias. Se realizó una revisión sistemática y un metaanálisis. Se incluyeron ensayos clínicos controlados aleatorizados comparando gabapentinoides frente a placebo, para la prevención de fasciculaciones y mialgias inducidas por succinilcolina. Se incluyeron seis estudios clínicos aleatorizados. El número total de pacientes fue de 481, de los cuales 241 se incluyeron en el grupo de intervención y 240 en el grupo de placebo. Los gabapentinoides redujeron la incidencia de mialgia inducida por succinilcolina (RR=0,69; IC95%: 0,56-0,84; p<0,001), que siguió siendo estadísticamente significativa para pregabalina (RR=0,71; IC95%: 0,54-0,93; p=0,013) y gabapentina (RR=0,61; IC95%: 0,45-0,82; p=0,001) por separado. No hubo diferencia entre los grupos en cuanto a fasciculaciones (RR=0,92; IC95%: 0,82-1,03; p=0,148). El uso preoperatorio de gabapentinoides se asocia a una menor incidencia de mialgias inducidas por succinilcolina dentro de las primeras 24horas posteriores al procedimiento. (AU)

Succinylcholine is the gold standard neuromuscular blocker for rapid sequence induction, however, its use is associated with fasciculations and myalgias. A systematic review and meta-analysis including randomized controlled clinical trials was performed comparing gabapentinoids versus placebo for the prevention of fasciculations and succinylcholine-induced myalgias. Six randomized clinical studies were included. The total number of patients was 481, of which 241 were in the intervention group and 240 in the placebo group. Gabapentinoids reduced the incidence of succinylcholine-induced myalgia (RR=.69; 95%CI: .56-.84; P<.001), which remained statistically significant for pregabalin (RR=.71; 95%CI: .54-.93; P=.013) and gabapentin (RR=.61; 95%CI: .45-.82; P=.001) separately. There was no difference between the groups in fasciculations (RR=.92; 95%CI: .82-1.03; P=.148). Preoperative use of gabapentinoids is associated with lower incidence of succinylcholine-induced myalgias within the first 24hours after the procedure. (AU)

Whole-body fasciculation detection in amyotrophic lateral sclerosis using motor unit MRI.

OBJECTIVE: Compare fasciculation rates between amyotrophic lateral sclerosis (ALS) patients and healthy controls in body regions relevant for diagnosing ALS using motor unit MRI (MUMRI) at baseline and 6 months follow-up, and relate this to single-channel surface EMG (SEMG). METHODS: Tongue, biceps brachii, paraspinals and lower legs were assessed with MUMRI and biceps brachii and soleus with SEMG in 10 healthy controls and 10 patients (9 typical ALS, 1 primary lateral sclerosis [PLS]). RESULTS: MUMRI-detected fasciculation rates in typical ALS patients were higher compared to healthy controls for biceps brachii (2.40 ± 1.90 cm-3min-1vs. 0.04 ± 0.10 cm-3min-1, p = 0.004), paraspinals (1.14 ± 1.61 cm-3min-1vs. 0.02 ± 0.02 cm-3min-1, p = 0.016) and lower legs (1.42 ± 1.27 cm-3min-1vs. 0.13 ± 0.10 cm-3min-1, p = 0.004), but not tongue (1.41 ± 1.94 cm-3min-1vs. 0.18 ± 0.18 cm-3min-1, p = 0.556). The PLS patient showed no fasciculation. At baseline, 6/9 ALS patients had increased fasciculation rates compared to healthy controls in at least 2 body regions. At follow-up every patient had increased fasciculation rates in at least 2 body regions. The MUMRI-detected fasciculation rate correlated with SEMG-detected fasciculation rates (τ = 0.475, p = 0.006). CONCLUSION: MUMRI can non-invasively image fasciculation in multiple body regions and appears sensitive to disease progression in individual patients. SIGNIFICANCE: MUMRI has potential as diagnostic tool for ALS.

Neuromuscular ultrasound changes in unilateral symptomatic subacute lumbosacral radiculopathy: A prospective simple blinded cohort study.

INTRODUCTION/AIMS: Lumbosacral radiculopathy (LR) is a common disorder. Neuromuscular ultrasound (NMU) is a rapidly evolving technique for the investigation of peripheral nerve and muscle disorders, but studies using NMU in LR are lacking. The aim of the present study was to investigate ultrasonographic neuromuscular changes distant from root compression in patients with subacute to chronic compressive LR with motor impairment. METHODS: Patients with unilateral subacute to chronic L4, L5, or S1 radiculopathy with motor impairment and confirmed by magnetic resonance imaging were included. The sciatic and femoral nerve cross-sectional areas (CSA), the CSA of lower limb muscles, and muscle fasciculation detection rate were assessed using a pre-specified neuromuscular ultrasound evaluation with blinded side-to-side comparison. RESULTS: Of the 18 included patients, 66% were male and the mean age was 51 years. Overall, 16.7% had L4, 55.5% L5, and 27.8% S1 radiculopathy, mostly due to disc herniation (83%). Sciatic nerve CSA of the symptomatic side was increased (61.4 mm2 vs. 51.3 mm2; p = .001), and the fasciculation detection rate was higher in the affected muscles (delta = 13%, p = .007) compared to unaffected ones. Muscle CSA in affected and nonaffected muscles was decreased on the symptomatic side. DISCUSSION: NMU evaluation in patients with symptomatic subacute to chronic LR revealed sciatic nerve enlargement distant from nerve root compression and higher fasciculation rates. These structural findings on NMU might be due to an axonal repair mechanism and an inflammatory response with endoneurial edema induced by ongoing nerve damage and potentially reflect progressive axonal loss.

Split Phenomenon of Fasciculation between Antagonistic Muscles in Amyotrophic Lateral Sclerosis: An Ultrasound Study.

OBJECTIVE: Paresis of muscle groups in patients with amyotrophic lateral sclerosis (ALS) tends to present split phenomena. We explored the split phenomenon of fasciculation in multiple antagonistic muscle groups in ALS patients. METHODS: One hundred and forty ALS patients and 66 non-ALS patients were included from a single ALS center. Muscle ultrasonography (MUS) was performed to detect fasciculation in elbow flexor-extensor, wrist flexor-extensor, knee flexor-extensor, and ankle flexor-extensor. Split phenomena of fasciculation between different antagonistic muscle groups were summarized, and the possible influence factors were analyzed through stratified analysis. RESULTS: The frequency of split phenomenon of fasciculation intensity was significantly higher than those of muscle strength (26.1% vs. 7.1% for elbow flexor-extensor, 38.3% vs. 5.7% for wrist flexor-extensor, 37.9% vs. 3.0% for knee extensor-flexor, and 33.6% vs. 14.4% for ankle flexor-extensor) (P < 0.01). For muscles with 0-1 level of muscle strength (the Medical Research Council, MRC, score), significance difference in mean fasciculation intensity was observed only in ankle flexor-extensor. For muscles with 2-5 level of muscle strength, significant dissociation of fasciculation grade was common, especially among patients with slow rapid progression rate and both upper and lower motor neuron (UMN and LMN) involvement. As for non-ALS patients, no significant difference was observed in fasciculation intensity between antagonistic muscles. CONCLUSION: Split phenomenon of fasciculation between antagonistic muscles was common and relatively specific in ALS patients. Muscle strength, progression rate, and UMN involvement were influence factors of the split phenomenon of fasciculation intensity.

Methods for motion artifact reduction in online brain-computer interface experiments: a systematic review.

Brain-computer interfaces (BCIs) have emerged as a promising technology for enhancing communication between the human brain and external devices. Electroencephalography (EEG) is particularly promising in this regard because it has high temporal resolution and can be easily worn on the head in everyday life. However, motion artifacts caused by muscle activity, fasciculation, cable swings, or magnetic induction pose significant challenges in real-world BCI applications. In this paper, we present a systematic review of methods for motion artifact reduction in online BCI experiments. Using the PRISMA filter method, we conducted a comprehensive literature search on PubMed, focusing on open access publications from 1966 to 2022. We evaluated 2,333 publications based on predefined filtering rules to identify existing methods and pipelines for motion artifact reduction in EEG data. We present a lookup table of all papers that passed the defined filters, all used methods, and pipelines and compare their overall performance and suitability for online BCI experiments. We summarize suitable methods, algorithms, and concepts for motion artifact reduction in online BCI applications, highlight potential research gaps, and discuss existing community consensus. This review aims to provide a comprehensive overview of the current state of the field and guide researchers in selecting appropriate methods for motion artifact reduction in online BCI experiments.

Differentiation Between Amyotrophic Lateral Sclerosis and Mimics Using Quantitative Analysis of Fsciculation with Muscle Ultrasound.

Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis (ALS). Methods We prospectively recruited patients who had ALS and neuropathy-radiculopathy attending Peking Union Medical College Hospital from 2017 to 2020. Healthy adults from a community were recruited as healthy controls. Muscle strength was assessed using the Medical Research Council (MRC) scale. At the first visit to the hospital, patients were assessed for maximal grade of fasciculations, total fasciculation score, and fasciculation grade in 16 muscle groups of bilateral upper and lower limbs using ultrasonography. The sensitivity and specificity of maximal grade of fasciculations, total fasciculation score, and fasciculation grade for the diagnosis of ALS were assessed by receiver operating characteristic analyses. Results The percentage of limb muscles with a maximal fasciculation grade higher than grade 2 in ALS patients and neuropathy-radiculopathy patients was 84.9% and 9.8%, respectively (χ2 = 172.436, P < 0.01). Of the 16 limb muscles detected, the total fasciculation score [median (interquartile range)] was 29 (15, 41) in ALS patients and 3 (0, 8) in neuropathy-radiculopathy patients (Z = 9.642, P < 0.001). Remarkable fasciculations were seen in ALS patients whose muscles with a MRC score ranging from 2 to 4, followed by patients with MRC score 5, and then in those with MRC score 0 and 1. The sensitivity and specificity of total fasciculation score for diagnosis of ALS were 80.6% and 93.4%, respectively (cut-off value 14). In patients with ALS, for muscles with MRC score 4 and 5, the percentage of muscles with fasciculation grades ≥ 3 was 42.3% and 24.1% respectively, while in neuropathy-radiculopathy patients, the percentage for muscles with MRC score 4 and 5 was only 1.7% and 0, respectively. Conclusion A combined analysis of fasciculation intensity and MRC score of the limb muscles may be helpful for differential diagnosis of ALS.

Preoperative pregabalin prevents succinylcholine-induced fasciculation and myalgia: A meta-analysis of randomized trials.

Succinylcholine is the gold standard neuromuscular blocker for rapid sequence induction; however, its use is associated with fasciculation and myalgia. We performed a systematic review and meta-analysis of randomized controlled clinical trials comparing gabapentinoids versus placebo for the prevention of fasciculations and succinylcholine-induced myalgias. Six randomized clinical studies were included with a total of 481 patients - 241 in the intervention group and 240 in the placebo group. Gabapentinoids reduced the incidence of succinylcholine-induced myalgia (RR = 0.69, 95% CI 0.56-0.84, P < .001), which remained statistically significant for pregabalin (RR = 0.71, 95% CI 0.54-0.93, P = .013) and gabapentin (RR = 0.61, 95% CI 0.45-0.82, P = .001) separately. There was no difference in fasciculations between the groups (RR = 0.92, 95% CI 0.82-1.03, P = .148). Preoperative use of gabapentinoids is associated with lower incidence of succinylcholine-induced myalgias within the first 24 h of surgery.

Clinical, neurophysiological and serological clues for the diagnosis of neuromyotonia and distinction from cramp-fasciculation syndrome.

Neuromyotonia and cramp-fasciculation syndrome diagnosis currently relies on neurophysiological examination. In this study we investigated the clinical features and neural antibody profile of patients with neuromyotonia and cramp-fasciculation syndrome to assess the diagnostic value of serological testing. Available sera from adult patients with electromyography-defined neuromyotonia and cramp-fasciculation syndrome were tested for neural antibodies by indirect immunofluorescence on mouse brain sections and live cell-based assays. Forty patients were included, 14 with neuromyotonia and 26 with cramp-fasciculation syndrome. Neural antibodies were detected in 10/10 neuromyotonia sera, most commonly against contactin-associated protein 2 (7/10, 70%), and in 1/20 (5%) cramp-fasciculation syndrome sera. Clinical myokymia, hyperhidrosis, and paresthesia or neuropathic pain were more common in neuromyotonia and mostly associated with contactin-associated protein 2 antibodies. Central nervous system involvement was present in 4/14 (29%) neuromyotonia patients. A tumor was detected in 13/14 (93%) neuromyotonia patients (thymoma, 13), and in 4/26 (15%) with cramp-fasciculation syndrome (thymoma, 1; other neoplasms, 3). Twenty-one/27 (78%) patients achieved a significant improvement or complete remission. Our findings highlight clinical, neurophysiological and serological clues that can be useful in the diagnosis of neuromyotonia and cramp-fasciculation syndrome. Antibody testing is valuable for neuromyotonia diagnosis, while its usefulness in cramp-fasciculation syndrome confirmation is limited.

A Case Series of Patients With MYBPC1 Gene Variants Featuring Undulating Tongue Movements as Myogenic Tremor.

Myosin-binding protein C1 (MYBPC1) encodes myosin-binding protein C, slow type (sMyBP-C), an accessory protein that regulates actomyosin cross-linking, stabilizes thick filaments, and modulates contractility in muscle sarcomeres and has recently been linked to myopathy with tremor. The clinical features of MYBPC1 mutations manifesting in early childhood bear some similarities to those of spinal muscular atrophy (SMA), such as hypotonia, involuntary movement of the tongue and limbs, and delayed motor development. The development of novel therapies for SMA has necessitated the importance of differentiating SMA from other diseases in the early infancy period. We report the characteristic tongue movements of MYBPC1 mutations, along with other clinical findings, such as positive deep tendon reflexes and normal peripheral nerve conduction velocity testing, which could help in considering other diseases as differential diagnoses.

The Effects of Prophylactic Intravenous Lignocaine vs Vecuronium on Succinylcholine-Induced Fasciculation and Postoperative Myalgia in Patients Undergoing Elective Surgery at Debre Markos Comprehensive Specialized Hospital, Ethiopia, 2022: Prospective Cohort Study.

Background: The incidence and severity of succinylcholine-induced fasciculation and postoperative myalgia have been shown to decrease when vecuronium bromide or preservative-free 2% plain lignocaine hydrochloride is administered before induction. The aim of this study is to examine the effectiveness of defasciculation dosages of vecuronium bromide and 2% preservative-free plain lignocaine hydrochloride in decreasing succinylcholine-induced fasciculation and postoperative myalgia in patients undergoing elective surgery. Methods: A total of 110 participants were included in a prospective observational cohort study that was located in an institution. Patients were randomly assigned to (Group L) and (Group V) based on the prophylactic measures they received from the responsible anesthetist utilizing preservative-free 2% plain lignocaine and defasciculation dose of vecuronium bromide, respectively. We recorded, socio-demographic variables, fasciculation, postoperative myalgia, total number of analgesics administered following surgery in 48hrs, and kind of procedure. The descriptive data were compiled using descriptive statistics. Categorical and continuous data were evaluated, respectively, using chi-square statistics and the independent sample t-test. To compare the prevalence of fasciculation and myalgia across the various groups, the Fischer exact test was performed. A 0.05 p-value was deemed statistically significant. Results: This study found that the incidence of fasciculation in the groups receiving the defasciculation doses of vecuronium bromide and preservative-free 2% plain lignocaine hydrochloride was 14.6% and 20% (p-value 0.007), respectively. The rate of mild-to-moderate postoperative myalgia in the vecuronium bromide group was 23.7%, 30.9%, and 16.4% in the first, 24th, and 48th hours, respectively (p-value 0.001), as opposed to 0%, 37.3%, and 9.1%, respectively (p-value 0.008) in the group receiving preservative-free 2% plain lignocaine hydrochloride. Conclusion: Pretreatment with 2% plain lignocaine that is preservative-free is more efficient than vecuronium bromide at reducing the frequency and intensity of postoperative succinylcholine-induced myalgia, whereas defasciculation dose of vecuronium was more effective prevention of succinylcholine-induced fasciculation.

Semaphorin-6D and Plexin-A1 Act in a Non-Cell-Autonomous Manner to Position and Target Retinal Ganglion Cell Axons.

Semaphorins and Plexins form ligand/receptor pairs that are crucial for a wide range of developmental processes from cell proliferation to axon guidance. The ability of semaphorins to act both as signaling receptors and ligands yields a multitude of responses. Here, we describe a novel role for Semaphorin-6D (Sema6D) and Plexin-A1 in the positioning and targeting of retinogeniculate axons. In Plexin-A1 or Sema6D mutant mice of either sex, the optic tract courses through, rather than along, the border of the dorsal lateral geniculate nucleus (dLGN), and some retinal axons ectopically arborize adjacent and lateral to the optic tract rather than defasciculating and entering the target region. We find that Sema6D and Plexin-A1 act together in a dose-dependent manner, as the number of the ectopic retinal projections is altered in proportion to the level of Sema6D or Plexin-A1 expression. Moreover, using retinal in utero electroporation of Sema6D or Plexin-A1 shRNA, we show that Sema6D and Plexin-A1 are both required in retinal ganglion cells for axon positioning and targeting. Strikingly, nonelectroporated retinal ganglion cell axons also mistarget in the tract region, indicating that Sema6D and Plexin-A1 can act non-cell-autonomously, potentially through axon-axon interactions. These data provide novel evidence for a dose-dependent and non-cell-autonomous role for Sema6D and Plexin-A1 in retinal axon organization in the optic tract and dLGN.SIGNIFICANCE STATEMENT Before innervating their central brain targets, retinal ganglion cell axons fasciculate in the optic tract and then branch and arborize in their target areas. Upon deletion of the guidance molecules Plexin-A1 or Semaphorin-6D, the optic tract becomes disorganized near and extends within the dorsal lateral geniculate nucleus. In addition, some retinal axons form ectopic aggregates within the defasciculated tract. Sema6D and Plexin-A1 act together as a receptor-ligand pair in a dose-dependent manner, and non-cell-autonomously, to produce this developmental aberration. Such a phenotype highlights an underappreciated role for axon guidance molecules in tract cohesion and appropriate defasciculation near, and arborization within, targets.

Axon Fasciculation, Mediated by Transmembrane Semaphorins, Is Critical for the Establishment of Segmental Specificity of Corticospinal Circuits.

Axon fasciculation is thought to be a critical step in neural circuit formation and function. Recent studies have revealed various molecular mechanisms that underlie axon fasciculation; however, the impacts of axon fasciculation, and its corollary, defasciculation, on neural circuit wiring remain unclear. Corticospinal (CS) neurons in the sensorimotor cortex project axons to the spinal cord to control skilled movements. In rodents, the axons remain tightly fasciculated in the brain and traverse the dorsal funiculus of the spinal cord. Here we show that plexinA1 (PlexA1) and plexinA3 (PlexA3) receptors are expressed by CS neurons, whereas their ligands, semaphorin-5A (Sema5A) and semaphorin-5B (Sema5B) are expressed in the medulla at the decussation site of CS axons to inhibit premature defasciculation of these axons. In the absence of Sema5A/5B-PlexA1/A3 signaling, some CS axons are prematurely defasciculated in the medulla of the brainstem, and those defasciculated CS axons aberrantly transverse in the spinal gray matter instead of the spinal dorsal funiculus. In the absence of Sema5A/Sema5B-PlexA1/A3 signaling, CS axons, which would normally innervate the lumbar spinal cord, are unbundled in the spinal gray matter, and prematurely innervate the cervical gray matter with reduced innervation of the lumbar gray matter. In both Sema5A/5B and PlexA1/A3 mutant mice (both sexes), stimulation of the hindlimb motor cortex aberrantly evokes robust forelimb muscle activation. Finally, Sema5A/5B and PlexA1/A3 mutant mice show deficits in skilled movements. These results suggest that proper fasciculation of CS axons is required for appropriate neural circuit wiring and ultimately affect the ability to perform skilled movements.SIGNIFICANCE STATEMENT Axon fasciculation is believed to be essential for neural circuit formation and function. However, whether and how defects in axon fasciculation affect the formation and function of neural circuits remain unclear. Here we examine whether the transmembrane proteins semaphorin-5A (Sema5A) and semaphorin-5B (Sema5B), and their receptors, plexinA1 (PlexA1) and plexinA3 (PlexA3) play roles in the development of corticospinal circuits. We find that Sema5A/Sema5B and PlexA1/A3 are required for proper axon fasciculation of corticospinal neurons. Furthermore, Sema5A/5B and PlexA1/A3 mutant mice show marked deficits in skilled motor behaviors. Therefore, these results strongly suggest that proper corticospinal axon fasciculation is required for the appropriate formation and functioning of corticospinal circuits in mice.

Needle electromyography abnormalities in the upper trapezius muscle in neuromuscular disorders.

BACKGROUND: : Needle electromyography (EMG) abnormalities in the trapezius muscle (TM) can be seen in neuromuscular disorders. The aim was to determine the characteristics of needle EMG abnormalities observed in the TM in neuromuscular disorders. METHODS: The data of patients who applied to the Clinical Neurophysiology Laboratory of University of Health Sciences Adana City Training and Research Hospital between December 2018 and October 2021 were reviewed. Polio survivors, amyotrophic lateral sclerosis (ALS) patients, patients with sensorimotor polyneuropathy, patients with spinal cord lesions involving C2/C3/C4 segments, patients with spinal accessory nerve (SAN) lesions, neuralgic amyotrophy (NA) patients, and patients with myopathy were included. Needle EMG findings of the upper TM of the patients were analyzed. Positive sharp waves, fibrillation potentials, fasciculation potentials, myotonic discharges, and motor unit action potential (MUAP) changes were considered needle EMG abnormalities. RESULTS: Eighty-one polio survivors, 23 ALS patients, 39 patients with sensorimotor polyneuropathy, 10 patients with cervical spinal lesions, eight NA patients, seven patients with SAN lesions, and three patients with myopathy were included in the study. Fifteen (65.2%) ALS patients, 18 (22.2%) polio survivors, three (30%) patients with cervical spinal lesions, two (5.1%) patients with sensorimotor neuropathy, one (12.5%) NA patient, seven (100%) patients with SAN lesions, and two (66.7%) patients with myopathies had at least one needle EMG abnormality in the TM. Fasciculation potentials in the TM were seen in 10 (43.5%) ALS patients. In four patients with SAN lesions and one polio survivor, MUAP could not be obtained from the TM. DISCUSSION: There may be more frequent needle EMG abnormalities, particularly in ALS patients and patients with SAN lesions. Since the number of patients with myopathy included in this study was low, it is difficult to comment on the needle EMG features of the TM for these patients. In addition, this study indicated that fasciculation potentials in the TM are typical in ALS patients and that MUAP may not be obtained from the TM in patients with SAN lesions.

Spectrum of SPTLC1-related disorders: a novel case of 'Ser331 syndrome' that expand the phenotype of hereditary sensory and autonomic neuropathy type 1A and motor neuron diseases.

We report a patient with early-onset hereditary sensory and autonomic neuropathy type 1A (HSAN-1A) who developed a distinct phenotype, with tongue fasciculation and atrophy, due to a mutation at serine 331 in the SPTLC1 gene. HSAN-1A manifestation causing tongue fasciculation and atrophy have been rarely found. Our report adds to the growing evidence of the existence of an overlap between hereditary neuropathy and motor neuron disease caused by pathogenic p.S331Y variant in SPTLC1 gene.

Fasciculation electromechanical latency is prolonged in amyotrophic lateral sclerosis.

OBJECTIVE: In amyotrophic lateral sclerosis (ALS), motor neurons become hyperexcitable and spontaneously discharge electrical impulses causing fasciculations. These can be detected by two noninvasive methods: high-density surface electromyography (HDSEMG) and muscle ultrasonography (MUS). We combined these methods simultaneously to explore the electromechanical properties of fasciculations, seeking a novel biomarker of disease. METHODS: Twelve ALS patients and thirteen healthy participants each provided up to 24 minutes of recordings from the right biceps brachii (BB) and gastrocnemius medialis (GM). Two automated algorithms (Surface Potential Quantification Engine and a Gaussian mixture model) were applied to HDSEMG and MUS data to identify correlated electromechanical fasciculation events. RESULTS: We identified 4,197 correlated electromechanical fasciculation events. HDSEMG reliably detected electromechanical events up to 30 mm below the skin surface with an inverse correlation between amplitude and depth in ALS muscles. Compared to Healthy-GM muscles (mean = 79.8 ms), electromechanical latency was prolonged in ALS-GM (mean = 108.8 ms; p = 0.0458) and ALS-BB (mean = 112.0 ms; p = 0.0128) muscles. Electromechanical latency did not correlate with disease duration, symptom burden, sum muscle power score or fasciculation frequency. CONCLUSIONS: Prolonged fasciculation electromechanical latency indicates impairment of the excitation-contraction coupling mechanism, warranting further exploration as a potential novel biomarker of disease in ALS. SIGNIFICANCE: This study points to an electromechanical defect within the muscles of ALS patients.

Differentiation Between Amyotrophic Lateral Sclerosis and Mimics Using Quantitative Analysis of Fsciculation with Muscle Ultrasound / 中国医学科学杂志(英文版)

Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis (ALS). Methods We prospectively recruited patients who had ALS and neuropathy-radiculopathy attending Peking Union Medical College Hospital from 2017 to 2020. Healthy adults from a community were recruited as healthy controls. Muscle strength was assessed using the Medical Research Council (MRC) scale. At the first visit to the hospital, patients were assessed for maximal grade of fasciculations, total fasciculation score, and fasciculation grade in 16 muscle groups of bilateral upper and lower limbs using ultrasonography. The sensitivity and specificity of maximal grade of fasciculations, total fasciculation score, and fasciculation grade for the diagnosis of ALS were assessed by receiver operating characteristic analyses. Results The percentage of limb muscles with a maximal fasciculation grade higher than grade 2 in ALS patients and neuropathy-radiculopathy patients was 84.9% and 9.8%, respectively (χ2 = 172.436, P < 0.01). Of the 16 limb muscles detected, the total fasciculation score [median (interquartile range)] was 29 (15, 41) in ALS patients and 3 (0, 8) in neuropathy-radiculopathy patients (Z = 9.642, P < 0.001). Remarkable fasciculations were seen in ALS patients whose muscles with a MRC score ranging from 2 to 4, followed by patients with MRC score 5, and then in those with MRC score 0 and 1. The sensitivity and specificity of total fasciculation score for diagnosis of ALS were 80.6% and 93.4%, respectively (cut-off value 14). In patients with ALS, for muscles with MRC score 4 and 5, the percentage of muscles with fasciculation grades ≥ 3 was 42.3% and 24.1% respectively, while in neuropathy-radiculopathy patients, the percentage for muscles with MRC score 4 and 5 was only 1.7% and 0, respectively. Conclusion A combined analysis of fasciculation intensity and MRC score of the limb muscles may be helpful for differential diagnosis of ALS.

Bulbar onset amyotrophic lateral sclerosis: A case report.

Introduction: Amyotrophic lateral sclerosis is a rare progressive neurodegenerative disease that affects the brain and spinal cord nerve cells. The study highlights the role of early diagnosis and prognosis of bulbar onset ALS. Case presentation: We present a case of 60 years old female who presented with slurring of speech with a deviation of tongue and progressive dysphagia. With the role of Magnetic Resonance Imaging (MRI), Electromyography (EMG) and Nerve Conduction Study (NCS), a diagnosis of ALS was made. Clinical discussion: ALS is a progressive neurodegenerative disorder, and the presence of genioglossus muscle involvement at diagnosis implies a shorter survival. It mandates multidisciplinary aspects requiring a professional opinion from neurologists, speech therapists, otolaryngologists, and electrophysiologists for effective treatment. Edaravone has antioxidant properties which counteract oxidative stress leading to neuronal injury in patients with ALS. Conclusion: ALS with bulbar onset can have a grave prognosis and hence requires a multidisciplinary approach toward effective treatment.

Premedication with Diclofenac and Precurarization with Atracurium on Succinylcholine-Induced Myalgia in Laparoscopic Cholecystectomy: A Double-Blinded Randomized Study.

Background and Aims: Succinylcholine is the only available depolarizing neuromuscular blocker that has been widely used in the induction of anesthesia, and it is the drug of choice for rapid-sequence induction of anesthesia due to its rapid onset of effect and ultrashort duration of action owing to its rapid hydrolysis by acetyl-cholinesterase. Postoperative muscle pain (myalgia) and muscle stiffness are the most common side effects and are observed most frequently on the 1st postoperative day in ambulatory surgery. The use of succinylcholine in the induction of anesthesia and intubation in routine cases has been discouraged because of such adverse effects. However, because of its cost-effectiveness and easy availability, it is still used by some institutions routinely. This study aimed to study the efficacy of preoperative diclofenac along with atracurium precurarization in alleviating succinylcholine-induced myalgia. Materials and Methods: It is a double-blind randomized comparative study carried out in a tertiary care hospital. The study sample was 60 and divided into two equal groups. All data entered in MS-Excel Sheet and Wilcoxon signed-rank test were done for nonparametric data and one-way ANOVA for the parametric data. The normal distribution of the study sample was tested by the Shapiro - Wilk test. Results: The fasciculations in the test group were much less than in the control group with P < 0.00001. The results for the incidence of myalgia in the two groups were as follows: P value at 24 h was 0.00018 and at 48 h was 0.0028, respectively. Creatine kinase levels at preoperative and 24 h postoperative periods were 49.47 ± 7.24 in Group D, 53.30 ± 7.98 in Group B and 87.38 ± 15.16 in Group D, and 188.41 ± 33.27 in Group B, respectively. Conclusion: Succinylcholine-induced myalgia has a complex pathophysiology. However, the preemptive use of diclofenac in combination with precurarization can alleviate the incidence and severity of succinylcholine-induced myalgia. Therefore, its use may be considered in routine cases for induction of anesthesia for facilitating laryngoscopy and endotracheal intubation.