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This case report presents a 55-year-old man, with a history of hypertension, who developed an unusual and atypical presentation of chronic inflammatory demyelinating polyneuropathy (CIDP). Over 3 months, the patient experienced progressive limb weakness ascending from lower limbs to upper limbs with marked tongue fasciculations and facial muscle weakness without dysarthria or dysphagia. While cranial nerve involvement has been documented in CIDP, cases featuring the hypoglossal nerve are much less frequent than those with the facial nerve. This makes the occurrence of both happening at the same time quite rare. Diagnosing atypical presentations of CIDP can be tricky, and offer a challenge for neurologists in daily practice. A comprehensive diagnostic workup that could include blood work, cerebrospinal fluid analysis, and a nerve conduction study is required to confirm the diagnosis and rule out potential other causes. Effective management in this case included several inpatient plasmapheresis sessions and administration of steroids, which led to significant clinical improvement in the patient’s symptoms. These breakthroughs are essential for clinicians to promptly diagnose and treat patients suffering from the same diseases. [ABSTRACT FROM AUTHOR]

Abstract Protocadherin 19 (Pcdh19) is a homophilic cell adhesion molecule and is involved in a variety of neuronal functions. Here, we tested whether Pcdh19 has a regulatory role in axon guidance using the developing Xenopus retinotectal system. We performed targeted microinjections of a translation blocking antisense morpholino oligonucleotide to knock down the expression of Pcdh19 selectively in the central nervous system. Knocking down Pcdh19 expression resulted in navigational errors of retinal ganglion cell (RGC) axons specifically at the optic chiasm. Instead of projecting to the contralateral optic tectum, RGC axons in the Pcdh19-depleted embryo misprojected ipsilaterally. Although incorrectly delivered into the ipsilateral brain hemisphere, these axons correctly reached the optic tectum. These data suggest that Pcdh19 has a critical role in preventing mixing of RGC axons originating from the opposite eyes at the optic chiasm, highlighting the importance of cell adhesion in bundling of RGC axons.

Protocadherin 19 (Pcdh19) is a homophilic cell adhesion molecule and is involved in a variety of neuronal functions. Here, we tested whether Pcdh19 has a regulatory role in axon guidance using the developing Xenopus retinotectal system. We performed targeted microinjections of a translation blocking antisense morpholino oligonucleotide to knock down the expression of Pcdh19 selectively in the central nervous system. Knocking down Pcdh19 expression resulted in navigational errors of retinal ganglion cell (RGC) axons specifically at the optic chiasm. Instead of projecting to the contralateral optic tectum, RGC axons in the Pcdh19-depleted embryo misprojected ipsilaterally. Although incorrectly delivered into the ipsilateral brain hemisphere, these axons correctly reached the optic tectum. These data suggest that Pcdh19 has a critical role in preventing mixing of RGC axons originating from the opposite eyes at the optic chiasm, highlighting the importance of cell adhesion in bundling of RGC axons. [ABSTRACT FROM AUTHOR]

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.

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 a-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. [ABSTRACT FROM AUTHOR]

[Display omitted] • Whole body motor unit MRI (MUMRI) offers a sensitive and non-invasive method to image fasciculation in multiple body regions. • Whole body MUMRI fasciculation rates correlated with single channel surface EMG fasciculation rates. • MUMRI discriminated well between ALS patients and healthy people for proximal limb and paraspinal muscles, but not the tongue. 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). 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]). MUMRI-detected fasciculation rates in typical ALS patients were higher compared to healthy controls for biceps brachii (2.40 ± 1.90 cm-3min−1 vs. 0.04 ± 0.10 cm-3min−1, p = 0.004), paraspinals (1.14 ± 1.61 cm-3min−1 vs. 0.02 ± 0.02 cm-3min−1, p = 0.016) and lower legs (1.42 ± 1.27 cm-3min−1 vs. 0.13 ± 0.10 cm-3min−1, p = 0.004), but not tongue (1.41 ± 1.94 cm-3min−1 vs. 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). MUMRI can non-invasively image fasciculation in multiple body regions and appears sensitive to disease progression in individual patients. MUMRI has potential as diagnostic tool for ALS. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis (ALS). 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. 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 (χ 2 = 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. A combined analysis of fasciculation intensity and MRC score of the limb muscles may be helpful for differential diagnosis of ALS. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Samuel Debas Bayable,1 Netsanet Temesgen Ayenew,1 Abebaw Misganaw,1 Melaku Bantie Fetene,2 Wubet Dessie Amberbir3 1Department of Anesthesia, College of Medicine and Health Science, Debre Markos University, Debre Markos, Ethiopia; 2Department of Anesthesia, College of Medicine and Health Science, Debre Berhan University, Debre Berhan, Ethiopia; 3Department of Anesthesia, Menelik II Health Science College, Addis Ababa, EthiopiaCorrespondence: Samuel Debas Bayable, Tel +251 911437507, Email Samuel_debas@dmu.edu.et; samueldebas88@yahoo.comBackground: 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.Keywords: fasciculation, muscle pain, postoperative myalgia, succinylcholine, vecuronium

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

See article on pages 148–156 in this issue. [ABSTRACT FROM AUTHOR]

• Aligned, automated outputs from muscle ultrasound and surface electromyography improved characterisation of fasciculations. • The latency between electrical and mechanical peaks was prolonged in amyotrophic lateral sclerosis. • Such insight into impaired excitation–contraction coupling could be translated into a novel biomarker of disease. 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. 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. 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. Prolonged fasciculation electromechanical latency indicates impairment of the excitation–contraction coupling mechanism, warranting further exploration as a potential novel biomarker of disease in ALS. This study points to an electromechanical defect within the muscles of ALS patients. [ABSTRACT FROM AUTHOR]

Abstract Background and purpose Muscle ultrasonography has been increasingly recognized as a useful tool for detection of fasciculations. Separately, concordance between dominant hand and onset side has been reported in amyotrophic lateral sclerosis (ALS). The aim of this study was to reveal the distribution of fasciculations in the whole body, focusing on handedness. Methods In 106 consecutive patients with ALS, muscle ultrasonography was systematically performed in 11 muscles (the tongue, and bilateral biceps brachii, 1st dorsal interosseous [FDI], T10-paraspinalis, vastus lateralis and tibialis anterior muscles). The fasciculation intensity was scored from 0 to 3 for each muscle. Results Fasciculations were more frequently found in the limb muscles than the tongue and paraspinalis. Side and handedness analyses revealed that fasciculation intensity in FDI was significantly more prominent on the right (median [inter-quartile range] 2 [0 - 3]) than left (1.5 [0 - 3]; p = 0.016), and in the dominant hand (2 [1 - 3]) than non-dominant side (1.5 [0 - 3]; p = 0.025). The differences were greater in patients with upper limb onset. There were no side differences in the lower limb muscles. Multivariate analyses showed that male patients had more frequent fasciculations in the dominant FDI (β = 0.22, p < 0.05). Conclusion More intensive fasciculations are present in the FDI in the dominant hand and gender might be associated with fasciculation intensities. This distribution pattern of fasciculations might be associated with pathogenesis of ALS.

Introduction/Aims: Fasciculations are an early clinical hallmark of amyotrophic lateral sclerosis (ALS), amenable to detection by high‐density surface electromyography (HDSEMG). In conjunction with the Surface Potential Quantification Engine (SPiQE), HDSEMG offers improved spatial resolution for the analysis of fasciculations. This study aims to establish an optimal recording duration to enable longitudinal remote monitoring in the home. Methods: Twenty patients with ALS and five patients with benign fasciculation syndrome (BFS) underwent serial 30 min HDSEMG recordings from biceps brachii and gastrocnemii. SPiQE was independently applied to abbreviated epochs within each 30‐min recording (0–5, 0–10, 0–15, 0–20, and 0–25 min), outputting fasciculation frequency, amplitude median and amplitude interquartile range. Bland–Altman plots and intraclass correlation coefficients (ICC) were used to assess agreement with the validated 30‐min recording. Results: In total, 506 full recordings were included. The 5 min recordings demonstrated diverse and relatively poor agreement with the 30 min baselines across all parameters, muscles and patient groups (ICC = 0.32–0.86). The 15‐min recordings provided more acceptable and stable agreement (ICC = 0.78–0.98), which did not substantially improve in longer recordings. Discussion: For the detection and quantification of fasciculations in patients with ALS and BFS, HDSEMG recordings can be halved from 30 to 15 min without significantly compromising the primary outputs. Reliance on a shorter recording duration should lead to improved tolerability and repeatability among patients, facilitating longitudinal remote monitoring in patients' homes. [ABSTRACT FROM AUTHOR]

Background: The fasciculus retroflexus is the prominent efferent pathway from the habenular complex. Medial habenular axons form a core packet whereas lateral habenular axons course in a surrounding shell. Both groups of fibers share the same initial pathway but differ in the final segment of the tract, supposedly regulated by surface molecules. The gene Amigo2 codes for a membrane adhesion molecule with an immunoglobulin‐like domain 2 and is selectively expressed in the medial habenula. We present it as a candidate for controlling the fasciculation behavior of medial habenula axons. Results: First, we studied the development of the habenular efferents in an Amigo2 lack of function mouse model. The fasciculus retroflexus showed a variable defasciculation phenotype. Gain of function experiments allowed us to generate a more condensed tract and rescued the Amigo2 knock‐out phenotype. Changes in Amigo2 function did not alter the course of habenular fibers. Conclusion: We have demonstrated that Amigo2 plays a subtle role in the fasciculation of the fasciculus retroflexus. Key Findings: Amigo2 participate in the fasciculation process of the fasciculus retroflexus 2. Amigo2 do not participate in the guidance mechanisms of the fasciculus retroflexus 3. The lateral habenula is born between E10.5 and E11.5 and the medial habenula between E12.5 and E13.5 in mouse. [ABSTRACT FROM AUTHOR]

Abstract Background Fasciculation is an important sign for the diagnosis of amyotrophic lateral sclerosis (ALS). Our study aimed to analyze the difference in fasciculation detected with muscle ultrasonography (MUS) between ALS patients and non-ALS patients with symptoms resembling ALS. Methods Eighty-eight ALS patients and fifty-four non-ALS (eight multifocal motor neuropathy, 32 chronic inflammatory demyelinating polyneuropathy/Charcot-Marie-Tooth, and 14 cervical spondylopathy or lumbar spondylopathy) patients were recruited. MUS was performed on 19 muscle groups in cervical, lumbosacral, bulbar, and thoracic regions for each patient. The intensity of fasciculation was divided into five grades based on firing frequency and number in the involved muscle groups. Results The overall detection rates were 72.8% in ALS and 18% in non-ALS patients. The fasciculation grades (median [IQR]) were 2 (0–3) in ALS and 0 (0–0) in non-ALS patients (P

Misuse of organophosphate insecticides, even in case of domestic application, can be life threatening. We report a case of accidental consumption of organophosphorus substance by a 3-year-old female child presented to us with unconsciousness and nicotinic manifestation of organophosphate poisoning unlike classical toxidrome of muscarinic manifestation.

1 Muscle fasciculation are frequent in SMA. 2 Fasciculation differs later-onset SMA from genetic muscle disease. 3 Physical examination and muscular ultrasound are feasible for fasciculation detection. 4 Muscular ultrasound was the best modality to look for fasciculation in SMA. Diagnosis of later-onset spinal muscular atrophy (SMA) can be challenging. This study aimed to evaluate the diagnostic properties of the detection of muscle fasciculations for SMA diagnosis in adolescents and adults with proximal muscle weakness. A cross-sectional diagnostic accuracy study was performed, in which 10 subjects with SMA (5 with type II and 5 with type III) and 9 subjects with genetic muscle diseases were evaluated by physical examination, muscle ultrasound (MUS) and electromyography (EMG). Inter-rater reliability of MUS was higher than physical examination and in a sensitivity analysis of MUS, all SMA subjects and a single patient with genetic muscle disease presented fasciculations in at least 2 different muscle groups, resulting in a sensitivity of 1 (95% CI: 0.69 to 1) and a specificity of 0.89 (95% CI: 0.52 to 1) for SMA diagnosis. Forty-two percent of evaluated subjects did not agree to perform EMG, limiting this method results. Muscle ultrasound presented the best diagnostic accuracy and physical examination combined with MUS seemed to be a good strategy for screening adolescents and adults with proximal muscle weakness for SMA. These results might improve diagnostic guidelines for later-onset SMA, leading to earlier diagnosis, treatment and specific care. [ABSTRACT FROM AUTHOR]

• Fasciculations were abundantly detected on muscle ultrasonography from the early stage of amyotrophic lateral sclerosis. • Fasciculation in the brainstem and thoracic regions has high specificity in the diagnosis of amyotrophic lateral sclerosis. • Via machine learning, we developed a muscle ultrasonography-based diagnostic tool for early-stage amyotrophic lateral sclerosis. Although fasciculation on muscle ultrasonography (MUS) is useful in diagnosing amyotrophic lateral sclerosis (ALS), its applicability to early diagnosis remains unclear. We aimed to develop and validate diagnostic models especially beneficial to early-stage ALS via machine learning. We investigated 100 patients with ALS, including 50 with early-stage ALS within 9 months from onset, and 100 without ALS. Fifteen muscles were bilaterally observed for 10 s each and the presence of fasciculations was recorded. Hierarchical clustering and nominal logistic regression, neural network, or ensemble learning were applied to the training cohort comprising the early-stage ALS to develop MUS-based diagnostic models, and they were tested in the validation cohort comprising the later-stage ALS. Fasciculations on MUS in the brainstem or thoracic region had high specificity but limited sensitivities and predictive profiles for diagnosis of ALS. A machine learning-based model comprising eight muscles in the four body regions had a high sensitivity (recall), specificity, and positive predictive value (precision) for both early- and later-stage ALS patients. We developed and validated MUS-fasciculation-based diagnostic models for early- and later-stage ALS. Fasciculation detected in relevant muscles on MUS can contribute to the diagnosis of ALS from the early stage. [ABSTRACT FROM AUTHOR]

Introduction: due to the wide indications for the use of rapid sequencing induction with succinylcholine and the high prevalence of fasciculation and myalgia, as well as increasing the dose of succinylcholine by up to 50% when using atracurium and long-term relaxation of atracurium, this plan Studies were performed Evaluation of experiences of using Atracurium and lidocaine on succinylcholine-induced fasciculation in anesthesia surgeries: a study from clinical experience. Material And Methods: This study is a clinical experience that was performed in gold in 2019 in Imam Reza Hospital [Tabriz University of Medical Sciences] with the participation of patients undergoing general anesthesia [requiring rapid sequencing anesthesia and succinylcholine injection]. One group received 0.05 mg/kg tracorium with a volume of 5 cc three minutes before induction of anesthesia and the other group received 1.5 mg/kg lidocaine with a volume of 5 cc 30 seconds before induction of anesthesia. The fasciculation status caused by succinylcholine injection was compared between the two groups. Results: The number and severity of fasciculation between the two groups of atracurium and lidocaine to prevent fasciculation were not statistically significant [p> 0.05]. Conclusion: Atracurium and lidocaine administration to prevent saccharin-induced fasciculation had similar effects in patients undergoing general anesthesia. [ABSTRACT FROM AUTHOR]

The correct wiring of neuronal circuits depends on outgrowth and guidance of neuronal processes during development. In the past two decades, great progress has been made in understanding the molecular basis of axon outgrowth and guidance. Genetic analysis in Caenorhabditis elegans has played a key role in elucidating conserved pathways regulating axon guidance, including Netrin signaling, the slit Slit/Robo pathway, Wnt signaling, and others. Axon guidance factors were first identified by screens for mutations affecting animal behavior, and by direct visual screens for axon guidance defects. Genetic analysis of these pathways has revealed the complex and combinatorial nature of guidance cues, and has delineated how cues guide growth cones via receptor activity and cytoskeletal rearrangement. Several axon guidance pathways also affect directed migrations of non-neuronal cells in C. elegans, with implications for normal and pathological cell migrations in situations such as tumor metastasis. The small number of neurons and highly stereotyped axonal architecture of the C. elegans nervous system allow analysis of axon guidance at the level of single identified axons, and permit in vivo tests of prevailing models of axon guidance. C. elegans axons also have a robust capacity to undergo regenerative regrowth after precise laser injury (axotomy). Although such axon regrowth shares some similarities with developmental axon outgrowth, screens for regrowth mutants have revealed regeneration-specific pathways and factors that were not identified in developmental screens. Several areas remain poorly understood, including how major axon tracts are formed in the embryo, and the function of axon regeneration in the natural environment.

Muscle fasciculations, resulting from the spontaneous activation of motor neurons, may be associated with neurological disorders, and are often assessed with intramuscular electromyography (EMG). Recently, however, both ultrasound (US) imaging and multichannel surface EMG have been shown to be more sensitive to fasciculations. In this study we combined these two techniques to compare their detection sensitivity to fasciculations occurring in different muscle regions and to investigate the effect of EMG electrodes’ configuration on their agreement. Monopolar surface EMGs were collected from medial gastrocnemius and soleus with an array of 32 electrodes (10 mm Inter-Electrode Distance, IED) simultaneously with b-mode US images detected alongside either proximal, central or distal electrodes groups. Fasciculation potentials (FP) were identified from single differential EMGs with 10 mm (SD1), 20 mm (SD2) and 30 mm (SD3) IEDs, and fasciculation events (FE) from US image sequences. The number, location, and size of FEs and FPs in 10 healthy participants were analyzed. Overall, the two techniques showed similar sensitivities to muscle fasciculations. US was equally sensitive to FE occurring in the proximal and distal calf regions, while the number of FP revealed by EMG increased significantly with the IED and was larger distally, where the depth of FE decreased. The agreement between the two techniques was relatively low, with a percentage of fasciculation classified as common ranging from 22% for the smallest IED to 68% for the largest IED. The relevant number of events uniquely detected by the two techniques is discussed in terms of different spatial sensitivities of EMG and US, which suggest that a combination of US-EMG is likely to maximise the sensitivity to muscle fasciculations.

Background and Purpose: Muscle ultrasonography has been increasingly recognized as a useful tool for detection of fasciculations. Separately, concordance between dominant hand and onset side has been reported in amyotrophic lateral sclerosis (ALS). The aim of this study was to reveal the distribution of fasciculations in the whole body, focusing on handedness.Methods: In 106 consecutive patients with ALS, muscle ultrasonography was systematically performed in 11 muscles (the tongue, and bilateral biceps brachii, 1st dorsal interosseous [FDI], T10-paraspinalis, vastus lateralis and tibialis anterior muscles). The fasciculation intensity was scored from 0 to 3 for each muscle.Results: Fasciculations were more frequently found in the limb muscles than the tongue and paraspinalis. Side and handedness analyses revealed that fasciculation intensity in FDI was significantly more prominent on the right (median [inter-quartile range] 2 [0 - 3]) than left (1.5 [0 - 3]; p = 0.016), and in the dominant hand (2 [1 - 3]) than non-dominant side (1.5 [0 - 3]; p = 0.025). The differences were greater in patients with upper limb onset. There were no side differences in the lower limb muscles. Multivariate analyses showed that male patients had more frequent fasciculations in the dominant FDI (β = 0.22, p < 0.05).Conclusion: More intensive fasciculations are present in the FDI in the dominant hand and gender might be associated with fasciculation intensities. This distribution pattern of fasciculations might be associated with pathogenesis of ALS. [ABSTRACT FROM AUTHOR]

Background: Succinylcholine is a preferred muscle relaxant for rapid sequence intubation. Postoperative myalgia (POM) is one of its commonest adverse effects with unknown pathogenesis. Various modalities were examined to reduce POM. We hypothesized that duloxetine may reduce the incidence and severity of fasciculation or succinylcholine‐induced POM in outpatient surgeries. Methods: This randomized double‐blinded trial involved controlled 70 adult participants scheduled for elective direct microlaryngoscopic surgeries. Before induction of general anesthesia by 2 h, 35 patients received duloxetine 30 mg orally (group D) and 35 patients received similar oral starch placebo capsules (group C). Fasciculations, POM, sedation score, time to first rescue analgesia, total analgesic consumption 24 h after surgery, patients' satisfaction, and adverse effects were recorded. Results: Incidence of fasciculation was 77.1% and 94.3% in groups D and C (p value = 0.04), whereas its severity was not significant between groups (p value = 0.09). Incidence and severity of POM were significantly lower in group D (p values = 0.004 and 0.021). Positive correlation was recorded between fasciculations and POM scores (r = 0.732 and p < 0.001). Time required for first analgesia was prolonged in group D (p value < 0.001) with less total analgesic consumption (p value = 0.039). The potassium and creatine kinase levels showed significant differences between both groups after 30 min and 24 h, respectively (p value < 0.05). Sedation scores and patients' satisfaction were better in duloxetine group (p value < 0.05) with no severe complications. Conclusions: Preoperative oral duloxetine 30 mg administration decreased incidence and severity of succinylcholine‐related muscle POM and fasciculations. It was also effective in reducing postoperative rescue analgesic requirement with better patients' satisfaction and no serious adverse effects. Preoperative oral duloxetine 30 mg administration decreased the incidence and the severity of succinylcholine‐related muscle postoperative myalgia and fasciculations. It was also effective in reducing postoperative rescue analgesic requirement with better patients' satisfaction and no serious adverse effects. [ABSTRACT FROM AUTHOR]