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Key Clinical Message The nicotinic symptom in Organo‐Phosphate (OP) poisoning is an unusual presentation in an adult, as in our case of a 38‐year‐old man who also experienced decreased motor power of all limbs and diffuses fasciculation of the tongue and lower limbs.

Background: Succinylcholine with quick onset, ultra short duration of action, and complete predictable paralysis has been the best drug for endotracheal intubation but the occurrence of side effects such as muscle fasciculations, postoperative myalgia, rise in serum potassium levels and myoglobinuria limits its use in the full stomach, burns, massive trauma, metabolic acidosis, and few myopathies. For attenuation of these effects, many drugs have been studied but pre-treatment with non-depolarizing muscle relaxant found to be successful. Aims and Objectives: This study was designed to assess the effect of Rocuronium and Vecuronium pre-treatment on post-succinylcholine fasciculations, rise in serum potassium levels, and postoperative myalgia. Materials and Methods: 100 patients between the age of 20–50 years of either sex (American Society of Anaesthesiologists grade I and II) undergoing general anesthesia for various surgical procedures were randomly allocated into two groups according to pre-treatment with Rocuronium (Group R) and Vecuronium (Group V) before Succinylcholine administration. After Succinylcholine administration severity of fasciculations, rise in serum potassium after 5 min and myalgia on postoperative days 1, 2, and 3 were recorded. Results: About 74% patients had no fasciculation in Group R as compared to 36% in group V. Statistically insignificant (P>0.05) rise in serum potassium level was observed in both groups. Higher number of patients had mild-to-moderate degree of myalgia with Vecuronium on postoperative days 2 and 3. Conclusion: Pre-treatment with Rocuronium before Succinylcholine provided better attenuation of post-succinylcholine muscle fasciculations, postoperative myalgia with statistically insignificant rise in serum potassium levels.

Amyotrophic lateral sclerosis is a neurodegenerative disease with a steady and rapid progression, manifested, in addition to the loss of strength of the muscles of the limbs and trunk, the development of fatal bulbar and respiratory disorders. Due to the need for early detection of this pathology, a large number of diagnostic tests have been proposed, which make it possible to suspect a motor neuron disease in a patient with a high probability. However, due to the polymorphic clinical picture of the disease, currently there are diagnostic situations in which existing methods do not allow to establish a reliable diagnosis or it becomes possible only at the later stages of the disease. This review examines the modern possibilities of ultrasound examination of peripheral nerves and muscles in the diagnosis and differential diagnosis of amyotrophic lateral sclerosis.

Background: Succinylcholine has been extensively used for endotracheal intubation because of its ultrashort duration of action, quick onset with complete and predictable paralysis. However, the occurrence of some side effects like post injection fasciculations, postoperative myalgia, and increase in serum potassium levels limits its use in some situations like burns, myopathies, and myoglobinuria. For attenuation of these effects, many drugs have been studied but with limited success. Aims and objectives: To evaluate the role of vitamin C on attenuation of post-suxamethonium myalgia and increase in serum potassium levels. Materials and methods: Seventy-five females of American Society of Anaesthesiologists grade I and II and aged 20 to 50 years undergoing general anesthesia for various short surgical procedures were randomly allocated into three groups. Induction of anesthesia was performed with injection thiopentone 5 mg/kg body weight and suxamethonium 50 mg in all three groups. In group I, no drug was administered before induction of anesthesia. In group II, 500 mg tablet of vitamin C was administered twice orally a day before and on the day of surgery half an hour before induction of anesthesia. In group III, vitamin C 500 mg was administered intravenously in 50 ml saline half an hour before induction of anesthesia. The absence or presence of muscle pains and fasciculations were noted and if present, then their severity was also estimated. Serum potassium levels were measured in the venous samples collected just before induction and at 5-minute interval after the administration of suxamethonium. Patients were observed for myalgia on postoperative days 1, 2, and 3. Results: The patients receiving vitamin C had a little reduction in the incidents but marked reduction in severity of muscle pains and fasciculation. Furthermore, the administration of vitamin C markedly delayed the onset of muscle pains. There was no effect of vitamin C pretreatment on serum potassium levels. Conclusion: Pretreatment with vitamin C 500 mg may be useful in reduction of severity of post- suxamethonium myalgia.

INTRODUCTION:More than 90% of amyotrophic lateral sclerosis (ALS) patients have muscle cramps, but evidence-based treatments have not been available.METHODS:A multicenter, double-blind, placebo-controlled crossover trial of mexiletine 150 mg twice daily was conducted in ALS patients requesting treatment of symptomatic muscle cramps.RESULTS:Muscle cramp frequency was reduced in 18 of 20 patients; 13 reductions were attributed to treatment (P

Objective: There is a need for improved diagnostic tools in Amyotrophic Lateral Sclerosis (ALS). Our objective was to assess muscle ultrasound as a diagnostic tool in patients with ALS and determine a simplified screening protocol to aid implementation in clinical practice., Methods: Ultrasound of bulbar and limb muscles was prospectively performed on all patients referred to a single centre with suspected ALS. Clinical measures of disease severity and upper motor neuron impairment were also recorded. Receiver operating characteristic (ROC) curves were calculated to assess the diagnostic utility of muscle ultrasound., Results: 94 patients initially suspected of ALS were recruited to this observational cohort study. Forty-four were subsequently diagnosed as ALS and 50 as disease mimics. ALS patients demonstrated a higher frequency and more generalised distribution of fasciculations compared to mimics. A simplified 5 muscle screening protocol exhibited an AUC of 0.94 (95 %CI 0.89-0.99) in discriminating ALS from mimics. The presence of ≥ 3 fasciculating muscles detected using this screening protocol was 89 % sensitive and 88 % specific for the diagnosis of ALS., Conclusions: Muscle ultrasound, screening as few as 5 muscles, has diagnostic utility in ALS., Significance: Muscle ultrasound enhances clinical diagnosis in ALS., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 International Federation of Clinical Neurophysiology. All rights reserved.)

Background: Succinylcholine has been extensively used for endotracheal intubation because of its ultrashort duration of action, quick onset with complete and predictable paralysis. However, the occurrence of some side effects like post injection fasciculations, postoperative myalgia, and increase in serum potassium levels limits its use in some situations like burns, myopathies, and myoglobinuria. For attenuation of these effects, many drugs have been studied but with limited success. Aims and objectives: To evaluate the role of vitamin C on attenuation of post-suxamethonium myalgia and increase in serum potassium levels. Materials and methods: Seventy-five females of American Society of Anaesthesiologists grade I and II and aged 20 to 50 years undergoing general anesthesia for various short surgical procedures were randomly allocated into three groups. Induction of anesthesia was performed with injection thiopentone 5 mg/kg body weight and suxamethonium 50 mg in all three groups. In group I, no drug was administered before induction of anesthesia. In group II, 500 mg tablet of vitamin C was administered twice orally a day before and on the day of surgery half an hour before induction of anesthesia. In group III, vitamin C 500 mg was administered intravenously in 50 ml saline half an hour before induction of anesthesia. The absence or presence of muscle pains and fasciculations were noted and if present, then their severity was also estimated. Serum potassium levels were measured in the venous samples collected just before induction and at 5-minute interval after the administration of suxamethonium. Patients were observed for myalgia on postoperative days 1, 2, and 3. Results: The patients receiving vitamin C had a little reduction in the incidents but marked reduction in severity of muscle pains and fasciculation. Furthermore, the administration of vitamin C markedly delayed the onset of muscle pains. There was no effect of vitamin C pretreatment on serum potassium levels. Conclusion: Pretreatment with vitamin C 500 mg may be useful in reduction of severity of post-suxamethonium myalgia. [ABSTRACT FROM AUTHOR]

Background: Succinylcholine with quick onset, ultra short duration of action, and complete predictable paralysis has been the best drug for endotracheal intubation but the occurrence of side effects such as muscle fasciculations, postoperative myalgia, rise in serum potassium levels and myoglobinuria limits its use in the full stomach, burns, massive trauma, metabolic acidosis, and few myopathies. For attenuation of these effects, many drugs have been studied but pre-treatment with non-depolarizing muscle relaxant found to be successful. Aims and Objectives: This study was designed to assess the effect of Rocuronium and Vecuronium pre-treatment on post-succinylcholine fasciculations, rise in serum potassium levels, and postoperative myalgia. Materials and Methods: 100 patients between the age of 20–50 years of either sex (American Society of Anaesthesiologists grade I and II) undergoing general anesthesia for various surgical procedures were randomly allocated into two groups according to pre-treatment with Rocuronium (Group R) and Vecuronium (Group V) before Succinylcholine administration. After Succinylcholine administration severity of fasciculations, rise in serum potassium after 5 min and myalgia on postoperative days 1, 2, and 3 were recorded. Results: About 74% patients had no fasciculation in Group R as compared to 36% in group V. Statistically insignificant (P>0.05) rise in serum potassium level was observed in both groups. Higher number of patients had mild-to-moderate degree of myalgia with Vecuronium on postoperative days 2 and 3. Conclusion: Pre-treatment with Rocuronium before Succinylcholine provided better attenuation of post-succinylcholine muscle fasciculations, postoperative myalgia with statistically insignificant rise in serum potassium levels. [ABSTRACT FROM AUTHOR]

Background and objectives: Muscle ultrasound (MUS) is an emerging noninvasive tool to identify fasciculations in amyotrophic lateral sclerosis (ALS). We assessed the utility of MUS in detecting fasciculations in suspected ALS patients. Methods: Thirty‐three patients (25 men) with possible (n = 7), probable (n = 12), or definite ALS according to Awaji criteria were studied. Electromyography was done in biceps brachii, quadriceps, and thoracic paraspinal muscles and MUS in biceps, triceps, deltoid, abductor‐digiti‐minimi, quadriceps, hamstrings, tibialis anterior, thoracic paraspinal, and tongue muscles. Results: The age at onset and illness duration was 49.73 ± 12.7 years and 13.57 ± 9.7 months, respectively. Limb‐onset = 24 patients (72.7%) and bulbar‐onset = 9 (27.3%). Totally 561 muscles were examined by MUS. Fasciculations were detected in 84.3% of muscles, 98.4% with and 73% without clinical fasciculations (p < 0.001). Fasciculation detection rate (FDR) by MUS was significantly higher in muscles with wasting (95.6%) than without wasting (77.6%, p < 0.001). Compared with EMG, FDR was significantly higher with MUS in quadriceps (81.8% vs. 51.5%, p = 0.002) and thoracic paraspinal muscles (75.8% vs. 42.4%, p = 0.013). The proportion of patients with definite ALS increased from 42% by clinical examination to 70% after combining EMG and MUS findings. Conclusions: MUS is more sensitive in detecting fasciculations than electromyography (EMG) and provides a safer, faster, painless, and noninvasive alternative to EMG in detecting fasciculations in ALS. [ABSTRACT FROM AUTHOR]

Fasciculations are the most common form of spontaneous muscle contraction. They frequently occur in healthy individuals. However, there are a minority of situations that fasciculations are observed in association with specific neurologic disorders. Publications concerning the evaluation of pediatric patients experiencing fasciculations are limited. These children may undergo invasive or expensive diagnostic investigations that are unnecessary. Moreover, without careful consideration of differential diagnoses, rare neuromuscular disorders that present with fasciculations in the pediatric age group can be under-recognized by pediatric neurologists. This review examines the most important pediatric disorders presenting with fasciculations and other spontaneous muscle contractions to guide pediatric neurologists in evaluating these children. [ABSTRACT FROM AUTHOR]

Background: Arizona's rugged desert landscape harbors many venomous animals, including a small nocturnal scorpion, Centruroides sculpturatus, whose venom can cause severe neuromotor disturbance. An effective antivenom is available at selected health care facilities in the state.Methods: We analyzed 4398 calls of scorpion stings to the Arizona Poison and Drug Information Center (APDIC) in Tucson over a period of 3 years, from January 2017 to December 2019.Results: We followed 1952 (44.4%) of the victims to resolution. We excluded 2253 callers with minimal effects of the sting and 193 victims with possible toxic effects who were lost to follow-up. The most common complaints among callers were pain at the sting site in 88.9% and local numbness in 62.2%. Detailed clinical information was obtained from 593 calls from a health care facility. Neuromotor signs consistent with C. sculpuratus envenomation included nystagmus in 163 (27.5%), hypersalivation in 91 (15.3%), and fasciculations in 88 (14.8%). Antivenom (Anascorp; Rare Disease Therapeutics, Inc., Franklin, Tenn) was administered to 145 patients. Most were children <5 years old (n = 76, or 54.4%); 27 (18.6%) were 5-9 years old and 42 (30.0%) were ≥10 years of age. About half, 79 of 145 (54.5%) victims who received antivenom, met the APDIC recommended use criteria.Conclusions: Patients treated with antivenom exhibited a rapid resolution of symptoms without immediate or delayed hypersensitivity reactions. We recommend broadened availability of antivenom at sites where it is most needed. [ABSTRACT FROM AUTHOR]

We report a case of a 32 year old patient of peripheral nerve hyper excitability who presented with fasciculation in all four limbs, muscle aches and fatigue. Electromyography study showed spontaneous, continuous activity in the form of discharges with high frequency. His symptoms disappeared when plasma exchange was employed along with steroid administration.

Background and purpose: We evaluated muscle echointensity as a marker for secondary axonal damage in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) using ultrasonography. Findings were correlated with clinical disability and muscular strength. Methods: Eighty patients with CIDP (40 with typical and 40 with atypical CIDP) were examined clinically, including assessment of Medical Research Council (MRC) sum score and Inflammatory Neuropathy Cause and Treatment Overall Disability Sum Score (INCAT‐ODSS). Echointensity in eight proximal and distal muscles of the arms and legs was evaluated by muscle ultrasonography using the Heckmatt scale. Results: Alterations of echointensity occurred most frequently in the distal leg muscles, with a median (range) Heckmatt score of 1.5 (1–4). There were no differences between typical and atypical CIDP patients with regard to Heckmatt score. Alterations of echointensity correlated to disability and muscle strength. The arm score of the INCAT‐ODSS correlated to Heckmatt score for the distal arm muscles (r = 0.23, p = 0.046) and the leg score of the INCAT‐ODSS correlated to Heckmatt scores for the proximal (r = 0.34, p = 0.002) and distal leg muscles (r = 0.33, p = 0.004). MRC sum score, as well as individual MRC scores for arm and leg muscles, correlated to Heckmatt scores of the corresponding muscle groups (r = −0.25, p = 0.02 for MRC sum score). Conclusion: Increased muscle echointensity, reflecting fibrosis and fatty infiltration due to secondary axonal damage, correlated to muscular strength and disability in a large cohort of CIDP patients. Alterations of echointensity occur in both typical and atypical CIDP patients and are pronounced in the distal leg muscles. [ABSTRACT FROM AUTHOR]

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Fenitrothion is one of the most globally used organophosphorus pesticides, which can cause neurological symptoms, including involuntary movements. However, due to the limited number of case report, information on its treatment is also scarce. Here we presented a 74-year-old Japanese woman who was admitted to our hospital due to a persistent nausea and vomiting after ingesting 200 mL of 50% fenitrothion for a suicidal attempt. She received continuous intravenous infusion of atropine and 2-pyridine aldoxime methiodide under mechanical ventilation and continuous hemodiafiltration. However, she developed fasciculations of the face and right arm on day 11, which raised suspicions of delayed neuropathy associated with organophosphorus poisoning. To reduce the risk of respiratory depression, she received intravenous levetiracetam at a dosage of 1,000 mg/day. However, as her fasciculations persisted, the levetiracetam dosage was adjusted to 2,000 mg/day on day 14. On the following day, her fasciculations subsided. Neurologic symptoms of lipid-soluble organophosphorus poisoning, including fenitrothion, can sometimes delay following ingestion. Temporary administration of levetiracetam may prove effective in alleviating fasciculations.

Background: In the current study, we aimed to determine normative values for muscle thickness and fasciculation prevalence in healthy subjects.Methods: We performed a prospective study from October to December 2018 in 65 healthy subjects. All subjects underwent quantitative sonographic evaluation of muscle thickness and fasciculation prevalence in the following 8 muscles: Biceps brachii, abductor pollicis brevis, first dorsal interosseous, abductor digiti minimi, quadriceps, tibialis anterior, extensor digitorum brevis, and abductor hallucis brevis.Results: Subject ages ranged from 21 to 82 years, with 63% women. Normative values for muscle thickness were determined using the fifth percentile. Multivariate regression analysis showed that sex, age, body mass index, and hand dominance affected muscle thickness. Fasciculations were observed frequently only in distal muscles.Conclusions: Normal values for muscle thickness were determined, and may enhance neuromuscular ultrasound sensitivity and serve as a basis for future studies. Larger series are needed to confirm these values. [ABSTRACT FROM AUTHOR]

Oral and maxillofacial procedures can potentially injure one or more cranial nerves, leading to facial nerve palsy. Iatrogenic trauma resulting in facial twitching due to aberrant regeneration of nerve fibers is a potential consequence. However, facial twitches have a broad differential diagnosis and are seen in both benign and life-threatening neurological conditions. Few reports describe twitches like orofacial fasciculations, myokymia, or other movement disorders after maxillofacial surgery. This case series aims to raise awareness of this phenomenon by reviewing three patients with different types of facial twitches after oral and maxillofacial surgery. The literature concerning facial movement disorders and their possible etiology is discussed.

Fasciculation is a minor adverse effect of succinylcholine and may be an unpleasant experience for patient. The aim of this study was to compare the use of atracurium and methocarbamol to decrease the occurrence and severity of succinylcholine-induced muscle fasciculation. Fifty-nine adults with American Society of Anesthesiologists I or II hospitalized for elective surgery were randomly assigned to two groups: Group A (n = 29) who received succinylcholine 1 mg/kg body weight (BW) intravenously followed by 0.2–0.5 mg/kg BW atracurium and patients in Group B (n= 29) who received succinylcholine 1 mg/kg BW intravenously followed by methocarbamol 0.2–0.5 mg/kg BW. Anesthesia was induced in all patients with thiopental sodium 3–5 mg/kg. Fasciculation was scored on a four-point (0–4) Likert scale. There were no statistically significant differences in demographic variables between two groups, whereas in Group A, 27 patients (93.1%) suffered from mild fasciculation and two (6.9%) from moderate fasciculation. In Group B, twenty patients (68.9%) suffered from mild fasciculation, five (17.2%) from moderate fasciculation, and four (13.9%) from severe fasciculation. The difference between the groups was statistically significant (P < 0.05). Atracurium is more effective than methocarbamol in decreasing the occurrence and severity of succinylcholine-induced fasciculations. In addition, the use of methocarbamol before succinylcholine administration can decrease the incidence of severe fasciculation.

Key Clinical Message: The nicotinic symptom in Organo‐Phosphate (OP) poisoning is an unusual presentation in an adult, as in our case of a 38‐year‐old man who also experienced decreased motor power of all limbs and diffuses fasciculation of the tongue and lower limbs. [ABSTRACT FROM AUTHOR]

Neuromuscular ultrasound is complementary to electrodiagnostic (EDx) testing and is useful in enhancing the diagnosis of mononeuropathies, peripheral nerve trauma, and demyelinating polyneuropathies. There is increasing interest in using ultrasound both to aid in the diagnosis of amyotrophic lateral sclerosis (ALS) and to monitor its progression. In this article we review the relevant literature on ultrasound in ALS. Ultrasound is more sensitive than EDx in identifying fasciculations in patients with ALS. It can detect decreased muscle thickness, increased muscle echointensity and echovariance, and reduced peripheral nerve size in these patients. Ultrasound is also a helpful tool in assessment of diaphragm function. Although additional studies are required to define the exact role of ultrasound in the evaluation and monitoring of ALS, it can improve the diagnostic yield in patients when ALS is suspected, but insufficiently supported, by clinical and EDx examinations. Muscle Nerve 60: 114-123, 2019. [ABSTRACT FROM AUTHOR]

Introduction: Fasciculations are a marker for the diagnosis of amyotrophic lateral sclerosis (ALS) and reflect increased lower motor neuron (LMN) excitability.Methods: We investigated modulation of fasciculation frequency in the first dorsal interosseous (FDI) muscle of the right hand following peripheral sensory nerve electrical stimulation, and vibration over the muscle-tendon region (50 and 100 Hz), in patients with ALS, spinal muscular atrophy, and benign fasciculation syndrome. FDI muscles of ALS patients were classified by the presence or absence of neurogenic changes on needle electromyography.Results: Both sensory nerve electrical stimulation and vibration significantly increased the frequency of fasciculations in neurogenic FDI muscles of ALS patients, but not in the remaining groups.Discussion: Our results favour increased excitability of LMNs when affected by the disease process in ALS. We found that some fasciculations originating in these neurons in ALS are susceptible to modulation by sensory input. Muscle Nerve 59:688-693, 2019. [ABSTRACT FROM AUTHOR]

We report a case of a 32 year old patient of peripheral nerve hyper excitability who presented with fasciculation in all four limbs, muscle aches and fatigue. Electromyography study showed spontaneous, continuous activity in the form of discharges with high frequency. His symptoms disappeared when plasma exchange was employed along with steroid administration. [ABSTRACT FROM AUTHOR]

Copyright of Mezdunarodnyj Nevrologiceskij Zurnal is the property of Zaslavsky O.Yu and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Highlights • We developed an automatic algorithm for detecting fasciculations in muscle ultrasound videos. • With algorithm guidance, observers found more fasciculations compared to visual analysis alone. • Our findings affirm the potential clinical usefulness of automated analysis of muscle ultrasound. Abstract Objective To develop an automated algorithm for detecting fasciculations and other movements in muscle ultrasound videos. Fasciculation detection in muscle ultrasound is routinely performed online by observing the live videos. However, human observation limits the objective information gained. Automated detection of movement is expected to improved sensitivity and specificity and increase reliability. Methods We used 42 ultrasound videos from 11 neuromuscular patients for an iterative learning process between human observers and automated computer analysis, to identify muscle ultrasound movements. Two different datasets were selected from this, one to develop the algorithm and one to validate it. The outcome was compared to manual movement identification by clinicians. The algorithm also quantifies specific parameters of different movement types, to enable automated differentiation of events. Results The algorithm reliably detected fasciculations. With algorithm guidance, observers found more fasciculations compared to visual analysis alone, and prescreening the videos with the algorithm saved clinicians significant time compared to reviewing full video sequences. All videos also contained other movements, especially contraction pseudotremor, which confused human interpretation in some. Conclusions Automated movement detection is a feasible and attractive method to screen for fasciculations in muscle ultrasound videos. Significance Our findings affirm the potential clinical usefulness of automated movement analysis in muscle ultrasound. [ABSTRACT FROM AUTHOR]

Highlights • Fasciculations are more frequent in proximal and cervical muscles and in upper limb onset patients. • Fasciculations associate directly with BMI loss and upper motor neuron impairment and inversely with disability. • Fasciculations increase with the initial lower motor neuron impairment but they decrease when it progresses. Abstract Objective To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance. Methods Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90 days) of ALS. The number of fasciculations was recorded in each muscle and the muscle thickness and strength were additionally measured in limb muscles. A subgroup of patients were electromyographically assessed. Results US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations' number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increase with the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy. Conclusions Our study suggests that both UMN and LMN degeneration trigger fasciculations causing BMI loss. The degree of LMN impairment could account for differences in fasciculations' rates within and between muscles. Significance In ALS, fasciculations could explain the link between hyperexcitability and BMI loss. [ABSTRACT FROM AUTHOR]

The purpose of this study was to assess the sonoelastographic features of four different muscles in patients with amyotrophic lateral sclerosis compared with healthy controls and to evaluate the relationship of these features to muscle strength and other ultrasonographic variables. Fourteen patients with amyotrophic lateral sclerosis and 20 controls were examined using strain sonoelastography scanning. The RGB channel fraction ratio was analyzed with ImageJ software (Version 1.48). Two main sonoelastographic patterns could be distinguished in the controls: a clear predominance of the blue channel (hard areas) and a more heterogeneous pattern with predominance of the green channel (intermediate stiffness). These patterns were also observed in patients, although a higher green channel score was observed in mildly impaired muscles, whereas a higher blue channel score was observed in the most severely impaired muscle. Sonoelastography may be a good complementary biomarker in the detection and monitoring of muscle changes in amyotrophic lateral sclerosis. [ABSTRACT FROM AUTHOR]

The development of a functional nervous system requires tight control of neurite growth and guidance by extracellular chemical cues. Neurite growth is astonishingly sensitive to shallow concentration gradients, but a widely observed feature of both growth and guidance regulation, with important consequences for development and regeneration, is that both are only elicited over the same relatively narrow range of concentrations. Here we show that all these phenomena can be explained within one theoretical framework. We first test long-standing explanations for the suppression of the trophic effects of nerve growth factor at high concentrations, and find they are contradicted by experiment. Instead we propose a new hypothesis involving inhibitory signalling among the cell bodies, and then extend this hypothesis to show how both growth and guidance can be understood in terms of a common underlying signalling mechanism. This new model for the first time unifies several key features of neurite growth regulation, quantitatively explains many aspects of experimental data, and makes new predictions about unknown details of developmental signalling. [ABSTRACT FROM AUTHOR]

Introduction: Cranial muscle fasciculations may be difficult to detect in amyotrophic lateral sclerosis (ALS). Ultrasound (US) detection of fasciculations in these muscles may have clinical utility.Methods: Patients with suspected ALS were prospectively enrolled. Nerve conduction studies, needle electromyography (EMG), and US examination of cranial muscles were performed. Controls were examined by US only. Fasciculations were counted and scored for each muscle after 10 or 30 seconds.Results: There were 84 patients with ALS. Fasciculations were most frequently found in the genioglossus muscle. Overall, detection rates by US and EMG were similar, but US was more likely to detect frequent fasciculations. Fasciculations were rare in controls, seen in 7 of 1,090 (0.6%) muscles. No control had > 5 fasciculations in any muscle.Discussion: Fasciculations were frequently detected in cranial muscles of patients with ALS. US was found to be a sensitive method, and was not impaired by factors such as anxiety and the inability of the patient to relax. Muscle Nerve 56: 1072-1076, 2017. [ABSTRACT FROM AUTHOR]

Axon-guidance by Slit-Roundabout (Robo) signaling at the midline initially guides growth cones to synaptic targets and positions longitudinal axon tracts in discrete bundles on either side of the midline. Following the formation of commissural tracts, Slit is found also in tracts of the commissures and longitudinal connectives, the purpose of which is not clear. The Slit protein is processed into a larger N-terminal peptide and a smaller C-terminal peptide. Here, I show that Slit and Slit-N in tracts interact with Robo to maintain the fasciculation, the inter-tract spacing between tracts and their position relative to the midline. Thus, in the absence of Slit in post-guidance tracts, tracts de-fasciculate, merge with one another and shift their position towards the midline. The Slit protein is proposed to function as a gradient. However, I show that Slit and Slit-N are not freely lpresent in the extracellular milieu but associated with the extracellular matrix (ECM) and both interact with Robo1. Slit-C is tightly associated with the ECM requiring collagenase treatment to release it, and it does not interact with Robo1. These results define a role for Slit and Slit-N in tracts for the maintenance and fasciculation of tracts, thus the maintenance of the hardwiring of the CNS. [ABSTRACT FROM AUTHOR]

Objectives We examined the clinical utility of muscle ultrasound (MUS) in detecting fasciculations in patients with nerve and muscle disorders (NMD) and investigated the impact on diagnostic sensitivity when combining electromyography (EMG) and MUS. Methods We included 58 consecutive patients suspected to have NMD and 38 healthy subjects (HS). Patients and HS underwent MUS in 14 skeletal and two bulbar muscles and the video recordings of the MUS were anonymised. Only patients underwent EMG. Results The follow-up diagnoses were: 15 Amyotrophic lateral sclerosis (ALS), 15 polyneuropathy, 14 patients had other diagnoses (disease-control group) and 14 patients had no pathological findings. MUS detected more muscles with fasciculations among ALS patients compared to all other groups. In ALS patients, the dominating pattern of fasciculations was continuous (45%). More proximal muscles showed fasciculations among ALS patients compared to all other patient groups. MUS was more sensitive than EMG in detecting fasciculations (58% vs. 48%). When combining the two methods, the sensitivity in detecting fasciculations increased to 65%. Fasciculations in nine muscles could predict the ALS diagnosis with high sensitivity and specificity. Conclusions MUS is a sensitive tool in detecting fasciculations in patients with NMD and performs well compared to EMG in diagnosing ALS. Significance MUS may add valuable information in the clinic, especially in diagnosing ALS. [ABSTRACT FROM AUTHOR]

Resumo Justificativa e objetivos A ação epidural de agentes bloqueadores neuromusculares pode ser explicada à luz de suas características físico‐químicas e propriedades do espaço epidural. Na literatura existem poucos casos sobre a administração acidental em espaço epidural de agente neuromuscular que se manifesta principalmente com a instituição de bloqueio neuromuscular ou fasciculações. Relato de caso Relatamos um caso de administração acidental de succinilcolina como uma dose teste epidural em uma paciente submetida à laparotomia programada, sob anestesia combinada geral e peridural. Aproximadamente dois minutos após a injeção de succinilcolina, a paciente queixou‐se de falta de ar, enquanto fasciculações leves apareceram em seu tronco e rosto, tratadas com a instituição imediata de anestesia geral. Exceto pela duração relativamente longa do bloqueio neuromuscular em comparação com a administração intravenosa, sequelas neurológicas ou cardiovasculares ou outros sintomas de toxicidade local ou sistêmica não foram observados. Conclusões A administração oral de diazepam parece diminuir os efeitos adversos da administração epidural acidental de succinilcolina. A meticulosidade e discriminação dos rótulos das seringas, bem como os cuidados persistentes mantidos durante todos os procedimentos de anestesia, continuam a ser de importância crucial. Background and objectives Epidural action of neuromuscular blocking agents could be explained under the light of their physicochemical characteristics and epidural space properties. In the literature there are few cases of accidental neuromuscular agent's epidural administration, manifesting mainly with neuromuscular blockade institution or fasciculations. Case report We report a case of accidental succinylcholine administration as an epidural test dose, in a female patient undergoing scheduled laparotomy, under combined general and epidural anesthesia. Approximately 2 min after the succinylcholine injection the patient complained for shortness of breath, while mild fasciculations appeared in her trunk and face, managed by immediate general anesthesia institution. With the exception of a relatively longer duration of neuromuscular blockade compared with intravenous administration, no neurological or cardiovascular sequelae or other symptoms of local or systemic toxicity were observed. Conclusions Oral administration of diazepam seems to lessen the adverse effects from accidental epidural administration of succinylcholine. The meticulous and discriminative labeling of syringes, as well as keeping persistent cautions during all anesthesia procedures remains of crucial importance. [ABSTRACT FROM AUTHOR]

Fasciculation is a minor adverse effect of succinylcholine and may be an unpleasant experience for patient. The aim of this study was to compare the use of atracurium and methocarbamol to decrease the occurrence and severity of succinylcholine-induced muscle fasciculation. Fifty-nine adults with American Society of Anesthesiologists I or II hospitalized for elective surgery were randomly assigned to two groups: Group A (n = 29) who received succinylcholine 1 mg/kg body weight (BW) intravenously followed by 0.2-0.5 mg/kg BW atracurium and patients in Group B (n = 29) who received succinylcholine 1 mg/kg BW intravenously followed by methocarbamol 0.2-0.5 mg/kg BW. Anesthesia was induced in all patients with thiopental sodium 3-5 mg/kg. Fasciculation was scored on a four-point (0-4) Likert scale. There were no statistically significant differences in demographic variables between two groups, whereas in Group A, 27 patients (93.1%) suffered from mild fasciculation and two (6.9%) from moderate fasciculation. In Group B, twenty patients (68.9%) suffered from mild fasciculation, five (17.2%) from moderate fasciculation, and four (13.9%) from severe fasciculation. The difference between the groups was statistically significant (P < 0.05). Atracurium is more effective than methocarbamol in decreasing the occurrence and severity of succinylcholine-induced fasciculations. In addition, the use of methocarbamol before succinylcholine administration can decrease the incidence of severe fasciculation. [ABSTRACT FROM AUTHOR]

Until early 80′s most patients presenting with lower motor neuron syndrome characterized by weakness, wasting, diminished reflexes, fasciculations, cramps and minimal sensory involvement would have been diagnosed as motor neuron disease- LMN variant. But similar clinical picture can be seen in various disorders right from degenerative illnesses like motor neuron disease to inflammatory ones like multifocal motor neuropathy (MMN). Some of them are potentially treatable conditions with immunomodulatory therapies and are associated with characteristic electrophysiological features and high serum antibody titers. Thus every patient presenting with pure motor LMN weakness and fasciculations should not be labeled as motor neuron disease and extensive electrophysiological search is warranted to diagnose these potentially treatable conditions, changing the grim prognosis to more favorable one.

Introduction: Fasciculations, the spontaneous activity of single motor units (MUs) are characteristic, but nonspecific for motor neuron disease (MND). We aimed to identify MU discharge properties to optimally differentiate MND patients from healthy controls.Methods: High-density surface electromyography recordings were performed in the thenar muscles during 10 min of rest. MU discharges were classified as "isolated" when the interspike intervals (ISIs) before and after were > 250 ms, "continual" when both ISIs were ≤ 250 ms, or as "other".Results: In patients (n = 30) compared with controls (n = 14), more MUs were active (9 vs. 3, P < 0.001) and generated relatively more isolated discharges (35% vs. 10%, P = 0.01). Two or more MUs with isolated discharges occurred more frequently in patients compared with controls (24% vs. <1% of 10-s windows, P < 0.001).Conclusions: More frequent occurrence of multiple MUs showing isolated discharges may improve identification of patients with MND. [ABSTRACT FROM AUTHOR]

Introduction: Benign fasciculations are common. Despite the favorable prognosis of benign fasciculation syndrome (BFS), patients are often anxious about their symptoms. In this study, we prospectively followed 35 patients with BFS over a 24-month period.Methods: We conducted serial questionnaires to assess anxiety, associated symptoms, and duration.Results: 71.4% of patients were men, and 34.4% were employed in the medical field. Most reported anxiety, but only 14% were anxious as measured by the Zung self-rating anxiety scale. Fasciculations were most common in the calves and persisted in 93% of patients. Anxiety levels did not change over time. Associated symptoms (subjective weakness, sensory symptoms, and cramps) were common and resolved to varying degrees. No patients developed motor neuron disease.Discussion: BFS is a benign disorder that usually persists over time. Commonly associated symptoms include subjective weakness, sensory symptoms, and cramps. BFS is usually not associated with pathologic anxiety. Muscle Nerve 58:852-854, 2018. [ABSTRACT FROM AUTHOR]

We report the case of a 54-year-old right-handed man who presented with a 2-year history of progressive upper-limb weakness with mild dysarthria and prominent involuntary perioral abnormal movements that were characterized as fasciculations. Electromyography disclosed motor neuron disease. The diagnosis of Kennedy's disease was established by polymerase chain reaction. Perioral abnormal movements and fasciculations may represent important clinical clues to the diagnosis of Kennedy's disease, particularly when associated with proximal muscle atrophy and gynecomastia. In suspected cases, genetic testing for elevated CAG repeats in the androgen receptor Xq12 gene is warranted. [ABSTRACT FROM AUTHOR]

Our objective was to explore if creatine kinase (CK) levels correlate with survival in amyotrophic lateral sclerosis (ALS), and whether a correlation is independent of other well-studied predictors such as location of onset, gender, age, fat free mass, spasticity, cramps, and fasciculations. We analyzed data from 80 ALS patients from a 48-week non-interventional longitudinal multicenter nutrition study with long term follow-up. The overall mean CK was 214 ± 191.8 U/l (range 22–1992 U/l). Forty-five percent of patients had at least one high CK value (> 200 U/l), and about half maintained a high CK value, but there was no trend over the study period. Male gender and extremity onset were significantly associated with high CK. In univariate analysis, age, bioelectric impedance spectroscopy (BIS) fat free mass, spasticity, and fasciculations were not associated with CK level. There was an association between CK and muscle cramps (p <0.001). In survival analysis, low CK (≤ 200 U/l) was associated with a longer overall survival (p =0.02), when adjusting for location of onset, age, race, gender, BIS fat free mass, and study site. In conclusion, CK may be a useful marker for ALS survival, which has implications for clinical care and the design of future clinical trials. [ABSTRACT FROM AUTHOR]

Objective. The goal of this study is to make a connection between physical exercise and clinical features of fasciculation anxiety syndrome. We randomized a cohort of 30 patients in three groups: first group included 10 patients with symptomatic fasciculation and anxiety, second group included 10 patients fasciculations associated with sensory symptoms or muscle weakness and were diagnosed with neuropathy or SLA and the third group included 10 patients with fasciculations associated with cramps ( cramp- fasciculation-syndrome). All three groups were included in a 3 times/week programme of exercises of 30 minutes each and we evaluated the electomyographic changes. Methods: We randomized 30 patients in three groups: first group included 10 patients with symptomatic fasciculation and anxiety, second group includes 10 patients fasciculations associated with sensory symptoms or muscle weakness and were diagnosed with neuropathy or SLA and the third group included 10 patients with fasciculations associated with cramps ( cramp - fasciculation-syndrome). All three groups were included in a 3 times/week programme of exercises of 30 minutes each on a 3 month period of following and we evaluate electomiographic changes. Results: In the present study, a cohort of 30 patients with sympthomatic fasciculations was assessed with clinical, neurophysiological and laboratory studies.The majority of cases with fasciculations were female. Triggers in all 3 groups was stress, caffeine and exercises. Anxiety appeared as a proeminent feature in the patients in the present cohort and may contribute to pathogenesis of symptomatic fasciculations in symptomatic fasciculation and anxiety, acting to promote fasciculations in Symptomatic fasciculation and anxiety. Conclusions: The present study described a cohort of 30 patiens presenting for evaluation of fasciculations and identified group which includes 10 patients with symptomatic fasciculation and anxiety, second group which includes 10 patients fasciculations associated with sensory symptoms or muscle weakness and were diagnosed with neuropathy or SLA and the third group which includes 10 patients with fasciculations associated with cramps ( crampfasciculation- syndrome). All three groups were included in a 3 times/week programme of exercises of 30 minutes each and we evaluate electomiographic changes. In the Symptomatic fasciculation and anxiety cramp- fasciculation-syndrome role of exercise appear to aggravate their simptomatology, but in the second group of patients with neuropathy and lateral amyotrophic sclerosis role of exercise is unclear but for sure it doesn't have a negative role. [ABSTRACT FROM AUTHOR]

We report on the clinical characteristics of amyotrophic lateral sclerosis (ALS) in Fortaleza (Northeastern Brazil). For this, we analyzed retrospectively (from 1980 to 1999) 78 cases of ALS from the Service of Neurology of the University Hospital of Fortaleza diagnosed clinically and laboratorially (EMG, muscle biopsy, myelography, blood biochemistry, muscle enzymes and cranio-cervical X-ray). The results showed that they were mostly sporadic ALS (76/78), and they were divided into definite (n= 36), probable (n= 20), possible (n= 15) and suspected (n= 7), according to the level of diagnostic certainty. They were also subdivided into juvenile (n= 17), early-onset adult (n= 18), age-specific (n= 39) and late-onset (n= 4) groups. Clinically, they presented as initials symptoms, principally, asymmetrical (30/78) and symmetrical (24/78) weakness of extremities, besides bulbar signs, fasciculations, and atrophy. Curiously, pain as first symptom occurred in an expressive fashion (17/78). The predominant initial anatomic site, in this series, was the spinal cord, and mainly affecting the arms. As to the symptom accrual from region to region, this occurs more quickly in contiguous areas, and fasciculations are predominant when bulbar region was associated.

Objective The aim of the study was to confirm the diagnostic performance of dynamic myosonography with regard to its reliability to correctly identify fasciculations and to distinguish them from artefacts. Furthermore, interrater agreement regarding the identification of different muscle movements was investigated. Methods A total of 11 observers analysed 25 muscle ultrasound videos acquired using a standardized protocol. The video files illustrated fasciculations and artefacts (voluntary probe movements, voluntary contractions or swallowing and pulsating vessels) in different muscle groups. Results Fasciculations could be distinguished from artefacts with a sensitivity of 90.9% and specificity of 98.5%. Interrater agreement regarding the presence or absence of fasciculations showed an overall median of 100% (interquartile range, IQR: 96–100%). In every investigated muscle group, the median of the interpreter agreement was found to be 100% (correct ratings of all observers: submental muscles: 43 of 44; biceps muscles: 22 of 22; forearm flexors: 31 of 33; rectus abdominis muscles: 33 of 33; quadriceps muscles: 19 of 22; tibialis anterior muscles: 51 of 55; undefinable muscles: 65 of 66). Conclusion Dynamic myosonography is an extremely reliable tool with excellent interrater agreement to correctly identify fasciculations and to distinguish them from artefacts. Significance Myosonography should be further incorporated in clinical routine diagnostic work-up. [ABSTRACT FROM AUTHOR]

Ultrasound detection of muscle fasciculations was recently proposed for assessing lower motor neuron (LMN) dysfunction in ALS patients. Given the continuum between ALS and frontotemporal degeneration (FTD), the aim of the present study was to evaluate muscle ultrasound (MUS) in FTD both for feasibility and prevalence of fasciculations. Twenty-two FTD patients were examined (five muscles bilaterally: biceps brachii, first dorsalis interosseous, T10 paraspinalis, vastus lateralis, tibialis anterior) with a 7-MHz linear array transducer and a fasciculation score (FS) computed. Twenty-two matched cognitively-intact control subjects and six ALS patients were also included. Results showed that MUS was feasible, reliable and well tolerated in all subjects. Two FTD/MND patients displayed very high FS values, similar to those in ALS patients. The remaining 20 FTD patients displayed a mean FS value significantly higher than the control group with six patients (30%) having FS values out of the range of controls. Disease progression rate correlated with the FS. In conclusion, MUS can be easily applied to FTD patients and represents a non-invasive technique for defining LMN involvement in these patients. LMN dysfunction is a frequent condition in FTD and might identify a subset of patients with a different clinical course. [ABSTRACT FROM AUTHOR]

Fasciculations are visible, fine and fast, sometimes vermicular contractions of fine muscle fibers that occur spontaneously and intermittently. The aim of this article is to discuss the main causes for fasciculations and their pathophysiology in different sites of the central/peripheral injury and in particular to disprove that the presence of this finding in the neurological examination is indicative of amyotrophic lateral sclerosis. Undoubtedly, most fasciculations have a distal origin in the motor nerve both in normal subjects and in patients with motor neuron disease. Most of them spread to other dendritic spines often producing an antidromic impulse in the main axon. The clinical and neurophysiological diagnosis must be thorough. It may often take long to record fasciculations with electroneuromyography. In other cases, temporal monitoring is necessary before the diagnosis. The treatment, which may be adequate in some cases, is not always necessary. [ABSTRACT FROM AUTHOR]