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

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

[Cramps and fasciculations: is it amyotrophic lateral sclerosis?] / Crampes et fasciculations : est-ce une sclérose latérale amyotrophique ?

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease of the adult age. It is an aggressive condition with a mean disease duration of only 3 to 5 years, characterized by progressive weakness and atrophy of limb, bulbar, and respiratory muscles. In general, death is caused by chronic hypoventilation due to respiratory insufficiency. No causal treatment is known today, but the two therapeutic agents authorized in Switzerland for the treatment of ALS can slow disease progression significantly. Other important therapeutic strategies include invasive/non-invasive ventilation, pain therapy, as well as physio-, ergo- and speech therapy on a regular basis.

La sclérose latérale amyotrophique (SLA) est la maladie du motoneurone la plus fréquente de l'adulte. C'est une maladie sévère (la survie moyenne est d'environ 3 à 5 ans), caractérisée par une dégénérescence des premier et deuxième motoneurones. Elle se manifeste par un déficit moteur amyotrophiant progressif des membres, de la langue, des muscles bulbaires et respiratoires. En général, le décès est causé par une hypoventilation chronique. Il n'existe actuellement aucun traitement curatif. Les deux médicaments autorisés en Suisse peuvent ralentir significativement la progression de la maladie et plusieurs nouvelles molécules sont à l'essai. Les traitements non médicamenteux/symptomatiques constituent le deuxième pilier de la prise en charge : ventilation non invasive, traitement des symptômes bulbaires, stabilisation du poids, physio et ergothérapie.

Fasciculation intensity and limb dominance in amyotrophic lateral sclerosis: a muscle ultrasonographic study.

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 (ß = 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.

Fasciculaciones y calambres: bases fisiológicas y enfrentamiento clínico de un fenómeno complejo / Fasciculations and cramps: physiological bases and clinical approach of a complex phenomenon

Fasciculations and cramps originate in the motor unit, a functional unit that includes the lower motor neuron and their innervated muscle fibres. Both are common complaints in outpatient practice. These symptoms can be secondary to neurological or medical pathology, presenting a broad differential diagnosis and a complex approach. Recent neurophysiological studies have increased the knowledge of their origin mainly in amyotrophic lateral sclerosis. The symptomatic management of fasciculations and cramps depends on their etiology and includes pharmacological and non-pharmacological treatments. This article aims to present an updated review of the most relevant aspects of physiopathology, clinical approach, and differential diagnosis of both phenomena.

Fasciculations in Children.

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.

Characterization of Fasciculation Potentials (FPs) in Amyotrophic Lateral Sclerosis (ALS) and Peripheral Nerve Hyperexcitability Syndromes (PNH).

This study is aimed at investigating the features of fasciculation potentials (FPs) in amyotrophic lateral sclerosis (ALS) and peripheral nerve hyperexcitability syndromes (PNH). Needle electrophysiologic examination (EMG) was performed for 5-15 muscles in the ALS and PNH patients. The spontaneous activity of fasciculations and fibrillations/sharp-waves (fibs-sw) was recorded. The distribution, firing frequency, and waveform parameters of FPs in muscles were calculated and compared. In total, 361 muscles in ALS patients and 124 muscles in PNH patients were examined, with the FP detection rates of 45.1% and 53.2%. Moreover, the ALS patients with the upper limb onset had the highest FP detection rate. Fasciculations occurred more frequently in the upper limbs than in the lower limbs in ALS and PNH. The detection rate of fibs-sw in the bulbar muscle was relatively low, which could be elevated when combining fibs-sw and FPs. Benign FPs in PNH were of smaller amplitude, shorter duration, and fewer phases/turns, compared with malignant FPs in ALS. The FP area in PNH was significantly smaller than that in ALS. The incidence of polyphasic FPs in ALS was distinctly greater than that in PNH. The firing frequency of FPs in PNH was higher than that in ALS. There was no significant difference in the amplitude, duration, phases and turns, and area of FPs between groups with and without fibs-sw in the muscles of normal strength in ALS. Conclusively, it is necessary to detect the FPs in the thoracic and bulbar muscles of patients suspected having ALS. FP parameters in ALS are significantly different from PNH.

Fasciculation score: a sensitive biomarker in amyotrophic lateral sclerosis.

OBJECTIVE: The aim of our study was to elucidate the characteristic of fasciculation distributions in amyotrophic lateral sclerosis (ALS) using a fasciculation score (FS) of muscle ultrasound (MUS) and to compare the diagnostic values of three MUS fasciculation parameters in patients. METHODS: Thirty ALS patients, 16 ALS mimics, and 10 healthy subjects were involved. MUS of unilateral 10 muscles in each patient and needle electromyography (EMG) of total 204 muscles were performed to detect fasciculations and spontaneous activity respectively in ALS. Control groups underwent only MUS. Fasciculation was graded semiquantitatively with FS. RESULTS: Three hundred fifty muscles in ALS and 260 in controls were examined. The fasciculation detection rates, total FS, the number of muscles with fasciculation, and the total number of fasciculations in ALS were all significantly higher than those of controls (P < 0.001). ALS patients exhibited a multifocal continuous pattern of fasciculation in limbs, whereas there were few fasciculations in controls. Compared with other parameters, total FS had the largest area under the curve (AUC) (AUC = 0.899, P < 0.001) in ALS diagnosis. The detection rates of lower motor neuron (LMN) acute lesions by MUS (70.6%) and EMG (72.1%) were nearly the same, and a positive correlation between the FS and spontaneous activity grades (P < 0.001, r = 0.359) was proved. CONCLUSIONS: ALS patients exhibited the multifocal continuous pattern of fasciculation in limbs. FS showed high sensitivity and specificity in differentiating ALS from non-ALS patients, and the optimal cut-off value was determined as 4. The combination of MUS and EMG can provide additional information about specific muscles.

Utility of transoral motion-mode ultrasonography to detect tongue fasciculation in patients with amyotrophic lateral sclerosis.

INTRODUCTION: Increasing evidence suggests the utility of the submandibular approach for ultrasonography to detect tongue fasciculation in amyotrophic lateral sclerosis (ALS). We hypothesized that transoral motion-mode ultrasonography (TOMU) would be useful to detect tongue fasciculation in patients with ALS. METHODS: Patients with sporadic ALS showing clinically definite tongue fasciculation were enrolled, and the ultrasonography findings of patients' tongues on TOMU and ultrasonography by the conventional submandibular approach were analyzed. RESULTS: Six patients with clinically definite ALS were enrolled in this study. Although small, irregular muscle movements of 5 to 10 mm in amplitude and 0.1 to 0.2 second in duration were detected in all patients by TOMU, similar muscle movements were detected in only two of the six patients by the submandibular approach. DISCUSSION: TOMU appeared to be useful for detecting tongue fasciculation in ALS patients. Further study is needed to better determine its role as a diagnostic tool for ALS.

[Fasciculations and cramps: physiological bases and clinical approach of a complex phenomenon]. / Fasciculaciones y calambres: bases fisiológicas y enfrentamiento clínico de un fenómeno complejo.

Fasciculations and cramps originate in the motor unit, a functional unit that includes the lower motor neuron and their innervated muscle fibres. Both are common complaints in outpatient practice. These symptoms can be secondary to neurological or medical pathology, presenting a broad differential diagnosis and a complex approach. Recent neurophysiological studies have increased the knowledge of their origin mainly in amyotrophic lateral sclerosis. The symptomatic management of fasciculations and cramps depends on their etiology and includes pharmacological and non-pharmacological treatments. This article aims to present an updated review of the most relevant aspects of physiopathology, clinical approach, and differential diagnosis of both phenomena.

Muscle ultrasonography in the diagnosis of amyotrophic lateral sclerosis.

Objectives: The aims of this study were to investigate the differences of fasciculations detected by muscle ultrasonography (MUS) among patients with amyotrophic lateral sclerosis (ALS), patients with ALS mimics and healthy controls, and to propose a simplified MUS fasciculation score for the diagnosis of ALS.Methods: We included 16 patients with ALS (ALS group), 10 patients with ALS mimics (disease-control group), and 10 healthy adults (healthy control group). Subjects underwent MUS in 11 muscles, including the tongue, and bilateral upper trapezius, biceps brachii, abductor pollicis brevis, rectus femoris, and tibialis anterior.Results: The number of muscles with fasciculations per person was more in the ALS group (6.44 ± 2.56) than in the disease-control group (1.20 ± 1.87, P = 0.001) and healthy control group (0.50 ± 1.08, P < 0.001). Fasciculations in 3 of 11 muscles could predict the ALS diagnosis with high sensitivity (88.2%) and specificity (94.7%).Conclusions: Fasciculations detected by MUS can be a simple and useful diagnostic tool for ALS.

Chronic inflammatory demyelinating polyneuropathy with tongue fasciculation: A case report.

Chronic inflammatory demyelinating polyneuropathy is one of the immune-mediated polyneuropathies responsive to immunotherapy. Its usual clinical presentation is a chronic course of symmetric sensorimotor affectation of both proximal and distal extremities with signs of demyelination on electrophysiologic studies. Cranial nerve signs in this condition is not commonly encountered, particularly involvement of the hypoglossal nerve. To date, there are only three published cases of hypoglossal involvement in this condition. This case report presents a patient with tongue fasciculation, which is an uncommon finding in chronic inflammatory demyelinating polyneuropathy. This paper highlights the importance of considering chronic inflammatory demyelinating polyneuropathy in the differential diagnoses of a patient with tongue fasciculation as it has been found to be responsive to immunotherapy in comparison to other lower motor neuron syndromes.

Fasciculating toxicity.

Organophosphorus (OP) compound poisoning with suicidal intent is common. It is one of the frequent cause for admission to hospital Emergencies and intensive care units in our region. We describe here a case of 19-year old girl who presented to the Emergency Department with atypical features of OP poisoning. She had perioral, tongue and lower limb fasciculations along with generalized muscle weakness with no or minimal muscarinic effects. OP poisoning with isolated nicotinic receptor mediated effect is often reported in children but in adult it is extremely rare. Based on history and clinical suspicion of nicotinic receptor mediated effect of OP, she was given intravenous atropine along with other supportive treatment. Patient got completely recovered from fasciculations and her motor weakness improved after 6 h of atropine therapy. Emergency physician should keep a high index of suspicion of isolated nicotinic and ganglionic mediated effect of OP and a trial of atropine should be given to the patient.

Slow, slurred speech as an initial complaint in amyotrophic lateral sclerosis.

OBJECTIVE: Otorhinolaryngologic examinations at an early stage, particularly those conducted by vocal specialists, can make potentially important contributions to the diagnosis of bulbar-onset ALS patients. METHODS: We analyzed 2623 patients (2010-2017) visited the ENT Voice Clinic, National Hospital Organization Tokyo Medical Center, with the primary complaint of speech or vocal dysfunction at the initial visit. Among those, 12 patients visited the voice clinic after consultations with other physicians but before receiving a diagnosis and we initially suspected bulbar-onset ALS due to slow, slurred speech (SSS). We analyzed the detail of those suspected ALS cases. RESULTS: Every patient suspected ALS patients consulted an average of 2.2 physicians before visiting the voice clinic and a total of 3.2 physicians before receiving the final diagnosis. The mean speech symptom duration before visiting the vocal clinic was 7.83 months in ALS, 24 months in MSA patients. The duration until final diagnosis after we referred them to neurologists was 2.16 months and 15.3 months, respectively. CONCLUSION: Otolaryngologists and primary care physicians to consider the possibility of ALS when patients present even with an only symptom of SSS. They should then refer such patients to neurologists for definitive diagnoses, leading to early detection and treatment of ALS.

New insights into the pathophysiology of fasciculations in amyotrophic lateral sclerosis: An ultrasound study.

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.