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Progressive neurogenic muscle atrophy with fasciculation in mice treated with IDPN.

Spontaneous discharges recorded on the EMG appeared in experimental mice given repeated doses of IDPN according to a time schedule. Spontaneous discharges showing a polyphasic pattern and duration of over 10 ms were considered to be fasciculations. One group of mice, treated with IDPN for 4 weeks became active with an increase in muscle volume 2 months after initiation of experiment; spontaneous discharges were recorded in this group at 3-4 weeks after first IDPN injection and disappeared at 4 months. Histologically, neurogenic muscle atrophy, in terms of group atrophy of muscles, was seen in muscles of mice exhibiting spontaneous discharge patterns. Peripheral nerves showed marked atrophy with degeneration. Dissociation of neurofilaments and other organelles was observed. Axonal swellings, filled with numerous maloriented neurofilaments, were confirmed in the lumbar cord. IDPN given repeatedly to mice at a certain dose causes degeneration of the peripheral nerves and results in neurogenic muscle atrophy.

Action Potentials↗

Fasciculation potentials in foot and leg muscles of healthy young adults.

The occurrence of fasciculation potentials (FPs) was studied in healthy subjects aged 18-25. In 25 males and 25 females 3 intrinsic foot muscles, the tibialis anterior and the gastrocnemius muscles on both sides were monitored with surface electrodes for 2 min periods. Only potentials with a peak-to-peak amplitude of at least 50 microV were counted. The number of FPs per minute (FPs/min) was significantly higher in the abductor hallucis (AH) and significantly lower in the tibialis anterior as compared to all other muscles (P < 0.001). Men had significantly more FPs in the AH than women (P < 0.05). In all subjects FPs were found in at least 1 AH. Cooling of the foot did not influence the numbers of FPs/min in the foot muscles. To study diurnal variation, all 5 muscles on both sides were monitored 3 times/day on 10 different days in another 10 subjects (5 males, 5 females). Only in the tibialis anterior did the number of FPs never exceed 3/min. In the other muscles considerable fluctuations were found, especially in the AH, where more than 100 FPs/min were occasionally recorded. In the course of the day a significant (P = 0.05) decrease in FPs/min was found for the AH muscle. In 8 subjects there was a significant correlation between the numbers of FPs in the left and right AH during successive recordings. This indicates that an, as yet unknown, general factor determines the fluctuations in numbers of FPs.

Adolescent↗

EMG spike trains of succinylcholine-induced fasciculations in myalgic patients.

Single spike activity from the surface electromyogram (EMG) of fasciculations induced by succinylcholine (Sch) were studied from limb muscles (biceps, triceps, anterior tibialis and gastrocnemius) in 100 female patients. About 2/3 of them (n = 72) also received nondepolarizing neuromuscular pretreatment (atracurium or vecuronium). We observed from 20% of EMG records in the myalgic (but not in the nonmyalgic) patients, sustained spike trains (mean duration 1.47 s) that resembled motor units firing at physiologically high rates (mean 21.7 spikes/s). The finding reflects Sch's distal actions at the muscle spindle. The implications for myalgia and the possible involvement of micro damage at the extrafusal muscles are discussed.

Action Potentials↗

Myasthenia gravis--a rare presentation with tongue atrophy and fasciculation.

We report the case of an unusual presentation of myasthenia gravis with tongue atrophy and fasciculation. Myasthenia gravis is an autoimmune condition associated with weakness and fatigability of voluntary muscles. In >50%, the initial symptoms and signs are related to extraocular muscle weakness, such as diplopia or ptosis [Tsung K, Seggev JS. An unusual cause of dysphagia. West J Med 1995; 163: 159-60]. Rarely, it is known to affect bulbar muscles and can lead to dysphagia and respiratory compromise.

Aged↗

Succinylcholine-induced fasciculations in denervated rat muscles as measured using 31P-NMR spectroscopy: the effect of pretreatment with dantrolene or vecuronium.

BACKGROUND: We have previously demonstrated by 31P nuclear magnetic resonance (NMR) that succinylcholine (SCh) induces metabolic changes in denervated muscle. To specify those changes, we attempted to inhibit them using two different kinds of drugs, dantrolene and vecuronium. METHODS: Three weeks after unilateral sciatic nerve section, 75 male Wistar rats were randomly assigned to one of the following 5 groups: (1) non-pretreated normal muscle group; (2) non-pretreated denervated muscle group; (3) denervated muscle group pretreated with a low dose of vecuronium (0.02 mg.kg(-1)); (4) denervated muscle group pretreated with a high dose of vecuronium (0.2 mg.kg(-1)); (5) denervated muscle group pretreated with dantrolene (2 mg.kg(-1)). The change of the inorganic phosphate/phosphocreatine (Pi/PCr) ratio of each muscle was measured by 31P-NMR before and after SCh (1 mg.kg(-1)) administration and the corresponding peak amplitude of the electromyograms (EMG) was determined. RESULTS: The high dose of vecuronium totally inhibited SCh-induced fasciculation on EMG (100%-->2%). In this group, though the Pi/PCr ratio significantly increased 10 min after SCh, the peak after 5 min disappeared. The inhibition with dantrolene was about the same order of magnitude as with the low dose of vecuronium (35%:21%). However, the increase in the Pi/PCr only lasted about 10 min, in contrast to the other drugs. CONCLUSION: Our findings indicate that the Pi/PCr increases 5 and 10 min after SCh, respectively, as a result of two different processes. The first peak is caused by an excessive energy consumption in response to excessive muscle contraction. This in turn triggers the second peak, caused by breakdown of glycogen, initiated by an increased Ca2+ concentration.

Animals↗

Fasciculation and focal loss of nerve accommodation in peripheral neuropathies.

Fasciculation of muscle and repetitive response of single motor units to brief electric stimuli in diffuse demyelinative and focal (compression) neuropathies are associated with localised loss of accommodation of motor nerve fibres. The pathogenesis of spontaneous and triggered repetitive firing of neurones is discussed and it is proposed that the discrete sites of abnormally low accommodation may be in regenerating (sprouting) neurones. An improved rapid method for measuring single fibre accommodation may be used to identify regenerating neurones.

Alcoholism↗

Fibrillation potentials, positive sharp waves and fasciculation in the intrinsic muscles of the foot in healthy subjects.

The extensor digitorum brevis and abductor digiti minimi muscles were examined bilaterally with electromyography in 53 healthy subjects. In 72% of the subjects either fibrillation potentials, positive sharp waves or fasciculation was seen in at least one muscle examined. These slight, usually symmetric neuropathic signs are believed to be associated with normal aging and to some extent also with external trauma to the nerves and muscles in the distal parts of the foot.

Adolescent↗

The muscular pain-fasciculation syndrome.

Five cases of a chronic neuromuscular syndrome consisted of muscular aching and sometimes burning pain, fasciculations, cramps, fatigue, and occasional paresthesia. The disorder affected the legs and, less commonly, the girdle, trunk, and arm muscles. The symptoms were enhanced by physical activity and were usually improved by rest. Neither muscular wasting nor weakness was found, although the condition was present for an average of 4.7 years and, in one patient, as long as 10 years. Electrophysiologic studies showed motor abnormalities indicative of axonal degeneration and muscle fiber denervation, most marked in the legs. Light microscopy of skeletal muscle and spinal cord in one case disclosed evidence of mild denervation atrophy in muscle, but no loss of anterior horn cells. The findings are compatible with a benign polyneuropathy.

Adult↗

Slow resolution of multifocal weakness and fasciculation: a reversible motor neuron syndrome.

A 25-year-old man with multifocal weakness and fasciculation was thought to have motor neuron disease. Signs progressed for 1 year, plateaued, and 3 years later resolved almost completely. There was no evidence of paraproteinemia, lymphoproliferative disorder, or vasculitis, and myelography was normal. Electrodiagnostic study disclosed multifocal, acute and chronic denervation that evolved into a picture consistent with residuals of old multifocal radiculopathy without active denervation. Prolongation of F response, absence of H-reflex, and conduction block in a proximal nerve segment suggested multifocal demyelination. A proximal motor neuropathy, perhaps demyelinating, may cause some of the benign motor neuron syndromes that simulate motor neuron disease.

Adult↗

Two consecutive fasciculation potentials having different motor unit origins are an electromyographically pathognomonic finding of ALS.

To electrophysiologically diagnose amyotrophic lateral sclerosis (ALS), fasciculation potentials (FPs) were evaluated in each wasted muscle in 12 ALS and 14 other neurogenic disorders (non-ALS patients). Various types of FPs were observed in ALS. The number of discharged FPs and firing rate of FPs were significantly increased in ALS compared to those in non-ALS. These findings indicate that more motor units take part in discharging FPs in ALS than in non-ALS, and that injured lower motor neurons exhibit hyperexcitability. Although nine of 12 ALS patients showed two consecutive FPs having different motor unit origins, non-ALS patients did not show such FPs. Since the two consecutive FPs having different motor unit origins reflect an involvement of both upper and lower motor neurons, these potentials are an electrophysiologically pathognomonic finding to ALS.

Action Potentials↗

[Syndrome of painful muscle fasciculations. Case report and differential diagnostic review].

The case of a patient with spontaneous painful muscular activity is described. We diagnosed the muscular pain fasciculation syndrome. Biopsy of the sural nerve and electrophysiological examination provided evidence of the neurogenic nature of the disturbance showing a remarkable axonal degeneration. In addition, when immunohistochemical studies were performed, the detection of immunoglobulins and complement components suggested an immunological process in the etiology of the disease. The patient made a good response to carbamazepine therapy. This effect is found in various syndromes of abnormal continuous muscle activity. Clinical, neurophysiological and morphological features of this case are discussed in relation of pathogenesis, possible drug effects and related diseases.

Adult↗

Fasciculation frequency changes at different length of relaxed muscle.

We have studied, in chronically denervated muscles, fasciculation potentials (FPs) which were clearly identified when the muscles were either relaxed or passively stretched. The frequency of 10 FPs was determined in both positions in seven patients. In stretched muscles, two FPs were no longer recorded, and the mean FP frequency decreased significantly (P < .01). There was also a decrease of the FP amplitude. As FPs are frequently associated with ordinary muscle cramps, stretching of the muscle may lessen the frequency of FPs by the same mechanism which stops cramps.

Adult↗

Neurogenic benign fasciculations, pseudomyotonia, and pseudotetany. A disease in search of a name.

We studied two patients with abnormal spontaneous muscular activity. The first had widespread fasciculations, painful spasms, delayed muscular relaxation, and hyperhidrosis. Improvement occurred after several years. The second case had generalized paresthesia, mild stiffness, a positive result from Trusseau's test, and was relieved by administration of carbamazepine. Both patients had abnormal conduction velocity. Examination of muscle biopsy specimens disclosed fiber type grouping and increased collateral ramification of motor axons. These observations exemplify symptoms and signs that resemble those of myotonia and tetany and occasionally occur in partial denervation. they provide additional evidence of the neurogenic nature of Isaacs-Mertens syndrome.

Adult↗

Fasciculation potentials in healthy people.

The aim of this study was to investigate the fasciculation potentials (FPs) in the small-peripheral muscles of the foot and hand and the possible associated factors, in a healthy population. One hundred-twenty-two normal individuals (65 men and 57 women), aged 17-67 years (mean 39.96, SD=12.76) participated in the study. A special questionnaire consisting of 47 questions was devised as the basic instrument of the interview, which included the Hamilton anxiety rating scale. The extensor digitorum brevis (EDB), the flexor hallucis brevis (FHB) and the first dorsal interosseous (FDI) muscles were studied bilaterally using surface electrodes. In 94 (58 men and 36 women) from 122 participants (65 men and 57 women) FPs were recorded (men 89.2%, women 63.1%, all 77%). The mean FPs per minute and muscle, in all three muscles, was 8.0 (SD=4.6). More FPs were recorded in the muscles of foot than in FDI (p<0.01) and in FHB than in EDB (p<0.001). FPs were correlated to gender, body height and weight and to the score of the Hamilton scale (r2>0.1, p<0.01). The syndrome of benign FPs was observed in 2 men (1.6% of men). These results suggest that FPs are a very common phenomenon in the peripheral muscles of healthy persons.

Action Potentials↗

A functional role for the middle extracellular region of the neural cell adhesion molecule (NCAM) in axonal fasciculation and orientation.

A new monoclonal antibody (mAb HR1) was used to study the function of a previously neglected region in the extracellular domain of the neural cell adhesion molecule (NCAM). Application of mAb HR1 in retina organ culture interferes with several axonal functions in the developing eye. The antibody disturbs the orientation of axons growing in the peripheral retina and the tracking of axons in the middle retina. In the central retina, fasciculation is disturbed and a proportion of the ganglion cell axons do not leave the eye at the optic fissure but are misrouted to the contralateral side of the retina. Analysis of peptide fragments of NCAM indicates that the epitope of mAb HR1 resides in the region C-terminal to the fifth immunoglobulin (Ig) domain. Moreover, mAb HR1 binds to the oligopeptide comprising the 15 amino acids immediately C-terminal to the fifth Ig domain of NCAM. In addition, binding of mAb HR1 to NCAM is increased by removal of the large polysialic acid chains of the fifth Ig domain. Taken together, the data show that this region of NCAM--which has been previously reported to represent the flexible hinge region of the molecule--is crucial for the function of this molecule, in particular on cell surfaces in motion, e.g., those of growing axons in the developing nervous system.

Amino Acid Sequence↗

NCAM is essential for axonal growth and fasciculation in the hippocampus.

The neural cell adhesion molecule (NCAM), probably the best characterized and most abundant cell adhesion molecule on neurons, is thought to be a major regulator of axonal growth and pathfinding. Here we present a detailed analysis of these processes in mice deficient for all NCAM isoforms, generated by gene targeting. The hippocampal mossy fiber tract shows prominent expression of polysialylated NCAM and the generation of new axonal projections throughout life. Focusing on this important intrahippocampal connection, we demonstrate that in the absence of NCAM, fasciculation and pathfinding of these axons are strongly affected. In addition we show alterations in the distribution of mossy fiber terminals. The phenotype is more severe in adult than in young animals, suggesting an essential role for NCAM in the maintenance of plasticity in the mature nervous system.

Aging↗

Expression of an L1-related cell adhesion molecule on developing CNS fiber tracts in zebrafish and its functional contribution to axon fasciculation.

E587 antigen, an L1-related cell adhesion molecule, is expressed by growing axons and has previously been shown to enhance axon growth and to mediate fasciculation of axons from newborn retinal ganglion cells in goldfish. In zebrafish, the monoclonal antibody E17 against E587 antigen stains all axons in the primary tracts and commissures from 17 h postfertilization (pf) onward and axons which are added subsequently to this scaffold. Moreover, Fab fragments of an E587 antiserum (E587 Fabs) injected into the ventricle of 30-h pf zebrafish embryos caused a marked defasciculation of distinct axon bundles in the posterior commissure, in hindbrain commissures, and in longitudinal tracts of the hindbrain, where they also caused increased crossings between fascicles. The regulated expression of E587 antigen by all developing axons and the effects caused by E587 Fabs show that E587 antigen contributes to the formation of tight and orderly fascicles in the developing CNS.

Animals↗