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PubMed1998

Strength-duration properties of sensory and motor axons in amyotrophic lateral sclerosis.

In normal subjects, the strength-duration time constant is longer for cutaneous afferents than for motor axons, probably because the former express a greater non-inactivating (persistent) Na+ conductance that is active at threshold. Using a threshold-tracking system the strength-duration properties of cutaneous afferents and motor axons were recorded from 23 patients with amyotrophic lateral sclerosis, and compared with those of 32 healthy subjects. In control subjects and patients, the strength-duration time constant of sensory fibres declined with age, and there was no difference between the two groups when age was taken into account. The motor time constant did not change with age when expressed as a percentage of the time constant for sensory fibres in the same nerve, but was significantly longer for the patients than control subjects. In addition, motor rheobase was significantly lower for the patients, when expressed as a percentage of sensory rheobase. There was an inverse relationship between the time constant and rheobase for sensory and motor axons, and this was the same for the patients and the control subjects, suggesting that the variations in time constant within and between the groups were related to the expression of a common factor. Measurements of refractoriness and supernormality provided no evidence for a difference in resting membrane potential between the patients and control subjects. These findings are consistent with the interpretation that motor axons of the patients with amyotrophic lateral sclerosis have a greater persistent Na+ conductance than normal motor axons. This could contribute to the ectopic activity responsible for fasciculation.

Adult↗
PubMed1988

Isolated phasic discharges in anterior tibial muscle: a stable feature of paradoxical sleep.

Phasic muscle discharges known currently as "fasciculations" are more frequently encountered in the limb skeletal muscles and predominate in the lower limbs when compared with the upper limbs. Of the lower limbs, the most active during paradoxical sleep (PS) is the anterior tibial muscle. When quantifying phasic discharges of the eyes versus a lower limb muscle during PS, the former are 14 times more frequent than the latter. During PS there is a sporadic and nonsignificant decrease in the tonus of the antigravitational extensor muscles. Isolated phasic discharges in anterior tibial muscles are a stable feature of PS.

Adult↗
PubMed1985

Oral dantrolene prevents rise of myoglobin due to suxamethonium.

Forty patients were studied in a randomised trial to investigate the effect of pre-operative oral dantrolene on the increase of serum myoglobin and creatinine kinase due to suxamethonium administration. Twenty patients in the treatment group were given 1.5 mg/kg dantrolene orally 4 hours pre-operatively. Blood was drawn immediately pre-induction and 5, 10 and 20 minutes after suxamethonium 1.2 mg/kg administration, following which surgery could commence. Myoglobin was measured by radioimmunoassay. The increase in mean myoglobin values was greatly reduced following suxamethonium in the treatment group (10.6 micrograms/litre at 20 minutes) compared to the control group (54.8 micrograms/litre at 20 minutes), (p less than 0.01). Total fasciculation score was not significantly reduced by the dantrolene pretreatment. No increase in creatinine kinase values occurred in any patient and the changes in mean potassium values in both groups were negligible. The only side effect attributed to dantrolene was pre-operative nausea in two patients. No interference with the action of suxamethonium, or difficulty with reversal was noticed. Oral dantrolene may be almost as effective as pretreatment with non-depolarising neuromuscular blocking drugs in preventing suxamethonium-induced increase in myoglobin with less interaction with other anaesthetic agents.

Administration, Oral↗
PubMed1993

Myalgia and biochemical changes following suxamethonium after induction of anaesthesia with thiopentone or propofol.

The incidence and severity of muscle pains and changes in creatine kinase were assessed following administration of 1 mg.kg-1 suxamethonium either immediately or 2 min after induction of anaesthesia with propofol or thiopentone in patients undergoing elective dental and ophthalmic surgery. The incidence of muscle pains was 35 and 60% respectively in the groups given suxamethonium immediately and 2 min after propofol, and 35 and 55% when given immediately and 2 min after thiopentone, with no statistically significant differences among the groups. Creatine kinase levels increased in all the groups after operation with the least average increase in the group receiving suxamethonium immediately after propofol and the highest increase in the group receiving suxamethonium 2 min after thiopentone. There was no correlation between the incidence and severity of fasciculations, muscle pains and changes in creatine kinase within or between the groups. It is concluded that neither the induction agent nor the time between the induction agent and suxamethonium administration has any significant influence on the incidence of muscle pains or creatine kinase elevation following suxamethonium.

Adolescent↗
PubMed1983

Syndromes of abnormal muscular activity: overlap between continuous muscle fibre activity and the stiff man syndrome.

Four patients with muscular pain, fasciculations, contractures or cramps are presented. Evidence of peripheral nerve involvement was revealed by electromyography and nerve conduction studies. Muscle biopsy showed mild signs of denervation and reinnervation and, at electron microscopy, dilatations of terminal cisternae were found. All patients showed a remarkable improvement after therapy with diphenylhydantoin or carbamazepine. These clinical, neurophysiological and morphological data underline the role of peripheral nerve pathology in various syndromes of abnormal continuous muscular activity.

Adult↗
PubMed1987

Timber rattlesnake venom-induced myokymia: evidence for peripheral nerve origin.

Facial and limb myokymia occurred in four consecutive cases of timber rattlesnake envenomation and represents the "fasciculations" frequently reported in this entity. The facial myokymia disappears within hours of antivenin therapy and the limb myokymia by increasing serum ionized calcium. These observations suggest that the action of the venom is a biochemical one, increasing peripheral nerve excitability.

Action Potentials↗
PubMed1992

Neuromuscular pharmacology in rat neonates: development of responsiveness to prototypic blocking and reversal drugs.

The neonatal pharmacology of neuromuscular drugs was studied in vivo in newborn rats and in vitro in neonatal phrenic nerve-hemidiaphragm preparations. Drugs used to probe neuromuscular development in rat neonates were physostigmine, edrophonium, neostigmine, 4-aminopyridine, d-tubocurarine (dTc), and succinylcholine. The prejunctional actions of these drugs were monitored in relation to neonatal age by the appearance of stimulus-evoked repetitive discharge initiated by motor nerve endings and the occurrence and magnitude of the resulting enhancement of twitch tension. The occurrence and incidence of drug-induced fasciculations also served to track the development of functional motor nerve endings. Each of these prejunctional actions was inoperative until the third neonatal week, indicative of incomplete motor nerve development. In contrast, 4-aminopyridine, a nonanticholinesterase, evoked these prejunctional actions in 1-wk-old rat neonates. Neostigmine and edrophonium antagonized dTc as early as the first week; presumably, postsynaptic maturation had reached a functional level. 4-Aminopyridine also antagonized dTc at week 1. Rat neonates showed resistance to dTc blockade when tested by neonatal phrenic nerve-hemidiaphragm preparations in vitro. Relationships between age and 85%-95% transmission block declined to the adult level by week 5. This result indicates that in rat neonates, pharmacodynamic rather than pharmacokinetic mechanisms predominate in the development of responsiveness to dTc.

4-Aminopyridine↗
PubMed1979

Cervical spondylotic radiculopathy producing motor manifestations mimicking primary muscular atrophy.

Cervical radiculopathy unaccompanied by pain or sensory disorder but manifested only by paresis, atrophy, fasciculation, and reflex loss is unusual. Three such cases are presented. Considerable diagnostic difficulty may arise in distinguishing patients presenting with these features from those who have primary motor neuron disease. Two additional case reports demonstrate that this distinction is not always possible. Diagnosis, management, and prognosis are discussed. Differentiation between motor neuron disease and spondylosis will avert needless surgery in the former group and will result in beneficial, sometimes curative surgery in the latter group.

Aged↗
PubMed2002

[Poisoning with organophosphate compounds].

A 50-year-old man swallowed 200 ml of an insecticide containing the organophosphates dimethoate and phenitrotion in an attempted suicide. On admission, signs of a cholinergic syndrome were observed: miosis, rhinorrhoea, and fasciculations. This was followed by bradycardia with hypotension and vomiting. The patient was treated with the antidotes atropine and obidoxime. Decreasing consciousness necessitated intubation, mechanical ventilation and other supportive measures. Although the serum concentrations of both organophosphate compounds rapidly decreased, the activity of cholinesterase showed a prolonged inhibition. The clinical course was complicated by hypotension, acute respiratory distress syndrome, nosocomial pneumonia, and an epileptic seizure. A period with muscle weakness and a persisting depressive disorder then followed. This case is characteristic for acute intoxications with irreversible acetylcholinesterase inhibitors, such as organophosphate compounds. The treatment of these potentially severe intoxications includes rapid decontamination and the administration of high doses of atropine followed by obidoxime. Mechanical ventilation and circulatory support are also indicated.

Acetylcholine↗
PubMed2002

[Juvenile asymmetrical segmental spinal muscular atrophy].

We report the case of a man who had an insidious onset of asymmetrical distal muscle weakness of the upper extremity at the age of 17. Objective findings were 1) muscular atrophy of calf and forearm flexor muscles and intrinsic hand muscles; 2) fasciculations; and 3) hand tremor. EMG and muscle biopsy showed neurogenic changes. MRI of the medulla and plasma creatine kinase were normal. Genetic testing for SMA-III was negative.

Adolescent↗
PubMed1991

[Multifocal demyelinating neuropathy with persistent conduction blocks and continuous muscular activity].

A patient developed an initially asymmetric sensory-motor neuropathy, with definite predominance in upper limbs. The examination also disclosed a markedly impaired muscle relaxation. The neurophysiological study showed conduction blocks with continuous muscle activity consisting of myokymias fasciculations and muscle cramps which disappeared after the anesthetic block of the distal nerve segments. In the sural nerve biopsy significant abnormalities were not found. After prednisone and carbamazepine therapy in usual doses the symptoms reverted and the conduction blocks persisted. Neuropathy with persistent conduction blocks is an uncommon disease which exceptionally results in a clinically apparent syndrome of continuous muscle activity. Its recognition is important, as symptoms can disappear after correct therapy.

Adult↗
PubMed1989

Intractable orbicularis myokymia: treatment alternatives.

Orbicularis myokymia frequently occurs in young, otherwise health individuals. The intermittent muscle fasciculations are transient and generally disappear with time. If the myokymia is persistent or progressive, neurologic assessment and investigation may be necessary. Muscle relaxants, botulinum-A toxin, and surgical myectomy are methods of treatment that only occasionally need to be considered. We present limited orbicularis myectomy and botulinum-A toxin injections as efficacious treatments in five selected intractable cases of orbicularis myokymia.

Adult↗
PubMed1989

[A case of myokymia-hyperhidrosis syndrome with muscle involvement].

The present report deals with an unique case of myokymia-hyperhidrosis syndrome. A 46-year-old man developed generalized relatively slow, undulating movement of the muscles, excessive sweating, muscle cramps and easy fatigability since three years ago. On admission, he had generalized myokymia, abnormal movements consisting of slow, undulating and worm-like muscular contractions. There was no muscle atrophy or weakness, or sensory deficits. Deep tendon reflexes were diminished and no Babinski sign or other pathological reflexes were present. Muscle tone was in normal range. Neither fasciculation nor myotonia was found. Laboratory examinations revealed elevation of BMR with normal thyroid function. The value of anti-Ach receptor antibody was below 0.6nmol/l. EMG recordings showed (1) continuous spontaneous repetitive discharges of NMU at a frequency of 30-50Hz, with duration of 1-2 seconds and amplitude of about 1 mV, were demonstrated at rest and the findings were consistent with myokymia, (2) waxing phenomenon was observed on repetitive peripheral nerve stimuli, which didn't improve by the tension test, and (3) high amplitude, long duration and polyphasic NMU, a sign of denervation, were observed on voluntary contraction. Muscle biopsy specimens from the left quadriceps showed a number of internal nuclei. On electron microscopy, clustered mitochondria and filamentous bodies were very often found in subsarcolemma. From these observations, damage to peripheral nerves, neuromuscular junctions and muscles may be responsible for the generalized myokymia in our patient.

Electromyography↗
PubMed1987

[Histopathology of the skeletal musculature in acute peroral carbophos poisoning].

Clinical studies of 378 patients with acute carbophose poisoning were carried out, as well as histological studies of striated muscles (gastrocnemius, anterior tibial, intercostal and phrenic) from 7 poisoned with lethal outcome due to acute oral intake of carbophose (malation). Besides, deltoid muscles (biopsy samples) of 2 severely poisoned with carbophose patients were studied histologically. Generalized paresis and paralysis of muscles of the body and the extremities, fasciculations, muscular hypotonia were found. Histologic studies revealed extensive vascular lesions, marked necrotic and necrobiotic changes in sceletal muscles and degenerative changes in the intermuscular nerve Fibers and motor endings.

Acute Disease↗
PubMed1979

Neuromyotonia in the spinal form of Charcot-Marie-Tooth disease.

The term neuromyotonia has been applied to spontaneous activity of peripheral motor nerves which gives rise to pseudomyotonia, muscular fasciculations and myokymia. A family is described in which 8 members of 3 generations suffer from the spinal form of Charcto-Marie-Tooth disease (distal type of chronic spinal atrophy). 5 of the 8 members were examined and found to have myokymia, accentuated by voluntary muscle contraction. Pseudomyotonia was present in 2 patients and, in the 1 patient treated, was abolished by carbamazepine. The association between neuromyotonia and charcto-Marie-Tooth disease has been reported in only 7 patients before but may be more common than previously thought because muscle cramps are reported to be a feature of this disorder.

Adult↗
PubMed1984

Fasciculins, anticholinesterase toxins from the venom of the green mamba Dendroaspis angusticeps.

Two toxins that are potent inhibitors of acetylcholinesterase have been isolated from the venom of the green mamba, Dendroaspis angusticeps. The toxins have been called fasciculins since after injection into mice (i.p. 0.5-3 micrograms/g body weight) they cause severe, generalized and long-lasting (5-7 h) fasciculations. Homogenates of diaphragm, tibialis anterior and gastrocnemius muscles from mice injected with fasciculins showed a decrease in acetylcholinesterase activity by 45-60% compared to muscles from control animals. Histochemical staining revealed a greatly reduced acetylcholinesterase activity at neuromuscular junctions. Fasciculins have 61 amino acid residues and four disulfides. The molecular weights are 6765 (fasciculin 1) and 6735 (fasciculin 2). The sequences of the two toxins differ probably only at one position by a replacement of Tyr with Asp/Asn. 1 g of venom contained about 40 mg of fasciculins, 2/3 of which was fasciculin 2. A similar inhibitor has also been isolated from D. polylepis (black mamba) venom. The sequence of fasciculin 2 is known. Most of the positive charges are concentrated in a small section of the central part of the molecule, and most of the negative charges are in the C-terminal region. Fasciculins appear to have a pronounced dipole character. Fasciculin binds to the peripheral anionic site, since it can displace propidium, a probe for that site, from acetylcholinesterase. In vitro, in Krebs-Henseleit solution containing 2 mM NaH2PO4 (pH 7.4), fasciculin 2 inhibits acetylcholinesterase from human erythrocytes (Ki = 1.1 X 10(-10) M, 37 degrees C), rat muscle (Ki = 1.2 X 10(-10) M, 37 degrees C) and Electrophorus electricus (Ki = 3.0 X 10(-10) M, 22 degrees C).(ABSTRACT TRUNCATED AT 250 WORDS)

Amino Acid Sequence↗
PubMed1982

Spontaneous electrical activity: an overview.

Spontaneous electrical activity of muscle originates either from one, a few, or a large number of muscle fibers; the responses from the latter are similar to those of motor units. Responses from one or a few fibers are those arising in the end-plate zone of normal muscle and the electrical activity is associated with denervation and myotonia. The motor unit-like activity may either be discharged at randomly varying intervals, as in fasciculations, or at intervals similar to the discharges during voluntary effort, as in hemifacial spasm, Isaacs' syndrome, and in infantile spinal muscular atrophy.

Action Potentials↗
PubMed1982

Post-suxamethonium pains in Nigerian surgical patients.

Contrary to an earlier report by Coxon, scoline pain occurs in African negroes. Its incidence was determined in a prospective study involving a total of 100 Nigerian patients (50 out-patients and 50 in-patients). About 62% of the out-patients developed scoline pain as compared with about 26% among the in-patients. The abolition of muscle fasciculations (by 0.075mg/kg dose of Fazadinium) did not influence the occurrence of scoline pain. Neither the type of induction agent (Althesin or Thiopentone) nor the salt preparation of suxamethonium used (chloride or bromide), affected the incidence of scoline pain.

Fasciculation↗