Cases reported "Myotonia"

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11/24. Twitch response in a myopathy with impaired relaxation but no myotonia.

    A patient with slow muscle relaxation but without accompanying motor unit or myotonic electrical activity had a unique staircase twitch response to repeated nerve stimulation. During 1-Hz stimulation, twitches recorded by measurement of the ankle dorsiflexor group displayed progressively increasing relaxation times with successive stimuli (37% increase) unlike the progressively decreasing relaxation time of the normal response (12-37% decrease). The response may be diagnostic of this unusual myopathy; the test methods are noninvasive and easily tolerated.
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ranking = 1
keywords = nerve
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12/24. myokymia, muscle hypertrophy and percussion "myotonia" in chronic recurrent polyneuropathy.

    Three unusual features were observed in a patient with chronic relapsing polyneuropathy: myokymia, muscle hypertrophy, and prolonged contraction in response to muscle percussion. Low nerve conduction velocity and conduction block were demonstrated in all motor nerves tested, indicating a demyelinating peripheral neuropathy. myokymia was caused by spontaneous motor unit activity which was shown to originate in peripheral nerves, since it persisted after nerve block and was abolished by regional curarization. Muscle hypertrophy was attributed to increased peripheral nerve activity, and the prolonged contraction of muscle in response to direct percussion was attributed to irritability of intramuscular nerve terminals.
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ranking = 165.42942467486
keywords = peripheral neuropathy, peripheral nerve, peripheral, neuropathy, nerve
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13/24. syndrome of continuous muscle-fiber activity: increased CSF GABA and effect of dantrolene.

    In a patient with a syndrome of continuous muscle fiber activity, peripheral nerve block completely abolished the EMG discharges. Reduction of spontaneous discharges by epidural block and demonstration of a silent period after the H response suggested that the disorder may originate in the spinal cord or ventral roots, sparing inhibitory influences on the alpha-motoneuron to some extent. GABA (gamma-aminobutyric acid) content in CSF was remarkably increased. Therapeutically, dantrolene sodium was as effective as phenytoin or carbamazepine.
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ranking = 45.90270418714
keywords = peripheral nerve, peripheral, nerve
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14/24. Electromyographic and nerve conduction findings in a patient with neuromyotonia, normocalcemic tetany and small-cell lung cancer.

    A 57-year-old man with neuromyotonia and normocalcemic tetany as probable paraneoplastic symptoms of small-cell lung cancer was examined neurophysiologically. In EMG, spontaneous action potential generation was demonstrated in peripheral motor axons. There were also time-locked high-frequency discharges after some voluntarily activated motor unit potentials. After electrical stimulation of motor axons, the M-response as well as spinal F-response or h-reflex was often followed by a sequence of oscillating potentials which could last several hundred msec. There was no evident peripheral polyneuropathy. tetany subsided when carbamazepine therapy was started. The only striking abnormality in extensive laboratory studies was an increase in the plasma noradrenaline value.
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ranking = 27.546284439062
keywords = peripheral, neuropathy, nerve
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15/24. Continuous muscle fiber activity syndrome.

    A 5.5 year old Turkish boy who suffered from progressive muscle stiffness was diagnosed as having continuous muscle fiber activity syndrome. electromyography showed continuous motor neuron activity at rest and following intravenous injection of diazepam. Peripheral nerve block, spinal and general anesthesia diminished the spontaneous activities. The clinical and electrophysiological findings of our case were compared to the previously reported cases with emphasis on the heterogeneity of the syndrome.
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ranking = 1
keywords = nerve
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16/24. association of neuromyotonia with peripheral neuropathy, myasthenia gravis and thymoma: a case report.

    A case of neuromuscular hyperactivity syndrome associated with a thymoma, high serum titres of anti-acetylcholine receptor and anti-dna antibodies is reported. The study of peripheral nerve conduction revealed a peripheral neuropathy. Repetitive stimulation showed a decrease in the fifth M response. Myasthenic symptoms were anamnestically reported but were absent at clinical observation. The clinical picture and EMG examination improved after phenytoin administration.
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ranking = 341.69066649904
keywords = peripheral neuropathy, peripheral nerve, peripheral, neuropathy, nerve
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17/24. Gaze-induced strabismus.

    A 20-year-old woman with a past history of a sinonasal undifferentiated carcinoma involving the skull base and cavernous sinus, previously treated with surgery and radiation therapy, presented with intermittent horizontal diplopia induced by gaze to the right side. The gaze in the direction of the action of the right VI nerve produced sustained contraction of the right lateral rectus and an exodeviation increasing on left gaze. Ocular neuromyotonia involving the right VI nerve was believed to be secondary to the patient's previous radiation therapy. The discussants review the prior literature regarding ocular neuromyotonia involving the III, IV and VI cranial nerves, its relationship to prior radiation therapy, and possible modes of therapy including the use of carbamazepine.
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ranking = 3
keywords = nerve
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18/24. Neuromyotonia, peripheral neuropathy and myasthenia gravis.

    A patient with neuromyotonia, peripheral neuropathy and myasthenia gravis (MG) is described. Neurophysiological studies, at rest, showed continuous muscle discharges of motor unit action potentials (MUAPs) in duplets and triplets. Motor (MNCV) and sensory (SNCV) nerve conduction studies revealed mild axonal and demyelinating peripheral neuropathy. plasma exchange was followed by disappearance of clinical and electrophysiological signs of neuromyotonia and MG, as well as peripheral neuropathy.
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ranking = 415.10314723665
keywords = peripheral neuropathy, peripheral, neuropathy, nerve
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19/24. Generator sites for spontaneous activity in neuromyotonia. An EMG study.

    A 16-year-old female patient with symptoms and signs compatible with neuromyotonia was studied with various neurophysiological tests and with muscle biopsy. Nerve conduction studies revealed signs of axonal motor neuropathy. EMG showed denervation in distal muscles, and moderate neurogenic changes in other muscles. Abundant spontaneous motor unit activity was recorded in all muscles. This activity did not disappear upon proximal nerve blockade with local anaesthetics. Based on the shape of spontaneous discharges and their behaviour on nerve stimulation and during voluntary effort, the site of generation was suggested. This varied for different discharges, from proximally in the nerve, to various sites along the intramuscular nerve tree. In some axons there were signs of conduction block proximal to the generation site for the spontaneous discharges. Different axons showed various degrees of abnormality; local hyperexcitability triggering new impulses only after the passage of a preceding impulse, increased hyperexcitability generating spontaneous activity, total impulse blocking, and finally axonal degeneration. Treatment with dihydantoin reduced the spontaneous activity with concomitant clinical improvement.
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ranking = 6.0932847676402
keywords = neuropathy, nerve
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20/24. Ocular neuromyotonia.

    AIMS/BACKGROUND: Ocular neuromyotonia is characterised by spontaneous spasm of extraocular muscles and has been described in only 14 patients. Three further cases, two with unique features, are described, and the underlying mechanism reviewed in the light of recent experimental evidence implicating extracellular potassium concentration in causing spontaneous firing in normal and demyelinated axons. methods: Two patients had third nerve neuromyotonia, one due to compression by an internal carotid artery aneurysm, which has not been reported previously, while the other followed irradiation of a pituitary tumour, a common association in the published reports. Selective activation occurred in both, where neuromyotonic activity was triggered by prolonged voluntary activation of specific extraocular muscles with or without spread of activity to other third nerve muscles. The other patient had fourth nerve involvement, where spasms of the superior oblique muscle were induced only by alcohol, a phenomenon which has not been described. RESULTS: The two patients with third nerve involvement responded to carbamazepine and in one, an improvement in a chronic partial third nerve paresis occurred. The other has not required treatment and remains asymptomatic by refraining from alcohol. CONCLUSIONS: A careful examination, including the effects of prolonged voluntary muscle action is required to initiate episodes and to demonstrate selective activation. Imaging is mandatory to exclude compressive intracranial lesions, particularly where there is no history of pituitary fossa irradiation. A trial of anticonvulsants should be considered in all patients. Extracellular potassium may play a role in spontaneous firing and ephatic transmission in ocular neuromyotonia.
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ranking = 5
keywords = nerve
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