Cases reported "Myotonia"

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1/24. A case of ocular neuromyotonia with tonic pupil.

    A 48-year old woman with hypertension experienced painful oculomotor nerve palsy. After surgery for a giant aneurysm of the internal carotid artery in the cavernous sinus, phasic constrictions of the pupil developed. Two years later, this phenomenon disappeared and was replaced by intermittent involuntary cyclic spasms elevating the ptosed lid. These cyclic lid movements were not elicited with any eye movement or by increased accommodation. The pupil now manifested the pharmacologic features of a tonic pupil. The explanation for this unique case of ocular neuromyotonia is based on a misdirection phenomenon, possibly caused by ephaptic transmission.
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ranking = 1
keywords = nerve
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2/24. Neuromyotonia of the abducens nerve after hypophysectomy and radiation.

    The clinical signs of the rarely encountered ocular neuromyotonia consist of transient involuntary tonic contraction and delayed relaxation of single or multiple extraocular muscles, resulting in episodic diplopia. With a mean time delay of 3.5 years, this motility disorder frequently follows tumor excision or adjuvant radiation near the skull base. Ocular neuromyotonia may reflect inappropriate discharge from oculomotor neurons with unstable cell membranes because of segmental demyelinization by tumor compression and radiation-induced microangiopathy. In the present paper, the authors present the case of a 53-year-old patient with a history of transsphenoidal hypophysectomy and adjuvant radiotherapy, who underwent strabismus surgery for abducens palsy.
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ranking = 4
keywords = nerve
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3/24. Neuromyotonia, myocloni, sensory neuropathy and cerebellar symptoms in a patient with antibodies to neuronal nucleoproteins (anti-Hu-antibodies).

    A middle-aged patient presented with subacute muscular stiffness, myocloni of both extremity and facial muscles, gait ataxia and symmetrical distal painful paraesthesias. Electrophysiologically, neuromyotonia was confirmed. High titer anti-Hu antibodies were detected, but no other paraneoplastic antibodies were found. Small-cell lung cancer was diagnosed. Under chemotherapy tumor remission was achieved and, except for minor sensory deficits, neurological symptoms disappeared. This report shows that paraneoplastic syndromes associated with antibodies to neuronal nucleoproteins (anti-Hu antibodies) may be associated with a syndrome including neuromyotonia, sensory neuropathy, cerebellar symptoms and myocloni.
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ranking = 10.466423838201
keywords = neuropathy
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4/24. Laryngeal spasm complicating pseudomyotonia.

    Pseudomyotonia is a syndrome characterized by progressive muscle stiffness, weakness, myokymia, hyperhydrosis, and wasting of muscle. It especially affects the distal aspect of the extremities. The electromyogram is characteristic, and shows continuous muscle activity at rest. We discuss a case that is believed to be the first recorded instance of pseudomyotonia complicated by airway obstruction secondary to laryngeal spasm. Treatment with phenytoin sodium resulted in rapid improvement of the peripheral symptoms and simultaneous resolution of the laryngeal spasm.
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ranking = 10.726499835711
keywords = peripheral
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5/24. Neuromyotonia: report of a case.

    Neuromyotonia is a rare condition of peripheral nerve dysfunction characterized by the signs of motor nerve hyperactivity, namely, myokymia, fasciculations, and muscular stiffness. relaxation of voluntary muscle contraction is delayed, and fluid movements are impaired to a variable degree. Signs of sensory involvement are less frequent. Usually, therapy with carbamazepine or phenytoin provides rapid improvement. In the present communication, a typical case is reported, and the most important differential diagnoses are outlined.
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ranking = 46.90270418714
keywords = peripheral nerve, peripheral, nerve
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6/24. adult-onset acquired oculomotor nerve paresis with cyclic spasms: relationship to ocular neuromyotonia.

    PURPOSE: To describe the characteristics and significance of acquired oculomotor nerve paresis with cyclic spasm. METHOD: Retrospective case series of two patients with a history of previous skull base irradiation for intracranial tumor who developed double vision and were found to have oculomotor nerve paresis with cyclic spasm. Both patients underwent a complete neuroophthalmologic assessment, including testing of eyelid position, pupillary size and reactivity, and ocular motility and alignment during both the paretic and spastic phases of the condition. RESULTS: Both patients developed unilateral lid retraction and ipsilateral esotropia with limitation of abduction during the spastic phase of the cycle, with ipsilateral ptosis, exotropia, and variable limitation of adduction during the paretic phase. The cycles were continuous and were not induced or altered by eccentric gaze. CONCLUSIONS: Cyclic oculomotor nerve paresis with spasms may occur years after irradiation of the skull base. This condition is different from the more common ocular motor disturbance that occurs in this setting-ocular neuromyotonia. However, in view of the similarity between these two disorders, it seems likely that they are caused by a similar peripheral mechanism.
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ranking = 17.726499835711
keywords = peripheral, nerve
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7/24. Effects of acute and chronic denervation on human myotonia.

    A 32-year-old man with myotonia congenita (Becker type) sustained multiple gunshot wounds. These produced a partial thoracic spinal cord injury and a severe sciatic nerve injury. Six days following the incident, clinical (percussion) and electrophysiologic (EMG) myotonia could be elicited in paretic leg muscles resulting both from the myelopathy and peripheral nerve disruption. Eight months later, the myotonia was no longer present in denervated muscles from the sciatic nerve injury, but was still noted in muscles with upper-motor neuron weakness from the myelopathy. Although myotonia is related principally to abnormalities in the muscle fiber itself, it appears that it is also dependent upon the structural integrity of the peripheral nerve supply to the muscle for myotonia to continue to occur. The findings in this patient suggest that myotonia may well have diminished and disappeared in muscles shortly after the nerves had undergone wallerian degeneration. Myotonia does not recur if there is no significant reinnervation.
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ranking = 94.80540837428
keywords = peripheral nerve, peripheral, nerve
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8/24. Neurological manifestations of ehlers-danlos syndrome.

    ehlers-danlos syndrome (EDS) is more identified for its cutaneous features but its neurological manifestations have not received the focused attention. Four patients of ehlers-danlos syndrome (EDS) with neurological manifestations were evaluated for phenotypic data. These four men were from three families and two had consanguineous parentage. The mean age at onset and presentation of neurological symptoms were 10.5 years and 19 years respectively. Patient 1 presented with bilateral optic atrophy, sensorineural deafness, cerebellar ataxia and neuropathy. Patient 2 had marfanoid habitus, chorea and cerebellar ataxia. Patient 3 had action and percussion myotonia, wasting and weakness of sternocleidomastoid and distal limb muscles. Patient 4 had action myotonia, mirror movements of both hands and neuropathy. MRI of brain showed right parietal polymicrogyria. Neuroaxis involvement at multiple levels in EDS may have prognostic significance.
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ranking = 4.1865695352804
keywords = neuropathy
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9/24. Mistaken diagnoses in continuous muscle fiber activity of peripheral nerve origin.

    The syndrome of continuous muscle fiber activity of peripheral nerve origin has manifestations that resemble those of many other more common neurologic disorders during childhood and infancy. This similarity often leads to misdiagnosis when an adequate index of suspicion is not entertained and a comprehensive electromyographic examination is not performed. Two affected patients from 1 family are reported to illustrate the type of diagnostic errors that were made before the establishment of the correct diagnosis.
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ranking = 229.5135209357
keywords = peripheral nerve, peripheral, nerve
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10/24. Hyperexcitability of motor and sensory neurons in neuromyotonia.

    Two members of a family with a neuropathy resembling charcot-marie-tooth disease were unable to relax their muscles after voluntary contraction. Muscle spasm often outlasted voluntary contraction by 30 seconds or more before subsiding into myokymia and fasciculations. The posture of the hand during muscle spasm resembled that of tetany, and Trousseau's and Chvostek's signs were present although no abnormality of calcium or magnesium metabolism could be demonstrated. Muscle spasms ceased during medication with carbamazepine, 600 mg daily. Nerve stimulation, electromyography, and regional neuromuscular blockade with curare indicated that the condition originated in peripheral nerve, while microneurographic recordings showed that sensory as well as motor fibers were hyperexcitable. sural nerve biopsy revealed axonal degeneration involving myelinated and unmyelinated fibers. It was concluded that the neural hyperexcitability is caused by a membrane defect resulting in a low threshold for excitation throughout the length of the axon.
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ranking = 48.99598895478
keywords = peripheral nerve, peripheral, neuropathy, nerve
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