Cases reported "Myokymia"

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1/6. Remission of superior oblique myokymia after microvascular decompression.

    Superior oblique myokymia (SOM) is an ocular motility disorder characterized by oscillopsia, vertical or torsional diplopia, sometimes combined with pressure sensation. Although the pathophysiological basis is unclear, isolated case reports have documented its association with intracranial pathological processes. We present a case of SOM associated with a vascular compression of the fourth nerve at the root exit zone. Following microneurosurgical decompression, SOM completely resolved and paralysis of the fourth nerve occurred. This was less disturbing.
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2/6. Functional changes and adverse reactions after successful treatment of hereditary myokymia: a case report.

    myokymia is a vermiform motion of muscle that can be found in conditions in which there is pathology to the peripheral nerve to that muscle. There are inherited syndromes that can present with muscle cramping and myokymia that have been described, but little information exists on long-term follow-up. In this case study, a 12-year-old girl had an inherited form of myokymia, as determined by clinical examination, electromyographic findings, and family history. The mode of inheritance appeared to be autosomal dominant with reduced penetrance. carbamazepine was used successfully to treat this disorder. Measurements were made of strength and endurance before and after treatment; significant improvement was seen in endurance with little effect on strength. The patient was able to discontinue carbamazepine without return of symptoms and subsequently was not limited in her activities. Return of symptoms was seen with prednisone treatment for exacerbated asthma. The symptoms resolved with restarting the carbamazepine and weaning of the prednisone. Discussion is presented on the possible mechanism by which anticonvulsant and steroid therapy exert their effects on myokymia.
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3/6. Vascular compression as a cause of superior oblique myokymia disclosed by thin-slice magnetic resonance imaging.

    PURPOSE: To describe a case of superior oblique myokymia in which thin-slice magnetic resonance imaging (MRI) appeared to show vascular compression of the trochlear nerve. methods: A 50-year-old woman presented with episodic monocular oscillopsia. Neuro-ophthalmologic examinations showed intermittent intorsional microtremor of her right eye, diagnosed as right superior oblique myokymia. Thin-slice (1.6 mm) MRI, using spoiled gradient recalled acquisition in the steady state, was employed to examine the trochlear nerve in its course through the ambient cistern. RESULTS: Imaging disclosed a branch of the posterior cerebral artery immediately adjacent to the right trochlear nerve. CONCLUSION: These magnetic resonance findings suggest that a cause of superior oblique myokymia may be vascular compression of the trunk of the trochlear nerve.
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ranking = 11679.652716844
keywords = trochlear nerve, trochlear, nerve
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4/6. Superior oblique myokymia: magnetic resonance imaging support for the neurovascular compression hypothesis.

    Superior oblique myokymia is a rare movement disorder thought to be caused by vascular compression of the trochlear nerve. Direct display of such neurovascular compression by magnetic resonance imaging has been lacking. The goal of this study was to assess the presence of neurovascular contacts in patients with superior oblique myokymia, using a specific magnetic resonance imaging protocol. A total of 6 patients suffering from right superior oblique myokymia underwent detailed neuro-ophthalmological examination, which showed tonic or phasic eye movement. All patients underwent magnetic resonance imaging, using a magnetic resonance imaging Fourier transform constructive interference in steady-state sequence in combination with magnetic resonance imaging time of flight magnetic resonance arteriography both before and after the administration of Gd-DTPA. With this protocol, the trochlear nerve could be visualized on 11 of 12 sides (92%). Arterial contact was detected at the root exit zone of the symptomatic right trochlear nerve in all 6 patients (100%). No arterial contact was identified at the root exit zone of the asymptomatic left trochlear nerve in any of the 5 left nerves visualized. In conclusion, superior oblique myokymia can result from neurovascular contact at the root exit zone of trochlear nerve, and therefore should be considered among the neurovascular compression syndromes.
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ranking = 14600.065896055
keywords = trochlear nerve, trochlear, nerve
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5/6. Recurrent superior oblique myokymia in a patient with retinitis pigmentosa.

    Superior oblique myokymia is an infrequently encountered condition, presenting with episodes of oscillopsia and/or vertical or oblique nystagmus, accompanied by a fine, monocular, cyclorotational nystagmus. Recent research suggests it is caused by vascular compression of the trunk of the trochlear nerve. The clinical features of a patient reporting three episodes of superior oblique myokymia, each following childbirth, are described. She had previously been diagnosed with retinitis pigmentosa. The possible aetiologies of superior oblique myokymia are described and appropriate assessment and possible referral for further testing detailed.
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ranking = 2919.9131792111
keywords = trochlear nerve, trochlear, nerve
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6/6. Morvan's syndrome: clinical, laboratory, and in vitro electrophysiological studies.

    Morvan's syndrome is a rare disorder characterized by neuromyotonia, hyperhidrosis, and central nervous system dysfunction. We report a patient with features of this syndrome, but who initially presented with breathing difficulties. Concentric needle electromyography showed an abundance of myokymic and neuromyotonic discharges. exercise tests and repetitive nerve stimulation showed a decrement-increment response of compound muscle action potentials. antibodies against voltage-gated potassium channels were not detected on repeated testing, but the presence of oligoclonal bands in the cerebrospinal fluid (CSF) suggested an autoimmune etiology. At follow-up over 3 years, no cancer was found. Electrophysiological in vitro studies of effects of patient serum and CSF on rat nerves provided no evidence of altered voltage-gated sodium or potassium conductances. We conclude that putative humoral factors do not block ion channels acutely but may cause channel dysfunction with chronic exposure.
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