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Facial myokymia in multiple system atrophy.

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Two patients are described with clinical and neuroimaging features consistent with a diagnosis of multiple system atrophy (MSA). The patients are unusual in that facial myokymia became apparent clinically at some stage in their illness. In each patient, the nature and severity of the involuntary

[Syndrome of myokymia, pseudo-myotonia, muscular atrophy, and hyperhidrosis. Apropos of a case].

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Brainstem reflexes in patients with olivopontocerebellar atrophy.

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In 4 patients with familial olivopontocerebellar atrophy (OPCA) we have recently described an abnormal movement of facial muscles characterized by rhythmic muscle twitching during voluntary activation (facial action myoclonus). In the present article, we present the results of a neurophysiological

Continuous muscle activity and distal spinal muscular atrophy.

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A young man presented with myokymias, cramp-like difficulty in muscle relaxation and peroneal atrophy. EMG studies revealed continuous muscle activity (CMA) manifested as grouped potentials and high frequency discharges. Sensory nerve conduction studies and sural nerve biopsy gave normal results,

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

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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,

Familial dyskinesia and facial myokymia (FDFM): a novel movement disorder.

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We describe here familial dyskinesia and facial myokymia (FDFM), a novel autosomal dominant disorder characterized by adventitious movements that sometimes appear choreiform and that are associated with perioral and periorbital myokymia. We report a 5-generation family with 18 affected members (10

Myokymia and neuromyotonia in veterinary medicine: a comparison with peripheral nerve hyperexcitability syndrome in humans.

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Involuntary muscle hyperactivity can result from muscle or peripheral nerve hyperexcitability or central nervous system dysfunction. In humans, diseases causing hyperexcitability of peripheral nerves are grouped together under the term 'peripheral nerve hyperexcitability' (PNH). Hyperexcitability of

Myokymia and neuromyotonia in a cat.

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A 6-year-old spayed female domestic shorthair cat was examined because of a 2-week history of rhythmic muscle movements. Physical examination revealed thoracic limb rigidity, contracture of the carpi, generalized muscle atrophy, and rhythmic rippling of the muscles of all 4 limbs. Results of a CBC

Myokymia and neuromyotonia of the tongue: a case report of complication of irradiation.

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A 51-year-old man has suffered from progressive dysarthria since 1989. He himself noted slight weakness and tightness of the tongue, so that he was unable to perform motor tasks in a normal fashion. He was diagnosed as having nasopharyngeal carcinoma and had irradiation 70 Gy in 32 divided doses in

Post-irradiation myokymia and neuromyotonia in unilateral tongue and mentalis muscles: report of a case.

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We report a 52-year-old man with slowly progressive dysarthria and dysphagia for about 11 years after radiation therapy of nasopharyngeal carcinoma. Neurological examination revealed atrophy and myokymia on the left side of the tongue and in the left mentalis muscles. Electrical discharges of

Successful treatment of hemifacial myokymia and dystonia associated to linear scleroderma "en coup de sabre" with repeated botox injections.

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Linear scleroderma "en coup de sabre" (LSCS) is a form of localized scleroderma presents as band-like sclerotic lesions of the frontoparietal area. It has been reported in association with diverse neurological manifestations like seizures, migraine, neuromyotonia, dystonia and abnormalities in MRI

AAEM minimonograph #46: neurogenic muscle hypertrophy.

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Muscle hypertrophy occurs uncommonly in several neurogenic disorders including neuropathies, radiculopathies, spinal muscular atrophy, and post-polio syndrome. Its pathogenesis varies in different circumstances. In the presence of generalized myokymia and neuromyotonia (Isaacs' syndrome),

Epilepsy and paroxysmal movement disorders in families: evidence for shared mechanisms.

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The epilepsies have been regarded as clinically distinct from the paroxysmal movement disorders. Recently, a variety of ion channel defects have been identified as the biological basis of certain familial epilepsies and paroxysmal movement disorders. We studied two families with the co-occurrence of

[Isaacs' syndrome. Report of three cases].

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We report two females, and one male with Isaacs' syndrome. The patients presented with clinical myokymia activity, muscle cramps, delayed relaxation, and muscle hypertrophy and increased sweating. Needle electromyography in several muscles showed generalized continuous motor unit discharges,

Axonal channelopathies: an evolving concept in the pathogenesis of peripheral nerve disorders.

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Abnormalities of peripheral nerve Na+ and K+ channels result in clinical manifestations unrelated to axonal degeneration or demyelination. Na+ channel blockade causes weakness and sensory loss associated with slowed conduction and decreased motor and sensory action potential amplitudes. K+ channel

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