Cases reported "Myoclonus"

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1/189. Bulbar myoclonus without palatal myoclonus. A hypothesis on pathophysiology.

    A 40-year-old woman with myoclonic contractions bilaterally in the infrahyoid neck muscles, especially in the left cricothyroid muscle, was presented and successfully treated with botulinum toxin injections. The patient had a wide, aberrant vessel curving into the left dorsolateral reticular formation of the medulla oblongata. Based on our observations, we propose that symptomatic bulbar and palatal myoclonus is caused by pathology in the dorsolateral reticular formation, and not by inferior olivary dysfunction as is currently thought.
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2/189. Palatal myoclonus as a cause of objective tinnitus: a report of six cases and a review of the literature.

    We describe 6 new cases of palatal myoclonus (PM), a rare disorder that is characterized by involuntary rhythmic contraction of the palatal musculature. Although it has been reported that PM is a lifelong condition, one of our patients experienced a complete resolution of PM, which casts doubt on the accepted beliefs about the persistence of PM. Included in this report is the first published case of voluntary PM with objective tinnitus.
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3/189. melatonin treatment of non-epileptic myoclonus in children.

    Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non-epileptic, and one had epileptic and non-epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.
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4/189. Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease.

    We describe a patient with an unusual clinical presentation of progressive multiple cranial nerve palsies, cerebellar ataxia, and palatal tremor (PT) resulting from an unknown etiology. magnetic resonance imaging showed evidence of hypertrophy of the inferior olivary nuclei, brain stem atrophy, and marked cerebellar atrophy. This combination of progressive multiple cranial nerve palsies, cerebellar ataxia, and PT has never been reported in the literature. We have also reviewed the literature of PT secondary to neurodegenerative causes. In a total of 23 patients, the common causes are sporadic olivopontocerebellar atrophy (OPCA; 22%), Alexander's disease (22%), unknown etiology (43.4%), and occasionally progressive supranuclear palsy (4.3%) and spinocerebellar degeneration (4.3%). Most patients present with progressive cerebellar ataxia and approximately two thirds of them have rhythmic tremors elsewhere. Ear clicks are observed in 13% and evidence of hypertrophy of the inferior olivary nucleus in 25% of the patients. The common neurodegenerative causes of PT are OPCA/multiple system atrophy, Alexander's disease, and, in most of them, the result of an unknown cause.
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keywords = tremor
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5/189. Disappearance of rhythmic involuntary movements during sleep in a case of olivopontocerebellar atrophy.

    We report on a 54-year-old woman with an 8 or so year history of olivopontocerebellar atrophy associated with the rhythmic involuntary movements of the left upper and lower limbs, and cervical region. Surface electromyogram of the left upper limb revealed rhythmicity (about 3 Hz) and reciprocity between antagonistic muscles, which disappeared on polysomnography at all sleep stages including rapid eye movement sleep without atonia. These were characterized by the co-existence of rhythmic skeletal myoclonus and parkinsonian tremor. These findings suggest that a disturbance of the striatonigral system as well as the dentato-rubro-olivary circuit may be involved in these movements. It also seems that their fate is dependent on the level of wakefulness and that the ascending reticular activating system also plays a role in the development of these movements.
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keywords = tremor
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6/189. Ventricular pseudo-bigeminy due to sustained myoclonus.

    An elderly unconsciocus patient with anteroseptal myocardial infarction showing ventricular pseudo-bigeminy (artifact) due to sustained myoclonus is reported. The reason why the artifacts coincided with his cardiac contraction is not clearly understood. The artifact is completely eliminated following intravenous injection of succinylcholine chloride. This is the first reported case of such a puzzling electrocardiographic finding to our knowledge. It is extremely important to distinguish between a true and pseudo-arrhythmia. Otherwise, an erroneous diagnosis frequently leads to an erroneous therapeutic approach.
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7/189. Localization of a gene for myoclonus-dystonia to chromosome 7q21-q31.

    Essential myoclonus-dystonia is a neurological condition characterized by myoclonic and dystonic muscle contractions and the absence of other neurological signs or laboratory abnormalities; it is often responsive to alcohol. The disorder may be familial with apparent autosomal dominant inheritance. We report a large kindred with essential familial myoclonus-dystonia and map a locus for the disorder to a 28-cM region of chromosome 7q21-q31.
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8/189. An unusual concomitant tremor and myoclonus after a contralateral infarct at thalamus and subthalamic nucleus.

    A 72-year-old woman experienced a sudden onset of spontaneous tremor and myoclonus of right extremities that completely subsided 24 hours after onset. neuroimaging study revealed an infarct at the left ventral portion of thalamus and subthalamic nucleus. Concomitant dyskinetic movement disorders after stroke are extremely rare and the mechanism is herein discussed.
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keywords = tremor
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9/189. Postural and action myoclonus in patients with parkinsonian type multiple system atrophy.

    patients with a parkinsonian syndrome and features of multisystem atrophy (pMSA) may exhibit abnormal movements of the hands and fingers, which are reported in the literature either as "jerky" tremor or myoclonus. We studied clinically and electrophysiologically these movements in 11 consecutive patients with pMSA. No abnormal movements were observed when the patients were at complete rest, except for a characteristic parkinsonian "pill-rolling" tremor in one patient. Abnormal small-amplitude, nonrhythmic movements involving just one or a few fingers, or more rarely the whole hand, were observed in nine patients when holding a posture or at the beginning of an action. Accelerometric recordings showed small-amplitude irregular oscillations which, contrary to those of patients with tremor, had no predominant peak in the Fast Fourier frequency spectrum analysis. Electromyographic recordings in the forearm and hand muscles showed brief jerks of less than 100 ms duration which were synchronous in antagonist muscles of the forearm and alternated with brief periods of silence. Electrical stimulation of the digital nerves evoked consistent reflex responses in the wrist flexor and extensor muscles at a latency of 55.3 /-4.1 ms (range, 50-63 ms). Routine electroencephalographic (EEG) and somatosensory evoked potentials to median nerve stimulation were normal. back-averaging of the EEG activity time-locked to the jerks was performed in two patients with no evidence of abnormal cortical activity. Two patients had episodes of transient respiratory failure related to pneumonia. This caused a long-lasting enhancement of the abnormal hand and finger movements, which became larger and more widespread, with features of posthypoxic myoclonus. We conclude that the abnormal hand and finger movements of patients with pMSA are a form of postural and action myoclonus, and can be described as mini-polymyoclonus.
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ranking = 391.78785427149
keywords = tremor, action
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10/189. myoclonus from selective dentate nucleus degeneration in type 3 gaucher disease.

    OBJECTIVE: To describe a case with a new genetic variant of type 3 gaucher disease presenting with stimulus-sensitive and action myoclonus in the presence of selective dentate abnormalities. DESIGN: Clinical, pathologic, and molecular genetic studies. SETTING: Medical school departments. PATIENT: A 6-year-old girl with type 3 gaucher disease experienced progressively crippling generalized stimulus-sensitive and action myoclonus. Repeated electroencephalographic examination did not show cortical activity associated with the myoclonus, suggesting its subcortical origin. Neuropathological examination revealed selective degeneration of the cerebellar dentate nucleus and dentatorubrothalamic pathway in the face of essentially complete lack of storage in the brain. mutation analysis identified the following 2 mutant alleles: one with a V394L mutation and the other with the lesion RecTL (D409H L444P A456P V460V), which resulted from a recombination event, with the pseudogene located 16 kilobases downstream from the structural gene. CONCLUSION: Given the restricted abnormalities, this genetically unique case provides insight into the pathogenesis of myoclonus and suggests a prominent role for the cerebellar dentate nucleus in its genesis.
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