Cases reported "Myoclonus"

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1/10. 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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ranking = 1
keywords = parkinsonian
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2/10. 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 = 6
keywords = parkinsonian
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3/10. Myoclonic involuntary movement associated with chronic manganese poisoning.

    We report a 17-year-old man showing myoclonic involuntary movement (IVM) associated with chronic manganese (Mn) poisoning. The patient, a welder, showed myoclonic IVM mainly in the right upper and lower extremities, elevated levels of Mn in the blood and hair and high-intensity signals in the globus pallidus on T1-weighted MR images. chelation therapy resulted in improvement of the myoclonic IVM and MRI abnormalities. This is the first report of Mn poisoning characterized by myoclonic IVM without parkinsonism.
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ranking = 0.90989903070189
keywords = parkinsonism
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4/10. EEG characteristics in juvenile Huntington's disease: a case report and review of the literature.

    The clinical features of Juvenile Huntington's disease (J-HD) differ from those of the more common adult-onset form, and include cognitive decline, parkinsonism, myoclonus and seizures. A paucity of literature is available describing the electroencephalographic (EEG) findings. We describe the clinical and EEG characteristics of a patient with genetically confirmed J-HD. A review of previously published cases yielded EEG descriptions in only 23 patients whose disease onset was prior to the age of 32, and only 14 of these were prior to the age of 20. Epileptiform abnormalities were noted in 17 (74%), which was considerably more common than in the adult form. Generalized discharges were noted in nine, with six having polyspike and wave. The remainder had focal or multifocal epileptiform discharges. With genetic testing now available, refinement of the EEG data will be possible.
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ranking = 0.90989903070189
keywords = parkinsonism
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5/10. myoclonus and parkinsonism.

    We have observed three patients in whom the onset of parkinsonian signs was clinically associated with myoclonic movements. In each case, the parkinsonian signs were relatively mild and myoclonus was the major abnormality. The presentation of these three unusual cases within 1 year may be coincidental or may reflect a common pathogenetic mechanism. adrenocorticotropic hormone was given to all three patients. It ameliorated the myoclonus in two patients but had no effect on the third.
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ranking = 5.6395961228075
keywords = parkinsonian, parkinsonism
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6/10. Computed tomography in the management of complications due to ventricular shunting.

    A 13-year-old girl first underwent ventricular drainage at the age of 5. She developed voluntary movement myoclonus of the left hand 7 1/2 years later. A CT scan of the brain 6 months later demonstrated a thalamic cyst consistent with a granulomatous reaction to the shunt.
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ranking = 0.39777810123392
keywords = hunt
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7/10. movement disorders in childhood organic acidurias. Clinical, neuroimaging, and biochemical correlations.

    Over the last 5 years the Pediatric neurology service at King Faisal Specialist Hospital and research Centre (KFSH&RC) has seen 131 infants and children with movement disorders. Forty-nine (37%) had identifiable biochemical defects, 25 of which were organic acidemias. Nineteen of 29 patients with dystonia had organic acidemias, primarily glutaric aciduria type 1 (7 patients), bilateral striatal necrosis (4 patients), and 3-methyl glutaconic aciduria (3 patients). All patients with parkinsonian rigidity (n = 11) had organic acidemias; again, the greatest number accounted for by glutaric aciduria type 1 (7 patients), who had both parkinsonian rigidity combined with dystonia. myoclonus occurred in only 1 of 25 and chorea in 7 of 25 patients with organic acidemias. At the least all patients had bilateral lesions of putamen and head of caudate, seen best in MRI brain scans as increased T2 signal intensities with normal volume, and later with volume loss.
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ranking = 2
keywords = parkinsonian
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8/10. Brainstem myoclonus in a patient with non-dopa-responsive parkinsonism.

    In the few reports where electrophysiologic techniques have been used to characterise stimulus-sensitive myoclonus in the setting of a parkinsonian syndrome, the origin of the myoclonus has usually been found to be cortical. We describe a patient with parkinsonism unresponsive to levodopa who had myoclonus that was both spontaneous and induced by somatosensory stimuli. In addition, autonomic symptoms and a marked sleep disturbance were present early in his illness. Results of electrophysiologic investigations including electromyography (EMG) studies, routine electroencephalography (EEG) recording, jerked locked back-averaging of EEG, and somatosensory evoked potentials were consistent with a brainstem origin for the myoclonic jerks. Following ipsilateral digital and supraorbital electrical stimulation, the earliest muscle activation occurred in the trapezius. An all-night sleep study showed frequent myoclonic jerks during sleep and markedly abnormal sleep architecture. We believe that this patient's myoclonus was related to pathologic changes in brainstem reticular nuclei that occurred as part of his disease process. To our knowledge, brainstem myoclonus has not been described as a feature of parkinsonian syndromes.
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ranking = 6.5494951535094
keywords = parkinsonian, parkinsonism
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9/10. Postural myoclonus induced by phenytoin.

    Involuntary movements are an infrequent complication of treatment with phenytoin and include tremor, asterixis, myoclonus, parkinsonism, and dyskinesias. The mechanism by which phenytoin exerts its actions is unclear. phenytoin has been observed to exert variable effects on dopamine metabolites and also may induce changes in serotonergic activity. In this report, we discuss the available experimental evidence concerning the possible mechanisms of involuntary movements induced by phenytoin. We describe a case of postural myoclonus during treatment with phenytoin.
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ranking = 0.90989903070189
keywords = parkinsonism
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10/10. Cortical myoclonus in levodopa-responsive parkinsonism.

    We observed myoclonic movements of the fingers and wrists in two patients with a levodopa-responsive parkinsonian syndrome most consistent with Parkinson's disease. These patients were studied with electrophysiological techniques. Brief (<50 ms) myoclonic electromyographic discharges showed a time-locked relationship to a focal premovement electroencephalographic potential. Somatosensory-evoked potentials were not enlarged and long-latency reflexes were not grossly exaggerated. This pattern of electrophysiological findings can be distinguished from those previously found in other parkinsonian syndromes. These results provide evidence for a cortical origin of the myoclonus seen in these patients.
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ranking = 5.6395961228075
keywords = parkinsonian, parkinsonism
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