Cases reported "Myoclonus"

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1/6. Focal myoclonus and propriospinal propagation.

    OBJECTIVE: To investigate the relationship between axial segmental myoclonus and propriospinal myoclonus. methods: A patient with a 3-year history of axial jerks evoked by physical effort and unexpected somesthetic and auditory stimulations was investigated. Polygraphy with multiple-channel axial and limb EMG recording was performed with off-line analysis. RESULTS: Spontaneous, somesthetic and acoustic evoked jerks always began in the left rectus abdominis muscle with a single or repetitive EMG burst that could spread to other rostral and caudal muscles without engagement of cranial nerve innervated muscles, consistent with propriospinal propagation. Spontaneous and evoked jerks could however also appear focally in abdominal muscles and remain localized without any diffusion to other muscle segments. CONCLUSION: Focal axial myoclonus and propriospinal myoclonus may coexist. Conceivably the same spinal generator responsible for a monomeric segmental myoclonus may, under conditions of heightened excitability, cause a multimeric propriospinally propagated muscular activation.
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2/6. unconsciousness, automatisms, and myoclonic jerks with diffuse rhythmic 9-Hz discharges.

    A 25-year-old man presented with seizures characterized by unconsciousness, automatic behavior, and myoclonic jerks lasting 0.5 to 8 sec. Videotape analysis of 300 simultaneously recorded electroencephalographic and clinical attacks revealed 8- to 9-Hz 150 to 200 muV sharp waves beginning in both medial temporal areas during eyelid flutters. When discharges spread diffusely on both sides and lasted 2 to 3 sec, 20 degrees forward flexion of the head, unconsciousness, pupil dilation, and staring became evident. Asymmetrical myoclonic jerks invariably appeared. When paroxysms were greater than 3 sec, automatisms and tonic posturing occurred. The electroencephalographic and clinical seizures appeared during the awake and relaxed states, i.e., with normal alpha rhythm. They were suppressed during physical and mental activities and during sleep, i.e., in the absence of alpha rhythm. Our studies suggest that these seizures associated with diffuse electroencephalographic alpha-like activities are subcortical in origin and should be tested in their responsivity to alpha suppressants and inducers.
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3/6. cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma.

    cerebellar ataxia and opsoclonus were the initial manifestations of an associated neuroblastoma in a 20-month-old girl. Two months after the initial symptomatology, a physical examination revealed an abnormal mass palpable left to the midline. Urinary catecholamines were within normal limits. The child's neurological findings improved immediately after surgery, and steroid treatment and the follow-up on her after 2 years revealed normal general and neurological development. The syndrome of myoclonic encephalopathy including cerebellar ataxia, myoclonus and opsoclonus, and its relationship to neuroblastoma is reviewed. Failure to recognize this association can result in delays in both diagnosis and treatment and could be fatal.
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keywords = physical
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4/6. Action myoclonus following acute cerebral anoxia.

    Action myoclonus secondary to posthypoxic encephalopathy is being seen increasingly with improved resuscitation techniques. A case report describes 5 specific physical and occupational therapeutic techniques for achieving independence in ambulation, transfers and self-care: (1) analysis and segmentation of complex motions into small steps; (2) controlled progression of training; (3) voluntary cessation of abnormal activity (pacing); (4) progressive densensitization to external stimuli; and (5) quantification of progress. literature review suggests that posthypoxic action myoclonus is secondary to a loss of inhibitory synapses in the brainstem reticular formation due to low serotonin levels. The proposed therapeutic effect of clonazepam, the drug used in this patient, is decreased serotinin degredation. L-5-hydroxytryptamine, an investigative drug, is also therapeutic, for it stimulates increased serotonin production.
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keywords = physical
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5/6. Benign neonatal sleep myoclonus.

    Benign neonatal sleep myoclonus is a disorder characterised by abnormal episodic jerky movements, which occur exclusively during sleep. When the history is suggestive of the diagnosis, and physical examination and basic investigations are normal, a confident diagnosis can usually be reached, and other potentially more serious neonatal seizure disorders excluded. This allows the doctor to reassure the parents as to the prognosis--the condition resolves spontaneously within the first year, most often within the first three months, with no sequelae. We describe four patients who presented to one unit with this condition.
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6/6. Oculopalatal myoclonus after the one-and-a-half syndrome with facial nerve palsy.

    PURPOSE: The one-and-a-half syndrome is an eye movement disorder characterized by a unilateral gaze palsy and an ipsilateral internuclear ophthalmoplegia. The authors describe a previously unrecognized association between the one-and-a-half syndrome and oculopalatal myoclonus (OPM). methods: Five clinical cases are presented, with pertinent physical findings and radiologic studies. RESULTS: A previously unrecognized association of the one-and-a-half syndrome with subsequent development of OPM appears to exist. Involvement of the facial nerve in patients with the one-and-a-half syndrome may be a predictor of the subsequent development of OPM. CONCLUSION: patients with the one-and-a-half syndrome and facial nerve palsy should be followed closely for possible future development of OPM.
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keywords = physical
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