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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acetazolamide treatment significantly improves action myoclonus in Ramsay Hunt Syndrome. A family with two brothers and a sister, and a sporadic case with Ramsay Hunt Syndrome and uncontrollable action myoclonus, are described where addition of oral acetazolamide lead to marked improvement in their action myoclonus.
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PMID:Acetazolamide therapy improves action myoclonus in Ramsay Hunt Syndrome. 907 41

The author first reviews the definition of myoclonia as an epileptic crisis differentiated from tonic crises and infantile spasms. He reviews the prevalence and incidence found in bibliographic data, under the following headings 1. Early myoclonic encephalopathy or neonatal myoclonic encephalopathy 2. Early epileptic syndrome with bursts of suppression or Otahara's syndrome. 3. West's syndrome. 4. The benign myoclonic epilepsy syndrome of children. 6. Syndrome of myoclonic epilepsy in non-progressive encephalopathy. 7. Early myoclonic epilepsy of children or Dose's syndrome. 8. Lennox-Gastaut syndrome. 9. Syndrome of epilepsy with absences in children. 10. Myoclonic absence epilepsy syndrome. 11. Landau-Kleffner syndrome and the syndrome of continuous slow spike-and-wave epilepsy during slow sleep. 12. Photosensitive epilepsy. 13. Absence epilepsy in young patients. 14. Juvenile myoclonic epilepsy. 15. Syndrome of gran mal epilepsy on waking. 16. Progressive myoclonic epilepsies. The author reviews 6,450 cases, 408 patients who had myoclonic crises, that is 6.3%. The differences seen in this total group of patients were: the myoclonic crises which presented alone, myoclonic crises accompanied by simple typical absences, those initially accompanied by generalized tonic-clonic crises and those presenting typical absences, tonic-clonic generalized crises and myoclonus simultaneously. The course of the different groups is analyzed.
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PMID:[Severity and epidemiology of myoclonic epilepsy]. 1071 95

The neuroleptic malignant syndrome and the serotonin syndrome share many clinical features and may exist on a spectrum of the same disorder. The neuroleptic malignant syndrome, however, is an idiosyncratic drug reaction, whereas the serotonin syndrome is an effect of drug toxicity. Both syndromes present with varying degrees of mental status changes and autonomic instability. In general, patients with the neuroleptic malignant syndrome have higher fevers and pronounced extrapyramidal signs with muscle rigidity, whereas patients with the serotonin syndrome have lower fevers, more gastrointestinal dysfunction, and myoclonus. Treatment for both syndromes consists of removing the offending agent and providing intensive supportive care. Syndrome-specific therapies are controversial.
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PMID:The neuroleptic malignant and serotonin syndromes. 1076 87

The serotonin(5-HT) syndrome(SS) is a condition of both the central and peripheral 5-HTergic hyperstimulation, characterized by a constellation of 5-HT-related side effects(confusion, agitation, restlessness, myoclonus, hyperreflexia, diaphoresis, shivering or tremor in the setting of the recent addition of 5-HTergic agents. The SS is produced most often by the concurrent use of monoamine oxidase inhibitors and other 5-HTergic agents. However, more recent reports suggest that the tricyclic antidepressant or selective serotonin reuptake inhibitor(SSRI) monotherapy induces the SS. Recently, for the operationalized assessment of both the presence and the severity of the core symptoms of the SS, Hegerl et al. developed the Serotonin Syndrome Scale(SSS) as a modification of the diagnostic criteria of the SS proposed by Sternbach. Since, in Japan, some novel 5-HTergic agents have been, and will be in use, recognition of the SS is quite important. Therefore, for clinical application of SSS, we prepared the Japanese-language version (JSSS).
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PMID:[The Japanese version of the serotonin syndrome scale (JSSS)]. 1087 22

Eye movements are necessary for clear and stable vision, for which images of the world should be brought to the fovea and be held steady on the retina. The main types of eye movements consist of saccadic eye movements, vergence, vestibular eye movements, smooth pursuit eye movements and eye movements for visual stabilization. Saccades are fast eye movements and have consistent relationship between their peak velocity and the size of the movements. The burst neurons in the brainstem are the generator of saccades and receive projections from the frontal eye field, supplementary eye field, parietal eye field, basal ganglia, superior colliculus and cerebellum. Saccades are evaluated by a speed and accuracy of the size. There are different types of saccade, such as visually triggered saccades, antisaccades, memory-guided saccade and predictive saccades. The specific test paradigms of saccades may show the localization and the type of diseases. Opsoclonus, flatter-like oscillation, ocular myoclonus, square-wave jerks are characteristic involuntary eye movements. Syndromes of the paramedic pontine reticular formation, medial longitudinal fascicules and one-and-a-half syndrome are caused by disease of the pons.
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PMID:[Neurology of eye movements]. 1146 61

Myoclonus presents as a sudden brief involuntary jerk triggered by the central nervous system. Electromyographic studies enable determining whether the jerk is caused by a muscular contraction, i.e. positive myoclonus, or by an interruption of muscular activity, i.e. negative myoclonus. Many classifications have been proposed, reflecting our lack of understanding about myoclonus. Myoclonus is a symptom and should never be considered as a diagnosis. Clinical history and physical examination are the basis to diagnosis. Clinical neurophysiology testing can reveal a neuroanatomical localization and certain patterns have some etiological specificity. Etiological hypotheses can be put forward on the basis of clinical and neurophysiological data. The cortex is the most commonly identified source of myoclonus, but the subcortical area and spinal area can also be involved. Myoclonus is considered epileptic when it is combined with an epileptiform discharge on the EEG. The International Classification of Epileptic Syndromes should be applied in this situation. Myoclonic epilepsies are a collection of syndromes in which myoclonic seizures are a prominent feature. Myoclonus can occur as one among several seizure components, as the only manifestation of seizure, or as one of multiple seizure types within an epileptic syndrome. Neurophysiological studies are needed to investigate the pathophysiological mechanisms of the myoclonus. Electrophysiological studies report that myoclonic seizures are produced through a cortical generator via a polysynaptic mechanism acting on muscles. Apparently, the epileptiform discharges stimulate the motor cortex resulting in myoclonus jerk. Despite recent progress, advances are still needed to achieve a better understanding of the pathophysiological mechanisms involved in myoclonus. In myoclonic epileptic syndromes, more useful information can probably be obtained from studies grouping several patients with a same epileptic syndrome than from single case reports.
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PMID:[Myoclonus and epilepsy: diagnosis and pathophysiology]. 1834 53

Limb apraxia is a neurological disorder of higher cognitive function characterized by an inability to perform purposeful skilled movements and not attributable to an elementary sensorimotor dysfunction or comprehension difficulty. Corticobasal Syndrome (CBS) is an akinetic rigid syndrome with asymmetric onset and progression with at least one basal ganglia feature (rigidity, limb dystonia or myoclonus) and one cortical feature (limb apraxia, alien hand syndrome or cortical sensory loss). Even though limb apraxia is highly prevalent in CBS (70-80%), very few studies have examined the performance of CBS patients on praxis measures in detail. This review aims to (1) briefly summarize the clinical, neuroanatomical and pathological findings in CBS, (2) briefly outline what limb apraxia is and how it is assessed, (3) to comprehensively review the literature on limb apraxia in CBS to date and (4) to briefly summarize the literature on other forms of apraxia, such as limb-kinetic apraxia and buccofacial apraxia. Overall, the goal of the review is to bring a model-based perspective to the findings available in the literature to date on limb apraxia in CBS.
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PMID:A model-based approach to understanding apraxia in Corticobasal Syndrome. 1908 35

Five patients with spontaneously recovering Dancing Eye Syndrome/Opsoclonus Myoclonus Syndrome are described. Age at presentation ranged from 4 to 19 months. Four had symptoms of fever and a coryzal illness within days to a few weeks prior to the onset. One of the 4 also had varicella zoster 4 weeks before presentation. All had opsoclonus, myoclonus/ataxia and irritability. Associated infective agents identified were Coxsackie virus and rotavirus. Spontaneous improvement of symptoms started within 9 days of presentation and total duration of illness ranged from 10 to 24 days. Developmental progress at follow-up was normal in all cases. A range of immunomodulatory therapies have been advocated for the treatment of Dancing Eye Syndrome/Opsoclonus Myoclonus Syndrome. However, in some children, early spontaneous recovery may occur, an observation which should be borne in mind when designing therapeutic trials in this condition.
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PMID:Dancing Eye Syndrome associated with spontaneous recovery and normal neurodevelopment. 1954 13

Paraneoplastic movement disorders are rare autoimmune nonmetastatic complications of cancer. Common paraneoplastic movement disorders include cerebellar syndrome, opsoclonus myoclonus, basal ganglia disorders, stiff person syndrome, and neuromyotonia. Syndromes usually present before cancer diagnosis and are commonly associated with one or more serum antibodies. Increasing numbers of antibodies have been identified (Hu, Yo, Ri, CV2, amphiphysin, Ma, Ta, Tr, NMDA, mGluR1, PCA2, ANNA-3, VGCCA). Antibodies are highly correlated with the likelihood of an underlying cancer and are closely associated with certain tumors. Clinical clues to paraneoplastic aetiology include speed of onset, severity, speed of progression, resistance to treatment, and more widespread neurological signs than one would expect from nonparaneoplastic aetiologies. Cancer should be sought in those with classical presentations and those with possible presentations who have paraneoplastic antibodies. If no tumor is found on initial investigation, interval screening is advisable. The most common associated cancers found are small cell lung cancer, breast, gynaecological, testicular, lymphoma, and thymoma. Early identification and treatment sometimes leads to neurological improvement and may improve cancer prognosis. Prognosis is dependent on the tumor type and its likely response to treatment.
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PMID:Paraneoplastic movement disorders. 1956 65

The phenomenon of continuous spikes and waves during slow-wave sleep (CSWS) is associated with a number of epileptic syndromes, which share a behavioral phenotype characterized by deterioration of cognitive, behavioral, or sensorimotor functions. Available evidence seems to suggest that spike-wave activity is a result of a complex interaction between cortical and subcortical inhibitory networks and can "per se" produce a transient loss of underlying cortical functions. Syndromes like Landau-Kleffner syndrome, CSWS, and phenomena such as negative myoclonus could share in common--at least at the neurophysiological level--some similarities. Differences in behavioral phenotypes could be explained in term of maturational and genetic differences, as well as by the functional specificity of the involved areas.
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PMID:Neurophysiology of CSWS-associated cognitive dysfunction. 1968 48


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