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Myoclonus

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101. Combined focal myoclonus and dystonia secondary to a cerebellar hemorrhage: a case report. (PubMed)

Combined focal myoclonus and dystonia secondary to a cerebellar hemorrhage: a case report. Myoclonus is a clinical sign characterized by sudden, brief jerky, shock-like involuntary movements of a muscle or group of muscles. Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. Cases of myoclonus or dystonia secondary to a structural lesion in the cerebellum have been reported. However, there has never been (...) a reported case of combined myoclonus and dystonia secondary to a cerebellar lesion.Herein, we report a 22-year-old female patient with sudden-onset myoclonic jerks, dystonic posture and mild ataxia in the right upper extremity. At age 19, she experienced sudden headache with vomiting. The neurological examination showed ataxia, myoclonus and dystonia in the right upper extremity. Brain images demonstrated a hemorrhage in the right cerebellar hemisphere secondary to a cavernous malformation. After

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2016 BMC Neurology

102. The network sustaining action myoclonus: a MEG-EMG study in patients with EPM1. (PubMed)

The network sustaining action myoclonus: a MEG-EMG study in patients with EPM1. To explore the cortical network sustaining action myoclonus and to found markers of the resulting functional impairment, we evaluated the distribution of the cortico-muscular coherence (CMC) and the frequency of coherent cortical oscillations with magnetoencephalography (MEG). All patients had EPM1 (Unverricht-Lundborg) disease known to present with prominent and disabling movement-activated myoclonus.Using (...) ipsilateral and contralateral to the activated limb. Moreover, the beta-CMC peak occurred at frequencies significantly slower and more stable frequencies in EPM1 patients with respect to controls. The frequency of the beta-CMC peak inversely correlated with the severity of myoclonus.the high and spatially extended beta-CMC peaking in a restricted range of low-beta frequencies in EPM1 patients, suggest that action myoclonus may result not only from an enhanced local synchronization but also from a specific

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2016 BMC Neurology

103. Altered functional brain connectivity in children and young people with opsoclonus-myoclonus syndrome. (PubMed)

Altered functional brain connectivity in children and young people with opsoclonus-myoclonus syndrome. Opsoclonus-myoclonus syndrome (OMS) is a rare, poorly understood condition that can result in long-term cognitive, behavioural, and motor sequelae. Several studies have investigated structural brain changes associated with this condition, but little is known about changes in function. This study aimed to investigate changes in brain functional connectivity in patients with OMS.Seven patients

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2016 Developmental Medicine and Child Neurology

104. [Effect of gabapentin pretreatment on myoclonus after etomidate: a randomized, double-blind, placebo-controlled study]. (PubMed)

[Effect of gabapentin pretreatment on myoclonus after etomidate: a randomized, double-blind, placebo-controlled study]. To evaluate the effects of three different doses of gabapentin pretreatment on the incidence and severity of myoclonic movements linked to etomidate injection.One hundered patients, between 18 and 60 years of age and risk category American Society of Anesthesiologists I-II, with planned elective surgery under general anesthetic were included in the study. The patients were (...) . Two minutes after induction, 2μgkg(-1) fentanyl and 0.8mgkg(-1) rocuronium were administered for tracheal intubation.Demographic data were similar. Incidence and severity of myoclonus in Group G1200 and Group G800 were significantly lower than in Group P; sedation incidence and level were appreciably higher compared to Group P and Group G400. While there was no difference in the incidence of myoclonus between Group P and Group G400, the severity of myoclonus in Group G400 was lower than

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2016 Revista brasileira de anestesiologia

105. Orthostatic myoclonus associated with Caspr2 antibodies (PubMed)

Orthostatic myoclonus associated with Caspr2 antibodies 26944274 2016 09 06 2018 11 13 1526-632X 86 14 2016 Apr 05 Neurology Neurology Orthostatic myoclonus associated with Caspr2 antibodies. 1353-5 10.1212/WNL.0000000000002547 Gövert Felix F From Christian-Albrecht University of Kiel (F.G., K.W., H.H., S.P., G.D., F.L.), Germany; University College London (UCL) Institute of Neurology (R.E.), UK; August Pi i Sunyer Biomedical Research Institute (IDIBAPS) (E.M.-H., J.D.), Hospital Clínic (...) administration & dosage pharmacology Male Membrane Proteins immunology Myoclonus diagnosis drug therapy Nerve Tissue Proteins immunology Posture 2015 08 18 2015 11 30 2016 3 6 6 0 2016 3 6 6 0 2016 9 7 6 0 ppublish 26944274 WNL.0000000000002547 10.1212/WNL.0000000000002547 PMC4826340 Parkinsonism Relat Disord. 2013 Nov;19(11):1013-7 23916653 Mov Disord. 2011 Jul;26(8):1563-5 21469203 Ann Neurol. 2011 Feb;69(2):303-11 21387375 Brain. 2010 Sep;133(9):2734-48 20663977 Arch Neurol. 2007 Sep;64(9):1318-22

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2016 Neurology

106. Myoclonus

Myoclonus Myoclonus Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Myoclonus Myoclonus Aka: Myoclonus From Related Chapters II (...) . Definition Brief, sudden onset, non-rhythmic, involuntary, shock-like mucle jerking III. Causes Myoclonic Exposure Anoxia Creutzfeldt-Jacob Disease Images: Related links to external sites (from Bing) These images are a random sampling from a Bing search on the term "Myoclonus." Click on the image (or right click) to open the source website in a new browser window. Related Studies (from Trip Database) Ontology: Myoclonus (C0027066) Definition (NCI) A sudden, involuntary contraction of a muscle or group

2018 FP Notebook

107. Low-Dose Ketamine Pretreatment Reduces the Incidence and Severity of Myoclonus Induced by Etomidate: A Randomized, Double-Blinded, Controlled Clinical Trial. (PubMed)

Low-Dose Ketamine Pretreatment Reduces the Incidence and Severity of Myoclonus Induced by Etomidate: A Randomized, Double-Blinded, Controlled Clinical Trial. Myoclonic movement induced by etomidate is a common but undesirable problem during general anesthesia induction. To investigate the influence of pretreatment with low-dose ketamine on the incidence and severity of myoclonus induced by etomidate, 104 patients were randomized allocated to 1 of 2 equally sized groups (n = 52) to receive (...) either intravenous low-dose ketamine 0.5 mg/kg (group K) or an equal volume of normal saline (group S) 1 minute before induction of anesthesia with 0.3-mg/kg etomidate. The incidence and severity of myoclonus were assessed for 2 minutes after administration of etomidate. Here, we found that the incidence and intensity of myoclonus were both significantly reduced in low-dose ketamine-treated group compared with saline-treated group. The incidence of adverse effects was low and similar between groups

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2016 Medicine

108. Efficacy and Safty of Opioids for the Prevention of Etomidate-Induced Myoclonus: A Meta-Analysis. (PubMed)

Efficacy and Safty of Opioids for the Prevention of Etomidate-Induced Myoclonus: A Meta-Analysis. Etomidate is a widely used hypnotic drug for induction of general anesthesia and sedation, especially in elderly patients and hemodynamically unstable patients. Myoclonus, however, is the most prominent problem during induction of anesthesia with etomidate. Many agents have been used to prevent it and opioid is one of them. This meta-analysis was to evaluate effects of opioids pretreatment (...) for preventing etomidate-induced myoclonus. We searched the PubMed, EMBASE, and the Cochrane Library databases and published studies in English updated to September 2015. Randomized controlled trials of opioids versus placebo/control in patients were included. We evaluated the prophylactic effect of opioids on etomidate-induced myoclonus. All statistical analysis was performed using RevMan 5.2 software. Nine randomized controlled trials involving 604 participants were included. The results indicated

2016 American journal of therapeutics

109. Clinical and Immunological Features of Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies. (PubMed)

Clinical and Immunological Features of Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies. Most studies on opsoclonus-myoclonus syndrome (OMS) in adults are based on small case series before the era of neuronal cell surface antibody discovery.To report the clinical and immunological features of idiopathic OMS (I-OMS) and paraneoplastic OMS (P-OMS), the occurrence of antibodies to cell surface antigens, and the discovery of a novel cell surface epitope.Retrospective

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2016 JAMA neurology

110. Autosomal dominant cortical tremor, myoclonus, and epilepsy (ADCME) in a unique south Indian community. (PubMed)

Autosomal dominant cortical tremor, myoclonus, and epilepsy (ADCME) in a unique south Indian community. Autosomal dominant cortical tremor, myoclonus, and epilepsy (ADCME)/familial adult onset myoclonic epilepsy (FAME) is a nonprogressive disorder characterized by (1) distal tremors that are usually precipitated by posture and action; (2) stimulus-sensitive myoclonus that is predominantly seen in the upper limb and is precipitated by photic stimuli, fatigue, emotional stress, and sleep

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2016 Epilepsia

111. Effect of gabapentin pretreatment on myoclonus after etomidate: a randomized, double-blind, placebo-controlled study. (PubMed)

Effect of gabapentin pretreatment on myoclonus after etomidate: a randomized, double-blind, placebo-controlled study. To evaluate the effects of three different doses of gabapentin pretreatment on the incidence and severity of myoclonic movements linked to etomidate injection.One hundered patients, between 18 and 60 years of age and risk category American Society of Anesthesiologists I-II, with planned elective surgery under general anesthetic were included in the study. The patients were (...) . Two minutes after induction, 2μgkg(-1) fentanyl and 0.8mgkg(-1) rocuronium were administered for tracheal intubation.Demographic data were similar. Incidence and severity of myoclonus in Group G1200 and Group G800 were significantly lower than in Group P; sedation incidence and level were appreciably higher compared to Group P and Group G400. While there was no difference in the incidence of myoclonus between Group P and Group G400, the severity of myoclonus in Group G400 was lower than

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2016 Brazilian journal of anesthesiology (Elsevier)

112. Myoclonus dystonia and muscular dystrophy: ɛ-sarcoglycan is part of the dystrophin-associated protein complex in brain. (PubMed)

Myoclonus dystonia and muscular dystrophy: ɛ-sarcoglycan is part of the dystrophin-associated protein complex in brain. Myoclonus-dystonia is a neurogenic movement disorder caused by mutations in the gene encoding ɛ-sarcoglycan. By contrast, mutations in the α-, β-, γ-, and δ-sarcoglycan genes cause limb girdle muscular dystrophies. The sarcoglycans are part of the dystrophin-associated protein complex in muscle that is disrupted in several types of muscular dystrophy. Intriguingly, patients (...) with myoclonus-dystonia have no muscle pathology; conversely, limb-girdle muscular dystrophy patients have not been reported to have dystonia-associated features. To gain further insight into the molecular mechanisms underlying these differences, we searched for evidence of a sarcoglycan complex in the brain.Immunoaffinity chromatography and mass spectrometry were used to purify ubiquitous and brain-specific ɛ-sarcoglycan directly from tissue. Cell models were used to determine the effect of mutations

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2016 Movement Disorders

113. KIF5A mutations cause an infantile onset phenotype including severe myoclonus with evidence of mitochondrial dysfunction. (PubMed)

KIF5A mutations cause an infantile onset phenotype including severe myoclonus with evidence of mitochondrial dysfunction. Missense mutations in kinesin family member 5A (KIF5A) cause spastic paraplegia 10. We report on 2 patients with de novo stop-loss frameshift variants in KIF5A resulting in a novel phenotype that includes severe infantile onset myoclonus, hypotonia, optic nerve abnormalities, dysphagia, apnea, and early developmental arrest. We propose that alteration and elongation

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2016 Annals of Neurology

114. A Rare Cause of Myoclonus: A Cupric Conundrum. (PubMed)

A Rare Cause of Myoclonus: A Cupric Conundrum. A woman aged 22 years presented with a 3-year history of jerks when brushing her teeth and a tremor when carrying drinks. Examination revealed a bilateral jerky tremor, stimulus-sensitive myoclonus, and difficulty with tandem gait. Thyroid and liver function test results were normal, but she had rapidly progressive renal failure. Serum copper, ceruloplasmin, and manganese levels were normal, but her urinary copper level was elevated on 2 occasions

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2016 JAMA neurology

115. Clinically distinct electroencephalographic phenotypes of early myoclonus after cardiac arrest. (PubMed)

Clinically distinct electroencephalographic phenotypes of early myoclonus after cardiac arrest. We tested the hypothesis that there are readily classifiable electroencephalographic (EEG) phenotypes of early postanoxic multifocal myoclonus (PAMM) that develop after cardiac arrest.We studied a cohort of consecutive comatose patients treated after cardiac arrest from January 2012 to February 2015. For patients with clinically evident myoclonus before awakening, 2 expert physicians reviewed (...) and classified all EEG recordings. Major categories included: Pattern 1, suppression-burst background with high-amplitude polyspikes in lockstep with myoclonic jerks; and Pattern 2, continuous background with narrow, vertex spike-wave discharges in lockstep with myoclonic jerks. Other patterns were subcortical myoclonus and unclassifiable. We compared population characteristics and outcomes across these EEG subtypes.Overall, 401 patients were included, of whom 69 (16%) had early myoclonus. Among

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2016 Annals of Neurology

116. Unmasking of myoclonus by lacosamide in generalized epilepsy (PubMed)

Unmasking of myoclonus by lacosamide in generalized epilepsy Lacosamide is a new-generation antiseizure medication that is approved for use as an adjunctive treatment and monotherapy in focal epilepsy. Its use in generalized epilepsy, however, has not been adequately evaluated in controlled trials. We report a 67-year-old woman who experienced new-onset myoclonic seizures after initiation of lacosamide. We presume that she had an undiagnosed generalized epilepsy syndrome, likely juvenile (...) myoclonic epilepsy. Myoclonic seizures were not reported before introducing lacosamide and completely resolved after lacosamide was discontinued. This suggests that lacosamide may have the potential to worsen myoclonus, similar to what has been reported with another sodium channel agent, lamotrigine, in some individuals with genetic generalized epilepsy (GGE).

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2016 Epilepsy & behavior case reports

117. Role of major and brain-specific Sgce isoforms in the pathogenesis of myoclonus-dystonia syndrome (PubMed)

Role of major and brain-specific Sgce isoforms in the pathogenesis of myoclonus-dystonia syndrome Loss-of-function mutations in SGCE, which encodes ε-sarcoglycan (ε-SG), cause myoclonus-dystonia syndrome (OMIM159900, DYT11). A "major" ε-SG protein derived from CCDS5637.1 (NM_003919.2) and a "brain-specific" protein, that includes sequence derived from alternative exon 11b (CCDS47642.1, NM_001099400.1), are reportedly localized in post- and pre-synaptic membrane fractions, respectively. Moreover (...) synthesis, cellular development, and cell death and survival. In comparison to WT littermates, Sgcem+/pGt mice exhibited "tiptoe" gait and stimulus-induced appendicular posturing between Postnatal Days 14 to 16. Abnormalities noted in older Sgcem+/pGt mice included reduced body weight, altered gait dynamics, and reduced open-field activity. Overt spontaneous or stimulus-sensitive myoclonus was not apparent on the C57BL/6J background or mixed C57BL/6J-BALB/c and C57BL/6J-129S2 backgrounds. Our data

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2016 Neurobiology of disease

118. Brain inflammation is accompanied by peripheral inflammation in Cstb−/− mice, a model for progressive myoclonus epilepsy (PubMed)

Brain inflammation is accompanied by peripheral inflammation in Cstb−/− mice, a model for progressive myoclonus epilepsy Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is an autosomal recessively inherited childhood-onset neurodegenerative disorder, characterized by myoclonus, seizures, and ataxia. Mutations in the cystatin B gene (CSTB) underlie EPM1. The CSTB-deficient (Cstb -/- ) mouse model recapitulates key features of EPM1, including myoclonic seizures. The mice

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2016 Journal of neuroinflammation

119. Opsoclonus myoclonus ataxia syndrome due to falciparum malaria in two Indian children (PubMed)

Opsoclonus myoclonus ataxia syndrome due to falciparum malaria in two Indian children Opsoclonus-myoclonus ataxia (OMA) syndrome is rare in children, mostly caused by neuroblastoma. Here, we present two very rare cases presenting with OMA due to falciparum malaria. Both of them responded to a high dose of adrenocorticotrophin hormone and intravenous immunoglobulin without recurrence and complication.

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2016 Indian journal of ophthalmology

120. The clinical heterogeneity of drug-induced myoclonus: an illustrated review (PubMed)

The clinical heterogeneity of drug-induced myoclonus: an illustrated review A wide variety of drugs can cause myoclonus. To illustrate this, we first discuss two personally observed cases, one presenting with generalized, but facial-predominant, myoclonus that was induced by amantadine; and the other presenting with propriospinal myoclonus triggered by an antibiotic. We then review the literature on drugs that may cause myoclonus, extracting the corresponding clinical phenotype and suggested (...) underlying pathophysiology. The most frequently reported classes of drugs causing myoclonus include opiates, antidepressants, antipsychotics, and antibiotics. The distribution of myoclonus ranges from focal to generalized, even amongst patients using the same drug, which suggests various neuro-anatomical generators. Possible underlying pathophysiological alterations involve serotonin, dopamine, GABA, and glutamate-related processes at various levels of the neuraxis. The high number of cases of drug

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2016 Journal of neurology

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