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Myoclonus

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181. Clinical diagnosis of propriospinal myoclonus is unreliable: An electrophysiologic study. (Abstract)

Clinical diagnosis of propriospinal myoclonus is unreliable: An electrophysiologic study. Propriospinal myoclonus is a rare movement disorder that is hypothesized to arise from a spinal generator that transmits activity up and down the spinal cord via long propriospinal pathways. Polymyography is mandatory for the diagnosis, but the typical electrophysiological pattern described for propriospinal myoclonus has been also found in patients with psychogenic axial jerks, supported by the presence (...) of a Bereitschaftspotential (BP; from German, "readiness potential," also called the premotor potential). We evaluated polymyographic findings in 65 patients referred to us with a clinical diagnosis of propriospinal myoclonus and also looked for the presence of the BP, as detected by jerk-locked back-averaging. At clinical reassessment by a movement disorder specialist, nearly one-half of the patients had clinical clues suggestive of a psychogenic cause of the jerks. Electrophysiological studies were carried out on all

2013 Movement Disorders

182. A descriptive study of myoclonus associated with etomidate procedural sedation in the ED. (Abstract)

A descriptive study of myoclonus associated with etomidate procedural sedation in the ED. Myoclonus is a well-recognized side effect of etomidate when given in induction doses for rapid sequence intubation. Most of the data reported on myoclonus with emergency department (ED) sedation doses are reported as a secondary finding.Our objective was to prospectively quantify the incidence and duration of myoclonus associated with the administration of etomidate in the lower doses given for procedural (...) sedation in the ED.This was a prospective descriptive study performed between September 2008 and September 2010 at an urban teaching hospital ED with approximately 50000 patient visits per year. Procedural sedation was performed at the discretion of the treating emergency physician, and adult patients receiving etomidate were eligible for enrollment. The occurrence and duration of myoclonus were observed and recorded. Any interference of myoclonus with the ability to complete the procedure was recorded

2013 American Journal of Emergency Medicine

183. Transitory stapedial myoclonus in a patient with benign fasciculation syndrome. (Abstract)

Transitory stapedial myoclonus in a patient with benign fasciculation syndrome. We report a previously undescribed association between transitory stapedial myoclonus, objective tinnitus and benign fasciculation syndrome.Case report and review of the world literature regarding stapedial myoclonus.A 30-year-old man with a diagnosis of benign fasciculation syndrome abruptly developed severe, low-pitched tinnitus on the right side. Otoscopic examination revealed rhythmic movement of the tympanic (...) membrane, which was synchronous with the tinnitus. No palatal spasm was noted on nasopharyngeal examination. Brain magnetic resonance imaging and pure tone audiometry were unremarkable. Based on these findings, a diagnosis of objective tinnitus due to stapedial myoclonus was made. The objective tinnitus spontaneously disappeared within 48 hours of its appearance, but in the following days the patient suffered frequent, brief episodes of objective tinnitus lasting only a few seconds.The occurrence

2013 Journal of Laryngology & Otology

184. 'North Sea' progressive myoclonus epilepsy: phenotype of subjects with GOSR2 mutation. Full Text available with Trip Pro

'North Sea' progressive myoclonus epilepsy: phenotype of subjects with GOSR2 mutation. We previously identified a homozygous mutation in the Golgi SNAP receptor complex 2 gene (GOSR2) in six patients with progressive myoclonus epilepsy. To define the syndrome better we analysed the clinical and electrophysiological phenotype in 12 patients with GOSR2 mutations, including six new unrelated subjects. Clinical presentation was remarkably similar with early onset ataxia (average 2 years of age (...) the North Sea, extending to the coastal region of Northern Norway. 'North Sea' progressive myoclonus epilepsy has a homogeneous clinical presentation and relentless disease course allowing ready identification from the other progressive myoclonus epilepsies.

2013 Brain

185. Clinical characteristics and therapeutic response of objective tinnitus due to middle ear myoclonus: A large case series. Full Text available with Trip Pro

Clinical characteristics and therapeutic response of objective tinnitus due to middle ear myoclonus: A large case series. To evaluate the clinical characteristics and therapeutic response of tinnitus due to middle ear myoclonus (MEM) and to suggest appropriate diagnostic methods.Retrospective chart review.This study included 58 patients with tinnitus diagnosed with MEM, who were seen from January 2004 to July 2011. Clinical and audiological characteristics were investigated. The therapeutic (...) with forceful eyelid closure was observed in 15% of patients. Impedance audiogram and otoendoscopic examinations of the tympanic membrane were helpful tools for diagnosing MEM. With medical therapy, more than 75% of patients exhibited complete or partial remission of their tinnitus. Patients with intractable MEM who underwent sectioning of the middle ear tendons had very good outcomes.Tinnitus due to middle ear myoclonus seems to occur in young patients and to be related to stress or noise. Information

2013 Laryngoscope

186. White Matter Degeneration with Unverricht-Lundborg Progressive Myoclonus Epilepsy: A Translational Diffusion-Tensor Imaging Study in Patients and Cystatin B-Deficient Mice. Full Text available with Trip Pro

White Matter Degeneration with Unverricht-Lundborg Progressive Myoclonus Epilepsy: A Translational Diffusion-Tensor Imaging Study in Patients and Cystatin B-Deficient Mice. To study white matter (WM) changes in patients with Unverricht-Lundborg progressive myoclonus epilepsy (EPM1) caused by mutations in the cystatin B gene and in the cystatin B-deficient (Cstb-/-) mouse model and to validate imaging findings with histopathologic analysis of mice.Informed consent was obtained and the study

2013 Radiology

188. Defining the epsilon-sarcoglycan (SGCE) gene phenotypic signature in myoclonus-dystonia: A reappraisal of genetic testing criteria. (Abstract)

Defining the epsilon-sarcoglycan (SGCE) gene phenotypic signature in myoclonus-dystonia: A reappraisal of genetic testing criteria. Mutations or exon deletions of the epsilon-sarcoglycan (SGCE) gene cause myoclonus-dystonia (M-D), but a subset of M-D patients are mutation-negative and the sensitivity and specificity of current genetic testing criteria are unknown. We screened 46 newly enrolled M-D patients for SGCE mutations and deletions; moreover, 24 subjects previously testing negative

2013 Movement Disorders

189. [Role of target controlled infusion of remifentanil for the prevention of etomidate induced myoclonus during general anesthesia]. (Abstract)

[Role of target controlled infusion of remifentanil for the prevention of etomidate induced myoclonus during general anesthesia]. To explore the preventive effect of etomidate-induced myoclonus by different concentrations of target-controlled infusion of remifentanil.A total of 120 cases undergoing general anesthesia were randomly divided into 4 groups according to different concentrations of target controlled infusion of remifentanil using plasma target controlled infusion ( Minto model (...) ) , in which the target concentration was set as 1 f.Lg/L ( group A) , 2 f.Lg/L ( group B) , 3 f.Lg/L ( group C) , and 4 f.Lg/L ( group D) . Five minutes after the balance of effect compartment, induction with etomidate 0. 3 mglkg was conducted intravenously. The intensity and duration of myoclonus was recorded.The incidence of etomidate-induced myoclonus was 70.9%, 33.3%, 26.7%, and 0 in groups A, B, C, and D, respectively. Along with the increase of the remifentanil concentration, the incidence of severe

2013 Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae Controlled trial quality: uncertain

190. Acute dyskinesia, myoclonus, and akathisa in an adolescent male abusing quetiapine via nasal insufflation: a case study. Full Text available with Trip Pro

Acute dyskinesia, myoclonus, and akathisa in an adolescent male abusing quetiapine via nasal insufflation: a case study. Although the benefits of antipsychotic pharmacotherapy can be pronounced, many patients develop unwanted adverse effects including a variety of movement disorders. Compared with the traditional antipsychotics, the atypical antipsychotics have a decreased risk for associated movement disorders. Drug-induced movement disorders can occur, however, and the risk of adverse events

2013 BMC Pediatrics

191. Comparison of effects of fentanyl and remifentanil on hemodynamic response to endotracheal intubation and myoclonus in elderly patients with etomidate induction Full Text available with Trip Pro

Comparison of effects of fentanyl and remifentanil on hemodynamic response to endotracheal intubation and myoclonus in elderly patients with etomidate induction Etomidate has a stable hemodynamic profile after induction, but hypertension and tachycardia are frequent after intubation as well as myoclonus. We compared the effects of fentanyl and remifentanil on the hemodynamic response to intubation and myoclonus during etomidate induction in elderly patients.Ninety ASA I or II patients aged over (...) of myoclonus were recorded.After intubation, group R showed significant decreases compared with groups C and F for all of the hemodynamic variables measured. The incidences of increases in SBP and HR of more than 30% of the baseline levels, SBP of > 200 mmHg, and HR of > 120 beats/min were significantly lower in group R (0%, 10%, 0%, and 0%, respectively) compared with groups C (83%, 83%, 30%, and 13%, respectively) and F (63%, 77%, 13%, and 7%, respectively). The frequency and intensity of myoclonus were

2013 Korean journal of anesthesiology Controlled trial quality: uncertain

192. Somatostatin receptor positron emission tomography/computed tomography (PET/CT) in the evaluation of opsoclonus-myoclonus ataxia syndrome Full Text available with Trip Pro

Somatostatin receptor positron emission tomography/computed tomography (PET/CT) in the evaluation of opsoclonus-myoclonus ataxia syndrome Opsoclonus-myoclonus ataxia (OMA) syndrome is the most common paraneoplastic neurological syndrome of childhood, associated with occult neuroblastoma in 20%-50% of all cases. OMA is the initial presentation of neuroblastoma in 1%-3% of children. Conventional radiological imaging approaches include chest radiography and abdominal computed tomography (CT

2013 Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India

193. Manual Quantitative Assessment of Amplitude and Sleep Stage Distribution of Excessive Fragmentary Myoclonus Full Text available with Trip Pro

Manual Quantitative Assessment of Amplitude and Sleep Stage Distribution of Excessive Fragmentary Myoclonus Excessive fragmentary myoclonus (EFM) consists of brief, asynchronous, twitch-like movements appearing asymmetrically in sleep. The new AASM Manual for the Scoring of Sleep and Associated Events identifies some EFM scoring criteria but does not provide amplitude criteria for scoring EFM. Older observational series have used 50 μVs. We report data from various amplitude criteria using

2013 Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine

194. Antiglycine receptor antibody and encephalomyelitis with rigidity and myoclonus (PERM) related to small cell lung cancer Full Text available with Trip Pro

Antiglycine receptor antibody and encephalomyelitis with rigidity and myoclonus (PERM) related to small cell lung cancer A 39-year-old man (a lifetime non-smoker) presented with a locked left jaw and leg myoclonus. Clinical and electromyographic findings were in keeping with progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome. A thoracic CT scan demonstrated a 19 mm right hilar nodule, which was proven to be small cell lung cancer on bronchoscopic biopsy. Serological

2013 BMJ case reports

195. Metabolic changes in DYT11 myoclonus-dystonia. Full Text available with Trip Pro

Metabolic changes in DYT11 myoclonus-dystonia. To identify brain regions with metabolic changes in DYT11 myoclonus-dystonia (DYT11-MD) relative to control subjects and to compare metabolic abnormalities in DYT11-MD with those found in other forms of hereditary dystonia and in posthypoxic myoclonus.[(18)F]-fluorodeoxyglucose PET was performed in 6 subjects with DYT11-MD (age 30.5 ± 10.1 years) and in 6 nonmanifesting DYT11 mutation carriers (NM-DYT11; age 59.1 ± 8.9 years) representing (...) increases in the inferior pons and in the posterior thalamus as well as reductions in the ventromedial prefrontal cortex. Significant phenotype-related increases were present in the parasagittal cerebellum. This latter abnormality was shared with posthypoxic myoclonus, but not with other forms of dystonia. By contrast, all dystonia cohorts exhibited significant metabolic increases in the superior parietal lobule.The findings are consistent with a subcortical myoclonus generator in DYT11-MD, likely

2013 Neurology

196. Surgical treatment of myoclonus dystonia syndrome. (Abstract)

Surgical treatment of myoclonus dystonia syndrome. Myoclonus dystonia (M-D) syndrome is a heritable movement disorder characterized by myoclonic jerks and dystonia primarily of the upper extremities. M-D remains poorly responsive to pharmacological treatment. Emerging reports suggest good response to DBS of the internal globus pallidus (GPi) and ventral intermediate nucleus (VIM) of the thalamus. This study aimed to appraise the value of these two DBS targets by evaluating reports available (...) in the literature. A systematic search of published case reports and case series was performed on Medline and Embase. Responses to DBS were evaluated. Myoclonus was assessed with the Unified Myoclonus Rating Scale (UMRS) and dystonia by the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). The primary outcome of interest was the relative improvements noted with GPi, compared to VIM stimulation. A total of 17 publications yielded 40 unique cases, with mean follow-up of 27.2 months. All patients demonstrated

2013 Movement Disorders

197. Middle ear myoclonus cured by selective tenotomy of the tensor tympani: strategies for targeted intervention for middle ear muscles. (Abstract)

Middle ear myoclonus cured by selective tenotomy of the tensor tympani: strategies for targeted intervention for middle ear muscles. To describe a case of middle ear myoclonus that was successfully cured by selective transection of the tensor tympani (TT) without sectioning the stapedius tendon (ST) and to review previously reported cases, elucidating precipitating factors for interventions targeting middle ear muscles.One case we encountered and a recent systematic review published in 2012 (...) .In addition to our case, 23 cases identified by the previous systematic review regarding middle ear myoclonus in which surgical interventions were conducted.Outcomes for selective tenotomy of TT or ST were analyzed focusing on the following 6 preoperative factors: 1) history of facial palsy, 2) provoking factors for tinnitus, 3) auscultation of the ear, 4) movement of the ear drum, 5) complication with palatal myoclonus, and 6) confirmation of myoclonus during surgery. Among these, the first 2 factors

2013 Otology and Neurotology

198. 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

2015 FP Notebook

199. Prevention of etomidate-related myoclonus in anesthetic induction by pretreatment with magnesium. (Abstract)

Prevention of etomidate-related myoclonus in anesthetic induction by pretreatment with magnesium. Etomidate frequently leads to myoclonic jerks during anesthetic induction. This study aimed to detect if pretreatment with magnesium decreases myoclonus incidence.A hundred ASA (the American Society of Anesthesiologists) I-II patients were included and randomized into 2 groups. Three minutes before etomidate induction of anesthesia (by 0.3 mg/kg intravenous etomidate), Group M received 2.48 mmol (...) (60 mg) intravenous magnesium sulphate and Group P received equal volume of intravenous saline. Myoclonus was evaluated as "present/absent".The rate of myoclonus was significantly lower in Group M than in Group P (p < 0.01). Hemodynamic parameters revealed no significant difference between the two groups.Low dose magnesium pretreatment before etomidate induction of anesthesia significantly reduces unwanted myoclonic jerks and also protects the hemodynamic stability.

2012 Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences Controlled trial quality: uncertain

200. A proof-of-concept trial of the whey protein alfa-lactalbumin in chronic cortical myoclonus. (Abstract)

A proof-of-concept trial of the whey protein alfa-lactalbumin in chronic cortical myoclonus. 22025266 2012 02 28 2015 11 19 1531-8257 26 14 2011 Dec Movement disorders : official journal of the Movement Disorder Society Mov. Disord. A proof-of-concept trial of the whey protein alfa-lactalbumin in chronic cortical myoclonus. 2573-5 10.1002/mds.23908 Errichiello Luca L Pezzella Marianna M Santulli Lia L Striano Salvatore S Zara Federico F Minetti Carlo C Mainardi Paolo P Striano Pasquale P eng (...) Letter Randomized Controlled Trial 2011 10 24 United States Mov Disord 8610688 0885-3185 0 Milk Proteins 0 Whey Proteins 9013-90-5 Lactalbumin IM Adult Chronic Disease Feasibility Studies Female Humans Lactalbumin administration & dosage Male Middle Aged Milk Proteins administration & dosage Myoclonus drug therapy Treatment Failure Whey Proteins Young Adult 2011 04 28 2011 06 16 2011 07 18 2011 10 26 6 0 2011 10 26 6 0 2012 3 1 6 0 ppublish 22025266 10.1002/mds.23908

2012 Movement disorders : official journal of the Movement Disorder Society Controlled trial quality: uncertain

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