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Myoclonus

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41. Progressive myoclonus ataxia: Time for a new definition? Full Text available with Trip Pro

Progressive myoclonus ataxia: Time for a new definition? The clinical demarcation of the syndrome progressive myoclonus ataxia is unclear, leading to a lack of recognition and difficult differentiation from other neurological syndromes.The objective of this study was to apply a refined definition of progressive myoclonus ataxia and describe the clinical characteristics in patients with progressive myoclonus ataxia and with isolated cortical myoclonus.A retro- and prospective analysis (...) was performed in our tertiary referral center between 1994 and 2014. Inclusion criteria for progressive myoclonus ataxia patients were the presence of myoclonus and ataxia with or without infrequent (all types, treatment responsive) epileptic seizures. Inclusion criteria for isolated cortical myoclonus was the presence of isolated cortical myoclonus. Clinical and electrophysiological characteristics data were systematically scored.A total of 14 progressive myoclonus ataxia patients (males, 7; females, 7

2018 Movement Disorders

42. Delineating cerebellar mechanisms in DYT11 myoclonus-dystonia. Full Text available with Trip Pro

Delineating cerebellar mechanisms in DYT11 myoclonus-dystonia. Recent research has highlighted the role of the cerebellum in the pathophysiology of myoclonus-dystonia syndrome as a result of mutations in the ɛ-sarcoglycan gene (DYT11). Specifically, a cerebellar-dependent saccadic adaptation task is dramatically impaired in this patient group.The objective of this study was to investigate whether saccadic deficits coexist with impairments of limb adaptation to provide a potential mechanism

2018 Movement Disorders

43. Long-term GPi-DBS improves motor features in myoclonus-dystonia and enhances social adjustment. (Abstract)

Long-term GPi-DBS improves motor features in myoclonus-dystonia and enhances social adjustment. Good short-term results of pallidal deep brain stimulation have been reported in myoclonus-dystonia. Efficacy and safety in the long term remain to be established. In addition, the actual impact of DBS treatment on social inclusion is unknown. The objective of this study was to assess the long-term clinical outcome, quality of life, and social adjustment of GPi-DBS in patients with ε-sarcoglycan (...) (DYT11)-positive myoclonus-dystonia.Consecutive myoclonus-dystonia patients with ε-sarcoglycan mutations who underwent GPi-DBS were evaluated at least 5 years postoperatively. Motor symptoms were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale including the Disability Scale, a composite score combining the rest and action parts of the Unified Myoclonus Rating Scale and modified Abnormal Involuntary Movement Scale. Standardized video-protocols were assessed by a blinded and external

2018 Movement Disorders

44. Effects of transcutaneous acupoint electrical stimulation combined with low-dose sufentanil pretreatment on the incidence and severity of etomidate-induced myoclonus: A randomized controlled trial. Full Text available with Trip Pro

Effects of transcutaneous acupoint electrical stimulation combined with low-dose sufentanil pretreatment on the incidence and severity of etomidate-induced myoclonus: A randomized controlled trial. Myoclonus is an undesirable phenomenon that occurs after induction of general anesthesia using etomidate. Opioids such as sufentanil are considered effective pretreatment drugs for myoclonus inhibition, although high doses are required. Transcutaneous acupoint electrical stimulation (TAES (...) ), a noninvasive technique involving electrical stimulation of the skin at the acupuncture points, exhibits analgesic effects, promotes anesthetic effects, decreases the dose of anesthetic drugs, and increases endogenous opioid peptide levels. In the present study, we investigated the effects of TAES combined with low-dose sufentanil pretreatment on the incidence and severity of etomidate-induced myoclonus in patients undergoing elective hysteroscopy.In a double-blind manner, 172 patients (American Society

2018 Medicine Controlled trial quality: uncertain

45. Acute inability to mobilise resulting from probable donepezil-induced myoclonus. Full Text available with Trip Pro

Acute inability to mobilise resulting from probable donepezil-induced myoclonus. Donepezil is a commonly prescribed cholinesterase inhibitor in Alzheimer's dementia. We present a case of probable donepezil-induced generalised myoclonus causing total inability to mobilise in a 93-year-old woman.

2018 Age and ageing

46. Electro-clinical Characteristics and Prognostic Significance of Post Anoxic Myoclonus. Full Text available with Trip Pro

Electro-clinical Characteristics and Prognostic Significance of Post Anoxic Myoclonus. To systematically examine the electro-clinical characteristics of post anoxic myoclonus (PAM) and their prognostic implications in comatose cardiac arrest (CA) survivors.Fifty-nine CA survivors who developed myoclonus within 72 h of arrest and underwent continuous EEG monitoring were included in the study. Retrospective chart review was performed for all relevant clinical variables including time of PAM onset (...) ("early onset" when within 24 h) and semiology (multi-focal, facial/ocular, whole body and limbs only). EEG findings including background, reactivity, epileptiform patterns and EEG correlate to myoclonus were reviewed at 6, 12, 24, 48 and 72 h after the return of spontaneous circulation (ROSC). Outcome was categorized as either with recovery of consciousness (Cerebral Performance Category (CPC) 1-3) or without recovery of consciousness (CPC 4-5) at the time of discharge.Seven of the 59 patients (11.9

2018 Resuscitation

47. Glutamate receptor δ2 serum antibodies in pediatric opsoclonus myoclonus ataxia syndrome. Full Text available with Trip Pro

Glutamate receptor δ2 serum antibodies in pediatric opsoclonus myoclonus ataxia syndrome. To identify neuronal surface antibodies in opsoclonus myoclonus ataxia syndrome (OMAS) using contemporary antigen discovery methodology.OMAS patient serum immunoglobulin G immunohistochemistry using age-equivalent rat cerebellar tissue was followed by immunoprecipitation, gel electrophoresis, and mass spectrometry. Data are available via ProteomeXchange (identifier PXD009578). This generated a list

2018 Neurology

48. Comparison of priming versus slow injection for reducing etomidate-induced myoclonus: a randomized controlled study Full Text available with Trip Pro

Comparison of priming versus slow injection for reducing etomidate-induced myoclonus: a randomized controlled study Etomidate injection is often associated with myoclonus. Etomidate injection technique influences the incidence of myoclonus. This study was designed to clarify which of the two injection techniques-slow injection or priming with etomidate-is more effective in reducing myoclonus.This prospective randomized controlled study was conducted on 189 surgical patients allocated to three (...) study groups. Control group (Group C, n = 63) received 0.3 mg/kg etomidate (induction dose) over 20 s. Priming group (Group P, n = 63) received pretreatment with 0.03 mg/kg etomidate, followed after 1 min by an etomidate induction dose over 20 s. Slow injection group (Group S, n = 63) received etomidate (2 mg/ml) induction dose over 2 min. The patients were observed for occurrence and severity of myoclonus for 3 min from the start of injection of the induction dose.The incidence of myoclonus

2018 Korean journal of anesthesiology Controlled trial quality: uncertain

49. Comparison of different doses of intravenous lignocaine on etomidate-induced myoclonus: A prospective randomised and placebo-controlled study Full Text available with Trip Pro

Comparison of different doses of intravenous lignocaine on etomidate-induced myoclonus: A prospective randomised and placebo-controlled study Etomidate-induced myoclonus (EM) is observed in 50%-80% of unpremedicated patients. Low-dose lignocaine has been shown to attenuate but not abolish the EM. The aim of this prospective, randomised controlled study was to compare the different doses of lignocaine on the incidence and severity of EM.Two hundred adult patients were randomly assigned into four (...) groups to receive saline placebo (Group I) or IV lignocaine 0.5 mg/kg (Group II), 1 mg/kg (Group III) or 1.5 mg/kg (Group IV) 2 min before injection etomidate 0.3 mg/kg IV. The patients were assessed for the EM using a four-point intensity scoring system. Our primary outcome was the incidence of myoclonus at 2 min (EM2). The incidence of myoclonus at 1 min (EM1) and severity of myoclonus constituted the secondary outcomes. ANOVA and Pearson Chi-square test were used for statistical analysis and P

2018 Indian journal of anaesthesia Controlled trial quality: uncertain

50. A rare case of isolated myoclonus in an elderly male without a history of epilepsy Full Text available with Trip Pro

A rare case of isolated myoclonus in an elderly male without a history of epilepsy Through this case report we attempt to highlight the presentation, initial investigation and management of lingual myoclonus as well as consolidate relevant literature.We present a unique case of a 72-year-old man who was admitted to the hospital for a sudden onset episodic speech arrest. Lingual myoclonus, an isolated movement disorder, manifested as an intermittent expressive aphasia secondary to the intrusion (...) strokes, seizures, medications/toxins or CNS infections. Without a clear etiology on initial diagnostic evaluation, the patient was empirically treated as no clear guidelines exist. This case presentation is an attempt to add to the current understanding of lingual myoclonus.

2018 Clinical Neurophysiology Practice

51. The interrelation between clinical presentation and neurophysiology of posthypoxic myoclonus Full Text available with Trip Pro

The interrelation between clinical presentation and neurophysiology of posthypoxic myoclonus Posthypoxic myoclonus (PHM) in the first few days after resuscitation can be divided clinically into generalized and focal (uni- and multifocal) subtypes. The former is associated with a subcortical origin and poor prognosis in patients with postanoxic encephalopathy (PAE), and the latter with a cortical origin and better prognosis. However, use of PHM as prognosticator in PAE is hampered by the modest (...) ).Clinically assessed origin of PHM did not match the result obtained with neurophysiology: cortical PHM was more likely present in generalized than in focal PHM. In addition, some cases demonstrated co-occurrence of cortical and subcortical myoclonus. Patients that recovered from PAE had cortical myoclonus (1 generalized, 1 focal).Hypoxic damage to variable cortical and subcortical areas in the brain may lead to mixed and varying clinical manifestations of myoclonus that differ of those patients

2018 Annals of clinical and translational neurology

52. Oxycodone for prevention of etomidate-induced myoclonus: a randomized double-blind controlled trial Full Text available with Trip Pro

Oxycodone for prevention of etomidate-induced myoclonus: a randomized double-blind controlled trial Objective This study was performed compare the effectiveness of oxycodone and fentanyl in reducing the incidence and severity of etomidate-induced myoclonus. Methods In total, 162 patients with an American Society of Anesthesiologists physical status of I or II were assigned at random to three groups. Patients assigned to Group O received 0.1 mg/kg of oxycodone (n = 54), those assigned to Group F (...) were given 1 µg/kg of fentanyl (n = 54), and those assigned to Group S were given an equal volume of saline intravenously 2 minutes prior to administration of 0.3 mg/kg of etomidate (n = 54). The incidence and severity of myoclonus was evaluated 2 minutes after etomidate administration. The patients' vital signs, coughing, nausea, dizziness, and other related adverse reactions were also recorded. Results The incidence of myoclonus was significantly lower in Group O (0.0%) than in Group F (31.5

2018 The Journal of international medical research Controlled trial quality: uncertain

53. Identification of a Novel Homozygous Splice-Site Mutation in SCARB2 that Causes Progressive Myoclonus Epilepsy with or without Renal Failure Full Text available with Trip Pro

Identification of a Novel Homozygous Splice-Site Mutation in SCARB2 that Causes Progressive Myoclonus Epilepsy with or without Renal Failure Progressive myoclonus epilepsies (PMEs) comprise a group of rare genetic disorders characterized by action myoclonus, epileptic seizures, and ataxia with progressive neurologic decline. Due to clinical and genetic heterogeneity of PMEs, it is difficult to decide which genes are affected. The aim of this study was to report an action myoclonus

2018 Chinese medical journal

54. Segmental Spinal Myoclonus Complicating Lumbar Transforaminal Epidural Steroid Injection. (Abstract)

Segmental Spinal Myoclonus Complicating Lumbar Transforaminal Epidural Steroid Injection. Lumbar transforaminal epidural steroid injection is commonly used for treating lumbar radicular pain. We report a case of segmental spinal myoclonus that developed during lumbar transforaminal epidural injection with local anesthetic and steroid.A 58-year-old woman with failed back surgery syndrome presented with low back and right L3 and L4 radicular pain. As she had responded well previously (...) no abnormalities from blood tests and magnetic resonance imaging of the lower thoracic and lumbar spine. A neurologist subsequently made the clinical diagnosis of segmental spinal myoclonus. The myoclonus improved over 1 month and eventually resolved completely.Segmental spinal myoclonus is a rare complication after lumbar transforaminal epidural steroid and local anesthetic injection. Pain physicians should be aware of this potential complication.

2018 Regional Anesthesia and Pain Medicine

55. Ultrasound as Diagnostic Tool for Diaphragmatic Myoclonus Full Text available with Trip Pro

Ultrasound as Diagnostic Tool for Diaphragmatic Myoclonus Diaphragmatic myoclonus is a rare disorder of repetitive diaphragmatic contractions, acknowledged to be a spectrum that includes psychogenic features. Electromyography has been the diagnostic tool most commonly used in the literature.To test if we could perform a noninvasive technique to delineate the diaphragm as the source of abnormal movements and demonstrate distractibility and entrainability, we used B-mode ultrasound in a patient (...) with diaphragmatic myoclonus.Ultrasound imaging clearly delineated the diaphragm as the source of her abdominal movements. We were able to demonstrate entrainability of the diaphragm to hand tapping to a prescribed rhythm set by examiner.We recommend the use of ultrasound as a noninvasive, convenient diagnostic tool for further studies of diaphragmatic myoclonus. We agree with previous findings that diaphragmatic myoclonus may be a functional movement disorder, as evidenced by distractibility and entrainability

2016 Movement disorders clinical practice

56. Improvement of Isolated Myoclonus Phenotype in Myoclonus Dystonia after Pallidal Deep Brain Stimulation Full Text available with Trip Pro

Improvement of Isolated Myoclonus Phenotype in Myoclonus Dystonia after Pallidal Deep Brain Stimulation Myoclonus-dystonia is a condition that manifests predominantly as myoclonic jerks with focal dystonia. It is genetically heterogeneous with most mutations in the epsilon sarcoglycan gene (SGCE). In medically refractory cases, deep brain stimulation (DBS) has been shown to provide marked sustainable clinical improvement, especially in SGCE-positive patients. We present two patients (...) with myoclonus-dystonia (one SGCE positive and the other SGCE negative) who have the isolated myoclonus phenotype and had DBS leads implanted in the bilateral globus pallidus internus (GPi).We review their longitudinal Unified Myoclonus Rating Scale scores along with their DBS programming parameters and compare them with published cases in the literature.Both patients demonstrated complete amelioration of all aspects of myoclonus within 6-12 months after surgery. The patient with the SGCE-negative mutation

2016 Tremor and Other Hyperkinetic Movements

57. 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

58. Improvement of Post-hypoxic Myoclonus with Bilateral Pallidal Deep Brain Stimulation: A Case Report and Review of the Literature Full Text available with Trip Pro

Improvement of Post-hypoxic Myoclonus with Bilateral Pallidal Deep Brain Stimulation: A Case Report and Review of the Literature Post-hypoxic myoclonus (PHM) is a syndrome that occurs when a patient has suffered hypoxic brain injury. The myoclonus is usually multifocal and generalized, often stemming from both cortical and subcortical origins. In severe cases, pharmacological treatments with antiepileptic medications may not satisfactorily control the myoclonus.We present a case of a 23-year (...) -old male with chronic medication refractory PHM following a cardiopulmonary arrest related to an asthmatic attack who improved with bilateral globus pallidus internus (GPi) deep brain stimulation (DBS). We review the clinical features of PHM, as well as the preoperative and postoperative Unified Myoclonus Rating Scale scores and DBS programming parameters in this patient and compare them with the three other published PHM-DBS cases in the literature.This patient experienced an alleviation

2017 Tremor and Other Hyperkinetic Movements

59. Epileptic Negative Myoclonus as the First and Only Symptom in a Challenging Diagnosis of Benign Epilepsy With Centrotemporal Spikes Full Text available with Trip Pro

Epileptic Negative Myoclonus as the First and Only Symptom in a Challenging Diagnosis of Benign Epilepsy With Centrotemporal Spikes To investigate the clinical and neurophysiological characteristics of epileptic negative myoclonus as the first and only ictal symptom of benign epilepsy with centrotemporal spikes.Electrophysiological evaluations included polygraphic recordings with simultaneous video electroencephalogram monitoring and tests performed with patient's upper limb outstretched (...) in standing posture. Epileptic negative myoclonus manifestations, electrophysiological features, and responses to antiepileptic drugs were analyzed.The authors report 2 patients with benign epilepsy with centrotemporal spikes, who had epileptic negative myoclonus as the first and only seizure type. Video electroencephalogram monitoring results showed that their negative myoclonus seizures were emanating from the contralateral central and the parietal regions. Epileptic negative myoclonus was controlled

2017 Child Neurology Open

60. The Inter-rater Variability of Clinical Assessment in Post-anoxic Myoclonus Full Text available with Trip Pro

The Inter-rater Variability of Clinical Assessment in Post-anoxic Myoclonus Acute post-anoxic myoclonus (PAM) can be divided into an unfavorable (generalized/subcortical) and more favorable ((multi)focal/cortical) outcome group that could support prognostication in post-anoxic encephalopathy; however, the inter-rater variability of clinically assessing these PAM subtypes is unknown.We prospectively examined PAM patients using a standardized video protocol. Videos were rated by three (...) neurologists who classified PAM phenotype (generalized/(multi)focal), stimulus sensitivity, localization (proximal/distal/both), and severity (Clinical Global Impression-Severity Scale (CGI-S) and Unified Myoclonus Rating Scale (UMRS)).Poor inter-rater agreement was found for phenotype and stimulus sensitivity (κ=-0.05), moderate agreement for localization (κ=0.46). Substantial agreement was obtained for the CGI-S (intraclass correlation coefficient (ICC)=0.64) and almost perfect agreement for the UMRS

2017 Tremor and Other Hyperkinetic Movements

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