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Myoclonus

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61. Post-hypoxic myoclonus: Differentiating benign and malignant etiologies in diagnosis and prognosis (PubMed)

Post-hypoxic myoclonus: Differentiating benign and malignant etiologies in diagnosis and prognosis Neurological function following cardiac arrest often determines prognosis. Objective tests, including formal neurological examination and neurophysiological testing, are performed to provide medical providers and decision-makers information to help guide care based on the extent of neurologic injury. The demonstration of post-hypoxic myoclonus on examination has been described to portend poor (...) outcome after cardiac arrest, but recent studies have challenged this idea given that different forms of post-hypoxic myoclonus predict disparate prognoses. The presence of myoclonus status epilepticus (MSE) usually signals a poor outcome, especially if generalized. Lance-Adams syndrome (LAS), another form of post-hypoxic myoclonus, carries a better prognosis. Differentiating subtypes of post-hypoxic myoclonus is therefore critical. This can be difficult in the acute setting with clinical examination

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2017 Clinical Neurophysiology Practice

62. Post-hypoxic myoclonus: The good, the bad and the ugly (PubMed)

Post-hypoxic myoclonus: The good, the bad and the ugly 30214981 2018 12 18 2467-981X 2 2017 Clinical neurophysiology practice Clin Neurophysiol Pract Post-hypoxic myoclonus: The good, the bad and the ugly. 105-106 10.1016/j.cnp.2017.04.002 Acharya Jayant N JN Department of Neurology, Penn State University Hershey Medical Center, 30 Hope Drive, EC037, Hershey, PA 17033, USA. eng Editorial 2017 05 05 Netherlands Clin Neurophysiol Pract 101684308 2467-981X 2018 9 15 6 0 2017 5 5 0 0 2017 5 5 0 1

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2017 Clinical Neurophysiology Practice

63. Classification of Involuntary Movements in Dogs: Myoclonus and Myotonia (PubMed)

Classification of Involuntary Movements in Dogs: Myoclonus and Myotonia Myoclonus is a sudden brief, involuntary muscle jerk. Of all the movement disorders, myoclonus is the most difficult to encapsulate into any simple framework. On the one hand, a classification system is required that is clinically useful to aid in guiding diagnosis and treatment. On the other hand, there is need for a system that organizes current knowledge regarding biological mechanisms to guide scientific research (...) . These 2 needs are distinct, making it challenging to develop a robust classification system suitable for all purposes. We attempt to classify myoclonus as "epileptic" and "nonepileptic" based on its association with epileptic seizures. Myotonia in people may be divided into 2 clinically and molecularly defined forms: (1) nondystrophic myotonias and (2) myotonic dystrophies. The former are a group of skeletal muscle channelopathies characterized by delayed skeletal muscle relaxation. Many distinct

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2017 Journal of Veterinary Internal Medicine

64. Pramipexole Overdose Associated with Visual Hallucinations, Agitation and Myoclonus (PubMed)

Pramipexole Overdose Associated with Visual Hallucinations, Agitation and Myoclonus Pramipexole is a dopamine D2 receptor agonist used to treat idiopathic Parkinson's disease and primary restless legs syndrome. There is limited information on pramipexole overdose.A 59-year-old male ingested 3 mg pramipexole, 2250 mg venlafaxine, 360 mg mirtazapine, with suicidal intent. He presented alert, had normal vital observations and normal pupillary reflexes. He was mildly agitated, reported visual (...) hallucinations and was given 5 mg diazepam. He had a mildly elevated lactate of 1.7 mmol/L, but otherwise normal laboratory investigations. Overnight, he remained agitated with visual hallucinations and developed myoclonus while awake. He had increasing difficulty passing urine on a background of mild chronic urinary retention. On review, 14 h post-ingestion, he was hypervigilant, jittery and mildly agitated. He had pressured speech and difficulty focusing on questioning. He had a heart rate of 110 bpm

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2017 Journal of Medical Toxicology

65. Improvement of Post-hypoxic Myoclonus with Bilateral Pallidal Deep Brain Stimulation: A Case Report and Review of the Literature (PubMed)

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

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2017 Tremor and Other Hyperkinetic Movements

66. Contrast induced spinal myoclonus after percutaneous coronary intervention (PubMed)

Contrast induced spinal myoclonus after percutaneous coronary intervention We present a case of a 77-year-old man diagnosed with contrast-induced spinal myoclonus following primary percutaneous coronary intervention. After being admitted with a diagnosis of anteroseptal myocardial infarction, he underwent primary percutaneous coronary intervention to the left anterior descending artery and was prescribed aspirin, clopidogrel, and intravenous heparin. The following day he developed non (...) -intentional irregular jerky movements confined to the truncal area. In view of rhythmic jerking confined to muscles innervated by a restricted segment of the spinal cord, resistance to supra-spinal influences and voluntary action, and no preceding electroencephalography activity in the contralateral sensorimotor cortex, a diagnosis of spinal myoclonus was made. Spinal myoclonus is a rare entity in which myoclonic movements occur in muscles originating from few (segmental), or many adjacent spinal motor

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2017 Journal of cardiology cases

67. 6‐Mercaptopurine modifies cerebrospinal fluid T cell abnormalities in paediatric opsoclonus–myoclonus as steroid sparer (PubMed)

6‐Mercaptopurine modifies cerebrospinal fluid T cell abnormalities in paediatric opsoclonus–myoclonus as steroid sparer The purpose of this study was to evaluate the capacity of 6-mercaptopurine (6-MP), a known immunosuppressant, to normalize cerebrospinal fluid (CSF) lymphocyte frequencies in opsoclonus-myoclonus syndrome (OMS) and function as a steroid sparer. CSF and blood lymphocytes were immunophenotyped in 11 children with OMS (without CSF B cell expansion) using a comprehensive panel

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2017 Clinical and experimental immunology

68. Progressive Encephalomyelitis with Rigidity and Myoclonus Associated With Anti-GlyR Antibodies and Hodgkin’s Lymphoma: A Case Report (PubMed)

Progressive Encephalomyelitis with Rigidity and Myoclonus Associated With Anti-GlyR Antibodies and Hodgkin’s Lymphoma: A Case Report A 60-year-old man presented with a 6-month history of low back pain and progressive rigidity of the trunk and lower limbs, followed by pruritus, dysphonia, hyperhydrosis, and urinary retention. Brain and spinal imaging were normal. EMG showed involuntary motor unit hyperactivity. Onconeural, antiglutamic acid decarboxylase (anti-GAD), voltage-gated potassium (...) lymphoma (HL), in the absence of bone marrow infiltration nor B symptoms. Polychemotherapy with ABVD protocol was completed with good clinical response and at 1-year follow-up the neurological examination is normal.Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and severe neurological syndrome characterized by muscular rigidity and spasms as well as brain stem and autonomic dysfunction. It can be associated with anti-GAD, GlyR, and DPPX antibodies. All

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2017 Frontiers in neurology

69. Multifocal Myoclonus as a Manifestation of Acute Cerebral Infarction Recovered by Carotid Arterial Stenting (PubMed)

Multifocal Myoclonus as a Manifestation of Acute Cerebral Infarction Recovered by Carotid Arterial Stenting 28122425 2018 11 13 2005-940X 10 1 2017 Jan Journal of movement disorders J Mov Disord Multifocal Myoclonus as a Manifestation of Acute Cerebral Infarction Recovered by Carotid Arterial Stenting. 64-66 10.14802/jmd.16040 Kim Hyangkyoung H Department of Surgery, Chung-Ang University College of Medicine, Seoul, Korea. Byun Jun Soo JS Department of Radiology, Chung-Ang University College

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2017 Journal of movement disorders

70. Metronidazole-Induced Craniocervical Myoclonus with Reversible Bilateral Dentate Nucleus Lesions (PubMed)

Metronidazole-Induced Craniocervical Myoclonus with Reversible Bilateral Dentate Nucleus Lesions 28122422 2018 11 13 2005-940X 10 1 2017 Jan Journal of movement disorders J Mov Disord Metronidazole-Induced Craniocervical Myoclonus with Reversible Bilateral Dentate Nucleus Lesions. 67-68 10.14802/jmd.16021 Lee Hyun Chang HC Department of Neurology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea. Kim Young Eun YE Department of Neurology, Hallym

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2017 Journal of movement disorders

71. Update on Pharmacological Treatment of Progressive Myoclonus Epilepsies (PubMed)

Update on Pharmacological Treatment of Progressive Myoclonus Epilepsies Progressive myoclonus epilepsies (PMEs) are a group of rare inherited diseases featuring a combination of myoclonus, seizures and variable degree of cognitive impairment. Despite extensive investigations, a large number of PMEs remain undiagnosed. In this review, we focus on the current pharmacological approach to PMEs.References were mainly identified through PubMed search until February 2017 and backtracking of references (...) myoclonus or seizures. Lamotrigine should be used with caution due to its unpredictable effect on myoclonus. Avoidance of drugs known to aggravate myoclonus and seizures, such as carbamazepine and phenytoin, is paramount. Psychiatric (in particular depression) and other comorbidities need to be adequately managed. Although a 3- to 4-drug regimen is often necessary to control seizures and myoclonus, particular care should be paid to avoid excessive pharmacological load and neurotoxic side effects. Target

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2017 Current pharmaceutical design

72. Relative Incidence of Seizures and Myoclonus in Alzheimer’s Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia (PubMed)

Relative Incidence of Seizures and Myoclonus in Alzheimer’s Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia Patients with Alzheimer's disease (AD) are more prone to seizures and myoclonus, but relative risk of these symptoms among other dementia types is unknown.To determine incidence of seizures and myoclonus in the three most common neurodegenerative dementias: AD, dementia with Lewy bodies (DLB), and frontotemporal dementia (FTD).Our institution's medical records were (...) reviewed for new-onset unprovoked seizures and myoclonus in patients meeting criteria for AD (n = 1,320), DLB (n = 178), and FTD (n = 348). Cumulative probabilities of developing seizures and myoclonus were compared between diagnostic groups, whereas age-stratified incidence rates were determined relative to control populations.The cumulative probability of developing seizures after disease onset was 11.5% overall, highest in AD (13.4%) and DLB (14.7%) and lowest in FTD (3.0%). The cumulative

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2017 Journal of Alzheimer's disease : JAD

73. Deep brain stimulation for myoclonus-dystonia syndrome with double mutations in DYT1 and DYT11 (PubMed)

Deep brain stimulation for myoclonus-dystonia syndrome with double mutations in DYT1 and DYT11 Myoclonus-dystonia syndrome (MDS) is a rare autosomal dominant inherited disorder characterized by the presentation of both myoclonic jerks and dystonia. Evidence is emerging that deep brain stimulation (DBS) may be a promising treatment for MDS. However, there are no studies reporting the effects of DBS on MDS with double mutations in DYT1 and DYT11. Two refractory MDS patients with double mutations

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2017 Scientific reports

74. Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study (PubMed)

Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study Pediatric-onset opsoclonus-myoclonus syndrome (OMS) is a devastating neuroinflammatory, often paraneoplastic, disorder. The objective was to characterize demographic, clinical, and immunologic aspects in the largest cohort reported to date. Cross-sectional data were collected on 389 children in an IRB-approved, observational study at the National Pediatric Myoclonus Center

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2017 Frontiers in neurology

75. Spinal Myoclonus Responding to Continuous Intrathecal Morphine Pump (PubMed)

Spinal Myoclonus Responding to Continuous Intrathecal Morphine Pump Spinal myoclonus is a sudden, brief, and involuntary movement of segmental or propriospinal muscle groups. Spinal myoclonus has occasionally been reported in patients undergoing opioid therapy, but the pathophysiology of opioid-induced myoclonus has not been elucidated yet. Here, we present two patients with spinal segmental myoclonus secondary to ischemic and radiation myelopathy. Conventional medications did not help treat (...) persistent myoclonus in both legs. Continuous intrathecal morphine infusion was implanted for pain control in one patient, which relieved spinal myoclonus entirely. This experience led to the application of this method with a second patient, leading to the same gratifying result. Spinal myoclonus reemerged as soon as the morphine pumps were off, which confirmed the therapeutic role of opioids. In contrast to the opioid-induced myoclonus, these cases show a benefit of opioids on spinal myoclonus, which

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2017 Journal of movement disorders

76. Negative Myoclonus Induced by Ciprofloxacin (PubMed)

Negative Myoclonus Induced by Ciprofloxacin Negative myoclonus is characterized by a brief sudden loss of muscle activity, and can be caused by a variety of acquired factors and epilepsy syndromes.We show a clear video example of a patient with an extensive negative myoclonus that was induced by ciprofloxacin.Several neurotoxic effects have been associated with the use of ciprofloxacin, but negative myoclonus has not been reported previously.

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2017 Tremor and Other Hyperkinetic Movements

77. TUBB2B Mutation in an Adult Patient with Myoclonus-Dystonia (PubMed)

TUBB2B Mutation in an Adult Patient with Myoclonus-Dystonia Tubulin mutations are a cause of neuronal migrational disorders referred to as tubulinopathies. Mutations in tubulin genes can have a severe impact on microtubule function and result in heterogeneous clinical presentations. Current understanding of the clinical spectrum of tubulinopathies is predominantly based on research in fetal tissue and early-childhood cases.Testing of candidate genes followed by whole-exome sequencing (...) pachygyria and dysmorphic basal ganglia. Her neurological examination demonstrated mild cognitive impairment, myoclonus-dystonia, and skeletal anomalies.Here, we report the unique phenotype of an adult TUBB2B mutation carrier. This case illustrates a relatively mild phenotype compared to previously described fetal and early childhood cases. This highlights the importance of obtaining molecular genetic testing in individuals with a high probability of a genetic disease, including undiagnosed adult

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2017 Case reports in neurology

78. Autosomal recessive inheritance of ADCY5-related generalized dystonia and myoclonus (PubMed)

Autosomal recessive inheritance of ADCY5-related generalized dystonia and myoclonus 28971144 2018 11 13 2376-7839 3 5 2017 Oct Neurology. Genetics Neurol Genet Autosomal recessive inheritance of ADCY5 -related generalized dystonia and myoclonus. 193 10.1212/NXG.0000000000000193 Barrett Matthew J MJ Department of Neurology (M.J.B., C.C.), and Department of Pathology (E.S.W.), University of Virginia Health System, Charlottesville; and Department of Clinical Genomics and Neurology (R.D.), Mayo

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2017 Neurology: Genetics

79. Molecular Diagnosis of Myoclonus Epilepsy Associated with Ragged-Red Fibers Syndrome in the Absence of Ragged Red Fibers (PubMed)

Molecular Diagnosis of Myoclonus Epilepsy Associated with Ragged-Red Fibers Syndrome in the Absence of Ragged Red Fibers Myoclonus epilepsy with ragged-red fibers (MERRFs), an inherited mitochondrial disorder, has characteristic morphological changes of ragged-red fibers (RRFs) in muscle biopsy, in the absence of which mitochondrial etiology is usually not considered in patients with phenotypes suggestive of MERRF. In these circumstances, MERRF can only be diagnosed using genetic analyses

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2017 Frontiers in neurology

80. Rapid Resolution of Enterovirus 71-Associated Opsoclonus Myoclonus Syndrome on Intravenous Immunoglobulin (PubMed)

Rapid Resolution of Enterovirus 71-Associated Opsoclonus Myoclonus Syndrome on Intravenous Immunoglobulin Nonparaneoplastic opsoclonus-myoclonus ataxia syndrome is a rare neuroinflammatory condition featured by opsoclonus, myoclonus, ataxia, and cognitive behavioral disturbance. The authors report an observation of enterovirus 71-associated opsoclonus-myoclonus ataxia syndrome evolving toward full recovery on intravenous intravenous immunoglobulin (IG) treatment. Based on this case report (...) , enterovirus 71 should be added to the list of infectious agents likely involved in opsoclonus-myoclonus ataxia syndrome, including the emerging subgroup of opsoclonus-myoclonus ataxia syndrome recovering without aggressive or prolonged immunosuppressive intervention. Further studies are mandatory to define the precise role, incidence, treatment, and outcome of enterovirus 71 and other infectious agents in benign forms of opsoclonus-myoclonus ataxia syndrome.

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2017 Child Neurology Open

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