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https://www.readbyqxmd.com/read/29643630/comparison-of-pretreatment-with-dexmedetomidine-with-midazolam-for-prevention-of-etomidate-induced-myoclonus-and-attenuation-of-stress-response-at-intubation-a-randomized-controlled-study
#1
Swarnendu Dey, Meenakshi Kumar
Background and Aims: Myoclonus is a common problem during induction of anesthesia with etomidate. A variety of drugs have been used to decrease the incidence of myoclonus. In this study we compared the effects of dexmedetomidine and midazolam pretreatment on the incidence of etomidate induced myoclonus. We also studied the effects of these drugs on attenuation of stress response at laryngoscopy and intubation on induction with etomidate. Material and Methods: Eighty adult patients (18 to 60 years age) of either sex, American Society of Anestheiologists physical status I and II undergoing elective general surgeries under general anesthesia were randomly allocated into two groups...
January 2018: Journal of Anaesthesiology, Clinical Pharmacology
https://www.readbyqxmd.com/read/29643517/-application-of-scalp-recorded-high-frequency-oscillations-in-epileptic-encephalopathy-with-continuous-spike-and-wave-during-sleep
#2
P Gong, Z X Yang, J Xue, P Qian, H P Yang, X Y Liu, K G Bian
OBJECTIVE: To investigate the clinical significance of high-frequency oscillations (HFOs) on scalp electroencephalography (EEG) in patients with epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS). METHODS: Twenty-one CSWS patients treated for epilepsy from January 2006 to December 2016 in Pediatric Department of Peking University First Hospital were enrolled into the study. Selected clinical variables including gender, age parameters, seizure frequencies and antiepileptic drugs were compared between (a)...
April 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29629926/neuroprognostication-postcardiac-arrest-translating-probabilities-to-individuals
#3
Clifton W Callaway
PURPOSE OF REVIEW: Predicting neurological recovery in patients who are comatose after cardiac arrest is an important activity during postarrest care, and this prediction can affect survival. As no early test or clinical finding perfectly predicts potential for recovery, guidelines recommend using data from multiple examinations or tests to estimate patient prognosis. RECENT FINDINGS: Studies reported accuracy of initial clinical examination, progression of clinical examination, early (<24 h) brain imaging, electroencephalography (EEG), evoked potentials, later (>24 h) brain imaging, blood markers of brain injury, and cerebral oximetry for predicting good or poor outcome...
April 6, 2018: Current Opinion in Critical Care
https://www.readbyqxmd.com/read/29628850/development-of-postoperative-central-anticholinergic-syndrome-due-to-low-dose-intravenous-fentanyl
#4
Hojae Cho, Jiyeon Kim
A 37-year-old female patient, 57 kg and 160 cm, underwent laparoscopic appendectomy. In the recovery room, fentanyl 100 mcg was intravenously administered for pain control. Three minutes after the administration, the patient developed intense and uncontrolled myoclonus, lower limb rigidity, agitation, aphasia, and periocular and neck swelling. The myoclonus and rigidity were suspected to be due to the opioid administration, and thus, naloxone was administered, but the symptoms were not improved. The patient's symptoms continued until the patient received administration of physostigmine...
April 2018: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29625811/further-characterization-of-capos-caos-syndrome-with-the-glu818lys-mutation-in-the-atp1a3-gene-a-case-report
#5
Takuya Hayashida, Yoshiaki Saito, Atsushi Ishii, Shinichi Hirose, Rika Hiraiwa, Yoshihiro Maegaki, Kousaku Ohno
A 38-year-old female patient experienced recurrent episodes of neurological deterioration during febrile illness at the age of 7 and 8 months, and 2, 4, and 37 years. Acute symptoms comprised unconsciousness, headache, abnormal ocular movements, flaccid paralysis with areflexia, ataxia, dysphagia, and movement disorders. Each episode of neurological deterioration was followed by partial recovery with residual symptoms of progressive disturbance of visual acuity with optic atrophy and hearing loss, moderate intellectual disability, strabismus, ophthalmoplegia, as well as fluctuating degree of gait ataxia, chorea, tremor, and myoclonus...
April 3, 2018: Brain & Development
https://www.readbyqxmd.com/read/29625105/myoclonus-epilepsy-retinitis-pigmentosa-leukoencephalopathy-and-cerebral-calcifications-associated-with-a-novel-m-5513g-a-mutation-in-the-mt-tw-gene
#6
Elena Cardaioli, Andrea Mignarri, Teresa Anna Cantisani, Alessandro Malandrini, Claudia Nesti, Anna Rubegni, Niccola Funel, Antonio Federico, Filippo Maria Santorelli, Maria Teresa Dotti
We sequenced the mitochondrial genome from a 40-year-old woman with myoclonus epilepsy, retinitis pigmentosa, leukoencephalopathy and cerebral calcifications. Histological and biochemical features of mitochondrial respiratory chain dysfunction were present. Direct sequencing showed a novel heteroplasmic mutation at nucleotide 5513 in the MT-TW gene that encodes tRNATrp . Restriction Fragment Length Polymorphism analysis confirmed that about 80% of muscle mtDNA harboured the mutation while it was present in minor percentages in mtDNA from other tissues...
April 3, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29623876/averaging-in-time-frequency-domain-reveals-the-temporal-and-spatial-extent-of-seizures-recorded-by-scalp-eeg
#7
Rafeed Alkawadri, Nicolas Gaspard
To demonstrate the importance of averaging in time-frequency space and the added localizing value in time and space in a case of cortical myoclonus. One hundred myoclonic jerks were averaged in time series and in spectral domain. For the latter, we chose 100 (10-second) segments from interictal background and used the unpaired t-test for the jerk-related and control spectral data to obtain the t value and corresponding p value at each pixel. We corrected for multiple comparisons using false discovery rate procedure...
April 6, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29610228/video-neuroimages-paraneoplastic-spinal-myoclonus-associated-with-caspr2-antibodies
#8
Harrison Hines, Nick M Murray, Sarah Ahmad, Safwan Jaradeh, Carl A Gold
No abstract text is available yet for this article.
April 3, 2018: Neurology
https://www.readbyqxmd.com/read/29607915/-a-case-of-limbic-encephalitis-associated-with-sj%C3%A3-gren-s-syndrome-mimicking-anti-n-methyl-d-aspartate-receptor-encephalitis
#9
Kenji Yoshimura, Rie Kanki, Satoshi Nakano
A 25-year-old woman in her 37 weeks and 5 days pregnant presented with abnormal behavior and memory impairment following a high fever. Her manifestations were diagnosed as limbic encephalitis, and she delivered a baby by Cesarean section. In the operation, bilateral ovarian tumors were found and resected, though they were revealed as non-teratoma afterward. After operation, she became agitated, and started to present oral dyskinesia, intractable systemic myoclonus, central hypoventilation, and autonomic manifestations such as labile blood pressure, but her symptoms responded well to immunotherapy...
March 31, 2018: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29607243/hereditary-myoclonus-dystonia-a-novel-sgce-variant-and-phenotype-including-intellectual-disability
#10
David G Coughlin, Tanya M Bardakjian, Meredith Spindler, Andres Deik
Background: Hereditary myoclonus dystonia is often due to changes in the SGCE gene. Dystonia (DYT)- SGCE has a variable phenotype that can involve focal or generalized myoclonus and various forms of task-specific, segmental, or generalized dystonia. Psychiatric comorbidities are common. Case Report: We report a case of a young woman with generalized myoclonus, dystonia, and intellectual disability. She was found to have a novel SGCE splice site variant. Discussion: This novel variant is very likely pathogenic by in silico analysis and has not been previously reported...
2018: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/29605618/progressive-myoclonus-epilepsy-without-renal-failure-in-a-chinese-family-with-a-novel-mutation-in-scarb2-gene-and-literature-review
#11
Wo-Tu Tian, Xiao-Li Liu, Yang-Qi Xu, Xiao-Jun Huang, Hai-Yan Zhou, Ying Wang, Hui-Dong Tang, Sheng-Di Chen, Xing-Hua Luan, Li Cao
PURPOSE: To describe the clinical and genetic features of a Chinese progressive myoclonus epilepsy (PME) patient related with SCARB2 mutation without renal impairment and review 27 SCARB2-related PME patients from 11 countries. METHODS: The patient was a 27-year-old man with progressive action myoclonus, ataxia, epilepsy, dysarthria and absence of cognitive deterioration. Renal functional test was normal. Electroencephalography (EEG) showed progressively slowed background activity and sporadic generalized spike-and-wave discharges...
March 14, 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29603387/genotype-phenotype-correlations-dystonia-and-disease-progression-in-spinocerebellar-ataxia-type-14
#12
Viorica Chelban, Sarah Wiethoff, Bjørn K Fabian-Jessing, Nourelhoda A Haridy, Alaa Khan, Stephanie Efthymiou, Esther B E Becker, Emer O'Connor, Joshua Hersheson, Katrina Newland, Allan Thomas Hojland, Pernille A Gregersen, Suzanne G Lindquist, Michael B Petersen, Jørgen E Nielsen, Michael Nielsen, Nicholas W Wood, Paola Giunti, Henry Houlden
BACKGROUND: Spinocerebellar ataxia type 14 is a rare form of autosomal dominant cerebellar ataxia caused by mutations in protein kinase Cγ gene. Clinically, it presents with a slowly progressive, mainly pure cerebellar ataxia. METHODS: Using next generation sequencing, we screened 194 families with autosomal dominant cerebellar ataxia and normal polyglutamine repeats. In-depth phenotyping was performed using validated clinical rating scales neuroimaging and electrophysiological investigations...
March 30, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29600275/ataxia-telangiectasia-a-new-remitting-form-with-a-peculiar-transcriptome-signature
#13
Vincenzo Leuzzi, Daniela D'Agnano, Michele Menotta, Caterina Caputi, Luciana Chessa, Mauro Magnani
Objective: Ataxia-telangiectasia (AT) is a rare, severe, and ineluctably progressive multisystemic neurodegenerative disease. Variant AT phenotypes have been described in patients with mild- and late-onset neurologic deterioration and atypical features (dystonia and myoclonus). We report on the clinical characteristics and transcriptome profile of patients with a typical AT presentation and genotype who experienced an unexpected favorable course. Methods: A 24-year-old woman developed, by the age of 3 years, all the classic symptoms of AT associated with increased alpha-fetoprotein levels, a compound AT-mutated ( ATM ) genotype with an inframe deletion c...
April 2018: Neurology. Genetics
https://www.readbyqxmd.com/read/29561731/paraneoplastic-movement-disorders
#14
Karolina Popławska-Domaszewicz, Jolanta Florczak-Wyspiańska, Wojciech Kozubski, Sławomir Michalak
Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Two types of antibodies can be distinguished: antibodies against nuclear and cytoplasmic neuronal antigens (anti-Hu, anti-Ri, anti-Yo, anti-Ma, anti-CV2/CRMP5, anti-Gephrin, and anti-GABATRAP) and antibodies recently identified against cell surface and synaptic proteins (anti-NMDAR, anti-LGI1, and anti-Caspr2)...
March 21, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29561417/myoclonic-movement-after-general-anesthesia-a-case-report-and-review-of-the-literature
#15
Je Jin Lee, Seho Lim, Yeon Sil Lee, Hwa-Yong Shin, Chong-Wha Baek, Yong Hun Jung, Young Cheol Woo, Yong-Hee Park
RATIONALE: Myoclonic movement is a rare side effect after general anesthesia. Since we use various intravenous agents during general anesthesia recently, it is troublesome to find out the exact cause of this neurologic complication. PATIENT CONCERNS: A 31-year-old female patient without any past medical history underwent hip arthroscopic surgery under general anesthesia. DIAGNOSES: Although there was no specific event during the operation, she showed a sudden myoclonic movement confined to left upper extremity in recovery room...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29560339/effectiveness-of-combining-behavioral-treatment-with-valproic-acid-for-dysphagia-caused-by-palatal-myoclonus-in-patients-with-stroke-two-case-reports
#16
Bo-Ram Kim, Yejin Lee, Soo Jin Kim, Hyuntae Kim, Jong Won Lee, Seunghwan Lee, Jongmin Lee
Palatal myoclonus (PM) is a rare disease that may induce dysphagia. Since dysphagia related to PM is unique and is characterized by myoclonic movements of the involved muscles, specific treatments are needed for rehabilitation. However, no study has investigated the treatment effectiveness for this condition. Therefore, the aim of this case report was to describe the benefit of combining behavioral treatment with valproic acid administration in patients with dysphagia triggered by PM. The two cases were treated with combined treatment...
February 2018: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/29557234/sudden-unexpected-death-in-epilepsy-in-a-14-year-old-girl-case-report-and-literature-review
#17
Xinyue Zhang, Lichao Sun, Li Cui, Weihong Lin
Background Sudden unexpected death in epilepsy (SUDEP) is the most common cause of death in people with epilepsy. The pathogenesis of SUDEP is still unclear. The majority of SUDEP cases occur in intractable epilepsy, especially in the aftermath of a generalized tonic-clonic seizure. We report an atypical SUDEP case with infrequent seizures in frontal lobe epilepsy. Case Report A 14-year-old girl presented with a 13-year history of paroxysmal convulsions. She experienced three episodes within 6-10 months after birth...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29555260/effect-of-low-dose-cyclophosphamide-acth-and-ivig-combination-immunotherapy-on-neuroinflammation-in-pediatric-onset-oms-a-retrospective-pilot-study
#18
Michael R Pranzatelli, Tyler J Allison, Elizabeth D Tate
INTRODUCTION: Flow cytometric cerebrospinal fluid (CSF) lymphocyte subset analysis has improved the diagnosis of neuroinflammation and identified multiple markers of inflammation in opsoclonus-myoclonus syndrome (OMS). The aim of this exploratory, retrospective study was to analyze the effect of immunotherapy on these markers to determine which agents are disease modifying. METHODS: Cross-sectional immunological observations were made in an IRB-approved case-control study, and patients were treated empirically...
March 5, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29555100/myoclonus-in-angelman-syndrome
#19
Sarah F Pollack, Olivia R Grocott, Kimberly A Parkin, Anna M Larson, Ronald L Thibert
Angelman syndrome (AS) is a neurogenetic imprinting disorder caused by loss of the maternally inherited Ube3a gene and is characterized by generalized epilepsy, limited expressive speech, sleep dysfunction, and movement disorders. Myoclonic seizures are often the first seizure type to appear, and myoclonic status, associated with developmental regression, may occur in the first few years of life. Additionally, there have been rare reports of prolonged episodes of myoclonus without electrographic correlate in adults with AS...
March 16, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29542410/dabco-catalyzed-green-and-efficient-synthesis-of-2-amino-4h-pyrans-and-their-biological-evaluation-as-antimicrobial-and-anticancer-agents
#20
Amit Shivajirao Waghmare, Shivaji Sandu Pandit, Dayanand M Suryawanshi
AIM AND OBJECTIVE: 4H-pyran is one of the most well-known groups of synthetic heterocyclic compounds and it has attracted considerable attention of chemists in recent years because of their extensive range of biological and pharmaceutical activities. These compounds are used as antibacterial, anticancer agents, anti-coagulants, spasmolytics and anti-anaphylactic. 4H-pyran derivatives are utilized in cosmetics, pigments, biodegradable agrochemicals as well as photoactive materials. In addition, 4H-pyrans are also helpful as cognitive enhancers for the treatment of neuro degenerative diseases, including Alzheimer's disease, as well as for the treatment of schizophrenia and myoclonus...
March 14, 2018: Combinatorial Chemistry & High Throughput Screening
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