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Myoclonus

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https://www.readbyqxmd.com/read/28442302/facial-twitches-in-adcy5-associated-disease-myokymia-or-myoclonus-an-electromyography-study
#1
Sinem Tunc, Norbert Brüggemann, Magdalena K Baaske, Corinna Hartmann, Karen Grütz, Ana Westenberger, Christine Klein, Alexander Münchau, Tobias Bäumer
OBJECTIVE: A clinical feature in patients with ADCY5 gene mutations are perioral muscle twitches initially described as facial myokymia. METHODS: Five patients with ADCY5-associated disease with facial twitches and truncal jerks underwent electrophysiological investigations of the orbicularis oris and trapezius muscles to delineate neurophysiological characteristics of these phenomena. RESULTS: Electromyography (EMG) recordings showed a complex electrophysiological pattern with brief bursts of less than 100 ms and longer bursts with a duration of 100-300 ms up to several seconds in keeping with myoclonus and chorea, respectively, as key findings...
April 20, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28432174/postintervention-acute-opsoclonus-myoclonus-syndrome
#2
Annelise Aquilina, Nicola Dingli, Josanne Aquilina
Opsoclonus myoclonus syndrome (OMS) is a rare, neurological condition affecting 1 in 10 000 000 people annually. Opsoclonus, defined as involuntary rapid, multivectorial oscillations of the eyes, together with ataxia and myoclonus are usually present. OMS may be paraneoplastic: often associated with occult neuroblastoma in childhood and with breast carcinoma or small cell lung carcinoma in adults. Other aetiologies include viral or toxic agents. The pathogenesis is thought to be immune mediated. A 37-year-old woman with previous inflammatory cranial mononeuropathies was admitted for elective dilatation and curettage (D&C)...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28431757/anti-ammonia-treatment-responsive-myoclonus-as-initial-presentation-of-acquired-hepatocerebral-degeneration
#3
Roberto Monreal-Robles, Guillermo Delgado-García, Erik García-Valadez, Carlos R Cámara-Lemarroy, Ingrid Estrada-Bellmann
No abstract text is available yet for this article.
April 18, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28431624/opsoclonus-myoclonus-ataxia-associated-with-west-nile-virus-infection-a-dramatic-presentation-with-benign-prognosis
#4
Roy Zaltzman, Colin Klein, Carlos R Gordon
Opsoclonus myoclonus and ataxia is a combination of severe neurological signs associated with several pathologic agents and conditions. Only few cases of opsoclonus have been related to West Nile virus infection. We report on a 61-year-old woman and on a 55-year-old man who had history of recent fever, who were hospitalized because of acute severe truncal ataxia, opsoclonus and tremor with minimal myoclonic jerks. A through work-up revealed the presence of both IgM and IgG antibodies against West Nile virus both in the serum and Cerebrospinal Fluid and excluded other causes known to be associated with this combination of neurological signs...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28428906/tbc1d24-mutations-in-a-sibship-with-multifocal-polymyoclonus
#5
Adeline Ngoh, Jose Bras, Rita Guerreiro, Amy McTague, Joanne Ng, Esther Meyer, W Kling Chong, Stewart Boyd, Linda MacLellan, Martin Kirkpatrick, Manju A Kurian
BACKGROUND: Advances in molecular genetic technologies have improved our understanding of genetic causes of rare neurological disorders with features of myoclonus. CASE REPORT: A family with two affected siblings, presenting with multifocal polymyoclonus and neurodevelopmental delay, was recruited for whole-exome sequencing following unyielding diagnostic neurometabolic investigations. Compound heterozygous mutations in TBC1D24, a gene previously associated with various epilepsy phenotypes and hearing loss, were identified in both siblings...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28416624/teaching-video-neuroimages-maneuvers-to-elicit-opsoclonus-in-opsoclonus-myoclonus-syndrome
#6
Kyle W Mahoney, Daniel R Gold, Jiaying Zhang
No abstract text is available yet for this article.
April 18, 2017: Neurology
https://www.readbyqxmd.com/read/28415947/effect-of-pretreatment-with-midazolam-on-etomidate-induced-myoclonus-a-meta-analysis
#7
Chengmao Zhou, Yu Zhu, Zhen Liu, Lin Ruan
Objective To investigate the effect of pretreatment with midazolam on myoclonus induced by etomidate injection. Methods A meta-analysis was performed using Review Manager software, version 5.2. Two researchers independently searched PubMed, Cochrane Library, and Embase® databases for randomized controlled trials involving patients who underwent etomidate induced general anaesthesia with or without midazolam pretreatment, published between 1990 and 2016. Outcome measures comprised overall myoclonus incidence rate and incidence rate classified by degree of myoclonus following etomidate injection...
April 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28411061/a-nuanced-view-of-post-anoxic-myoclonus
#8
EDITORIAL
Jonathan Elmer, Claudio Sandroni
No abstract text is available yet for this article.
April 11, 2017: Resuscitation
https://www.readbyqxmd.com/read/28406812/electroencephalography-predicts-poor-and-good-outcomes-after-cardiac-arrest-a-two-center-study
#9
Andrea O Rossetti, Diego F Tovar Quiroga, Elsa Juan, Jan Novy, Roger D White, Nawfel Ben-Hamouda, Jeffrey W Britton, Mauro Oddo, Alejandro A Rabinstein
OBJECTIVE: The prognostic role of electroencephalography during and after targeted temperature management in postcardiac arrest patients, relatively to other predictors, is incompletely known. We assessed performances of electroencephalography during and after targeted temperature management toward good and poor outcomes, along with other recognized predictors. DESIGN: Cohort study (April 2009 to March 2016). SETTING: Two academic hospitals (Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; Mayo Clinic, Rochester, MN)...
April 12, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28402042/mm1-type-sporadic-creutzfeldt-jakob-disease-with-1-month-total-disease-duration-and-early-pathologic-indicators
#10
Yasushi Iwasaki, Hiroko Kato, Tetsuo Ando, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
A 62-year-old man presented with abnormal behavior and cognitive impairment. Diffusion-weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent...
April 12, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28386402/opioid-induced-myoclonus-and-hyperalgesia-following-a-short-course-of-low-dose-oral-morphine
#11
Owen Bleddyn Woodward, Sangeeta Naraen, Akriti Naraen
A 76-year-old man was admitted to hospital with a right-sided fractured neck of femur requiring repair via a cemented hemiarthroplasty. Intraoperatively he received 10 mg of intravenous morphine. Post-operatively he received a short course of low-dose oral opioids and subsequently developed myoclonic jerks and hyperalgesia. The opioids were discontinued and both adverse effects resolved. This case report discusses the concurrent development of myoclonus and hyperalgesia following a low dose of opioids and explores possible management options...
February 2017: British Journal of Pain
https://www.readbyqxmd.com/read/28384730/intractable-epilepsy-and-progressive-cognitive-decline-in-a-young-man
#12
Alexander L Cohen, Lyell K Jones, Joseph E Parisi, James P Klaas
A young man with normal neurodevelopment presented with 3 years of medically refractory, progressive epilepsy and myoclonus. Initial examination included neuroimaging, electroencephalography, and biochemical analyses, all of which were unremarkable except for mildly enlarged ventricles. Over the following year, the patient experienced rapid cognitive decline with new-onset recurrent visual hallucinations and progressive lethargy. Results of subsequent electroencephalography and brain imaging were unchanged, and a fluorodeoxyglucose F 18 positron emission tomographic scan was normal...
April 3, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28382685/dimethyl-fumarate-ameliorates-myoclonus-stemming-from-protein-misfolding-in-oligodendrocytes
#13
Cherie M Southwood, Danielle M Garshott, Chelsea R Richardson, Navid Seraji-Bozorgzad, Andrew M Fribley, Alexander Gow
Multiple sclerosis (MS) is considered a primary autoimmune disease; however, this view is increasingly being challenged in basic and clinical science arenas because of the growing body of clinical trials data showing that exclusion of immune cells from the CNS only modestly slows disease progression to disability. Accordingly, there is significant need for expanding the scope of potential disease mechanisms to understand the etiology of MS. Concomitantly, the use of a broader range of preclinical animal models for characterizing existing efficacious clinical treatments may elucidate additional or unexpected mechanisms of action for these drugs that augment insight into MS etiology...
April 6, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28382611/parkinsonism-or-other-movement-disorders-presenting-as-stroke-mimics
#14
Cheng-Yang Hsieh, Chih-Hung Chen, Sheng-Feng Sung, Wen-Juh Hwang
PURPOSE: Patients with parkinsonism or other movement disorders may visit the emergency department due to acute deterioration of neurological status or consciousness disturbance. Under such circumstances, patients may be misdiagnosed as having a hyperacute stroke, i.e. stroke mimic. The purpose of the present study was to explore the clinical features and consequences of patients with parkinsonism or other movement disorders presenting as stroke mimics with activation of a stroke code...
December 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/28380698/myoclonus-epilepsy-and-ataxia-due-to-kcnc1-mutation-analysis-of-20-cases-and-k-channel-properties
#15
Karen L Oliver, Silvana Franceschetti, Carol J Milligan, Mikko Muona, Simone A Mandelstam, Laura Canafoglia, Anna M Boguszewska-Chachulska, Amos Korczyn, Francesca Bisulli, Carlo Di Bonaventura, Francesca Ragona, Roberto Michelucci, Bruria Ben-Zeev, Rachel Straussberg, Ferruccio Panzica, João Massano, Daniel Friedman, Arielle Crespel, Bernt A Engelsen, Frederick Andermann, Eva Andermann, Krystyna Spodar, Anetta Lasek-Bal, Patrizia Riguzzi, Elena Pasini, Paolo Tinuper, Laura Licchetta, Elena Gardella, Matthias Lindenau, Annette Wulf, Rikke S Møller, Felix Benninger, Zaid Afawi, Guido Rubboli, Christopher A Reid, Snezana Maljevic, Holger Lerche, Anna-Elina Lehesjoki, Steven Petrou, Samuel F Berkovic
OBJECTIVE: To comprehensively describe the new syndrome of myoclonus epilepsy and ataxia due to potassium (K(+) ) channel mutation (MEAK), including cellular electrophysiological characterization of observed clinical improvement with fever. METHODS: We analysed clinical, electroclinical and neuroimaging data for twenty patients with MEAK due to recurrent KCNC1 p.R320H mutation. In vitro electrophysiological studies were conducted using whole cell patch clamp to explore biophysical properties of wild-type and mutant KV 3...
April 5, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28373807/autosomal-dominant-cortical-tremor-myoclonus-and-epilepsy-syndrome-mimicking-juvenile-myoclonic-epilepsy
#16
Zeynep Aydin Özemir, Emel Oğuz Akarsu, Zeliha Matur, Ali Emre Öge, Betül Baykan
INTRODUCTION: Autosomal dominant cortical tremor, myoclonus, and epilepsy (ADCME) syndrome is a genetically heterogeneous and under-recognized disease characterized by tremulous movements mimicking essential tremor, myoclonus, and rare generalized tonic-clonic seizures. Here we described the clinical and electrophysiological features of three siblings with ADCME syndrome mimicking juvenile myoclonic epilepsy (JME). METHODS: Three siblings (two females and one male) diagnosed with ADCME were analyzed by electroencephalogram (EEG), somatosensory evoked potentials, and accelerometric recordings...
September 2016: Noro Psikiyatri Arsivi
https://www.readbyqxmd.com/read/28367296/famotidine-induced-reversal-of-meperidine-related-serotonin-syndrome-a-case-report
#17
Soohyun Joe, Eunju Kim, Junyi Park, Dongwon Lee, Jongchul Son, Hyun Kim
Serotonin syndrome is an unexpected fatal adverse event related to serotonergic medication. This case report is the first report describing the possible treatment effect of famotidine on serotonin syndrome. Furthermore, this is the first case report of serotonin syndrome induced by meperidine alone in a patient with no previous history suggesting a susceptibility to serotonin syndrome. A 70-year-old male with no recent history of serotonergic drug use presented with severe serotonin syndrome following ureteroscopy, possibly due to postoperative meperidine administration...
April 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/28364570/excessive-fragmentary-myoclonus-what-do-we-know
#18
REVIEW
Jiří Nepožitek, Karel Šonka
Excessive fragmentary myoclonus (EFM) is a polysomnographic finding registered by the surface electromyography (EMG) and characterized as a result of the muscle activity consisting of sudden, isolated, arrhythmic, asynchronous and asymmetric brief twitches. The EMG potentials are defined by the exact criteria in The International Classification of the Sleep Disorders, 3rd edition and they appear with high intensity in all sleep stages. Clinical significance of EFM is unclear. It was observed in combination with other diseases and features such as obstructive and central sleep apnea, narcolepsy, periodic limb movements, insomnia, neurodegenerative disorders and peripheral nerve dysfunction...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28352055/progressive-encephalomyelitis-with-rigidity-and-myoclonus-in-an-intellectually-disabled-patient-mimicking-neuroleptic-malignant-syndrome
#19
Zheyu Xu, Kalpana Prasad, Tianrong Yeo
We present a case of 32-year-old male with profound mental retardation and autism spectrum disorder who had presented with seizures, rigidity and elevated creatine kinase and was initially diagnosed as neuroleptic malignant syndrome (NMS). The patient subsequently had a complicated clinical course, developing refractory status epilepticus, which lead to the eventual diagnosis of progressive encephalomyelitis with rigidity and myoclonus (PERM). We discuss the clinical similarities and differences between NMS and PERM, and highlight the need to consider alternative diagnoses when the clinical picture of NMS is atypical, particularly in this patient group where the history and clinical examination may be challenging...
March 24, 2017: Journal of Movement Disorders
https://www.readbyqxmd.com/read/28342959/early-lance-adams-syndrome-after-cardiac-arrest-prevalence-time-to-return-to-awareness-and-outcome-in-a-large-cohort
#20
Irene Aicua Rapun, Jan Novy, Daria Solari, Mauro Oddo, Andrea O Rossetti
INTRODUCTION: Early myoclonus after cardiac arrest (CA) is traditionally viewed as a poor prognostic sign (status myoclonus). However, some patients may present early Lance-Adams syndrome (LAS): under appropriate treatment, they can reach a satisfactory functional outcome. Our aim was to describe their profile, focusing on pharmacologic management in the ICU, time to return of awareness, and long-term prognosis. METHODS: Adults with early LAS (defined as generalized myoclonus within 96h, with epileptiform EEG within 48h after CA) were retrospectively identified in our CA registry between 2006 and 2016...
March 22, 2017: Resuscitation
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