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https://www.readbyqxmd.com/read/28534142/teflon-granuloma-after-microvascular-decompression-for-hemifacial-spasm-a-case-report-and-literature-review
#1
Kazunori Oda, Tadashi Higuchi, Yasuo Murai, Fumio Yamaguchi, Akio Morita
Teflon has been commonly used as a surgical material. In particular, Teflon has been considered suitable for microvascular decompression of cranial nerves, as it is a stable, inert substance that does not resorb or migrate. Giant cell foreign body reactions after microvascular decompression (MVD) have been reported, but this rare complication has not been well recognized. Here, we report one case of Teflon granuloma that occurred 4 years after MVD for hemifacial spasm. We discuss the cause, histopathological analysis, particular MVD surgical methods, and management of Teflon granuloma...
May 22, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28533163/grin2b-gain-of-function-mutations-are-sensitive-to-radiprodil-a-negative-allosteric-modulator-of-glun2b-containing-nmda-receptors
#2
Brice Mullier, Christian Wolff, Zara Amanda Sands, Philippe Ghisdal, Pierandrea Muglia, Rafal Marian Kaminski, Véronique Marie André
De novo gain of function mutations in GRIN2B encoding the GluN2B subunit of the N-methyl-D-aspartate (NMDA) receptor have been linked with epileptic encephalopathies, including infantile spasms. We investigated the effects of radiprodil, a selective GluN2B negative allosteric modulator and other non-selective NMDA receptor inhibitors on glutamate currents mediated by NMDA receptors containing mutated GluN2B subunits. The experiments were performed in Xenopus oocytes co-injected with the following human mRNAs: GRIN1/GRIN2B, GRIN1/GRIN2B-R540H, GRIN1/GRIN2B-N615I and GRIN1/GRIN2B-V618G...
May 19, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28529467/radiotherapy-for-pertussis-an-historical-assessment
#3
Edward J Calabrese, Gaurav Dhawan, Rachna Kapoor
X-ray therapy was used to treat pertussis/whooping cough during a 13-year period from 1923 to 1936 in North America and Europe. Twenty studies from clinicians in the United States reported that approximately 1500 cases of pertussis were treated by X-ray therapy usually with less than 0.5 erythema dose. Young children (<3 years) comprised about 70% to 80% of the cases, with the age of cases ranging from as young as 1 month to 50 years. In general, symptoms of severe coughing, vomiting episodes, and spasms were significantly relieved in about 85% of cases following up to 3 treatments, while about 15% of the cases showed nearly full relief after only 1 treatment...
April 2017: Dose-response: a Publication of International Hormesis Society
https://www.readbyqxmd.com/read/28526473/coronary-flow-reserve-is-reduced-in-sarcoidosis
#4
Seref Kul, Gonul Aciksari Kutlu, Tolga Sinan Guvenc, Murat Kavas, Kenan Demircioglu, Yusuf Yilmaz, Halil Ibrahim Yakar, Asiye Kanbay, Sibel Boga, Mustafa Caliskan
BACKGROUND AND AIMS: Sarcoidosis is a multisystem disease with frequent cardiac involvement, albeit manifest cardiac disease is rare. Though epicardial coronary arteries are not frequently involved, microvascular disease is rather common in both symptomatic and asymptomatic patients. The mechanism of microvascular involvement has not been elaborated yet. The aim of this study is to investigate coronary flow velocity reserve (CFVR) using transthoracic echocardiography in patients with sarcoidosis but without known atherosclerotic coronary artery disease or risk factors for atherosclerosis...
May 4, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28524221/-epileptic-spasms-in-infants-beyond-hypsarrhythmia
#5
M Garcia-Fernandez
Epileptic spasms are the most frequent type of epileptic seizures in infants. They can also occur beyond the period of infancy, within the context of other epileptic encephalopathies or as an expression of a focal or generalised epilepsy. The clinical semiology of epileptic spasms varies greatly. They sometimes consist of very subtle clinical manifestations, which occur in series, without the typical axorhizomelic contraction, or in association with focal seizures. The critical EEG correlate is also very variable and basically consists of the combination of a hypervoltage slow wave, a bout of rapid low-amplitude activity or a diffuse attenuation of the trace...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28523890/handy-hints-about-raynaud-s-phenomenon-in-children-a-critical-review
#6
REVIEW
Donato Rigante, Michele Fastiggi, Francesco Ricci, Francesca D'Errico, Benedetta Bracci, Cristina Guerriero
Raynaud's phenomenon (RP) is a vasospastic disorder characterized by recurrent self-limited episodes of skin pallor, cyanosis, and hyperemia caused by paroxysmal spasms in the small arteries of the fingers and toes and can occur in any age group. Hands, feet, nose, ears, and nipples can be affected. The diagnosis is made clinically, assessing varying degrees of ischemia in the involved areas of skin, but this transient ischemia may also herald the onset of connective tissue disease. Investigation is recommended when RP starts in childhood to exclude an underlying autoimmune condition and close follow-up for its development...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28520647/altered-brain-structure-and-function-associated-with-sensory-and-affective-components-of-classic-trigeminal-neuralgia
#7
Yuan Wang, Dong-Yuan Cao, Bethany Remeniuk, Samuel Krimmel, David A Seminowicz, Ming Zhang
Classic trigeminal neuralgia (CTN) is a chronic neuropathic pain state characterized by intense, piercing spasms of the orofacial region, and may be attributable to abnormal pain processing in the central nervous system. Our study investigated neuronal alterations using voxel-based morphometry (VBM), diffuse tensor imaging (DTI), and resting state functional connectivity in 38 CTN patients and 38 matched healthy controls. For VBM analyses, CTN patients displayed gray matter volume (GMV) reductions in the anterior and mid cingulate cortex (ACC and MCC), insula, secondary somatosensory cortex (S2), primary motor cortex (M1), premotor area (PMA), and several regions in the temporal lobe...
May 16, 2017: Pain
https://www.readbyqxmd.com/read/28515470/a-novel-missense-mutation-in-the-hect-domain-of-nedd4l-identified-in-a-girl-with-periventricular-nodular-heterotopia-polymicrogyria-and-cleft-palate
#8
Koji Kato, Fuyuki Miya, Ikumi Hori, Daisuke Ieda, Kei Ohashi, Yutaka Negishi, Ayako Hattori, Nobuhiko Okamoto, Mitsuhiro Kato, Tatsuhiko Tsunoda, Mami Yamasaki, Yonehiro Kanemura, Kenjiro Kosaki, Shinji Saitoh
We identified a novel de novo heterozygous missense mutation in the NEDD4L gene (NM_015277: c.2617G>A; p.Glu873Lys) through whole-exome sequencing in a 3-year-old girl showing severe global developmental delay, infantile spasms, cleft palate, periventricular nodular heterotopia and polymicrogyria. Mutations in the HECT domain of NEDD4L have been reported in patients with a neurodevelopmental disorder along with similar brain malformations. All patients reported with NEDD4L HECT domain mutations showed periventricular nodular heterotopia, and most had seizures, cortex anomalies, cleft palate and syndactyly...
May 18, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28513609/application-of-rare-variant-transmission-disequilibrium-tests-to-epileptic-encephalopathy-trio-sequence-data
#9
(no author information available yet)
The classic epileptic encephalopathies, including infantile spasms (IS) and Lennox-Gastaut syndrome (LGS), are severe seizure disorders that usually arise sporadically. De novo variants in genes mainly encoding ion channel and synaptic proteins have been found to account for over 15% of patients with IS or LGS. The contribution of autosomal recessive genetic variation, however, is less well understood. We implemented a rare variant transmission disequilibrium test (TDT) to search for autosomal recessive epileptic encephalopathy genes in a cohort of 320 outbred patient-parent trios that were generally prescreened for rare metabolic disorders...
May 17, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28512048/combined-hyperactive-dysfunction-syndrome-of-the-cranial-nerves-a-retrospective-systematic-study-of-clinical-characteristics-in-44-patients
#10
Jingwei Cao, Jie Jiao, Zhenhui Du, Wenzhe Xu, Bin Sun, Feng Li, Yuguang Liu
BACKGROUND: Combined hyperactive dysfunction syndrome (HDS) is defined as the combination symptoms arising from overactivity in cranial nerves specifically, trigeminal neuralgia (TN), hemifacial spasm (HFS), and glossopharyngeal neuralgia (GPN) without an obvious explanatory structural lesion. The study aims to retrospectively analyze the clinical characteristics of combined HDS treated with microvascular decompression (MVD) in a single institution. METHODS: A total of 1450 patients with HDS were treated with MVD in our department during a 10-year period, among which 44 cases of combined HDS were identified...
May 13, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28507604/health-related-quality-of-life-outcomes-from-botulinum-toxin-treatment-in-hemifacial-spasm
#11
Daniel Weiss, Justine Sturm, Leonhard Hieber, Axel Börtlein, Ingo Mayr, Matthias Appy, Benedicta Kühnler, Joachim Buchthal, Christian Dippon, Guy Arnold, Tobias Wächter
BACKGROUND: Although botulinum neurotoxin (BoNT) injections may alleviate involuntary muscle contractions in hemifacial spasm substantially, it is less clear whether the motor effect would translate into improvements of health-related quality of life (HR-QoL). METHODS: In this open-label clinical observational study, we characterized outcomes on HR-QoL in terms of the EuroQol (EQ-5D-5L) from BoNT in a prospective cohort of patients with hemifacial spasm (n = 73)...
April 2017: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/28506505/the-utility-of-surveillance-electroencephalography-to-guide-early-antiepileptic-drug-therapy-in-infants-with-tuberous-sclerosis-complex
#12
Robyn Whitney, Saber Jan, Maria Zak, Bláthnaid McCoy
BACKGROUND: Seizures are a common early presentation in infants with tuberous sclerosis complex (TSC) and can be preceded by electrographic changes on electroencephalography (EEG) before clinical seizure onset. A limited number of studies have addressed the initial EEG findings in TSC and the outcome of early treatment with antiepileptic medication prior to clinical seizure onset. METHODS: We describe two infants with tuberous sclerosis complex whose surveillance EEG showed focal seizures that were not previously recognized by caregivers...
April 18, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28505064/changes-in-renal-artery-dimensions-are-associated-with-clinical-response-to-radiofrequency-renal-denervation-a-series-of-studies-using-quantitative-angiography-and-intravascular-ultrasound
#13
Thomas Weber, Jörg Kellermair, Karen T Witberg, Jurgen Ligthart, Robert J Melder, Thomas Forstner, Bernd Eber
OBJECTIVE: Renal denervation (RDN) can cause focal (notches) and global (spasms) changes in renal artery dimensions. We quantified these changes and related them to renal norepinephrin tissue content in animals and to blood pressure (BP) changes in patients. METHODS: We measured renal artery dimensions pre-RDN and post-RDN, utilizing quantitative renal angiography (QRA) in a porcine model and in a retrospective patient cohort, and intravascular ultrasound (IVUS) in a prospective patient cohort...
May 12, 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28500109/hemifacial-spasm-and-neurovascular-compression-syndrome
#14
Puya Dehgani Mobaraki, Ruggero Lapenna, Giampietro Ricci, Jacques Magnan
No abstract text is available yet for this article.
May 12, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28499220/effects-of-preadmission-beta-blockers-on-neurogenic-stunned-myocardium-after-aneurysmal-subarachnoid-hemorrhage-a-meta-analysis
#15
Hai Luo, Wei-Xin Song, Jin-Wen Jiang, Jian-Lan Zhao, Wei-Lin Rong, Mei-Hua Li
OBJECTIVE: Spontaneous subarachnoid hemorrhage is mostly caused by the rupture of an aneurysm. Neurogenic stunned myocardium (NSM) is one of the most frequent complications caused by aneurysmal subarachnoid hemorrhage (aSAH). The possible pathogenesis of NSM may be that the catecholamine peak resulting from aSAH leads to subendocardial ischemia or coronary artery spasm. We designed this meta-analysis to find out whether beta-blockers (BB) can significantly reduce the incidence of NSM and improve the outcomes of aSAH...
May 1, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28498272/paget-s-disease-of-the-temporal-bone-a-single-institution-contemporary-review-of-27-patients
#16
Nicholas L Deep, Jake G Besch-Stokes, John I Lane, Colin L W Driscoll, Matthew L Carlson
OBJECTIVES: To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB). STUDY DESIGN: Retrospective chart review of patients evaluated from 1998 to 2016. SETTING: Quaternary referral center. PATIENTS: Patients with radiographically confirmed PDTB. MAIN OUTCOME MEASURES: Clinical, audiological, and radiological features and management strategies of PDTB...
May 11, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28498061/microvascular-decompression-for-hemifacial-spasm-can-intraoperative-lateral-spread-response-monitoring-improve-surgical-efficacy
#17
Yongxu Wei, Wenlei Yang, Weiguo Zhao, Chunhua Pu, Ning Li, Yu Cai, Hanbing Shang
OBJECTIVE The purpose of this study was to evaluate whether intraoperative monitoring of lateral spread response (LSR) improves the efficacy of microvascular decompression (MVD) for hemifacial spasm (HFS). METHODS In this prospective study, patients undergoing MVD for HFS were assigned to one of 2 groups, Group A (MVD with intraoperative LSR monitoring) or Group B (MVD without LSR monitoring). Clinical outcome at 12 months after surgery was assessed through telephone survey. Data analysis was performed to investigate the effect of intraoperative LSR monitoring on efficacy of MVD...
May 12, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28496986/a-case-of-stiff-dog-syndrome-associated-with-anti-glutamic-acid-decarboxylase-antibodies
#18
Theresa E Pancotto, John H Rossmeisl
BACKGROUND: The stiff person syndrome (SPS) is a rare and debilitating autoimmune disorder with an unknown pathogenesis and variable clinical presentation that can present a diagnostic challenge. Although entities that clinically mimic stiff-person spectrum disorders (SPSD) have manifested in horses, they have not been reported in dogs. CASE PRESENTATION: We describe a 2-year-old beagle dog presented for progressive attacks of muscular rigidity and lordosis with superimposed spasms of the appendicular muscles triggered by tactile stimulation which resulted in marked gait impairment...
2017: Journal of Clinical Movement Disorders
https://www.readbyqxmd.com/read/28493170/eluxadoline-a-review-in-diarrhoea-predominant-irritable-bowel-syndrome
#19
Gillian M Keating
Eluxadoline (Truberzi(®)) is an orally administered, minimally absorbed agent that acts locally in the gastrointestinal tract as a mixed µ-opioid receptor agonist and δ-opioid receptor antagonist. The randomized, double-blind, placebo-controlled, multinational, phase 3 IBS-3001 and IBS-3002 trials examined the efficacy of eluxadoline in patients with diarrhoea-predominant irritable bowel syndrome (IBS-D). The composite response rate (i.e. the proportion of patients with improvement in both worst abdominal pain and stool consistency on ≥50% of days; primary endpoint), was significantly higher in patients receiving eluxadoline 100 mg twice daily than in those receiving placebo after 12 and 26 weeks' therapy...
June 2017: Drugs
https://www.readbyqxmd.com/read/28491725/recurrent-and-late-onset-coronary-spasms-after-cryoballoon-ablation-procedure-in-a-patient-with-atrial-fibrillation
#20
Tomonori Watanabe, Hitoshi Hachiya, Shinsuke Miyazaki, Hiroaki Nakamura, Hiroshi Taniguchi, Yoshito Iesaka
No abstract text is available yet for this article.
September 2016: HeartRhythm Case Reports
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