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Periodic paralysis

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https://www.readbyqxmd.com/read/29777778/anterior-approach-in-humeral-plating-osteosynthesis
#1
Daniel Handy Eone, Prudence Christelle Danielle Ngono, Marie Josée Essi, Gaël Leckpa, Kennedy Olivier Muluem, Farikou Ibrahima, Maurice Aurélien Sosso
CONTEXT: Humeral plating osteosynthesis is controversial, particularly regarding the choice between anterior and lateral approach, data for which in the context of a low-income country are lacking. HYPOTHESIS: The anterior approach is an easy surgical technique, allowing good anatomic reconstruction. We hypothesize that the anterior approach is associated with fewer neurovascular lesions and functional sequelae than the lateral approach. MATERIALS AND METHODS: A retrospective study with assessment update was carried out over a period of 6 years 4 months from January 2010 to June 2016, with consecutive recruitment in the city of Yaoundé, Cameroon...
May 16, 2018: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/29769724/structural-basis-for-gating-pore-current-in-periodic-paralysis
#2
Daohua Jiang, Tamer M Gamal El-Din, Christopher Ing, Peilong Lu, Régis Pomès, Ning Zheng, William A Catterall
Potassium-sensitive hypokalaemic and normokalaemic periodic paralysis are inherited skeletal muscle diseases characterized by episodes of flaccid muscle weakness1,2 . They are caused by single mutations in positively charged residues ('gating charges') in the S4 transmembrane segment of the voltage sensor of the voltage-gated sodium channel Nav 1.4 or the calcium channel Cav 1.11,2 . Mutations of the outermost gating charges (R1 and R2) cause hypokalaemic periodic paralysis1,2 by creating a pathogenic gating pore in the voltage sensor through which cations leak in the resting state3,4 ...
May 16, 2018: Nature
https://www.readbyqxmd.com/read/29762909/open-label-dose-escalation-phase-1-study-of-safety-and-single-and-multiple-dose-pharmacokinetics-of-dichlorphenamide-in-healthy-volunteers
#3
Fredric Cohen
Single-and multiple-dose pharmacokinetics and safety were investigated in this phase 1 study of dichlorphenamide, a carbonic anhydrase inhibitor approved in the United States for treatment of primary periodic paralysis. Dichlorphenamide was administered to 6 cohorts (n = 6 each) of healthy adults. Cohorts A through E received single doses of 25-400 mg followed by 50-800 mg/day in divided doses for 10 total doses. Cohort F (safety analysis only) received up to 28 titrated doses from 100-800 mg/day. Plasma for pharmacokinetics sampling was obtained predose and up to 48 hours postdose...
May 15, 2018: Clinical Pharmacology in Drug Development
https://www.readbyqxmd.com/read/29752813/the-long-exercise-test-in-periodic-paralysis-a-bayesian-analysis
#4
Daniel B Simmons, Julie Lanning, James C Cleland, Araya Puwanant, Paul T Twydell, Robert C Griggs, Rabi Tawil, Eric L Logigian
INTRODUCTION: The long exercise test (LET) is used to assess the diagnosis of periodic paralysis (PP), but LET methodology and normal "cut-off" values vary. METHODS: To determine optimal LET methodology and cut-offs, we reviewed LET data (abductor digiti minimi (ADM) motor response amplitude, area) from 55 PP patients (32 genetically definite) and 125 controls. Receiver operating characteristic (ROC) curves were constructed and area-under-the-curve (AUC) calculated to compare 1) peak-to-nadir versus baseline-to-nadir methodologies, and 2) amplitude versus area decrements...
May 12, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29745256/salvage-of-cervical-motor-radiculopathy-using-peripheral-nerve-transfer-reconstruction
#5
Fardad T Afshari, Taushaba Hossain, Caroline Miller, Dominic M Power
PURPOSE: Motor nerve transfer surgery involves re-innervation of important distal muscles using either an expendable motor branch or a fascicle from an adjacent functioning nerve. This technique is established as part of the reconstructive algorithm for traumatic brachial plexus injuries. The reproducible outcomes of motor nerve transfer surgery have resulted in exploration of the application of this technique to other paralysing conditions. The objective of this study is to report feasibility and increase awareness about nerve transfer as a method of improving upper limb function in patients with cervical motor radiculopathy of different aetiology...
May 10, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29735379/treatment-of-hematomas-after-anterior-cervical-spine-surgery-a-retrospective-study-of-15-cases
#6
Weiliang Miao, Xiaojun Ma, Deyong Liang, Yu Sun
OBJECTIVE: Postoperative hematoma is a rare and dangerous complication of cervical spine surgery. The aim of this study was to investigate the incidence and related factors of postoperative hematoma, and to report on 15 cases at our institution over a 6-year period. METHODS: Fifteen cases of postoperative hematoma were retrospectively identified. We investigated their neurological outcomes, characteristics, and surgical data, and identified risk factors associated with postoperative (PO) hematoma...
May 4, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29717916/risk-factors-associated-with-the-development-of-acquired-airway-disease-after-congenital-heart-surgery-a-retrospective-cohort-study
#7
Andrew J Matisoff, Pranathi Ari, David Zurakowski, Alexandra G Espinel, Nina Deutsch, Brian K Reilly
OBJECTIVE: In this single-center, retrospective review, we sought to determine the risk factors associated with the development of severe acquired airway disease (AAD; vocal cord paralysis [VCP] or subglottic stenosis [SGS]) in pediatric patients who had undergone surgery for congenital heart disease (CHD) with cardiopulmonary bypass. All patients who required surgical treatment for CHD using cardiopulmonary bypass at our institution between 2010 and 2015 were reviewed. We defined severe AAD as either clinically significant VCP, SGS, or both, requiring consultation with the otolaryngology (ENT) service for evaluation...
April 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29688841/dose-effects-of-mycophenolate-mofetil-in-chinese-patients-with-neuromyelitis-optica-spectrum-disorders-a-case-series-study
#8
Yujuan Jiao, Lei Cui, Weihe Zhang, Chunyu Zhang, Yeqiong Zhang, Xin Zhang, Jinsong Jiao
BACKGROUND: Neuromyelitis optica (NMO) spectrum disorder (NMOSD) is a devastating autoimmune inflammatory disorder of the central nervous system, which can result in blindness or paralysis. Currently, there is a dire need for new treatment options in the clinic. Several case series have shown that mycophenolate mofetil (MMF) may be an effective treatment for NMOSD patients. The dosing of MMF in the treatment of NMOSD has been poorly studied. Therefore, we evaluated the efficacy, tolerability, influential factors and optimal dosage of MMF in Chinese patients with NMOSD...
April 23, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29686816/evaluation-of-the-prognostic-value-of-multimodal-intraoperative-monitoring-in-posterior-fossa-surgery-patients-with-cerebellopontine-angle-tumors
#9
Mohsen Dalvandi, Ali Nazemi Rafie, Alireza Kamali, Alireza Jamshidifard
The aim of this study was to compare the outcomes of four intraoperative monitoring approaches in order to compare their prognostic value in surgical complications of the facial nerve. This quasi-experimental study was conducted on 25 patients with Cerebellopontine Angle (CPA) tumors, who were hospitalized in the Valiasr Hospital, affiliated to the Arak University of Medical Sciences, Arak, Iran. The degree of the facial nerve paralysis was assessed based on the House-Brackmann (HB) facial nerve grading system...
January 12, 2018: European Journal of Translational Myology
https://www.readbyqxmd.com/read/29674667/a-mixed-periodic-paralysis-myotonia-mutant-p1158s-imparts-ph-sensitivity-in-skeletal-muscle-voltage-gated-sodium-channels
#10
Mohammad-Reza Ghovanloo, Mena Abdelsayed, Colin H Peters, Peter C Ruben
Skeletal muscle channelopathies, many of which are inherited as autosomal dominant mutations, include myotonia and periodic paralysis. Myotonia is defined by a delayed relaxation after muscular contraction, whereas periodic paralysis is defined by episodic attacks of weakness. One sub-type of periodic paralysis, known as hypokalemic periodic paralysis (hypoPP), is associated with low potassium levels. Interestingly, the P1158S missense mutant, located in the third domain S4-S5 linker of the "skeletal muscle", Nav1...
April 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29668642/operative-treatment-of-terrible-triad-of-the-elbow-with-a-modified-pugh-standard-protocol-retrospective-analysis-of-a-prospective-cohort
#11
Guanyi Liu, Weihu Ma, Ming Li, Jianxiang Feng, Rongming Xu, Zhijun Pan
The aim of the present study was to investigate the efficacy of the surgical treatment of terrible triad injuries of the elbow using a modified standard surgical approach, and treatment determined by the mode and mechanism of injury, and intraoperative findings.The study was a retrospective analysis of a prospective cohort of patients presenting with terrible triad injuries of the elbow, who underwent surgical treatment between July 2009 and January 2014. All patients were treated surgically according to a modified Pugh standard protocol...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29668505/comparison-of-functional-results-after-cross-face-nerve-graft-spinal-accessory-nerve-and-masseter-nerve-innervated-gracilis-for-facial-paralysis-reconstruction-the-chang-gung-experience
#12
David Chwei-Chin Chuang, Johnny Chuieng-Yi Lu, Tommy Nai-Jen Chang, Vincent G Laurence
BACKGROUND: Using functioning free muscle transplantation (FFMT) for facial paralysis and postparalysis facial synkinesis reconstruction is our preferred technique. Gracilis was the first choice of muscle. Three motor neurotizers: cross-face nerve graft (CFNG), spinal accessory nerve (XI) and masseter nerve (V3) have been used as neurotizers for different indications. METHODS: A total of 362 cases of facial reanimation with FFMT were performed between 1986 and 2015...
April 17, 2018: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/29667944/proximal-muscle-weakness-with-overlying-hypokalemic-periodic-paralysis-in-sj%C3%A3-gren-syndrome-report-of-6-cases
#13
Vimal K Paliwal, Anmol Singh Rai, Surendra Kumar, Ritu Verma, Vikas Agarwal
No abstract text is available yet for this article.
April 5, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29657223/distal-renal-tubular-acidosis-in-sj%C3%A3-gren-s-syndrome
#14
Ram Narayan, Mansoor C Abdulla, Jemshad Alungal, N C Krishnadas
Interstitial nephritis and immune complex-mediated glomerulonephritis are the two common renal manifestations of primary Sjögren's syndrome (SS). Here, we discuss three cases of primary SS where presenting manifestation was distal renal tubular acidosis. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with distal tubular acidosis or recurrent hypokalemic periodic paralysis as treatment of primary disease improves the outcome of illness.
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29656635/clinical-characteristics-of-dizziness-associated-with-acute-peripheral-facial-palsy
#15
Kudamo Song, Sehun Chang, Jun Lee, Sun Ae Shin, Ho Yun Lee
Background and Objectives: We aimed to assess the clinical significance of dizziness associated with acute peripheral facial palsy (APFP). Subjects and Methods: Medical records of patients who visited an otorhinolaryngology clinic at a university hospital and were admitted for treatment of APFP between 2014 and 2016 were thoroughly reviewed. Results: In total, 15.3% (n=15) of patients had dizziness. Continuous, rotatory dizziness without exacerbating factors was most common and frequently accompanied by nausea/vomiting...
April 17, 2018: Journal of Audiology & Otology
https://www.readbyqxmd.com/read/29649187/surveillance-to-track-progress-toward-polio-eradication-worldwide-2016-2017
#16
Tracie J Gardner, Ousmane M Diop, Jaume Jorba, Smita Chavan, Jamal Ahmed, Abhijeet Anand
Global efforts to eradicate polio began in 1988, and four of the six World Health Organization (WHO) regions currently have achieved poliofree certification. Within the remaining two regions with endemic poliomyelitis (African and Eastern Mediterranean), Afghanistan, Nigeria, and Pakistan have never interrupted transmission of wild poliovirus (WPV). The primary means of detecting poliovirus transmission is surveillance for acute flaccid paralysis (AFP) among children aged <15 years, combined with collection and testing of stool specimens for detection of WPV and vaccine-derived polioviruses (VDPVs)* in WHO-accredited laboratories within the Global Polio Laboratory Network (GPLN) (1,2)...
April 13, 2018: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/29628155/-vocal-cord-paralysis-after-endotracheal-intubation-an-uncommon-complication-of-general-anesthesia
#17
Claudia Hurtado Nazal, Andrea Araneda Vilches, Carolina Vergara Marín, Karen García Contreras, Carla Napolitano Valenzuela, Pedro Badía Ventí
BACKGROUND: General anesthesia is a safe, frequent procedure in clinical practice. Although it is very unusual in procedures not related to head and or neck surgery, vocal cord paralysis is a serious and important complication. Incidence has been associated with patient age and comorbidities, as well as the position of the endotracheal tube and cuff. It can become a dangerous scenario because it predisposes aspiration. OBJECTIVES: To present a case and analyze the risk factors associated with increased risk of vocal cord paralysis described in the literature...
April 5, 2018: Revista Brasileira de Anestesiologia
https://www.readbyqxmd.com/read/29606556/prevalence-and-mutation-spectrum-of-skeletal-muscle-channelopathies-in-the-netherlands
#18
B C Stunnenberg, J Raaphorst, J C W Deenen, T P Links, A A Wilde, D J Verbove, E J Kamsteeg, A van den Wijngaard, C G Faber, G J van der Wilt, B G M van Engelen, G Drost, H B Ginjaar
Few reliable data exist on the prevalence of skeletal muscle channelopathies. We determined the minimum point prevalence of genetically-defined skeletal muscle channelopathies in the Netherlands and report their mutation spectrum. Minimum point prevalence rates were calculated as number of genetically-confirmed skeletal muscle channelopathy patients (CLCN1, SCN4A, CACNA1S and KCNJ2 gene mutations) in the Netherlands (1990-2015) divided by the total number of at-risk individuals. Rates were expressed as cases/100...
March 9, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29605429/dysfunction-of-nav1-4-a-skeletal-muscle-voltage-gated-sodium-channel-in-sudden-infant-death-syndrome-a-case-control-study
#19
Roope Männikkö, Leonie Wong, David J Tester, Michael G Thor, Richa Sud, Dimitri M Kullmann, Mary G Sweeney, Costin Leu, Sanjay M Sisodiya, David R FitzPatrick, Margaret J Evans, Iona J M Jeffrey, Jacob Tfelt-Hansen, Marta C Cohen, Peter J Fleming, Amie Jaye, Michael A Simpson, Michael J Ackerman, Michael G Hanna, Elijah R Behr, Emma Matthews
BACKGROUND: Sudden infant death syndrome (SIDS) is the leading cause of post-neonatal infant death in high-income countries. Central respiratory system dysfunction seems to contribute to these deaths. Excitation that drives contraction of skeletal respiratory muscles is controlled by the sodium channel NaV1.4, which is encoded by the gene SCN4A. Variants in NaV1.4 that directly alter skeletal muscle excitability can cause myotonia, periodic paralysis, congenital myopathy, and myasthenic syndrome...
March 28, 2018: Lancet
https://www.readbyqxmd.com/read/29598895/triple-innervation-for-re-animation-of-recent-facial-paralysis
#20
Federico Biglioli, Fabiana Allevi, Dimitri Rabbiosi, Silvia Cupello, Valeria Marinella Augusta Battista, Alberto Maria Saibene, Giacomo Colletti
Recent facial palsies are those in which fibrillations of the mimetic musculature remain detectable by electromyography (EMG). Such fibrillations generally cease 18-24 months after palsy onset. During this period, facial re-animation surgery seeks to supply new neural inputs to the facial nerve. Neural usable sources were divided into qualitative (contralateral facial nerve) and quantitative (hypoglossus and masseteric nerve), depending on the type of stimulus provided. To further improve the extent and quality of facial re-animation, we here describe a new surgical technique featuring triple neural inputs: the use of the masseteric nerve and 30% of the hypoglossus nerve fibres as quantitative sources was associated with the contralateral facial nerve (incorporated via two cross-face nerve grafts) as a qualitative source in order to restore facial movements in 24 consecutive patients...
May 2018: Journal of Cranio-maxillo-facial Surgery
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