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Periodic paralysis

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https://www.readbyqxmd.com/read/28087767/trial-by-trial-motor-cortical-correlates-of-a-rapidly-adapting-visuomotor-internal-model
#1
Sergey D Stavisky, Jonathan C Kao, Stephen I Ryu, Krishna V Shenoy
: Accurate motor control is mediated by internal models of how neural activity generates movement. We examined neural correlates of an adapting internal model of visuomotor gain in motor cortex while two macaques performed a reaching task in which the gain scaling between the hand and a presented cursor was varied. Previous studies of cortical changes during visuomotor adaptation focused on preparatory and peri-movement epochs and analyzed trial-averaged neural data. Here, we recorded simultaneous neural population activity using multielectrode arrays and focused our analysis on neural differences in the period before the target appeared...
January 13, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28063223/neurally-adjusted-ventilatory-assist-for-infants-under-prolonged-ventilation
#2
Juyoung Lee, Han-Suk Kim, Young Hwa Jung, Chang Won Choi, Yong Hoon Jun
BACKGROUND: Severe bronchopulmonary dysplasia (BPD) often leads to prolonged mechanical ventilation lasting several months. Cyanotic episodes frequently occur in these patients, which necessitate long-term sedation and/or intermittent muscle paralysis. Neurally adjusted ventilatory assist (NAVA) might provide precisely the amount of support that these patients need without sedation. METHODS: We reviewed the medical records of preterm infants who underwent a tracheostomy and required mechanical ventilation for more than 6 months during a period of 6 years...
January 7, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28058777/platysma-sparing-vascularized-submental-lymph-node-flap-transfer-for-extremity-lymphedema
#3
Igor Poccia, Chia-Yu Lin, Ming-Huei Cheng
BACKGROUND AND OBJECTIVES: Due to its consistent vascular and lymphatic anatomy, the vascularized submental lymph node flap is a reliable option for lymphedema treatment. Despite these advantages, flap harvest requires resection of platysma, which may cause a marginal mandibular nerve pseudo-paralysis. The aim of this study was to investigate the donor site morbidity of an innovative platysma-sparing vascularized submental lymph node flap transfer for treating extremity lymphedema. METHODS: Ten patients undergoing platysma sparing submental lymph-node flap harvest were prospectively enrolled in the study and compared with a control group of 10 patients who underwent standard submental lymph-node flap harvest...
January 6, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28050008/hypokalemic-paralysis-complicated-by-concurrent-hyperthyroidism-and-hyperaldosternoism-a-case-report
#4
Yu-Hsin Hsiao, Yu-Wei Fang, Jyh-Gang Leu, Ming-Hsein Tsai
BACKGROUND Thyrotoxic periodic paralysis (TPP) is commonly observed in patients with acute paralysis and hyperthyroidism. However, there is a possibility of secondary causes of hypokalemia in such a setting. CASE REPORT Herein, we present the case of a 38-year-old woman with untreated hypertension and hyperthyroidism. She presented with muscle weakness, nausea, vomiting, and diarrhea since one week. The initial diagnosis was TPP. However, biochemistry tests showed hypokalemia with metabolic alkalosis and renal potassium wasting...
January 4, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28024840/intrafamilial-phenotypic-variability-in-andersen-tawil-syndrome-a-diagnostic-challenge-in-a-potentially-treatable-condition
#5
A Ardissone, V Sansone, L Colleoni, P Bernasconi, I Moroni
Andersen-Tawil syndrome (ATS) is a rare autosomal dominant channelopathy characterized by periodic paralysis, cardiac dysrhythmias, and distinct facial and skeletal characteristics, that may be variably present in the affected members. Mutations in the KCNJ2 and KCNJ5 gene have been associated with this disorder. We describe a family in which several members presented with different ATS phenotypes. The proband, a 4-year-old boy, presented with recurrent episodes of muscle weakness from an early age; two siblings suffered cardiac arrhythmia but had never experienced episodes of paralysis; their mother reported occasional muscle pain after exercise and unspecified cardiac arrhythmias...
November 18, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28012096/a-sodium-channel-myotonia-presenting-with-intermittent-dysphagia-as-a-manifestation-of-a-rare-scn4a-variant
#6
Jihane N Benhammou, Jennifer Phan, Hane Lee, Kevin Ghassemi, William Parsons, Wayne W Grody, Joseph R Pisegna
The voltage gated sodium channel SCN4A mutations account for non-dystrophic myotonia and include a heterogeneous group of conditions that include hyperkalemic periodic paralysis, paramyotonica congenita, potassium-aggravated myotonia, and hypokalemic periodic paralysis type 2. This case report proposes that a rare variant p.Pro1629Leu in SCN4A can cause a skeletal muscle deficit with intermittent dysphagia.
December 23, 2016: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28012042/dihydropyridine-receptor-dhpr-cacna1s-congenital-myopathy
#7
Vanessa Schartner, Norma B Romero, Sandra Donkervoort, Susan Treves, Pinki Munot, Tyler Mark Pierson, Ivana Dabaj, Edoardo Malfatti, Irina T Zaharieva, Francesco Zorzato, Osorio Abath Neto, Guy Brochier, Xavière Lornage, Bruno Eymard, Ana Lía Taratuto, Johann Böhm, Hernan Gonorazky, Leigh Ramos-Platt, Lucy Feng, Rahul Phadke, Diana X Bharucha-Goebel, Charlotte Jane Sumner, Mai Thao Bui, Emmanuelle Lacene, Maud Beuvin, Clémence Labasse, Nicolas Dondaine, Raphael Schneider, Julie Thompson, Anne Boland, Jean-François Deleuze, Emma Matthews, Aleksandra Nadaj Pakleza, Caroline A Sewry, Valérie Biancalana, Susana Quijano-Roy, Francesco Muntoni, Michel Fardeau, Carsten G Bönnemann, Jocelyn Laporte
Muscle contraction upon nerve stimulation relies on excitation-contraction coupling (ECC) to promote the rapid and generalized release of calcium within myofibers. In skeletal muscle, ECC is performed by the direct coupling of a voltage-gated L-type Ca(2+) channel (dihydropyridine receptor; DHPR) located on the T-tubule with a Ca(2+) release channel (ryanodine receptor; RYR1) on the sarcoplasmic reticulum (SR) component of the triad. Here, we characterize a novel class of congenital myopathy at the morphological, molecular, and functional levels...
December 23, 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/28008586/association-of-kcnj2-genetic-variants-with-susceptibility-to-thyrotoxic-periodic-paralysis-in-patients-with-graves-disease
#8
Suyeon Park, TaeYong Kim, Soyoung Sim, Hye-Seon Oh, Eyun Song, Mijin Kim, Hyemi Kwon, YunMi Choi, MinJi Jeon, WonGu Kim, YoungKee Shong, WonBae Kim
Objective: Thyrotoxic periodic paralysis (TPP) is characterized by acute onset paralysis and hypokalemia predominantly in male patients with thyrotoxicosis. Recent studies have emphasized the importance of potassium channels, which might explain the underlying mechanism of TPP. The KCNJ2 gene encodes the inward-rectifying potassium channel. In this study, we evaluated the role of KCNJ2 in the development of TPP. Design: Case-control analysis of KCNJ2 genetic association with TPP. Patients: 83 male patients with Graves' disease (GD) were recruited for this study; 43 patients had TPP, whereas 40 patients had no history of TPP...
December 22, 2016: Experimental and Clinical Endocrinology & Diabetes
https://www.readbyqxmd.com/read/28008196/clinical-characteristics-of-primary-peritoneal-carcinoma-patients-a-single-institution-experience-involving-8-patients
#9
Satomi Hattori, Hiroaki Kajiyama, Utako Fuji, Yuko Furui, Yuki Ishibashi, Yuka Hattori, Noriko Takahashi, Fumitaka Kikkawa, Toshiya Misawa
Primary peritoneal carcinoma (PPC) is treated similarly to advanced epithelial ovarian carcinoma (aEOC); however, the standard approach for the management of PPC is controversial. The objective of this study was to evaluate the clinical features and prognosis of those patients. A retrospective analysis was performed of eight patients with PPC between January 2008 and December 2015. Clinicopathologic parameters, the diagnostic modality, treatment, and oncologic outcome were analyzed. The median age at the time of diagnosis was 72...
December 2016: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28006864/is-thyrotoxic-periodic-paralysis-a-disease-caused-by-muscle-membrane-dysfunction
#10
Yoo-Ri Son, Nam-Hee Kim, Dong-Gun Kim, Kyung-Seok Park
INTRODUCTION: Thyrotoxic periodic paralysis (TPP) is characterized by recurrent episodes of reversible paralysis with hyperthyroidism. It is clinically similar to hypokalemic periodic paralysis (HOPP), which features significant ion channel dysfunction and reduced muscle fiber conduction velocity (MFCV). However, the muscle membrane function in TPP is not known. METHODS: For 13 patients with TPP and 15 age-matched controls, clinical assessment and serial neurophysiological testing including nerve conduction, prolonged exercise (PE) testing, and MFCV were performed...
December 22, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/28005811/the-role-of-percutaneous-radiofrequency-thermocoagulation-for-persistent-or-recurrent-trigeminal-neuralgia-after-surgery
#11
Pengfei Liu, Wenxiang Zhong, Chenlong Liao, Min Yang, Wenchuan Zhang
Therapeutic strategy is controversial and not yet uniform for patients with trigeminal neuralgia (TN) and persistent or recurrent facial pain after microvascular decompression, percutaneous radiofrequency thermocoagulation (PRT), or Gamma Knife surgery. The outcomes and risks of PRT for these patients are not clearly understood. The authors performed a retrospective study of 84 patients with persistent or recurrent TN after surgery who then underwent PRT between 2007 and 2013. Data were obtained with chart review and telephone interviews...
November 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28003099/long-term-results-of-18-fat-injections-in-unilateral-vocal-fold-paralysis
#12
R Pagano, D Morsomme, S Camby, L Lejeune, C Finck
OBJECTIVE: The purposes of this study were to assess the long-term efficacy of medialization laryngoplasty via injection of autologous fat in patients with unilateral laryngeal paralysis, and to discuss the results based on the volume of fat injected and the size of the initial glottic gap. STUDY DESIGN: This was a retrospective study with clinical reevaluation of 18 patients treated for unilateral laryngeal paralysis at Liège University Hospital between April 1, 2011, and December 1, 2014...
December 19, 2016: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28000305/parotidectomy-under-sedation-and-locoregional-anesthesia-with-monitoring-of-brain-activity
#13
Marco Antonio Scirea Tesseroli, Fabricio Battistela Zasso, Humberto Hepp, Antonio Vitor Martins Priante, André Luiz Loureiro de Mattos Filho, Alvaro Sanabria
BACKGROUND: Parotidectomy is usually performed while the patient is under general anesthesia, however, sedation with locoregional anesthesia could be an alternative. METHODS: Fifteen adult patients with parotid tumors of the superficial lobe were included in this study. Anesthetic procedure consisted of sedation associated with cervical plexus and auriculotemporal nerve block. Sedation was managed based on the bispectral index. RESULTS: Superficial parotidectomies were performed in 13 patients, and combined partial resections were performed in 2 patients...
December 21, 2016: Head & Neck
https://www.readbyqxmd.com/read/27997055/role-of-immediate-recurrent-laryngeal-nerve-reconstruction-in-surgery-for-thyroid-cancers-with-fixed-vocal-cords
#14
Shinobu Iwaki, Tatsuyoshi Maeda, Miki Saito, Naoki Otsuki, Miki Takahashi, Emi Wakui, Hirotaka Shinomiya, Koichi Morimoto, Hiroyuki Inoue, Hiroo Masuoka, Akira Miyauchi, Ken-Ichi Nibu
BACKGROUND: Quality of voice after immediate recurrent laryngeal nerve (RLN) reconstruction in thyroid cancers has not been thoroughly studied. METHODS: Thirteen patients with fixed vocal cords (fixed vocal cord group) and 8 patients with intact or impaired mobile vocal cords (mobile vocal cord group) who had immediate RLN reconstruction simultaneously with total thyroidectomy, and patients who had arytenoid adduction and thyroplasty for vocal cord paralysis caused by previous surgery (arytenoid adduction thyroplasty group) were enrolled in this study...
December 20, 2016: Head & Neck
https://www.readbyqxmd.com/read/27993359/perioperative-management-and-outcomes-of-esophageal-atresia-and-tracheoesophageal-fistula
#15
Dave R Lal, Samir K Gadepalli, Cynthia D Downard, Daniel J Ostlie, Peter C Minneci, Ruth M Swedler, Thomas Chelius, Laura Cassidy, Cooper T Rapp, Katherine J Deans, Mary E Fallat, S Maria E Finnell, Michael A Helmrath, Ronald B Hirschl, Rashmi S Kabre, Charles M Leys, Grace Mak, Jessica Raque, Frederick J Rescorla, Jacqueline M Saito, Shawn D St Peter, Daniel von Allmen, Brad W Warner, Thomas T Sato
BACKGROUND/PURPOSE: Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care...
December 5, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27982765/the-epidemiology-of-spinal-tuberculosis-in-the-united-states-an-analysis-of-2002-2011-data
#16
Rafael De la Garza Ramos, C Rory Goodwin, Nancy Abu-Bonsrah, Ali Bydon, Timothy F Witham, Jean-Paul Wolinsky, Daniel M Sciubba
OBJECTIVE The aim of this study was to investigate the incidence of spinal tuberculosis (TB) in the US between 2002 and 2011. METHODS The Nationwide Inpatient Sample database from 2002 to 2011 was used to identify patients with a discharge diagnosis of TB and spinal TB. Demographic and hospital data were obtained for all admissions, and included age, sex, race, comorbid conditions, insurance status, hospital location, hospital teaching status, and hospital region. The incidence rate of spinal TB adjusted for population growth was calculated after application of discharge weights...
December 16, 2016: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/27981401/early-permanent-disappearance-of-abnormal-muscle-response-during-microvascular-decompression-for-hemifacial-spasm-a-retrospective-clinical-study
#17
Chengrong Jiang, Wu Xu, Yuxiang Dai, Tianyu Lu, Wei Jin, Weibang Liang
The objective of this study is to explore the cause of early abnormal muscle response (AMR) disappearance during microvascular decompression for hemifacial spasm and the clinical outcomes of these patients. Three hundred seventy-two patients received microvascular decompression (MVD) under intraoperative electrophysiological monitoring in Nanjing Drum Tower Hospital in 2014; the characteristic AMR of HFS was observed in 359 patients during the operation. And the 359 patients were divided into two groups based on whether AMR had remained before the beginning of the decompression procedure for offending vessels...
December 15, 2016: Neurosurgical Review
https://www.readbyqxmd.com/read/27973611/publication-trends-in-acupuncture-research-a-20-year-bibliometric-analysis-based-on-pubmed
#18
Yan Ma, Ming Dong, Kehua Zhou, Carol Mita, Jianping Liu, Peter M Wayne
OBJECTIVE: Acupuncture has become popular and widely practiced in many countries around the world. Despite the large amount of acupuncture-related literature that has been published, broader trends in the prevalence and scope of acupuncture research remain underexplored. The current study quantitatively analyzes trends in acupuncture research publications in the past 20 years. METHODS: A bibliometric approach was used to search PubMed for all acupuncture-related research articles including clinical and animal studies...
2016: PloS One
https://www.readbyqxmd.com/read/27955984/lower-extremity-weakness-in-a-teenager-due-to-thyrotoxic-periodic-paralysis
#19
Matthew D Thornton
BACKGROUND: Thyrotoxic hypokalemic paralysis is the hallmark of thyrotoxic periodic paralysis (TPP). TPP is a potentially deadly complication of hyperthyroidism that occurs because of rapid and dramatic intracellular shift of potassium. This transference results in severe hypokalemia and clinically manifests itself as muscle weakness or paralysis. This condition predominantly affects males of Asian descent, and its presentation can range from mild to severe, as seen in our case. CASE REPORT: We present the case of a 15-year-old Asian-American male who presented to a tertiary-care pediatric emergency department complaining of generalized weakness and flaccid paralysis of his lower extremities...
December 9, 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27943294/laryngeal-pacing-for-bilateral-vocal-fold-paralysis-voice-and-respiratory-aspects
#20
Andreas H Mueller, Rudolf Hagen, Claus Pototschnig, Gerhard Foerster, Wilma Grossmann, Katrin Baumbusch, Markus Gugatschka, Tadeus Nawka
OBJECTIVE: To evaluate the effects of neurostimulation with the laryngeal pacemaker (LP) system in patients with bilateral vocal fold paralysis (BVFP) in terms of voice quality and respiratory function. STUDY DESIGN: Feasibility study, first-in-human, single-arm, open-label, prospective, multicenter study with group-sequential design and 6-month follow-up period, as described in details in our previous publication. METHODS: Nine symptomatic BVFP subjects were unilaterally implanted with the LP system at three study sites in Germany and Austria...
December 10, 2016: Laryngoscope
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