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Journal of the Peripheral Nervous System: JPNS

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https://www.readbyqxmd.com/read/28436077/neuroprotective-effect-of-epidermal-growth-factor-in-experimental-acrylamide-neuropathy-an-electrophysiological-approach
#1
H Perez-Saad, N Subiros, J Berlanga, L Aldana, D Garcia Del Barco
The neuroprotective effect of epidermal growth factor (EGF) has been documented in different contexts, but its potential benefits in peripheral neuropathies have been little studied. We investigated the neuroprotective action of EGF in experimental neuropathy induced by acrylamide (ACR). Mice and rats were treated chronically with acrylamide for 6 and 8 weeks, respectively. Concurrently they received EGF in daily doses of 1 and 5 mg/kg in mice and 3 mg/kg in rats, or saline (PBS). ACR severely affected the neurological score, the muscle strength, and the muscle potential M, in mice, as well as F-waves, sensory potentials (SP), and apomorphine-induced penile erection, in rats...
April 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28432749/motor-nerve-excitability-after-childhood-guillain-barr%C3%A3-syndrome
#2
Judith Drenthen, Joyce Roodbol, Ellen M Maathuis, Coriene E Catsman-Berrevoets, Joleen H Blok, Marie-Claire Y de Wit, Bart C Jacobs
OBJECTIVE: To determine the residual motor nerve dysfunction after pediatric Guillain-Barré syndrome (GBS). METHODS: Observational cross-sectional cohort study in patients who previously developed GBS during childhood (<18 years). Ulnar motor nerve dysfunction was defined by compound motor action potential (CMAP) scan in patients after a follow-up of at least one year compared with age-matched healthy controls, in relation to clinical course and outcome. RESULTS: Thirty-seven persons previously diagnosed with GBS in childhood were included with a mean age at current examination of 20...
April 21, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28429515/a-device-to-measure-secretion-of-individual-sweat-glands-for-diagnosis-of-peripheral-neuropathy
#3
Adam J Loavenbruck, James S Hodges, Vincenzo Provitera, Maria Nolano, Gwen Wendelshafer-Crabb, William R Kennedy
INTRODUCTION: There is a need for quantitative, precise assessment of small fiber peripheral nerve function. We tested a customized camera device and protocol designed to quantify secretions of individual sweat glands (SGs). MATERIALS AND METHODS: Testing was performed on 178 healthy controls and 20 neuropathy subjects. Sweating was stimulated on a 2.25 cm(2) skin area by iontophoresis of pilocarpine. The camera imaged sweat from 50-400 sweat ducts. We calculated secretion rate of individual SGs, total sweat volume, and number of secreting SGs at four body sites...
April 21, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28429469/high-body-mass-and-kidney-dysfunction-relate-to-worse-nerve-function-even-in-adults-without-neuropathy
#4
Rens Hanewinckel, M Arfan Ikram, Oscar H Franco, Albert Hofman, Judith Drenthen, Pieter A van Doorn
Polyneuropathy is a prevalent and disabling disorder. Despite extensive evaluation, the cause often remains unknown. Factors that predispose for the development of polyneuropathy need to be identified. We investigated the effect of anthropometric and metabolic factors on peripheral nerve function in 908 participants of the population-based Rotterdam Study without any symptoms or signs of polyneuropathy. Participants underwent nerve conduction studies of the sural and peroneal nerve. Data on age, height, weight, waist circumference, diabetes, lipid levels, hypertension and kidney function were collected...
April 20, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28407329/the-six-spot-step-test-a-new-method-for-monitoring-walking-ability-in-patients-with-chronic-inflammatory-polyneuropathy
#5
Melissa Kreutzfeldt, Henrik B Jensen, Mads Ravnborg, Lars H Markvardsen, Henning Andersen, Søren H Sindrup
OBJECTIVE: To evaluate whether the Six-Spot-Step-Test (SSST) is more suitable for monitoring walking ability in patients with chronic inflammatory polyneuropathy than the Timed-25-Foot-Walking test (T25FW). METHOD: In the SSST, participants have to walk as quickly as possible across a field measuring 1x5 metres while kicking blocks out of five circles on the floor. Sixty-two patients and sixty-one controls performed the SSST and T25FW. Patients also performed the Overall Disability Sumscore, INCAT Sensory Sumscore, MRC Sumscore and 9-Hole-Peg-Test...
April 13, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28406555/international-guillain-barr%C3%A3-syndrome-outcome-study-igos-protocol-of-a-prospective-observational-cohort-study-on-clinical-and-biological-predictors-of-disease-course-and-outcome-in-guillain-barr%C3%A3-syndrome
#6
Bart C Jacobs, Bianca van den Berg, Christine Verboon, Govindsinh Chavada, David R Cornblath, Kenneth C Gorson, Thomas Harbo, Hans-Peter Hartung, Richard A C Hughes, Susumu Kusunoki, Pieter A van Doorn, Hugh J Willison
Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical presentation, course, and outcome. The factors that determine the clinical variation of GBS are poorly understood which complicates the care and treatment of individual patients. The protocol of the ongoing International GBS Outcome Study (IGOS), a prospective, observational, multi-centre cohort study that aims to identify the clinical and biological determinants and predictors of disease onset, subtype, course and outcome of GBS is presented here...
April 12, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28397393/early-discrimination-of-sensorimotor-guillain-barr%C3%A3-syndrome-into-demyelinating-or-axonal-subtype-by-automated-nerve-excitability-testing
#7
So Young Pyun, Mi-Ri Kang, Joo Young Lee, Kim Jong Kuk, Seong-Il Oh, Jong Seok Bae
In the early stage of disease, differentiating acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor sensory axonal neuropathy (AMSAN) using only a conventional nerve conduction studies (NCS) may be difficult. We evaluated the differences in the motor axonal excitability properties of 16 cases of sensorimotor Guillain-Barré syndrome by nerve excitability testing (NET). The antiganglioside antibody assay and follow-up NCS resulted in 12 patients diagnosed as AIDP and 4 patients as AMSAN. Clinical and excitability parameters in each group were compared with those in 30 normal controls...
April 11, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28177573/novel-ntrk1-mutations-associated-with-congenital-insensitivity-to-pain-with-anhidrosis-verified-by-functional-studies
#8
Tai-Seung Nam, Wenting Li, Somy Yoon, Gwang Hyeon Eom, Myeong-Kyu Kim, Sung Taek Jung, Seok-Yong Choi
Congenital insensitivity to pain with anhidrosis (CIPA), also known as hereditary sensory and autonomic neuropathy type IV (HSAN-IV), features loss of pain sensation, decreased or absent sweating (anhidrosis), recurrent episodes of unexplained fever, self-mutilating behavior and variable mental retardation. Mutations in neurotrophic receptor tyrosine kinase 1 (NTRK1) have been reported to be associated with CIPA. We identified four novel NTRK1 mutations in six Korean patients from four unrelated families. Of the four mutations, we demonstrated using a splicing assay that IVS14+3A>T causes aberrant splicing of NTRK1 mRNA, leading to introduction of a premature termination codon...
February 8, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28177572/diabetes-mellitus-may-affect-short-term-outcome-of-guillain-barr%C3%A3-syndrome
#9
Stojan Peric, Ivo Bozovic, Bogdan Bjelica, Ivana Berisavac, Olivera Stojiljkovic, Ivana Basta, Ljiljana Beslac-Bumbasirevic, Vidosava Rakocevic-Stojanovic, Dragana Lavrnic, Zorica Stevic
We sought to determine influence of diabetes mellitus on GBS course and short-term prognosis. Among the 257 GBS patients included in this retrospective study, diabetes mellitus was present in 17%. The degree of disability at admission and on discharge was assessed according to the GBS Disability Scale (mild disability = 0-3, severe disability = 4-6). Even after correction for age, diabetes mellitus was significantly associated with more severe disability at nadir (OR=3.4, P < 0.05) and on discharge (OR=2...
February 8, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28417601/abstracts-of-the-seventh-annual-meeting-of-the-associazione-italiana-sistema-nervoso-periferico-asnp-april-20-22-2017-bergamo-italy
#10
(no author information available yet)
No abstract text is available yet for this article.
April 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27991707/guillain-barr%C3%A3-syndrome-in-france-a-nationwide-epidemiological-analysis-based-on-hospital-discharge-data-2008-2013
#11
Alexandra Delannoy, Jérémie Rudant, Christophe Chaignot, Francis Bolgert, Yann Mikaeloff, Alain Weill
Guillain-Barré syndrome (GBS) is potentially life threatening and typically occurs after an infection. No detailed information is available concerning the epidemiological characteristics of GBS in France. We estimated age- and sex-specific incidence rates (IRs) based on a French nationwide hospital discharge database. All patients hospitalized for GBS between 2008 and 2013 were identified by International Classification of Diseases-10 code G61.0 as principal diagnosis. Patients previously hospitalized for GBS in 2006 and 2007 were excluded...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27982524/a-novel-ndrg1-mutation-in-a-non-romani-patient-with-cmt4d-hmsn-lom
#12
Giuseppe Piscosquito, Stefania Magri, Paola Saveri, Micaela Milani, Claudia Ciano, Laura Farina, Franco Taroni, Davide Pareyson
Charcot-Marie-Tooth disease type 4D (CMT4D), also known as hereditary motor and sensory neuropathy Lom type (HMSNL), is an autosomal recessive, early onset, severe demyelinating neuropathy with hearing loss, caused by N-Myc downstream-regulated gene 1 (NDRG1) mutations. CMT4D is rare with only three known mutations, one of which (p.Arg148Ter) is found in patients of Romani ancestry and accounts for the vast majority of cases. We report a 38-year-old Italian female with motor development delay, progressive neuropathy, and sensorineural deafness...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27982499/hereditary-gelsolin-amyloidosis-hga-a-neglected-cause-of-bilateral-progressive-or-recurrent-facial-palsy
#13
Anna Sagnelli, Giuseppe Piscosquito, Daniela Di Bella, Laura Fadda, Lisa Melzi, Antonio Morico, Claudia Ciano, Franco Taroni, Dante Facchetti, Ettore Salsano, Davide Pareyson
We report the first Italian family affected by hereditary gelsolin amyloidosis (HGA), a rare autosomal dominant disease characterized by adult-onset slowly progressive cranial neuropathy, lattice corneal dystrophy, and cutis laxa. The index case was a 39-year-old male with a 9-year history of progressive bilateral facial nerve palsy. His mother had two episodes of acute facial palsy, and his maternal aunt and grandfather were also affected. Electrophysiological studies confirmed bilateral facial nerve involvement, without signs of peripheral polyneuropathy, and ophthalmological examination showed bilateral lattice corneal dystrophy, in both the index case and his mother...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27935216/modulation-of-diet-induced-mechanical-allodynia-by-metabolic-parameters-and-inflammation
#14
Michael A Cooper, Janelle M Ryals, Pau-Yen Wu, Kellen D Wright, Katherine R Walter, Douglas E Wright
Dietary-associated diseases have increased tremendously in our current population, yet key molecular changes associated with high-fat diets that cause clinical pre-diabetes, obesity, hyperglycemia, and peripheral neuropathy remain unclear. This study examines molecular and metabolic aspects altered by voluntary exercise and a high-fat diet in the mouse dorsal root ganglion. Mice were examined for changes in mRNA and proteins encoding anti-inflammatory mediators, metabolic-associated molecules, and pain-associated ion channels...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27917570/handwriting-difficulties-of-children-with-charcot-marie-tooth-disease-type-1a
#15
Daniel Kunovsky, Reinie Cordier, Paula Bray, Joshua Burns
Hand weakness and impaired manual dexterity have been reported in children with Charcot-Marie-Tooth disease type 1A (CMT1A). This early onset of upper limb involvement might explain frequent clinical referrals for assessment and treatment of impaired handwriting performance. The aim of this study was to examine the impact of CMT1A on handwriting speed and legibility, and identify demographic, anthropometric, and physical measures that might relate to handwriting performance. Handwriting speed (Handwriting Speed Test), handwriting legibility (Evaluation Tool of Children's Handwriting-Cursive), and hand strength (hand-held dynamometry of tip pinch, lateral pinch and grip) were assessed in 30 children with CMT1A (aged 8-17 years) and 30 age- and sex-matched controls...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27868289/determinants-of-health-related-quality-of-life-in-anti-mag-neuropathy-a-cross-sectional-multicentre-european-study
#16
Emilien Delmont, Fu Liong Hiew, Julien Cassereau, Anne-Catherine Aubé-Nathier, Aude-Marie Grapperon, Shahram Attarian, Yusuf A Rajabally
Our objective was to assess determinants of quality of life (QoL) in anti-myelin associated glycoprotein antibody (MAG) neuropathy. The SF-36 questionnaire was assessed in 55 patients, from Marseille, Angers (France) and Birmingham (UK). Routine clinical evaluations included Medical Research Council (MRC) sum score, inflammatory neuropathy cause and treatment (INCAT) sensory score, inflammatory Rasch-built overall disability score (I-RODS), ataxia score, Jamar grip dynamometry, timed 10-m walk, neuropathic pain symptom inventory (NPSI) score, and fatigue severity score (FSS)...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27862672/clinical-and-genetic-spectra-of-charcot-marie-tooth-disease-in-chinese-han-patients
#17
Bo Sun, Zhaohui Chen, Li Ling, Fei Yang, Xusheng Huang
Charcot-Marie-Tooth disease (CMT) is a common hereditary motor and sensory neuropathy. Epidemiological data for Chinese CMT patients are few. This study aimed to analyze the electrophysiological and genetic characteristics of Chinese Han patients. A total of 106 unrelated patients with the clinical diagnosis of CMT were included. Clinical examination, nerve conduction studies (NCS), next-generation sequencing (NGS), and bioinformatic analyses were performed. Genetic testing was performed for 82 patients; 27 (33%) patients carried known CMT-associated gene mutations...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27859927/clinical-features-of-familial-amyloid-polyneuropathy-carrying-transthyretin-mutations-in-four-chinese-kindreds
#18
Gonglu Liu, Wang Ni, Hongxia Wang, Hongfu Li, Yue Zhang, Ning Wang, Zhiying Wu
Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) is a rare hereditary disorder, characterized by a length-dependent polyneuropathy and dysfunction of various organs. Wide phenotypic heterogeneity makes early diagnosis difficult. In this study, we reviewed the clinical and electrophysiological features of four unrelated Chinese families with genetically confirmed TTR-FAP. Sequence analysis of TTR gene revealed the presence of four different mutations: Thr49Ala(p.Thr69Ala), Leu55Arg(p.Leu75Arg), Tyr116Ser(p...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27801990/inhibition-of-complement-in-guillain-barr%C3%A3-syndrome-the-ica-gbs-study
#19
Amy I Davidson, Susan K Halstead, John A Goodfellow, Govind Chavada, Arup Mallik, James Overell, Michael P Lunn, Alex McConnachie, Pieter van Doorn, Hugh J Willison
The outcome of Guillain-Barré syndrome (GBS) remains unchanged since plasma exchange and intravenous immunoglobulin (IVIg) were introduced over 20 years ago. Pathogenesis studies on GBS have identified the terminal component of complement cascade as a key disease mediator and therapeutic target. We report the first use of terminal complement pathway inhibition with eculizumab in humans with GBS. In a randomised, double-blind, placebo-controlled trial, 28 subjects eligible on the basis of GBS disability grade of at least 3 were screened, of whom 8 (29%) were randomised...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27717230/asymptomatic-dengue-infection-may-trigger-guillain-barr%C3%A3-syndrome
#20
LETTER
Thirugnanam Umapathi, Christen Sheng Jie Lim, Eng Eong Ooi, Summer L Zhang, Eunice Jin Hui Goh, Hwee Cheng Tan, Kelly Yi Shi Chng, Hugh Willison
No abstract text is available yet for this article.
December 2016: Journal of the Peripheral Nervous System: JPNS
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