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Journal of the Peripheral Nervous System: JPNS

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https://www.readbyqxmd.com/read/30255608/nociceptin-orphanin-fq-opioid-peptide-receptor-expression-in-pachyonychia-congenita
#1
Baohan Pan, Wolfgang Schröder, Ruth Jostock, Mary Schwartz, Gedge Rosson, Michael Polydefkis
Nociceptin/orphanin FQ opioid peptide (NOP)-receptor (NOP-R) is a member of the opioid receptor family. NOP-R activation has demonstrated analgesic effects in preclinical pain models without the addiction risks associated with other opiate targets. Pachyonychia congenita (PC) is a palmoplantar keratoderma characterized by neuropathic pain in affected skin. A cohort of KRT6A gene mutation PC patients with no other explanation for their neuropathic pain offered a unique opportunity to assess potential of NOP-R as a therapeutic target...
September 25, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/30203907/rituximab-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy-with-associated-diseases
#2
Thomas Roux, Rabab Debs, Thierry Maisonobe, Timothée Lenglet, Cécile Delorme, Céline Louapre, Véronique Leblond, Karine Viala
OBJECTIVE: We aimed to analyse the response to rituximab in a cohort of CIDP patients with associated disorders. METHODS AND MATERIALS: We conducted a clinical and electrophysiological retrospective monocentric study in 28 CIDP patients. Response to rituximab was defined as i) a 5-point increase in the Medical Research Council (MRC) sum score or a one-point decrease in the Overall Neuropathy Limitations Scale (ONLS) score, compared to the score at the first rituximab infusion, or ii) the discontinuation of, or reduced need for, the last treatments before rituximab initiation...
September 11, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/30151941/second-ivig-course-in-guillain-barr%C3%A3-syndrome-patients-with-poor-prognosis-sid-gbs-trial-protocol-for-a-double-blind-randomized-placebo-controlled-clinical-trial
#3
Christa Walgaard, Bart C Jacobs, Hester F Lingsma, Ewout W Steyerberg, David R Cornblath, Pieter A van Doorn
One course of intravenous immunoglobulins (IVIg) of 2 g/kg is standard treatment in Guillain-Barré syndrome (GBS) patients unable to walk independently. Despite treatment some patients recover poorly, in part related to rapid consumption of IVIg, indicating that they may benefit from a second course of IVIg. The aim of the study is to determine whether a second course of IVIg, administered 1 week after start of the first course in patients with GBS and predicted poor outcome improves functional outcome on the GBS disability scale after 4 weeks...
August 27, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/30101437/clinical-relevance-of-serum-antibodies-to-gd1b-in-immune-mediated-neuropathies
#4
Noor E Taams, Nicolette C Notermans, Willem-Jan R Fokkink, Anne P Tio-Gillen, Ruth Huizinga, Marco W J Schreurs, Bart C Jacobs
Antibodies to the ganglioside GD1b have been reported in various forms of immune-mediated neuropathy, but their clinical relevance for diagnosis and prognosis is unknown. We investigated the prevalence of anti-GD1b antibodies in acute and chronic immune-mediated neuropathies, and the clinical presentation and outcome in Guillain-Barré syndrome (GBS) and Miller Fisher-GBS overlap syndrome (MF-GBS). Anti-GD1b, anti-GM1 and anti-GQ1b antibodies were tested in serum of patients with GBS (N = 165), Miller Fisher syndrome (N = 10), MF-GBS (N = 28), monoclonal gammopathy of unknown significance neuropathy (MGUS; N = 101), chronic inflammatory demyelinating polyneuropathy (N = 29), paraneoplastic syndrome with anti-Hu-associated neuropathy (PNS; N = 11), other auto-immune diseases (AID; N = 60), and healthy controls (HC; N = 60)...
August 12, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/30070025/the-2016-singapore-zika-virus-outbreak-did-not-cause-a-surge-in-guillain-barr%C3%A3-syndrome
#5
Thirugnanam Umapathi, Yiu-Wing Kam, O Ohnmar, Brandon C J Ng, Yixiang Ng, M Premikha, Yee-Sin Leo, Lisa F P Ng
Although individuals with Zika virus (ZIKV) antibodies were reported in Malaya in mid-1950s, entomological and human surveillance in Singapore did not identify autochthonous transmission until the outbreak of August-November, 2016. A total of 455 cases from 15 separate clusters were identified. We asked if this ZIKV outbreak increased the incidence of Guillain-Barré syndrome (GBS) and aimed to characterize these cases. Eleven GBS cases, consecutively enrolled into our prospective GBS database from onset to 4 weeks after outbreak, and six controls, comprising three GBS patients enrolled before outbreak and three non-GBS patients, were examined for evidence of recent ZIKV infection...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/30070007/mandating-nerve-biopsy-a-step-towards-personalizing-therapy-in-pure-neuritic-leprosy
#6
Dinkar Kulshreshtha, Kiran Preet Malhotra, Hardeep Singh Malhotra, Anup Kumar Thacker, Ravindra Kumar Garg, Ajai Kumar Singh, Pradeep Kumar Maurya
Pure neuritic leprosy (PNL) accounts for 5% to 10% of leprosy patients who usually present with asymmetrical neuropathy in the absence of lepra bacilli on slit-skin smears. However, nerve biopsies in PNL lack appropriate categorization in current immunologic terms. We aimed to classify nerve biopsies according to the immune spectrum of leprosy and assess the role of histologic classification of nerve biopsies in treating PNL. Patients from two tertiary care referral centres were enrolled in this incident case study...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/30027593/conventional-and-unconventional-therapies-in-typical-and-atypical-chronic-inflammatory-demyelinating-polyneuropathy-with-different-clinical-course-of-progression
#7
Stefanie K Y Hung, Fu L Hiew, Shanthi Viswanathan, Santhi Puvanarajah
Intravenous immunoglobulin (IVIG), corticosteroids and therapeutic plasma exchange (TPE) are evidence-based conventional treatments for chronic inflammatory demyelinating polyneuropathy (CIDP). In many centres, unconventional treatments are frequently used as alternatives. We evaluated the outcome of conventional and unconventional therapies in 31 CIDP patients. Overall response rate with conventional first-line immunotherapies was 77% (20/26), comparable between IVIG and corticosteroids (80% vs 70%). Use of TPE was limited...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29999199/employment-status-of-patients-with-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#8
Bogdan Bjelica, Ivana Basta, Ivo Bozovic, Aleksandra Kacar, Ana Nikolic, Aleksandra Dominovic-Kovacevic, Zoran Vukojevic, Vesna Martic, Aleksandar Stojanov, Gordana Djordjevic, Milutin Petrovic, Miroslav Stojanovic, Stojan Peric
It has been previously shown that patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are unemployed or retired have worse quality of life. The aim of this study was to assess predictors of early retirement in CIDP. One hundred five patients with CIDP were included. Following measures were used: questionnaire on employment status, Medical Research Council Sum Score, INCAT disability score, Beck Depression Inventory, and Krupp's Fatigue Severity Scale. At the moment of testing, 2% of patients were students, 15% were employed, 9% were unemployed due to CIDP, 9% were unemployed but not due to CIDP, 28% were retired early due to disability caused by CIDP, and finally 37% were in old-age pension...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29978519/a-novel-scn9a-splicing-mutation-in-a-compound-heterozygous-girl-with-congenital-insensitivity-to-pain-hyposmia-and-hypogeusia
#9
Margherita Marchi, Vincenzo Provitera, Maria Nolano, Marcello Romano, Simona Maccora, Ilaria D'Amato, Erika Salvi, Monique Gerrits, Lucio Santoro, Giuseppe Lauria
Congenital insensitivity to pain (CIP) is a rare autosomal recessive disorder presenting with a spectrum of clinical features caused by mutations in different genes. A 10-year-old girl with CIP, hyposmia and hypogeusia, and her unaffected twin and parents underwent next generation sequencing of SCN9A exons and flanking splice sites. Transcript analysis from whole blood successfully assayed the effect of the mutation on the mRNA splicing by polymerase chain reaction amplification on cDNA and Sanger sequencing...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29974556/serum-and-cerebrospinal-neurofilament-light-chain-levels-in-patients-with-acquired-peripheral-neuropathies
#10
Sara Mariotto, Alessia Farinazzo, Roberta Magliozzi, Daniela Alberti, Salvatore Monaco, Sergio Ferrari
Neurofilament light chain (NFL) levels reflect axonal damage in different inflammatory and neurodegenerative central nervous system conditions, in correlation with disease severity. Our aim was to determine the possible diagnostic and prognostic value of serum and cerebrospinal fluid (CSF) NFL levels in subjects with different forms of acquired peripheral neuropathies (PN). Paired serum and CSF samples of 25 patients with acquired PN were analysed for NFL using an ultrasensitive technique (Quanterix, Simoa, Lexington, MA, USA) and compared with a group of 25 age-matched healthy subjects...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29920851/a-unified-model-of-the-excitability-of-mouse-sensory-and-motor-axons
#11
Preet G S Makker, José M Matamala, Susanna B Park, Justin G Lees, Matthew C Kiernan, David Burke, Gila Moalem-Taylor, James Howells
Non-invasive nerve excitability techniques have provided valuable insight into the understanding of neurological disorders. The widespread use of mice in translational research on peripheral nerve disorders and by pharmaceutical companies during drug development requires valid and reliable models that can be compared to humans. This study established a novel experimental protocol that enables comparative assessment of the excitability properties of motor and sensory axons at the same site in mouse caudal nerve, compared the mouse data to data for motor and sensory axons in human median nerve at the wrist, and constructed a mathematical model of the excitability of mouse axons...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29603827/differences-between-acute-onset-chronic-inflammatory-demyelinating-polyneuropathy-and-acute-inflammatory-demyelinating-polyneuropathy-in-adult-patients
#12
Lucas Alessandro, José M Pastor Rueda, Miguel Wilken, Luis Querol, Mariano Marrodán, Julián N Acosta, Alberto Rivero, Fabio Barroso, Mauricio F Farez
Acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed...
September 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29896895/clinical-and-genetic-investigation-in-chinese-patients-with-demyelinating-charcot-marie-tooth-disease
#13
Jin He, Lingling Guo, Guorong Xu, Liuqing Xu, Shan Lin, Wanjin Chen, Ning Wang
Demyelinating Charcot-Marie-Tooth disease (CMT) is the most common subtype of CMT. It is caused mainly by 17p11.2 heterozygous duplication, but also by mutations in more than 20 genes which affect development and function of Schwann cells. To investigate the profile of genes mutated and clinical features in demyelinating CMT of Chinese descent, we collected a cohort of 44 demyelinating CMT patients and screened them using multiplex ligation-dependent probe amplification (MLPA) and targeted next-generation sequencing (NGS) technology...
June 12, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29906321/clinical-and-genetic-diversities-of-charcot-marie-tooth-disease-with-mfn2-mutations-in-a-large-case-study
#14
(no author information available yet)
No abstract text is available yet for this article.
June 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29799146/robert-r-myers-in-memoriam-april-15-1946-april-19-2018
#15
Veronica I Shubayev, Douglas W Zochodne
No abstract text is available yet for this article.
June 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29696771/risk-factors-for-the-development-of-paclitaxel-induced-neuropathy-in-breast-cancer-patients
#16
Jetter Robertson, Jeffrey Raizer, James S Hodges, William Gradishar, Jeffrey A Allen
Peripheral neuropathy is a common side effect of many chemotherapeutic agents including paclitaxel. We prospectively evaluated demographic and laboratory data in a cohort of 61 woman with breast cancer prior to paclitaxel exposure to explore factors that predispose to neuropathy development. Neuropathy was graded based on the total neuropathy score reduced version (rTNS) at baseline and at 4 months after initiation of chemotherapy. A multivariate analysis identified predictors with the strongest association with a change in rTNS...
June 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29693294/testing-overwork-weakness-in-charcot-marie-tooth-disease-is-it-true-or-false
#17
Valeria Prada, Laura Mori, Susanna Accogli, Matteo Rivarola, Sara Schizzi, Mernhaz Hamedani, Angelo Schenone
The occurrence of the overwork weakness (OW) in Charcot-Marie-tooth (CMT) disease has been debated for a long time. Especially at the hands level, it is still unclear as to whether OW occurs. Contrasting results may relate to the different muscle groups evaluated and the instruments used. We concentrated to the upper limbs (UL). We recruited 120 subjects, 60 CMT patients and 60 normal controls and evaluated the strength of the tripod pinch and of the hand-grip with a dynamometer, the opposition ability with the thumb opposition test (TOT) and applied an innovative instrumental testing of hand function using the sensor engineered glove test (SEGT), which previously demonstrated its sensitiveness to measure severity of hands dysfunction in CMT patients...
June 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29687564/clinical-spectrum-and-quality-of-life-in-patients-with-chronic-polyneuropathy-a-cross-sectional-study
#18
M S Panduranga, Deepti Vibha, Kameshwar Prasad, Achal Kumar Srivastava, Garima Shukla
Chronic polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical sensory motor symptoms and signs. There is paucity of studies on the etiological spectrum of polyneuropathy and its impact on quality of life (QoL). The present cross-sectional study in a referral based tertiary care center in North India found diabetic neuropathy as the commonest cause (25.5%) amongst 212 patients with chronic polyneuropathy. Idiopathic axonal polyneuropathy was present in 14.2% patients...
June 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29635876/intravenous-immunoglobulin-for-maintenance-treatment-of-multifocal-motor-neuropathy-a-multi-center-open-label-52-week-phase-3-trial
#19
Satoshi Kuwabara, Sonoko Misawa, Masahiro Mori, Yuta Iwai, Kazuhide Ochi, Hidekazu Suzuki, Hiroyuki Nodera, Akira Tamaoka, Masahiro Iijima, Tatsushi Toda, Hiroo Yoshikawa, Takashi Kanda, Ko Sakamoto, Susumu Kusunoki, Gen Sobue, Ryuji Kaji
Intravenous immunoglobulin (IVIg) therapy is currently the only established treatment in patients with multifocal motor neuropathy (MMN), and many patients have an IVIg-dependent fluctuation. We aimed to investigate the efficacy and safety of every 3 week IVIg (1.0 g/kg) for 52 weeks. This study was an open-label phase 3 clinical trial, enrolling 13 MMN patients. After an induction IVIg therapy (0.4 g/kg/d for 5 consecutive days), maintenance dose (1.0 g/kg) was given every 3 weeks for 52 weeks. The major outcome measures were the Medical Research Council (MRC) sum score and hand-grip strength at week 52...
June 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29603842/a-randomised-multi-centre-phase-iii-study-of-3-different-doses-of-intravenous-immunoglobulin-10-in-patients-with-chronic-inflammatory-demyelinating-polyradiculoneuropathy-procid-trial-study-design-and-protocol
#20
David R Cornblath, Hans-Peter Hartung, Hans D Katzberg, Ingemar S J Merkies, Pieter A van Doorn
Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) show varying degrees of response to intravenous immunoglobulin (IVIg) therapy. This randomised phase III study in patients with CIDP (ProCID trial) will compare the efficacy and safety of 3 different doses (0.5, 1.0, and 2.0 g/kg) of IVIg 10% (panzyga) administered every 3 weeks for 24 weeks. The primary efficacy endpoint is the rate of treatment response, defined as a decrease in adjusted inflammatory neuropathy cause and treatment disability score of ≥1 point, in the IVIg 1...
June 2018: Journal of the Peripheral Nervous System: JPNS
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