Early discrimination of sensorimotor Guillain ‐Barré syndrome into demyelinating or axonal subtype by automated nerve excitability testing
Abstract 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. Automate...
Source: Journal of the Peripheral Nervous System - March 1, 2017 Category: Neurology Authors: So Young Pyun, Mi ‐Ri Kang, Joo Young Lee, Kim Jong Kuk, Seong‐Il Oh, Jong Seok Bae Tags: RESEARCH REPORTS Source Type: research

Novel NTRK1 mutations associated with congenital insensitivity to pain with anhidrosis verified by functional studies
ABSTRACT 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 ...
Source: Journal of the Peripheral Nervous System - February 7, 2017 Category: Neurology Authors: Tai ‐Seung Nam, Wenting Li, Somy Yoon, Gwang Hyeon Eom, Myeong‐Kyu Kim, Sung Taek Jung, Seok‐Yong Choi Tags: RESEARCH REPORTS Source Type: research

A novel NDRG1 mutation in a non ‐Romani patient with CMT4D/HMSN‐Lom
We report a 38‐year‐old Italian female with motor development delay, progressive neuropathy and sensorineural deafness. MRI showed slight atrophy of cerebellum, medulla oblongata and upper cervical spinal cord. She had a novel homozygous NDRG1 frameshift mutation (c.739delC; p.His247ThrfsTer74). The identification of this NDRG1 mutation confirms that CMT4D is not a private Romani disease and should be considered in the differential diagnosis of recessive demyelinating CMT. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - December 15, 2016 Category: Neurology Authors: Giuseppe Piscosquito, Stefania Magri, Paola Saveri, Micaela Milani, Claudia Ciano, Laura Farina, Franco Taroni, Davide Pareyson Tags: RESEARCH REPORTS Source Type: research

Histopathological features of a patient with Charcot ‐Marie‐Tooth disease type 2U/AD‐CMTax‐MARS
We report the detailed histopathological findings in a patient with CMT2U/AD‐CMTax‐MARS. The findings are similar to those found in CMT2D caused by mutations in the GARS gene, encoding glycyl‐tRNA synthetase. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - December 6, 2016 Category: Neurology Authors: Makito Hirano, Nobuyuki Oka, Akihiro Hashiguchi, Shuichi Ueno, Hikaru Sakamoto, Hiroshi Takashima, Yujiro Higuchi, Susumu Kusunoki, Yusaku Nakamura Tags: CASE REPORT Source Type: research

Issue Information – Table of Contents
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - December 6, 2016 Category: Neurology Tags: ISSUE INFORMATION Source Type: research

Issue Information – Editorial Board
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - December 6, 2016 Category: Neurology Tags: ISSUE INFORMATION Source Type: research

Diabetes mellitus may affect short ‐term outcome of Guillain‐Barré syndrome
Abstract 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=2.0, P < 0.05). Linear regression analysis with multiple factors included showed that...
Source: Journal of the Peripheral Nervous System - November 30, 2016 Category: Neurology Authors: Stojan Peric, Ivo Bozovic, Bogdan Bjelica, Ivana Berisavac, Olivera Stojiljkovic, Ivana Basta, Ljiljana Beslac ‐Bumbasirevic, Vidosava Rakocevic‐Stojanovic, Dragana Lavrnic, Zorica Stevic Tags: RESEARCH REPORTS Source Type: research

Guillain ‐Barré syndrome in France: a nationwide epidemiological analysis based on hospital discharge data (2008–2013)
Abstract 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 (IR) based on a French nationwide hospital discharge database. All patients hospitalized for GBS between 2008 and 2013 were identified by ICD‐10 code G61.0 as principal diagnosis. Patients previously hospitalized for GBS in 2006 and 2007 were excluded. Sensitivity analyses were performed by considering alternative case definitions, based on more restri...
Source: Journal of the Peripheral Nervous System - November 30, 2016 Category: Neurology Authors: Alexandra Delannoy, J érémie Rudant, Christophe Chaignot, Francis Bolgert, Yann Mikaeloff, Alain Weill Tags: RESEARCH REPORTS Source Type: research

Hereditary gelsolin amyloidosis (HGA): a neglected cause of bilateral progressive or recurrent facial palsy
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 ...
Source: Journal of the Peripheral Nervous System - November 30, 2016 Category: Neurology Authors: Anna Sagnelli, Giuseppe Piscosquito, Daniela Di Bella, Laura Fadda, Lisa Melzi, Antonio Morico, Claudia Ciano, Franco Taroni, Dante Facchetti, Ettore Salsano, Davide Pareyson Tags: CASE REPORT Source Type: research

Modulation of diet ‐induced mechanical allodynia by metabolic parameters and inflammation
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. Proteins involved in the synaptosomal complex and pain associated TRP ion channels decrease in the dorsal root ganglion of high‐fat exercise animals relative to their sedentary controls. Exercise reversed high‐fat diet induced mechanical allodynia without affecting weight gain, elevated blood glucose, and utilization of fat a...
Source: Journal of the Peripheral Nervous System - November 30, 2016 Category: Neurology Authors: Michael A. Cooper, Janelle M. Ryals, Pau Yen Wu, Kellen D. Wright, Katherine R. Walter, Douglas E. Wright Tags: RESEARCH REPORTS Source Type: research

Top ‐100 cited articles on Guillain‐Barré syndrome: a bibliometric analysis
Abstract Since the first description of Guillain‐Barré syndrome (GBS) 100 years ago, the concept of this syndrome has changed remarkably. The purpose of our study was to identify and characterize the most‐cited articles that have contributed to advancing the understanding of GBS. Based on the database of Journal Citation Reports, we selected 554 journals that were considered as potential sources of reports on studies related to clinical neurology and general medicine. The Web of Science search tools were used to identify the most‐cited articles relevant to GBS or other variants in the selected journals. Of the selec...
Source: Journal of the Peripheral Nervous System - September 11, 2016 Category: Neurology Authors: Jee ‐Eun Kim, Jong Kuk Kim, Kang Min Park, Yerim Kim, Dae Young Yoon, Jong Seok Bae Tags: RESEARCH REPORTS Source Type: research

6th International Charcot ‐Marie‐Tooth and Related Neuropathy Consortium (CMTR) Meeting
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 3, 2016 Category: Neurology Tags: PROCEEDINGS Source Type: research

2016 Inflammatory Neuropathy Consortium and ‘GBS 100’ Centenary Symposium and Ceilidh
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 3, 2016 Category: Neurology Tags: PROCEEDINGS Source Type: research

Identification of fluocinolone acetonide to prevent paclitaxel ‐induced peripheral neuropathy
Abstract Paclitaxel (PTX) is among the most commonly used cancer drugs that cause chemotherapy‐induced peripheral neuropathy (CIPN), a debilitating and serious dose‐limiting side effect. Currently, no drugs exist to prevent CIPN, and symptomatic therapy is often ineffective. In order to identify therapeutic candidates to prevent axonal degeneration induced by PTX, we carried out a phenotypic drug screening using primary rodent dorsal root ganglion sensory neurons. We identified fluocinolone acetonide as a neuroprotective compound and verified it through secondary screens. Furthermore, we showed its efficacy in a mouse ...
Source: Journal of the Peripheral Nervous System - September 3, 2016 Category: Neurology Authors: Aysel Cetinkaya ‐Fisgin, Min Geol Joo, Xiang Ping, Nitish V. Thakor, Cengizhan Ozturk, Ahmet Hoke, In Hong Yang Tags: RESEARCH REPORT Source Type: research

Issue Information – Table of Contents
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 3, 2016 Category: Neurology Tags: ISSUE INFORMATION Source Type: research