Journal of the Peripheral Nervous System This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Abstracts
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 17, 2017 Category: Neurology Tags: ABSTRACTS Source Type: research

Issue Information
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 17, 2017 Category: Neurology Tags: ISSUE INFORMATION Source Type: research

International Guillain ‐Barré Syndrome Outcome Study (IGOS):Protocol of a prospective observational cohort study on clinical and biological predictors of disease course and outcome in Guillain‐Barré syndrome
Abstract 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. Patients fulfilling the diagnostic criteria for GBS, regardless of...
Source: Journal of the Peripheral Nervous System - April 12, 2017 Category: Neurology Authors: 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, Tags: RESEARCH REPORTS Source Type: research

Nodes of Ranvier in skin biopsies of patients with diabetes mellitus
Abstract Paranodal demyelination has been discussed as a potential mechanism of nerve fiber damage in diabetic neuropathy. Studies on human tissue are limited, as nerve biopsies are invasive and only rarely performed in patients with confirmed diabetic neuropathy. Skin biopsy has recently been suggested as a tool to analyze paranodal and nodal changes of myelinated fibers. We analyzed the myelinated fibers of skin biopsies of 35 patients with diabetic neuropathy, 17 patients with diabetes mellitus without neuropathy, and 30 normal controls. Immunofluorescence of skin sections with antibodies against Caspr, neurofascin, sod...
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Kathrin Doppler, Franziska Frank, Ann ‐Cathrin Koschker, Karlheinz Reiners, Claudia Sommer Tags: RESEARCH REPORTS Source Type: research

Axonal Charcot ‐Marie‐Tooth neuropathy concurrent with distal and proximal weakness by translational elongation of the 3’ UTR in NEFH
This study also identified a de novo c.3015_3027dup frameshift mutation predicting p.Lys1010Glnfs*57 in NEFH from a CMT2 family with an atypical clinical symptom of prominent proximal weakness. This mutation is located near the previously reported frameshift mutations, suggesting a mutational hotspot. Lower limb MRI revealed marked hyperintense signal changes in the thigh muscles compared to those in the calf muscles. Therefore, this study suggests that the stop loss and translational elongations by the 3’ UTR of the NEFH mutations may be a relatively frequent genetic cause of axonal peripheral neuropathy with the specif...
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Da Eun Nam, Sung ‐Chul Jung, Da Hye Yoo, Sun Seong Choi, Sung‐Yum Seo, Gwang Hoon Kim, Song‐Ja Kim, Soo Hyun Nam, Byung‐Ok Choi, Ki Wha Chung Tags: CASE REPORT Source Type: research

Waardenburg syndrome: a rare cause of inherited neuropathy due to SOX10 mutation
Abstract Waardenburg syndrome (WS) is a rare disorder comprising sensorineural deafness and pigmentation abnormalities. Four distinct subtypes are defined based on the presence or absence of additional symptoms. Mutations in six genes have been described in WS. SOX10 mutations are usually associated with a more severe phenotype of WS with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy and Hirschsprung disease. Here we report a 32 year‐old man with a novel heterozygous missense variant in SOX10 gene, who presented with congenital deafness, Hirschsprung disease, iris heterochromia, foot deformit...
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Petya Bogdanova ‐Mihaylova, Michael D Alexander, Raymond PJ Murphy, Sinéad M Murphy Tags: CASE REPORT Source Type: research

Deletion of Sarm1 gene is neuroprotective in two models of peripheral neuropathy
This study extends the role of Sarm1 to axon degeneration seen in peripheral neuropathies and identifies it as a likely target for therapeutic development. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Elliot Turkiew, Debbie Falconer, Nicole Reed, Ahmet H öke Tags: RESEARCH REPORTS Source Type: research

Drug ‐induced cutaneous lupus erythematosus after immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a case series
We describe six patients with cutaneous lupus erythematosus (cLE) during immunoglobulin G (IgG) treatment. Five patients were diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) and one patient with possible CIDP. Five patients received intravenous immunoglobulin (IVIg) and one patient subcutaneous immunoglobulin (SCIg). Skin lesions were systematically assessed by a dermatologist including skin biopsies. Patients showed disseminated erythematous plaques on several parts of the body with predominance of the chest and face. Skin biopsies showed perivascular and perifollicular vacuolar inflammation, consi...
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Max E Adrichem, Markus V Starink, Ester MM van Leeuwen, Christine Kramer, Ivo N van Schaik, Filip Eftimov Tags: CASE REPORT Source Type: research

Clinical characterization and genetic analysis of Korean patients with X ‐linked Charcot‐Marie‐Tooth disease type 1
This study provides a comprehensive summary of the clinical features and spectrum of GJB1 gene mutations in Korean CMTX1 patients. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Young Bin Hong, Jin ‐Mo Park, Jin Seok Yu, Da Hye Yoo, Da Eun Nam, Hyung Jun Park, Ji‐Su Lee, Sun Hee Hwang, Ki Wha Chung, Byung‐Ok Choi Tags: RESEARCH REPORTS Source Type: research

High mortality from Guillain ‐Barré syndrome in Bangladesh
Abstract Although Guillain‐Barré syndrome (GBS) has higher incidence and poor outcome in Bangladesh, mortality from GBS in Bangladesh has never been explored before. We sought to explore the frequency, timing and risk factors for deaths from GBS in Bangladesh. We conducted a prospective study on 407 GBS patients who were admitted to Dhaka Medical College Hospital, Dhaka, Bangladesh from 2010 to 2013. We compared deceased and alive patients to identify risk factors. Cox regression model was used to adjust for confounders. Out of 407 GBS patients, 50 (12%) died, with the median time interval between the onset of weakness ...
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Tanveen Ishaque, Mohammad B. Islam, Gulshan Ara, Hubert P. Endtz, Quazi D. Mohammad, Bart C. Jacobs, Zhahirul Islam Tags: RESEARCH REPORTS Source Type: research

Neuroprotective effect of epidermal growth factor in experimental acrylamide neuropathy: an electrophysiological approach
Abstract 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 p...
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: H Perez ‐Saad, N Subiros, J Berlanga, L Aldana, D Garcia del Barco Tags: RESEARCH REPORTS Source Type: research

Motor nerve excitability after childhood Guillain ‐Barré syndrome
ConclusionGBS in childhood results in residual motor nerve excitability disturbances, even in those completely recovered, probably reflecting altered physiology of regenerated peripheral nerves. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Judith Drenthen, Joyce Roodbol, Ellen M. Maathuis, Coriene E. Catsman ‐Berrevoets, Joleen H. Blok, Marie‐Claire Y. de Wit, Bart C. Jacobs Tags: RESEARCH REPORTS Source Type: research

A device to measure secretion of individual sweat glands for diagnosis of peripheral neuropathy
DiscussionThe device provides reliable, precise quantitative measures of sweat secretion from single SGs for characterization of sudomotor nerve function in healthy control subjects and in subjects with known peripheral neuropathy. The test combines the capabilities of existing tests of sudomotor function while providing additional capabilities. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 1, 2017 Category: Neurology Authors: Adam J. Loavenbruck, James S. Hodges, Vincenzo Provitera, Maria Nolano, Gwen Wendelshafer ‐Crabb, William R. Kennedy Tags: RESEARCH REPORTS Source Type: research

Issue Information
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - March 13, 2017 Category: Neurology Tags: ISSUE INFORMATION Source Type: research

The six spot step test – a new method for monitoring walking ability in patients with chronic inflammatory polyneuropathy
ConclusionThe SSST may be superior to the T25FW in terms of dynamic range, floor effect and responsiveness which makes the SSST a possible alternative for monitoring walking ability in patients with chronic inflammatory polyneuropathy. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - March 1, 2017 Category: Neurology Authors: Melissa Kreutzfeldt, Henrik B. Jensen, Mads Ravnborg, Lars H. Markvardsen, Henning Andersen, S øren H. Sindrup Tags: RESEARCH REPORTS Source Type: research