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Issue Information
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - April 5, 2016 Category: Neurology Tags: Issue Information Source Type: research

Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy, a time to start and a time to stop
Abstract Intravenous immunoglobulin (IVIg) is often used as preferred treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Several studies highlighted the short‐term efficacy of IVIg for CIDP yet many patients need maintenance therapy. Notwithstanding the fact IVIg has been used for over 30 years in CIDP, there is only limited evidence to guide dosage and interval during maintenance treatment. The variation in disease course, lack of biomarkers and fear of deterioration after stopping IVIg makes long‐term treatment challenging. Recent studies suggest a proportion of patients receive unnecessar...
Source: Journal of the Peripheral Nervous System - March 31, 2016 Category: Neurology Authors: Max E. Adrichem, Filip Eftimov, Ivo N. van Schaik Tags: REVIEW Source Type: research

Screening for SH3TC2 gene mutations in a series of demyelinating recessive Charcot‐Marie‐Tooth disease (CMT4)
Abstract Charcot‐Marie‐Tooth disease type 4C (CMT4C) is an autosomal recessive (AR) demyelinating neuropathy associated to SH3TC2 mutations, characterized by early onset, spine deformities, and cranial nerve involvement. We screened 43 CMT4 patients (36 index cases) with AR inheritance, demyelinating nerve conductions, and negative testing for PMP22 duplication, GJB1 and MPZ mutations, for SH3TC2 mutations. Twelve patients (11 index cases) had CMT4C as they carried homozygous or compound heterozygous mutations in SH3TC2. We found six mutations: three nonsense (p.R1109*, p.R954*, p.Q892*), one splice‐site (c.805+2T>...
Source: Journal of the Peripheral Nervous System - March 31, 2016 Category: Neurology Authors: Giuseppe Piscosquito, Paola Saveri, Stefania Magri, Claudia Ciano, Claudia Gandioli, Michela Morbin, Daniela Di Bella, Isabella Moroni, Franco Taroni F, Davide Pareyson Tags: RESEARCH REPORT Source Type: research

Severe, reversible nelarabine‐induced neuropathy and myelopathy
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - March 31, 2016 Category: Neurology Authors: Paola Alberti, Matteo Parma, Pietro Pioltelli, Enrico Maria Pogliani, Elisabetta Terruzzi, Alessandra Stasia, Elisa Doni, Caterina Cecchetti, Guido Cavaletti Tags: LETTER TO THE EDITOR 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 m...
Source: Journal of the Peripheral Nervous System - March 31, 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

Assessment scales for the diagnosis of polyneuropathy
Abstract Epidemiological studies that investigate the occurrence and determinants of chronic length‐dependent polyneuropathy are scarce. Population‐based studies on polyneuropathy require a valid and reliable screening protocol with both good sensitivity and specificity. Several questionnaires and scoring scales have been developed for the detection of polyneuropathy, grading the severity of the disease or evaluating the clinical course during follow‐up. This review summarizes the aims and content of existing diagnostic polyneuropathy screening tools in order to help future studies decide which scale to use for scree...
Source: Journal of the Peripheral Nervous System - March 10, 2016 Category: Neurology Authors: Rens Hanewinckel, M. Arfan Ikram, Pieter A. van Doorn Tags: REVIEW Source Type: research

Does ability to walk reflect general functionality in inflammatory neuropathies?
ConclusionOur findings show that assessing only one construct of functionality (e.g. walking ability) does not reflect the full scope of daily/social functional deficits perceived by patients. The ability to walk shows a patient is doing better, but not necessarily doing well. The I‐RODS© bypasses these limitations. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - March 10, 2016 Category: Neurology Authors: Thomas H.P. Draak, Kenneth C. Gorson, Els K. Vanhoutte, Sonja I. van Nes, Pieter.A. van Doorn, David R. Cornblath, Leonard H. van den Berg, Catharina G. Faber, Ingemar S.J. Merkies, Tags: RESEARCH REPORT Source Type: research

Fampridine‐PR (prolonged released 4‐aminopyridine) is not effective in patients with inflammatory demyelination of the peripheral nervous system
Abstract Fampridine‐PR is a voltage‐gated potassium channel inhibitor potentially improving nerve conduction in demyelinated axons. Based on its established clinical efficacy in patients with demyelination in the central nervous system we assessed if fampridine‐PR is also effective in patients with inflammatory demyelination of the peripheral nerve. In this small open‐label study ten patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were treated with fampridine‐PR 10 mg BID for 28 days and assessed clinically as well as by nerve conduction studies. In the present study Fampridine‐...
Source: Journal of the Peripheral Nervous System - March 1, 2016 Category: Neurology Authors: Verena‐Isabell Leussink, Mark Stettner, Clemens Warnke, Hans‐Peter Hartung Tags: RESEARCH REPORT Source Type: research

Erratum
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - March 1, 2016 Category: Neurology Tags: ERRATUM Source Type: research

Semi‐dominant mutations in MFN2‐related neuropathy and implications for genetic counselling
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - March 1, 2016 Category: Neurology Authors: Pedro J. Tomaselli, Alexander M. Rossor, James M. Polke, Roy Poh, Julian Blake, Mary M. Reilly Tags: LETTER TO THE EDITOR Source Type: research

Cerebrospinal fluid profile and seroprevalence of antiganglioside reactivity in patients with neuralgic amyotrophy
Abstract Neuralgic amyotrophy (NA), also known as acute brachial plexitis, is postulated as an autoimmune pathogenesis. In a well‐defined cohort of patients with NA, we analyzed the cerebrospinal fluid (CSF) profile and the prevalence of antiganglioside antibodies. Patients with Varicella zoster‐associated radiculitis and healthy blood donors served as controls. An abnormal routine laboratory CSF profile was found in 29% of those with NA, mostly showing a disruption of the blood–brain barrier. Antibodies predominantly from the immunoglobulin M (IgM) isotype against at least one human ganglioside were detected in 36% ...
Source: Journal of the Peripheral Nervous System - March 1, 2016 Category: Neurology Authors: Oliver Stich, Daniela Glos, Marie Brendle, Rick Dersch, Sebastian Rauer Tags: RESEARCH REPORT Source Type: research

Small and large fiber neuropathy in those with type 1 and type 2 diabetes: a 5‐year follow‐up study
Abstract The purpose of this study was to evaluate progression of diabetic polyneuropathy and differences in the spectrum and evolution of large‐ and small‐fiber involvement in patients with diabetes type 1 and 2 over 5 years. Fifty‐nine patients (35 type 1 and 24 type 2) were included. Nerve conduction studies (NCS), quantitative sensory testing, skin biopsy for quantification of intraepidermal nerve fiber density (IENFD), symptom scoring and clinical evaluations were performed. Z‐scores were calculated to adjust for the physiologic effects of age and height/gender. Neuropathic symptoms were not significantly more...
Source: Journal of the Peripheral Nervous System - March 1, 2016 Category: Neurology Authors: Sissel Løseth, Erik V. Stålberg, Sigurd Lindal, Edel Olsen, Rolf Jorde, Svein I. Mellgren 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 - March 1, 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 - March 1, 2016 Category: Neurology Tags: ISSUE INFORMATION Source Type: research

Guillain‐Barré syndrome in elderly
ConclusionsElderly patients, and especially old‐old patients, with GBS have more severe disease with slower recovery than do younger patients. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - February 16, 2016 Category: Neurology Authors: Stojan Peric, Ivana Berisavac, Olivera Stojiljkovic, Sonja Rajic, Milica Babic, Milan Cvijanovic, Aleksandra Dominovic‐Kovacevic, Ivana Basta, Ljiljana Beslac‐Bumbasirevic, Dragana Lavrnic Tags: RESEARCH REPORT Source Type: research