Intraneural perineuriomas: diagnostic value of magnetic resonance neurography
Abstract Intraneural perineurioma is an under‐recognized hypertrophic peripheral nerve tumor. It affects young patients involving frequently the sciatic nerve and its branches and presents with a progressive, painless and predominantly motor deficit. Magnetic resonance neurography is a useful tool to localize the lesion, evaluate its extension, and discriminate between different etiologies. We reviewed the clinical records of 11 patients with pathologically confirm intraneural perineurioma. Eight patients were males with mean age 19 years. Initial complains were unilateral stepagge (7 patients), bilateral stepagge (1 pat...
Source: Journal of the Peripheral Nervous System - November 2, 2017 Category: Neurology Authors: L Le ón Cejas, D Binaghi, M Socolovsky, A Dubrovsky, L Pirra, C Marchesoni, A Pardal, S Monges, G Peretti, A Taratuto, F Lubinieki, R Reisin Tags: RESEARCH REPORTS Source Type: research

Early electrophysiological findings in acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain ‐Barre syndrome in the Pakistani population‐ A comparison with global data
Abstract Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) are the most common variants of Guillian Barre syndrome documented in the Asian population. However the variability of early neurophysiologic findings in the Asian population compared to western data has not been documented. Eighty seven cases of AIDP were retrospectively reviewed for their demographic, clinical, electrophysiological and laboratory data. Mean age of subjects was 31 ± 8 years with males more commonly affected. Motor symptoms (97%) at presentation predominated. Common early nerve conduction findi...
Source: Journal of the Peripheral Nervous System - November 1, 2017 Category: Neurology Authors: Ahmed Wali, Sara Khan Tags: RESEARCH REPORTS Source Type: research

“Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical aspects and new animal models of auto‐immunity to nodal components”
Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and treatable disorder of the peripheral nerves with clinical and immunological heterogeneity. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - October 24, 2017 Category: Neurology Authors: Isabel Illa Tags: RESEARCH REPORT Source Type: research

Issue Information
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 5, 2017 Category: Neurology Tags: ISSUE INFORMATION Source Type: research

Changes in Intraepidermal Nerve Fiber and Langerhans Cell Densities in the Plantar Skin of Rats after Mercuric Chloride Exposure
ConclusionsThe present study demonstrated a decrease in IENFD and an increase in LCs density in the plantar skin of rats after HgCl2 poisoning, indicating that damage of the small nerve fibers occurs after mercury poisoning. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 1, 2017 Category: Neurology Authors: Binbin Sun, Shuangyi Fan, Kai Yao, Yifan Li, Xusheng Huang Tags: RESEARCH REPORTS Source Type: research

Protocol of a dose response trial of IV immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy (DRIP study)
Abstract High peak levels of serum IgG may not be needed for maintenance treatment of CIDP with IVIg. More frequent dosing of IVIg leads to more stable IgG levels and higher trough levels which may be related with improved clinical efficacy. More frequent lower dosing leads to lower peak levels and may induce less systemic side‐effects. The DRIP study is a double‐blind randomized controlled cross‐over intervention study. CIDP patients ≥ 18 years old, proven IVIg dependent and receiving an individually established but stable maintenance dose and interval of IVIg (Kiovig) can be included. One group (A) will be treate...
Source: Journal of the Peripheral Nervous System - September 1, 2017 Category: Neurology Authors: K. Kuitwaard, W.J.R. Fokkink, E Brusse, A.F.J.E. Vrancken, F. Eftimov, N.C. Notermans, A.J. van der Kooi, I.S.J. Merkies, B.C. Jacobs, P.A. van Doorn Tags: RESEARCH REPORTS Source Type: research

Epidemiology of Guillain ‐Barré syndrome in Finland 2004‐2014
Abstract At total mean incidence of 0.84‐1.1/100,000 the occurrence of Guillain‐Barré syndrome (GBS) is reported to be low in Finland compared to other Caucasian populations. However, a recent study from Southwestern Finland reported an incidence of 1.82/100,000 which is comparable to other Caucasian populations. We analyzed discharge data covering the years 2004 through 2014 on all neurological admissions in all Finnish university and central hospitals with a primary diagnosis of GBS. 989 admissions due to GBS (917 individuals) were identified. The standardized (European population) annual incidence rate was 1.70/100...
Source: Journal of the Peripheral Nervous System - September 1, 2017 Category: Neurology Authors: Jussi Sipil ä, Merja Soilu‐Hänninen, Jori O. Ruuskanen, Päivi Rautava, Ville Kytö Tags: RESEARCH REPORTS Source Type: research

Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy
Abstract CIDP patients treated with intravenous immunoglobulin (IVIg) usually start with a standard dosage of 2 g/kg body weight. Only a minority of patients have a sustained improvement, and most require ongoing maintenance treatment. Preferred IVIg regimens however vary considerably between doctors and at present it is unknown which is optimal. Since there are also large differences in IVIg dosage and interval requirements between patients, optimal IVIg maintenance treatment of CIDP is even more complex. The lack of evidence based guidelines on how IVIg maintenance treatment should be administered may potentially lead to...
Source: Journal of the Peripheral Nervous System - September 1, 2017 Category: Neurology Authors: K. Kuitwaard, W.J.R. Fokkink, E Brusse, A.F.J.E. Vrancken, F. Eftimov, N.C. Notermans, A.J. van der Kooi, I.S.J. Merkies, B.C. Jacobs, P.A. van Doorn Tags: REVIEW Source Type: research

Prediction of disease progression in Miller Fisher and overlap syndromes
ConclusionNo early predictors for progression from MFS to MFS‐GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least one week. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 1, 2017 Category: Neurology Authors: Christine Verboon, Heleen van Berghem, Pieter A. van Doorn, Liselotte Ruts, Bart C. Jacobs Tags: RESEARCH REPORT Source Type: research

The role of nutrition as risk factor for polyneuropathy: a case ‐control study
Abstract The aim of this case–control study is to investigate the role of nutrition as risk factor for polyneuropathy. Three hundred eighteen patients with chronic idiopathic axonal polyneuropathy and 636 matched controls completed a validated food frequency questionnaire that covered nutrient intake and alcohol consumption. As risk estimates, we calculated adjusted odds ratios for the intake of energy and nutrients. Energy and nutrient intake did not differ between patients and controls, regardless of moderate alcohol consumption. Nutrition is not a risk factor for chronic idiopathic axonal polyneuropathy. (Source: Jour...
Source: Journal of the Peripheral Nervous System - August 31, 2017 Category: Neurology Authors: Nora A. Visser, Nicolette C. Notermans, Jeanne H. M. de Vries, Leonard H. van den Berg, Alexander F. J. E. Vrancken Tags: RESEARCH REPORT Source Type: research

Clinical and genetic diversities of Charcot ‐Marie‐Tooth disease with MFN2 mutations in a large case study
We describe the clinical and mutational spectra of CMT patients harboring MFN2 mutations in Japan. We analyzed 1,334 unrelated patients with clinically suspected CMT referred by neurological and neuropediatric departments throughout Japan. We conducted mutation screening using a DNA microarray, targeted resequencing, and whole‐exome sequencing. We identified pathogenic or likely pathogenic MFN2 variants from 79 CMT patients, comprising 44 heterozygous and 1 compound heterozygous variants. A total of 15 novel variants were detected. An autosomal dominant family history was determined in 43 cases, and the remaining 36 case...
Source: Journal of the Peripheral Nervous System - July 30, 2017 Category: Neurology Authors: Masahiro Ando, Akihiro Hashiguchi, Yuji Okamoto, Akiko Yoshimura, Yu Hiramatsu, Junhui Yuan, Yujiro Higuchi, Jun Mitsui, Hiroyuki Ishiura, Ayako Umemura, Koichi Maruyama, Takeshi Matsushige, Shinichi Morishita, Masanori Nakagawa, Shoji Tsuji, Hiroshi Taka Tags: RESEARCH REPORT Source Type: research

2017 Peripheral Nerve Society Meeting July 8 –12, 2017 Sitges, Barcelona, Spain
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - June 22, 2017 Category: Neurology Tags: PROCEEDINGS Source Type: research

Minimum clinically important difference analysis confirms the efficacy of IgPro10 in CIDP: the PRIMA trial
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - June 8, 2017 Category: Neurology Authors: Ingemar S. J. Merkies, John ‐Philip Lawo, Jonathan M. Edelman, Jan L. De Bleecker, Claudia Sommer, Wim Robberecht, Mika Saarela, Jerzy Kamienowski, Zbigniew Stelmasiak, Orell Mielke, Björn Tackenberg, Jean‐Marc Léger, Tags: LETTER TO THE EDITOR Source Type: research

The six ‐spot‐step test – a new method for monitoring walking ability in patients with chronic inflammatory polyneuropathy
Abstract The aim of this study was 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). In the SSST, participants have to walk as quickly as possible across a field measuring 1 × 5 m, while kicking blocks out of five circles on the floor. Sixty‐two patients and 61 controls performed the SSST and T25FW. Patients also performed the overall disability sumscore, INCAT sensory sumscore, Medical Research Council sumscore, and 9‐hole‐peg‐test. Twenty‐one patients treated...
Source: Journal of the Peripheral Nervous System - June 8, 2017 Category: Neurology Authors: Melissa Kreutzfeldt, Henrik B. Jensen, Mads Ravnborg, Lars H. Markvardsen, Henning Andersen, S øren H. Sindrup Tags: RESEARCH REPORT Source Type: research

International Guillain ‐Barré Syndrome Outcome Study: 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, multicenter 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 ag...
Source: Journal of the Peripheral Nervous System - June 8, 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 REPORT Source Type: research