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.

Cerebrospinal fluid profile and seroprevalence of anti‐ganglioside 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 anti‐ganglioside 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 IgM isotype against at least one human ganglioside were detected in 36% of sera from patie...
Source: Journal of the Peripheral Nervous System - December 1, 2015 Category: Neurology Authors: Oliver Stich, Daniela Glos, Marie Brendle, Rick Dersch, Sebastian Rauer Tags: RESEARCH REPORTS Source Type: research

Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?
DiscussionNCS in CMT1a, HNPP and CIDP reflect demyelination. dCMAP duration, sensory AMNS and a 10 m/s CV difference parameter are most useful in the differential diagnosis of pediatric CIDP. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - December 1, 2015 Category: Neurology Authors: Anna Potulska‐Chromik, Barbara Ryniewicz, Karolina Aragon‐Gawinska, Dagmara Kabzinska, Andrzej Seroka, Marta Lipowska, Anna M. Kaminska, Anna Kostera‐Pruszczyk Tags: RESEARCH REPORTS Source Type: research

Caffeine prevents antihyperalgesic effect of gabapentin in an animal model of CRPS I: evidence for the involvement of spinal adenosine A1 receptor
This study was designed to determine whether 3 weeks of gabapentin treatment is effective in alleviating neuropathic pain‐like behavior in animal models of complex regional pain syndrome type‐I and partial sciatic nerve ligation (PSNL). We investigated the contribution of adenosine subtypes to the antihyperalgesic effect of gabapentin by examining the effect of caffeine, a non‐selective adenosine A1 and A2 receptor antagonist or 1,3‐dipropyl‐8‐cyclopentylxanthine (DPCPX), a selective adenosine A1 subtype receptor antagonist on this effect. Neuropathic pain was produced by unilateral prolonged hind paw ischemia ...
Source: Journal of the Peripheral Nervous System - October 12, 2015 Category: Neurology Authors: Daniel F. Martins, Marcos R. B. Prado, Eduardo Daruge, Ana P. Batisti, Aline A. Emer, Leidiane Mazzardo‐Martins, Adair R. S. Santos, Anna P. Piovezan Tags: RESEARCH REPORT Source Type: research

Impairment measures versus inflammatory RODS in GBS and CIDP: a responsiveness comparison
This study aimed to ‘define responder’ through the concept of minimum clinically important differences using the individually obtained standard errors (MCID‐SE) and a heuristic ‘external criterion’ responsiveness method in patients with Guillain‐Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). One hundred and fourteen newly diagnosed or relapsing patients (GBS: 55, CIDP: 59) were serially examined (1‐year follow‐up). The inflammatory Rasch‐built overall disability scale (I‐RODS), Rasch‐transformed MRC sum score (RT‐MRC), and Rasch‐transformed modified‐I...
Source: Journal of the Peripheral Nervous System - September 28, 2015 Category: Neurology Authors: Els K. Vanhoutte, Thomas H. P. Draak, Kenneth C. Gorson, Sonja I. van Nes, Janneke G. J. Hoeijmakers, W.‐Ludo Van der Pol, Nicolette. C. Notermans, Richard A. Lewis, Eduardo Nobile‐Orazio, Jean‐Marc Léger, Peter Y. K. Van den Bergh, Giuseppe Lauria Tags: RESEARCH REPORT Source Type: research

Comparing the NIS vs. MRC and INCAT sensory scale through Rasch analyses
Abstract We performed a comparison between Neuropathy Impairment Scale‐sensory (NISs) vs. the modified Inflammatory Neuropathy Cause and Treatment sensory scale (mISS), and NIS‐motor vs. the Medical Research Council sum score in patients with Guillain‐Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and IgM monoclonal gammopathy of undetermined significance‐related polyneuropathy (MGUSP). The ordinal data were subjected to Rasch analyses, creating Rasch‐transformed (RT)‐intervals for all measures. Comparison between measures was based on validity/reliability with an emph...
Source: Journal of the Peripheral Nervous System - September 28, 2015 Category: Neurology Authors: Thomas H. P. Draak, Els K. Vanhoutte, Sonja I. van Nes, Kenneth C. Gorson, W.‐Ludo Van der Pol, Nicolette C. Notermans, Eduardo Nobile‐Orazio, Richard A. Lewis, Jean‐Marc Léger, Peter Y. K. Van den Bergh, Giuseppe Lauria, Vera Bril, Hans Katzberg, Tags: RESEARCH REPORT Source Type: research

Grip strength comparison in immune‐mediated neuropathies: Vigorimeter vs. Jamar
In conclusion, validity, reliability, and responsiveness aspects were comparable between the Jamar dynamometer and Vigorimeter. However, based on patients' preference, the Vigorimeter is recommended in future studies in immune‐mediated neuropathies. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 28, 2015 Category: Neurology Authors: Thomas H. P. Draak, Mariëlle H. J. Pruppers, Sonja I. van Nes, Els K. Vanhoutte, Mayienne Bakkers, Kenneth C. Gorson, W.‐Ludo Van der Pol, Richard. A. Lewis, Nicolette C. Notermans, Eduardo Nobile‐Orazio, Jean‐Marc Léger, Peter Y. K. Van den Bergh Tags: RESEARCH REPORT Source Type: research

Rasch‐ionale for neurologists
Abstract Outcome measures are considered the most important tools to monitor patients' outcome in both clinical and research settings. Measuring the clinical state of patients is a fundamental part of our daily clinical practice and research that sometimes is taken for granted. In peripheral neuropathies, there are many scales available, but most of these are at the ordinal level. This paper will systematically address the types of scales available (being nominal, ordinal, interval, or ratio data‐based) in terms of their strengths and weaknesses. The differences between classical test theory‐based and modern test metho...
Source: Journal of the Peripheral Nervous System - September 28, 2015 Category: Neurology Authors: Els K. Vanhoutte, Mieke C. E. Hermans, Catharina G. Faber, Kenneth C. Gorson, Ingemar S. J. Merkies, Jean‐L. Thonnard, Tags: REVIEW Source Type: research

Outcome measures in neuromuscular disease: is the world still flat?
Abstract Valid, responsive, and meaningful outcome measures for the measurement of the impairment, activity limitations, and quality of life in patients with neuromuscular disease are crucial to identify the natural history of disease and benefits of therapy in clinical practice and trials. Although understanding of many aspects of neuromuscular diseases has advanced dramatically, the development of outcome measures has received less attention. The scales developed from Rasch theory by the PeriNomS Group represent the biggest significant shift in thought in neuromuscular outcome measures for decades. There remain problems ...
Source: Journal of the Peripheral Nervous System - September 28, 2015 Category: Neurology Authors: Michael P. Lunn, Peter Y. K. Van den Bergh Tags: REVIEW Source Type: research

Innovative quantitative testing of hand function in Charcot‐Marie‐Tooth neuropathy
ConclusionsThe SEGT test is sensitive to show hand dysfunction in CMT patients, with and without clinically impaired hands. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - September 1, 2015 Category: Neurology Authors: M. A. Alberti, L. Mori, L. Francini, I. Poggi, M. Monti Bragadin, E. Bellone, M. Grandis, G. Maggi, L. Reni, M.P. Sormani, A. Tacchino, L. Padua, V. Prada, M. Bove, A. Schenone Tags: RESEARCH REPORT Source Type: research

Microsurgeons do better –Tactile training might prevent the age‐dependent decline of the sensibility of the hand
ConclusionThis study demonstrates that longterm tactile training might prevent the known age‐dependent decline of the sensibility of the hand. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Daniel Schmauss, Kai Megerle, Andrea Weinzierl, Kariem Agua, Michael Cerny, Verena Schmauss, Joern A. Lohmeyer, Hans‐Guenther Machens, Holger Erne Tags: RESEARCH REPORTS Source Type: research

Mutational mechanisms in MFN2‐related neuropathy: compound heterozygosity for recessive and semidominant mutations
We describe a 49‐year‐old woman with CMT5 associated with compound heterozygosity for two MFN2 variants, one already reported missense mutation (c.748C>T, p.R250W) and a novel nonsense sequence change (c.1426C>T, p.R476*). Her mother, carrying the p.R250W variant, had very late‐onset minimal axonal neuropathy, whilst the father harboring the nonsense sequence change had neither clinical nor electrophysiological neuropathy. The missense mutation is likely pathogenic according to in silico analyses and a previous report, while the nonsense variant is predicted to behave as a null allele. The p.R250W variant behav...
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Giuseppe Piscosquito, Paola Saveri, Stefania Magri, Claudia Ciano, Daniela Di Bella, Micaela Milani, Franco Taroni, Davide Pareyson Tags: RESEARCH REPORTS Source Type: research

Pathological, biochemical, and biophysical characteristics of the transthyretin variant Y114H (p.Y134H) explain its very mild clinical phenotype
Abstract Transthyretin (TTR) is a homotetrameric protein that must misfold in order to form amyloid fibrils. Misfolding includes rate limiting tetramer dissociation, followed by fast tertiary structural changes of the monomer that enable aggregation. Hereditary ATTR amyloidosis is an autosomal dominant genetic disorder with systemic deposition of amyloid fibrils induced by TTR gene mutation. We identified a rare Y114H (p.Y134H) TTR variant in a Japanese patient presenting with late‐onset, very mild clinical course. The patient had an extremely low serum variant TTR concentration (18% of total TTR), whereas the compositio...
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Yoshiki Sekijima, Raúl I. Campos, Per Hammarström, K. Peter. R. Nilsson, Tsuneaki Yoshinaga, Kiyoshiro Nagamatsu, Masahide Yazaki, Fuyuki Kametani, Shu‐ichi Ikeda Tags: RESEARCH REPORTS Source Type: research

Epidermal innervation morphometry by immunofluorescence and bright‐field microscopy
Abstract We investigated the agreement between simple indirect immunofluorescence (IF) and bright‐field immunohistochemistry (BFI) on free‐floating sections for intraepidermal nerve fiber density (IENFD) quantification. Fifty‐five healthy subjects and 63 patients with probable small fiber neuropathy (SFN) underwent two adjacent skin biopsies at the distal leg processed by IF and BFI technique. Agreement between IENFD pairs obtained by each method was assessed by Bland‐Altman testing. The area under the curve of the receiving operating characteristics (ROC) curves was used to compare the discrimination ability. The ...
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Maria Nolano, Antonella Biasiotta, Raffaella Lombardi, Vincenzo Provitera, Annamaria Stancanelli, Giuseppe Caporaso, Lucio Santoro, Ingemar S.J. Merkies, Andrea Truini, Carla Porretta‐Serapiglia, Daniele Cazzato, Patrizia Dacci, Dino F. Vitale, Giuseppe Tags: RESEARCH REPORTS Source Type: research

Early onset Charcot‐Marie‐Tooth neuropathy type 2A and severe developmental delay: expanding the clinical phenotype of MFN2‐related neuropathy
We report a male child with psychomotor delay, dysmorphic features and weakness of lower limbs associated with electrophysiological features of severe, sensory‐motor, axonal neuropathy. The patient was diagnosed with early onset CMT2A and severe psychomotor retardation associated to c.310C>T mutation (p.R104W) in MFN2 gene. CMT2A should be considered in patients with both axonal sensory‐motor neuropathy and developmental delay. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Maria Tufano, Gerarda Cappuccio, Gaetano Terrone, Fiore Manganelli, Chiara Pisciotta, Alessandro Geroldi, Simona Capponi, Ennio Del Giudice Tags: CASE REPORT Source Type: research

Future needs in peripheral neuropathy outcome measures
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Peter YK Van den Bergh, Michael P Lunn Tags: EDITORIAL Source Type: research