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.

Rasch‐transformed Total Neuropathy Score clinical version (RT‐TNSc©) in patients with chemotherapy‐induced peripheral neuropathy (CIPN)
This study aims to improve impairment assessment in patients with chemotherapy‐induced peripheral neuropathy (CIPN) by subjecting TNSc© records to Rasch analyses. In particular, we wanted to investigate the influence of factors affecting the use of the TNSc© in clinical practice. TNSc© has 7 domains [sensory, motor, autonomic, pin‐prick, vibration, strength, and deep tendon reflexes (DTR)] each being scored 0‐4. Data obtained in 281 patients with stable CIPN were subjected to Rasch analyses to determine the fit to the model. The TNSc© did not meet Rasch model's expectations primarily due to misfit statistics in a...
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: D. Binda, G. Cavaletti, D. R. Cornblath, I.S.J. Merkies, Tags: RESEARCH REPORTS Source Type: research

Anti‐TNFalpha medications and neuropathy
Abstract We studied the clinical, electrophysiological and pathological features, outcome and frequency of anti‐TNF alpha (a‐TNF) medications induced neuropathies (ATIN) in patients with inflammatory disorders. Of 2017 patients treated with a‐TNF medication, 12 patients met our inclusion criteria for a prevalence of 0.60% and an incidence of 0.4 cases per 1000 person‐years. The median time from a‐TNF medication treatment to ATIN was 16.8 months (range 2 to 60 months). Six patients had focal or multifocal peripheral neuropathies. The other six had generalized neuropathies. For all, a‐TNF medication was stopped....
Source: Journal of the Peripheral Nervous System - August 26, 2015 Category: Neurology Authors: Pinelopi Tsouni, Olivier Bill, André Truffert, Christelle Liaudat, François Ochsner, Andreas J. Steck, Thierry Kuntzer Tags: RESEARCH REPORTS Source Type: research

2015 Peripheral Nerve Society Biennial Meeting
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - August 21, 2015 Category: Neurology Tags: PROCEEDINGS Source Type: research

SCN9A‐associated congenital insensitivity to pain and anosmia in an Irish patient
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - August 21, 2015 Category: Neurology Authors: Petya Bogdanova‐Mihaylova, Michael D. Alexander, Raymond P. J. Murphy, Sinéad M. Murphy Tags: LETTER TO THE EDITOR Source Type: research

MFN2 deletion of exons 7 and 8: founder mutation in the UK population
Abstract Mitofusin 2 (MFN2) mutations are the most common cause of axonal Charcot‐Marie‐Tooth disease (CMT2). The majority are inherited in an autosomal dominant manner but recessive and semi‐dominant kindreds have also been described. We previously reported a deletion of exon 7 and 8 resulting in nonsense mediated decay, segregating with disease when present in trans with another pathogenic MFN2 mutation. Detailed clinical and electrophysiological data on a series of five affected patients from four kindreds and, when available, their parents and relatives was collected. MFN2 Sanger sequencing, multiplex ligation pr...
Source: Journal of the Peripheral Nervous System - June 26, 2015 Category: Neurology Authors: Aisling S Carr, James M Polke, Jacob Wilson, Ana L Pelayo‐Negro, Matilde Laura, Tina Nanji, James Holt, Jennifer Vaughan, Julia Rankin, Mary G Sweeney, Julian Blake, Henry Houlden, Mary M Reilly Tags: RESEARCH REPORT Source Type: research

Grip strength comparison in immune‐mediated neuropathies: Vigorimeter versus 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 - June 26, 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, Nicolette C. Notermans, Eduardo Nobile‐Orazio, Jean‐Marc Léger, Peter Y.K. Van den Bergh, Giuseppe Lauria, Vera Tags: RESEARCH REPORT Source Type: research

Outcome measures in neuromuscular disease: Is the world still flat?
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - June 26, 2015 Category: Neurology Authors: Michael P Lunn, Peter Van den Bergh Tags: REVIEW Source Type: research

Severe childhood Guillain‐Barré syndrome associated with Mycoplasma pneumoniae infection: a case series
We report 7 children with recent M. pneumoniae infection and severe GBS that presented to two European medical centers from 1992–2012. Severe GBS was defined as the occurrence of respiratory failure, central nervous system (CNS) involvement, or death. Five children had GBS, one Bickerstaff brain stem encephalitis (BBE), and one acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP). The 5 patients with severe GBS were derived from an original cohort of 66 children with GBS. In this cohort, 17 children (26%) had a severe form of GBS and 47% of patients with M. pneumoniae infection presented with severe...
Source: Journal of the Peripheral Nervous System - June 26, 2015 Category: Neurology Authors: Patrick M. Meyer Sauteur, Joyce Roodbol, Annette Hackenberg, Marie‐Claire Y. de Wit, Cornelis Vink, Christoph Berger, Enno Jacobs, Annemarie M.C. van Rossum, Bart C. Jacobs Tags: REVIEW 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 modifi...
Source: Journal of the Peripheral Nervous System - June 26, 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, Eduardo Nobile‐Orazio, Jean‐Marc Léger, Peter Y.K. Van den Bergh, Giuseppe Lauria, Vera Bril, Hans Katzb Tags: RESEARCH REPORT Source Type: research

The Val30Met Familial Amyloid Polyneuropathy specific Rasch‐built Overall Disability Scale (FAP‐RODS©)
In conclusion, the 34‐item FAP‐RODS© is a disease‐specific interval measure suitable for detecting activity and participation restrictions in patients with FAP. The use of the FAP‐RODS© is recommended for future international clinical trials in patients with Val30Met FAP determining its responsiveness and its cross‐cultural validation. Its expansion to other forms of FAP should also be focus of future clinical studies. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - June 25, 2015 Category: Neurology Authors: Mariëlle H.J. Pruppers, Ingemar S.J. Merkies, Catharina G. Faber, Ana M. Da Silva, Vanessa Costa, Teresa Coelho Tags: RESEARCH REPORT Source Type: research

Outcome measures in MMN revisited: further improvement needed
The objectives of the current study were to provide an overview of the outcome measures (OMs) applied in clinical trials in MMN and to determine the responsiveness of a core set of selected OMs as part of the PeriNomS study. The following OMs were serially applied in 26 patients with newly diagnosed or relapsing MMN, receiving intravenous‐immunoglobulin (assessments: T0/T3/T12 months): 14 muscle‐pairs MRC scale, the Neuropathy‐Impairment‐Scale motor‐subset, a self‐evaluation scale, grip strength, and MMN‐RODS©. All data, except the grip strength, were subjected to Rasch analyses before determining responsive...
Source: Journal of the Peripheral Nervous System - June 25, 2015 Category: Neurology Authors: Mariëlle H.J. Pruppers, Thomas H.P. Draak, Els K. Vanhoutte, W. Ludo Van der Pol, Kenneth C. Gorson, Jean‐Marc Léger, Eduardo Nobile‐Orazio, Leonard H. van den Berg, Catharina G. Faber, Ingemar S.J. Merkies, Tags: RESEARCH REPORT Source Type: research

Mutational analysis of Greek patients with suspected hereditary neuropathy with liability to pressure palsies (HNPP): a 15‐year experience
In conclusion, the common HNPP deletion accounts for ~ 50% and PMP22 micromutations for ~ 2% of cases in a large consecutive cohort of patients with suspected HNPP. The mutational and phenotypic spectrum of HNPP is similar in the Greek population compared to other populations. Proposed electrophysiological diagnostic criteria perform satisfactorily in every day clinical practice. (Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - June 25, 2015 Category: Neurology Authors: Georgia Karadima, Georgios Koutsis, Maria Raftopoulou, Karolina‐Maria Karletidi, Thomas Zambelis, Nikolaos Karandreas, Marios Panas Tags: RESEARCH REPORT Source Type: research

Transthyretin Asp38Tyr: a new mutation associated to a late onset neuropathy
(Source: Journal of the Peripheral Nervous System)
Source: Journal of the Peripheral Nervous System - June 15, 2015 Category: Neurology Authors: Carolina Lavigne Moreira, Vanessa D. Marques, Charles M. Lourenço, Daisy I. Cabrini, Jose C. Nuñez, Ronaldo F. Herrera, Wilson Marques Tags: LETTER TO THE EDITOR Source Type: research

Effect of diet‐induced obesity or type 1 or type 2 diabetes on corneal nerves and peripheral neuropathy in C57Bl/6J mice
Abstract We determined the impact diet‐induced obesity (DIO) and types 1 and 2 diabetes have on peripheral neuropathy with emphasis on corneal nerve structural changes in C57Bl/6J mice. Endpoints examined included nerve conduction velocity, response to thermal and mechanical stimuli and innervation of the skin and cornea. DIO mice and to a greater extent type 2 diabetic mice were insulin resistant. DIO and both types 1 and 2 diabetic mice developed motor and sensory nerve conduction deficits. In the cornea of DIO and type 2 diabetic mice there was a decrease in sub‐epithelial corneal nerves, innervation of the corneal ...
Source: Journal of the Peripheral Nervous System - June 15, 2015 Category: Neurology Authors: Matthew S. Yorek, Alexander Obrosov, Hanna Shevalye, Amey Holmes, Matthew M. Harper, Randy H. Kardon, Mark A. Yorek Tags: RESEARCH REPORT Source Type: research