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multifocal motor neuropathy mmn

Thomas Musacchio, Ann-Kathrin Zaum, Nurcan Üçeyler, Claudia Sommer, Nora Pfeifroth, Karlheinz Reiners, Erdmute Kunstmann, Jens Volkmann, Simone Rost, Stephan Klebe
Silver syndrome/SPG17 is a motor manifestation of mutations in the BSCL2 gene and usually presents as a complicated form of hereditary spastic paraplegia (HSP). We present clinical data, follow-up, and genetic results of seven patients with Silver syndrome/SPG17 including a family with a variable intrafamilial phenotype ranging from subclinical signs to a severe and rapidly progressing amyotrophic lateral sclerosis (ALS)-like phenotype. For molecular diagnosis of the family, we used the TruSight Exome sequencing panel consisting of 2761 genes...
October 13, 2016: Journal of Neurology
Juan M Racosta, Luciano A Sposato, Kurt Kimpinski
INTRODUCTION: High-dose intravenous immunoglobulin (IVIg) is an evidence-based treatment for multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). Recently, subcutaneous immunoglobulin (SC-Ig) has received increasing attention. METHODS: We performed a meta-analysis of reports of efficacy and safety of SC-Ig versus IVIg for inflammatory demyelinating polyneuropathies. RESULTS: A total of 8 studies comprising 138 patients (50 with MMN and 88 with chronic CIDP) were included in the meta-analysis...
September 20, 2016: Muscle & Nerve
Peter G Erdmann, Laurien L Teunissen, Leonard H van den Berg, Nicolette C Notermans, Carin D Schröder, Bart C Bongers, Nico L U van Meeteren
PURPOSE: This study assessed the validity of the shuttle walk test (SWT) to evaluate walking ability in patients with polyneuropathy. METHODS: Forty-one patients with chronic idiopathic axonal polyneuropathy (CIAP) and 49 patients with multifocal motor neuropathy (MMN) performed both the 10-meter walk test (10MWT) and the SWT. Face validity was assessed by evaluating whether patients considered both tests to reflect their walking ability (Likert scale: 1 = not at all, 10 = very well)...
September 6, 2016: Disability and Rehabilitation
Bas A Jongbloed, Wieke Haakma, H Stephan Goedee, Jeroen W Bos, Clemens Bos, Jeroen Hendrikse, Leonard H Van Den Berg, W Ludo Van Der Pol
INTRODUCTION: Differentiating multifocal motor neuropathy (MMN) from amyotrophic lateral sclerosis (ALS) is important, as MMN is a difficult, but treatable disorder. METHODS: We studied peripheral nerve imaging techniques in differentiating MMN from ALS by measuring the cross-sectional area (CSA) of the median and ulnar nerves in the forearms using high resolution ultrasound (HRUS) and MRI. RESULTS: HRUS CSA values of the median nerve in the forearm (P = 0...
August 29, 2016: Muscle & Nerve
G Stoll, K Reiners
The Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are the most common immune-mediated polyneuropathies, which can show variable clinical and electrophysiological manifestations. Rarer immune-mediated neuropathies encompass paraproteinemic neuropathies (PPN), multifocal motor neuropathy (MMN) and vasculitic neuropathies. The diagnosis usually relies on the history of symptom evolution, distribution of nerve dysfunction and particularly on characteristic features in nerve conduction studies, aided by cerebrospinal fluid (CSF) examination and nerve biopsy findings...
August 2016: Der Nervenarzt
Lars Høj Markvardsen, Ingelise Christiansen, Johannes Jakobsen
BACKGROUND: Intravenous immunoglobulin (IVIG) is recommended treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). Recent studies have demonstrated that subcutaneous immunoglobulin (SCIG) is feasible, safe, and effective in both disorders. IVIG leads to transient hemolysis and, consequently, we hypothesized that frequent small doses of SCIG exerts less hemolytic activity than a few larger doses of IVIG. STUDY DESIGN AND METHODS: In an open-label study, 23 three patients treated with IVIG for CIDP or MMN were switched to SCIG at an equal dosage...
July 12, 2016: Transfusion
Trajche Ivanovski, Francesc Miralles
INTRODUCTION: Several studies have suggested that differential weakness in muscles supplied by the same motor nerve supports the diagnosis of multifocal motor neuropathy (MMN). METHODS: We describe the clinical, electrophysiological, neuroimaging, and laboratory findings of patients with a lower motor syndrome whose clinical presentation included differential finger extension weakness that we have seen in our neuromuscular clinic. RESULTS: We identified 3 patients with hand weakness and 1 patient with asymmetric weakness of the upper extremity...
July 11, 2016: Muscle & Nerve
Verena I Leussink, Hans-Peter Hartung, Bernd C Kieseier, Mark Stettner
Intravenous immunoglobulins represent an established therapy for the treatment of chronic immune-mediated neuropathies, specifically chronic inflammatory demyelinating polyradiculoneuropathies (CIDPs) as well as multifocal motor neuropathies (MMNs). For the treatment of antibody deficiency syndromes, subcutaneous immunoglobulins (SCIgs) have represented a mainstay for decades. An emerging body of evidence suggests that SCIg might also exhibit clinical efficacy in CIDP and MMN. This article reviews the current evidence for clinical effectiveness, as well as safety of SCIg for the treatment of immune-mediated neuropathies, and addresses remaining open questions in this context...
July 2016: Therapeutic Advances in Neurological Disorders
Aristide Merola, Michela Rosso, Alberto Romagnolo, Erdita Peci, Dario Cocito
Objective. This cross-sectional study analyzes the pattern of ultrasound peripheral nerve alterations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) at different stages of functional disability. Material and Methods. 22 CIDP and 10 MMN patients and a group of 70 healthy controls were evaluated with an ultrasound scan of the median, ulnar, peroneal, tibial, and sural nerves. Results were correlated with clinical disability scales and nerve conduction studies...
2016: Neurology Research International
Alexander Grimm, Debora Vittore, Victoria Schubert, Christina Lipski, Bianka Heiling, Bernhard F Décard, Hubertus Axer
OBJECTIVE: To investigate the use of nerve ultrasound in the differentiation between Charcot-Marie Tooth hereditary neuropathy (CMT1) and chronic inflammatory demyelinating polyradiculoneuropathies (CIDP), multifocal motor neuropathy (MMN) and multifocal acquired demyelinating sensory and motor neuropathies (MADSAM). METHODS: Ultrasound/electrophysiology of predefined nerves was performed in CMT1a/b, immunoneuropathies, and healthy controls. Ultrasound pattern sum score (UPSS, sum of the amount of 12 predefined measurement points), homogeneity score (HS) and regional nerve enlargement index (RNEI) in ulnar, median, and tibial nerve were used for evaluation of morphology...
July 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Martin Stangel, Ralf Gold, David Pittrow, Ulrich Baumann, Michael Borte, Maria Fasshauer, Manfred Hensel, Dörte Huscher, Marcel Reiser, Claudia Sommer
OBJECTIVES: The management of patients with multifocal motor neuropathy (MMN) under everyday clinical conditions has been insufficiently studied. We therefore collected comprehensive observational data on patients with MMN who received intravenous (IV) or subcutaneous (SC) immunoglobulins (IGs) as maintenance therapy. METHODS: This was a prospective, noninterventional study (registry) in neurological centres (hospitals and offices) throughout Germany. RESULTS: As of 1 December 2015, 80 patients with MMN were included (mean age 55...
May 2016: Therapeutic Advances in Neurological Disorders
Oliver Harschnitz, Leonard H van den Berg, Lill Eva Johansen, Marc D Jansen, Sandra Kling, Renata Vieira de Sá, Lotte Vlam, Wouter van Rheenen, Henk Karst, Corette J Wierenga, R Jeroen Pasterkamp, W Ludo van der Pol
OBJECTIVE: We investigated the pathogenicity of immunoglobulin M (IgM) anti-GM1 antibodies in serum from patients with multifocal motor neuropathy (MMN) using human induced pluripotent stem cell (iPSC)-derived motor neurons (MNs). METHODS: iPSCs were generated from fibroblasts and differentiated into MNs. We studied the binding of IgM to MNs, their complement-activating properties, and effects on structural integrity using fluorescence and electron microscopy. Live cell imaging was used to study effects of antibody binding on MNs in the presence and absence of complement...
July 2016: Annals of Neurology
Antonios Kerasnoudis, Kalliopi Pitarokoili, Aiden Haghikia, Ralf Gold, Min-Suk Yoon
INTRODUCTION: We evaluated a new neuropathy ultrasound protocol (NUP) for differentiating chronic immune-mediated neuropathies. METHODS: The NUP was evaluated in 110 patients with clinical presentations of chronic immune-mediated neuropathies. All patients were evaluated clinically and electrophysiologically and were divided into 4 groups: a) symmetric demyelinating, b) symmetric axonal, c) asymmetric demyelinating, and d) asymmetric axonal polyneuropathy. During Step 2, the NUP underwent prospective evaluation in all 4 study groups...
April 7, 2016: Muscle & Nerve
Hiroyuki Nodera, Yuishin Izumi, Naoko Takamatsu, Ryuji Kaji
To explore suggestive evidence of focal proximal demyelination in multifocal motor neuropathy (MMN) without overt evidence of conduction block, we conducted sonographical assessment of cervical nerve roots in 9 MMN patients, 22 ALS patients, and 17 control subjects. The mean diameters of the C5 and C6 roots in MMN patients were significantly larger than those in ALS and controls, especially on the clinically dominant side. Although non-specific, sonography can be a potentially useful diagnostic procedure to support the diagnosis of MMN, even when overt conduction block is lacking...
2016: Journal of Medical Investigation: JMI
Hans D Katzberg, Vilija Rasutis, Vera Bril
INTRODUCTION: Subcutaneous immunoglobulin (SCIG) has been used for treatment of immune neuropathies. METHODS: We evaluated safety and efficacy of 1.53:1 SCIG to intravenous immunoglobulin (IVIG) in individuals receiving <2 g/kg IVIG per month and1:1 in individuals receiving 2g/kg/month for treatment of multifocal motor neuropathy (MMN) in an open-label, 6-month trial. Medical Research Council sum score, grip strength, modified Guy's upper limb disability score, Health Utility Index Quality of Life, and immunoglobulin levels were evaluated at baseline, 3, and 6 months...
March 14, 2016: Muscle & Nerve
Mark Stettner, Lena Hinrichs, Rainer Guthoff, Silja Bairov, Ioannis N Petropoulos, Clemens Warnke, Hans-Peter Hartung, Rayaz A Malik, Bernd C Kieseier
OBJECTIVE: There is an unmet need for better diagnostic tools to further delineate clinical subsets of heterogeneous chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) to facilitate treatment decisions. Corneal confocal microscopy (CCM) is a noninvasive and reproducible nerve imaging technique. This study evaluates the potential of CCM as a diagnostic surrogate in CIDP and MMN. METHODS: In a cross-sectional prospective approach, 182 patients and healthy controls were studied using CCM to quantify corneal nerve damage and immune cell infiltration...
February 2016: Annals of Clinical and Translational Neurology
Francesca Gallia, Claudia Balducci, Eduardo Nobile-Orazio
High-dose intravenous immunoglobulin (IVIg) is effective in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). Not all brands of IVIg are however licensed for these neuropathies. We reviewed six patients with CIDP and seven with MMN treated with maintenance therapy with IVIg from 2009 to 2013. In all patients, we measured the Medical Research Council (MRC) and Overall Neuropathy Limitation Scale (ONLS) scores before each infusion, registered the monthly dose and brand of IVIg, and recorded adverse events...
June 2016: Journal of the Peripheral Nervous System: JPNS
Kai F Loewenbrück, Julia Liesenberg, Markus Dittrich, Jochen Schäfer, Beate Patzner, Beate Trausch, Jochen Machetanz, Andreas Hermann, Alexander Storch
The objective of the study was to investigate nerve ultrasound (US) in comparison to nerve conduction studies (NCS) for differential diagnosis of amyotrophic lateral sclerosis with predominant lower motoneuron disease(ALS/LMND) and multifocal motor neuropathy(MMN). A single-center, prospective, examiner-blinded cross-sectional diagnostic study in two cohorts was carried out. Cohort I: convenience sample of subjects diagnosed with ALS/LMND or MMN (minimal diagnostic criteria:possible ALS (revised EL-Escorial criteria), possible MMN (European Federation of Neurosciences guidelines)...
January 2016: Journal of Neurology
Els K Vanhoutte, Catharina G Faber, Sonja I van Nes, Elisabeth A Cats, W-Ludo Van der Pol, Kenneth C Gorson, Pieter A van Doorn, David R Cornblath, Leonard H van den Berg, Ingemar S J Merkies
Clinical trials in multifocal motor neuropathy (MMN) have often used ordinal-based measures that may not accurately capture changes. We aimed to construct a disability interval outcome measure specifically for MMN using the Rasch model and to examine its clinimetric properties. A total of 146 preliminary activity and participation items were assessed twice (reliability studies) in 96 clinically stable MMN patients. These patients also assessed the ordinal-based overall disability sum score (construct, sample-dependent validity)...
September 2015: Journal of the Peripheral Nervous System: JPNS
L Vlam, M Stam, W de Jager, E A Cats, L H van den Berg, W L van der Pol
Multifocal motor neuropathy (MMN) and progressive muscular atrophy (PMA) are associated with IgM monoclonal gammopathy or the presence IgM anti-GM1-antibodies. To further investigate the pathophysiology of MMN and PMA we determined concentrations of 16 mainly B-cell associated inflammatory markers in serum from 25 patients with MMN, 55 patients with PMA, 25 patients with amyotrophic lateral sclerosis (ALS) and 50 healthy controls. Median serum concentrations of the 16 tested cytokines and chemokines were not significantly increased in patients with MMN or patients with PMA, irrespective of the presence of IgM monoclonal gammopathy or high IgM anti-GM1 antibodies...
September 15, 2015: Journal of Neuroimmunology
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