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multifocal motor neuropathy mmn

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https://www.readbyqxmd.com/read/28320129/immunoglobulin-therapy-in-the-treatment-of-multifocal-motor-neuropathy
#1
REVIEW
Aditya Kumar, Huned S Patwa, Richard J Nowak
Multifocal motor neuropathy (MMN) is a chronic immune-mediated disorder leading to slowly progressive muscle weakness and wasting. Current treatments are aimed at modulating the immune system in order to avoid further decline and to maintain functional status. Intravenous immunoglobulin (IVIg) is widely used in the treatment of immune-mediated disorders and is the only treatment approved for MMN. While patients do remain stable with maintenance IVIg treatment, most patients will slowly deteriorate over many years...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28144763/high-dose-ig-vena-is-well-tolerated-and-efficacious-in-patients-with-multifocal-motor-neuropathy
#2
Eduardo Nobile-Orazio, Dario Cocito, Chiara Briani, Rosaria Plasmati, Angelo Schenone, Francesca Gallia, Ivan Marjanovic, Anna Lotti Suffredini
Multifocal motor neuropathy (MMN) is a rare, chronic, motor neuropathy that progressively impairs physical functioning and quality of life. Randomised controlled trials have shown that high-dose intravenous immunoglobulin (IVIg) is superior to placebo in improving muscle strength and disability, but many patients require periodic infusions to maintain long-term improvement. This observational, multicentre, retrospective study investigated the efficacy and tolerability of human normal immunoglobulin (Ig VENA) at high intravenous infusion rates in 20 MMN patients (14 male, 6 female)...
January 31, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/27988478/monitoring-the-short-term-effect-of-intravenous-immunoglobulins-in-multifocal-motor-neuropathy-using-motor-unit-number-index
#3
Manon Philibert, Aude-Marie Grapperon, Emilien Delmont, Shahram Attarian
OBJECTIVE: To determine whether motor unit number index (MUNIX) is pertinent to monitor the effect of intravenous immunoglobulins (IVIg) in multifocal motor neuropathy (MMN). METHODS: MUNIX was assessed longitudinally in 7 MMN patients and 17 healthy controls in the abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. A MUNIX sum-score and a compound muscle action potential (CMAP) sum-score were calculated by summing up the scores of APB and ADM...
January 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/27927940/diagnostic-value-of-sonography-in-treatment-naive-chronic-inflammatory-neuropathies
#4
H Stephan Goedee, W Ludo van der Pol, Jan-Thies H van Asseldonk, Hessel Franssen, Nicolette C Notermans, Alexander J F E Vrancken, Michael A van Es, Stavros Nikolakopoulos, Leo H Visser, Leonard H van den Berg
OBJECTIVE: To determine the diagnostic value of high-resolution ultrasound (HRUS) for detection of chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner syndrome (LSS), and multifocal motor neuropathy (MMN). METHODS: Between January 2013 and January 2015, we enrolled 75 consecutive treatment-naive patients with chronic inflammatory neuropathies and 70 disease controls. We performed extensive nerve conduction and standardized HRUS studies bilaterally of large arm and leg nerves and brachial plexus...
January 10, 2017: Neurology
https://www.readbyqxmd.com/read/27836184/serological-study-using-glycoarray-for-detecting-antibodies-to-glycolipids-and-glycolipid-complexes-in-immune-mediated-neuropathies
#5
Miyuki Morikawa, Motoi Kuwahara, Rino Ueno, Makoto Samukawa, Yukihiro Hamada, Susumu Kusunoki
We performed a serological investigation using glycoarray in Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and multifocal motor neuropathy (MMN). Antibodies to 10 glycolipids and 45 glycolipid complexes were tested. Anti-GM1/sulfatide and anti-GA1/sulfatide IgG antibodies were common in GBS (20.0% and 19.0%, respectively). Anti-GQ1b/sulfatide IgG antibody was detected in 14.0% of GBS patients. IgG antibodies to antigens containing GQ1b were significantly correlated with ophthalmoplegia in GBS (p<0...
December 15, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27796190/postoperative-physical-therapy-management-of-tendon-transfer-for-digital-wrist-extension-due-to-multifocal-motor-neuropathy
#6
Steve Volpe
Study Design Case report. Background Multifocal motor neuropathy is a progressive motor nerve disorder characterized by muscle weakness in the extremities. Muscle imbalance and weakness can become so severe that the involved extremity can be rendered nonfunctional. The purpose of this case report is to describe the physical therapy postoperative management of a patient who underwent a multiple tendon transfer to correct the loss of digital/wrist extension of the right upper extremity. Case Description A 38-year-old woman with a medical diagnosis of multifocal motor neuropathy, which caused muscle imbalance and weakness in the right hand, underwent a multiple tendon transfer to correct the loss of digit and wrist extension...
December 2016: Journal of Orthopaedic and Sports Physical Therapy
https://www.readbyqxmd.com/read/27738760/als-and-mmn-mimics-in-patients-with-bscl2-mutations-the-expanding-clinical-spectrum-of-spg17-hereditary-spastic-paraplegia
#7
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
https://www.readbyqxmd.com/read/27649063/subcutaneous-versus-intravenous-immunoglobulin-for-chronic-autoimmune-neuropathies-a-meta-analysis
#8
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
https://www.readbyqxmd.com/read/27599252/validity-of-the-shuttle-walk-test-as-a-functional-assessment-of-walking-ability-in-individuals-with-polyneuropathy
#9
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
https://www.readbyqxmd.com/read/27571543/comparative-study-of-peripheral-nerve-mri-and-ultrasound-in-multifocal-motor-neuropathy-and-amyotrophic-lateral-sclerosis
#10
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...
December 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27474733/-immune-mediated-neuropathies
#11
REVIEW
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
https://www.readbyqxmd.com/read/27401495/improvement-of-hemoglobin-levels-after-a-switch-from-intravenous-to-subcutaneous-administration-of-immunoglobulin-in-chronic-inflammatory-demyelinating-polyneuropathy-and-multifocal-motor-neuropathy
#12
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...
October 2016: Transfusion
https://www.readbyqxmd.com/read/27396516/differential-weakness-of-finger-extensor-muscles-a-clinical-pattern-of-multifocal-motor-neuropathy
#13
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...
March 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/27366241/subcutaneous-immunoglobulins-in-the-treatment-of-chronic-immune-mediated-neuropathies
#14
REVIEW
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
https://www.readbyqxmd.com/read/27313890/peripheral-nerve-ultrasonography-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy-and-multifocal-motor-neuropathy-correlations-with-clinical-and-neurophysiological-data
#15
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
https://www.readbyqxmd.com/read/27291881/ultrasound-pattern-sum-score-homogeneity-score-and-regional-nerve-enlargement-index-for-differentiation-of-demyelinating-inflammatory-and-hereditary-neuropathies
#16
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
https://www.readbyqxmd.com/read/27134672/treatment-of-patients-with-multifocal-motor-neuropathy-with-immunoglobulins-in-clinical-practice-the-signs-registry
#17
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
https://www.readbyqxmd.com/read/27130524/autoantibody-pathogenicity-in-a-multifocal-motor-neuropathy-induced-pluripotent-stem-cell-derived-model
#18
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
https://www.readbyqxmd.com/read/27061901/nerve-ultrasound-protocol-in-differentiating-chronic-immune-mediated-neuropathies
#19
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
https://www.readbyqxmd.com/read/27040062/cervical-root-sonography-to-differentiate-multifocal-motor-neuropathy-from-als
#20
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
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