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Multifocal motor neuropathy

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
Mark A Ferrante, Asa J Wilbourn
INTRODUCTION: The muscles commonly affected by neuralgic amyotrophy (NA) are well known, but the location of the responsible lesions is less clear (plexus versus extraplexus). METHODS: We report the lesion locations in 281 NA patients as determined by extensive electrodiagnostic (EDX) testing. RESULTS: Our 281 patients had 322 bouts of NA, 57 of which were bilateral, for a total of 379 assessable events. A single nerve was involved in 174 (46%), and 205 (54%) were multifocal...
September 28, 2016: Muscle & Nerve
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
Makoto Sudo, Kazuki Miyaji, Peter J Späth, Kana Morita-Matsumoto, Yoshiki Yamaguchi, Nobuhiro Yuki
Intravenous immunoglobulin (IVIG), consisting of IgG, is the first-line treatment for Guillain-Barré syndrome and multifocal motor neuropathy. IgG, but neither IgM nor IgA, has been demonstrated in vitro to inhibit complement deposition mediated by anti-ganglioside autoantibodies in sera from patients with both conditions. The objective of this study is to investigate the in vitro effectiveness of IgM and IgA in inhibiting complement deposition to ganglioside/anti-ganglioside antibody complexes. Serum samples were obtained from patients with multifocal motor neuropathy associated with anti-GM1 IgM antibodies, Guillain-Barré syndrome associated with anti-GM1 IgG antibodies and Miller Fisher syndrome associated with anti-GQ1b IgG antibodies...
August 24, 2016: International Immunopharmacology
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
Kanta Tanaka, Natsuko Ota, Yuzuru Harada, Ikko Wada, Toshihiko Suenaga
Focal nerve enlargements at sites of conduction blocks can be visualized sonographically in patients with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). However, little is known about association between nerve morphological changes and treatment responses. Here we present a 73-year-old female MADSAM patient whose sonographical multifocal nerve enlargements normalized following a good treatment response. She was admitted to our department with progressive asymmetrical muscle weakness and sensory disturbances for 6 months...
September 2016: Neuromuscular Disorders: NMD
A V Krasil'nikov, V A Naimushin
The article describes a case of multifocal motor neuropathy with conduction blocks in a female patient, aged 27 years. The development of the disease, results of neurological, laboratory, instrumental examinations, including electroneuromyography, and their role for the diagnosis and differential diagnosis are presented.
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
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
Hidetoshi Ishigaki, Takuya Hiraide, Yoshifumi Miyagi, Taiju Hayashi, Tomoko Matsubayashi, Ayumi Shimoda, Susumu Kusunoki, Tokiko Fukuda
Multifocal motor neuropathy is a rare immune-mediated neuropathy characterized by progressive asymmetric weakness and atrophy without sensory abnormalities. Although disease onset is usually in adulthood, a few childhood-onset cases have been reported. Here, we report the case of an 8-year-old boy with multifocal motor neuropathy who presented with a slowly progressive left and distal upper limb weakness without sensory loss. The initial high-dose intravenous immunoglobulin treatment significantly improved left upper limb muscle weakness...
September 2016: Pediatric Neurology
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
Pierre Lozeron, Vincent Ribrag, David Adams, Marion Brisset, Marguerite Vignon, Marine Baron, Marion Malphettes, Marie Theaudin, Bertrand Arnulf, Nathalie Kubis
To report the frequency of the different patterns of sensory and motor electrophysiological demyelination distribution in patients with anti-MAG neuropathy in comparison with patients with IgM neuropathy without MAG reactivity (IgM-NP). Thirty-five anti-MAG patients at early disease stage (20.1 months) were compared to 23 patients with IgM-NP; 21 CIDP patients and 13 patients with CMT1a neuropathy were used as gold standard neuropathies with multifocal and homogeneous demyelination, respectively. In all groups, standard motor and sensory electrophysiological parameters, terminal latency index and modified F ratio were investigated...
September 2016: Journal of Neurology
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
Attiyeh Vasaghi, Alireza Ashraf, Alireza Shirzadi, Peyman Petramfar
This report describes a patient with dysphonia for 16 years in combination with asymmetric and progressive decrease in sense and power of both upper and lower extremities for the past 3 years. Electrophysiological study revealed asymmetric conduction block and abnormal sensory action potential in 4 limbs. The vagus nerves palsy and abnormal electrodiagnosis of the limbs led us to diagnose the disease as Lewis and Sumner syndrome, also called multifocal acquired demyelinating sensory and motor neuropathy diagnosis, which improved by corticosteroid consumption to some extent...
May 12, 2016: American Journal of Physical Medicine & Rehabilitation
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
Nikolai Nikolov, Jürgen Reisinger, Hans P Schwarz
Intravenous immunoglobulins have been used to treat autoimmune disorders (ADs) for over 50 years. The etiologies of various ADs are not fully understood and although intravenous immunoglobulin treatment has proved its immunomodulatory properties, the roles of proposed mechanisms of action also remain a matter of speculation. A systemic search of the literature regarding KIOVIG(®) (Baxalta US, Inc., MA, USA) use in clinical trials on patients with ADs and a detailed review of retrieved articles revealed eight relevant publications...
July 2016: Immunotherapy
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