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Multifocal motor neuropathy

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https://www.readbyqxmd.com/read/28006835/cramp-fasciculation-syndrome-associated-with-monofocal-motor-neuropathy
#1
Nicolas J Dubuisson, Vincent Van Pesch, Peter Y K Van den Bergh
INTRODUCTION: Cramp-fasciculation syndrome (CFS) is a peripheral nerve hyperexcitability disorder (PNH), which could be caused by inflammatory neuropathy. CASE REPORT: We describe a 51-year-old woman who presented with a 4-5 year history of fasciculations and painful cramping of the right thenar eminence. Electrophysiological studies showed motor conduction block in the right median nerve between the axilla and the elbow with fasciculation potentials and cramp discharges on EMG in the right abductor pollicis brevis muscle...
December 22, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/28003344/differentiating-lower-motor-neuron-syndromes
#2
REVIEW
Nidhi Garg, Susanna B Park, Steve Vucic, Con Yiannikas, Judy Spies, James Howells, William Huynh, José M Matamala, Arun V Krishnan, John D Pollard, David R Cornblath, Mary M Reilly, Matthew C Kiernan
Lower motor neuron (LMN) syndromes typically present with muscle wasting and weakness and may arise from pathology affecting the distal motor nerve up to the level of the anterior horn cell. A variety of hereditary causes are recognised, including spinal muscular atrophy, distal hereditary motor neuropathy and LMN variants of familial motor neuron disease. Recent genetic advances have resulted in the identification of a variety of disease-causing mutations. Immune-mediated disorders, including multifocal motor neuropathy and variants of chronic inflammatory demyelinating polyneuropathy, account for a proportion of LMN presentations and are important to recognise, as effective treatments are available...
December 21, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28000233/multifocal-sensory-demyelinating-neuropathy-report-of-a-case
#3
Shin J Oh
INTRODUCTION: Multifocal sensory demyelinating neuropathy has not been reported. METHOD: A 42-year-old man with numbness of the left hand for 3 years and of the right hand for 6 months had a pure multifocal sensory neuropathy involving both hands, most prominently affecting 2-point discrimination, number writing, and object recognition in the left hand. Near-nerve needle sensory and mixed nerve conduction studies were performed in the left ulnar nerve. RESULTS: Studies of the left ulnar nerve documented a demyelinating neuropathy characterized by temporal dispersion and marked decrease in the amplitudes of the sensory and mixed compound nerve potentials in the above elbow-axilla segment...
December 21, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27988478/monitoring-the-short-term-effect-of-intravenous-immunoglobulins-in-multifocal-motor-neuropathy-using-motor-unit-number-index
#4
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
#5
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/27916751/-autoantibodies-in-chronic-inflammatory-neuropathies
#6
Ryo Yamasaki
Autoantibodies in chronic demyelinating neuropathies have been explored for several years. Recently, the peptides in the nodes of Ranvier have been the focus of attention in finding targets of autoantibodies. Until now, the most popular autoantibodies have been contactin-1 and neurofascin-155 for chronic demyelinating polyradiculoneuropathy (CIDP), GM1-ganglioside for multifocal motor neuropathy, and myelin-associated glycoprotein for polyneuropathy associated with monoclonal gammopathy of unknown significance...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27866728/what-is-new-in-2015-in-dysimmune-neuropathies
#7
REVIEW
T Kuntzer
This review discusses and summarizes the concept of nodopathies, the diagnostic features, investigations, pathophysiology, and treatment options of chronic inflammatory demyelinating polyradiculoneuropathy, and gives updates on other inflammatory and dysimmune neuropathies such as Guillain-Barré syndrome, sensory neuronopathies, small-fiber-predominant ganglionitis, POEMS syndrome, neuropathies associated with IgM monoclonal gammopathy and multifocal motor neuropathy. This field of research has contributed to the antigenic characterization of the peripheral motor and sensory functional systems, as well as helping to define immune neuropathic syndromes with widely different clinical presentation, prognosis and response to therapy...
December 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27861220/hereditary-neuropathy-with-liability-to-pressure-palsies-diverse-phenotypes-in-childhood
#8
Yohei Harada, Araya Puwanant, David N Herrmann
Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare autosomal-dominant disorder that most commonly produces recurrent painless focal sensory and motor neuropathies often preceded by minor, mechanical stress, or minor trauma. Herein, we report 2 pediatric cases of HNPP with atypical presentations; isolated muscle cramping and toe walking. Electrophysiologic testing disclosed multifocal sensorimotor polyneuropathy with slowing of sensory conduction velocities in both cases, which prompted PMP 22 gene deletion testing...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27836184/serological-study-using-glycoarray-for-detecting-antibodies-to-glycolipids-and-glycolipid-complexes-in-immune-mediated-neuropathies
#9
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
#10
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
#11
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/27680713/the-lesion-distribution-among-281-patients-with-sporadic-neuralgic-amyotrophy
#12
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
https://www.readbyqxmd.com/read/27649063/subcutaneous-vs-intravenous-immunoglobulin-for-chronic-autoimmune-neuropathies-a-meta-analysis
#13
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
#14
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
#15
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/27567246/polyclonal-igm-and-iga-block-in-vitro-complement-deposition-mediated-by-anti-ganglioside-antibodies-in-autoimmune-neuropathies
#16
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...
November 2016: International Immunopharmacology
https://www.readbyqxmd.com/read/27474733/-immune-mediated-neuropathies
#17
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/27460345/normalization-of-sonographical-multifocal-nerve-enlargements-in-a-madsam-patient-following-a-good-clinical-response-to-intravenous-immunoglobulin
#18
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
https://www.readbyqxmd.com/read/27456907/-multifocal-motor-neuropathy-with-conduction-blocks
#19
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
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
#20
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
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