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Multifocal motor neuropathy

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https://www.readbyqxmd.com/read/28523213/brachial-plexus-magnetic-resonance-imaging-differentiates-between-inflammatory-neuropathies-and-does-not-predict-disease-course
#1
Bas A Jongbloed, Jeroen W Bos, Dirk Rutgers, Willem Ludo van der Pol, Leonard H van den Berg
OBJECTIVE: The main objective of this study was to evaluate the correlation between the distribution of brachial plexus magnetic resonance imaging (MRI) abnormalities and clinical weakness, and to evaluate the value of brachial plexus MRI in predicting disease course and response to treatment in multifocal motor neuropathy (MMN), Lewis-Sumner syndrome (LSS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: Sixty-seven patients with an inflammatory neuropathy diagnosed at our tertiary referral center for neuromuscular diseases had undergone bilateral T2-weighted short tau inversion recovery (STIR) MRI of the brachial plexus...
May 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28434507/motor-neuropathies-and-lower-motor-neuron-syndromes
#2
REVIEW
A Verschueren
Motor or motor-predominant neuropathies may arise from disease processes affecting the motor axon and/or its surrounding myelin. Lower motor neuron syndrome (LMNS) arises from a disease process affecting the spinal motor neuron itself. The term LMNS is more generally used, rather than motor neuronopathy, although both entities are clinically similar. Common features are muscle weakness (distal or proximal) with atrophy and hyporeflexia, but no sensory involvement. They can be acquired or hereditary. Immune-mediated neuropathies (multifocal motor neuropathy, motor-predominant chronic inflammatory demyelinating polyneuropathy) are important to identify, as effective treatments are available...
April 20, 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28368901/fortuitous-diagnosis-of-preexisting-neuropathy-during-ultrasound-guided-regional-anesthesia-performance-a-case-report
#3
Philippe Marty, Bertrand Basset, Constance Marquis, Medhi Merouani, Olivier Rontes, Alain Delbos
Ultrasound-guided regional anesthesia requires the anesthesia provider to interpret new information. This article reports on the case of a 38-year-old man scheduled for a fifth metacarpal fracture repair. Ultrasound nerve examination revealed abnormal pathology of the axillary brachial plexus consisting of an increased volume of the terminal nerves of the brachial plexus. Ultrasound scanning initiated the subsequent diagnosis of multifocal motor neuropathy. Regional anesthesia was abandoned in favor of general anesthesia...
March 31, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28320129/immunoglobulin-therapy-in-the-treatment-of-multifocal-motor-neuropathy
#4
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/28251916/genetic-heterogeneity-of-motor-neuropathies
#5
Boglarka Bansagi, Helen Griffin, Roger G Whittaker, Thalia Antoniadi, Teresinha Evangelista, James Miller, Mark Greenslade, Natalie Forester, Jennifer Duff, Anna Bradshaw, Stephanie Kleinle, Veronika Boczonadi, Hannah Steele, Venkateswaran Ramesh, Edit Franko, Angela Pyle, Hanns Lochmüller, Patrick F Chinnery, Rita Horvath
OBJECTIVE: To study the prevalence, molecular cause, and clinical presentation of hereditary motor neuropathies in a large cohort of patients from the North of England. METHODS: Detailed neurologic and electrophysiologic assessments and next-generation panel testing or whole exome sequencing were performed in 105 patients with clinical symptoms of distal hereditary motor neuropathy (dHMN, 64 patients), axonal motor neuropathy (motor Charcot-Marie-Tooth disease [CMT2], 16 patients), or complex neurologic disease predominantly affecting the motor nerves (hereditary motor neuropathy plus, 25 patients)...
March 28, 2017: Neurology
https://www.readbyqxmd.com/read/28194064/therapeutic-plasma-exchange-in-patients-with-neurologic-disorders-review-of-63-cases
#6
Anil Tombak, Mehmet Ali Uçar, Aydan Akdeniz, Arda Yilmaz, Hakan Kaleagası, Mehmet Ali Sungur, Eyup Naci Tiftik
Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome (n = 22), myasthenia gravis (n = 21), chronic inflammatory demyelinating polyneuropathy (n = 7), polymyositis (n = 3), multifocal motor neuropathy (n = 2), acute disseminated encephalomyelitis (n = 2), neuromyelitis optica (n = 2), multiple sclerosis (n = 2), limbic encephalitis (n = 1) and transverse myelitis (n = 1)...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28144763/high-dose-ig-vena-is-well-tolerated-and-efficacious-in-patients-with-multifocal-motor-neuropathy
#7
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/28006835/cramp-fasciculation-syndrome-associated-with-monofocal-motor-neuropathy
#8
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
#9
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
#10
Shin J Oh
INTRODUCTION: Multifocal sensory demyelinating neuropathy has not been adequately reported in the literature. METHODS: 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 of the left hand. Near-nerve needle sensory and mixed nerve conduction studies were performed on the left ulnar nerve...
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
January 2017: Journal of Neurology
https://www.readbyqxmd.com/read/27680713/the-lesion-distribution-among-281-patients-with-sporadic-neuralgic-amyotrophy
#19
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-versus-intravenous-immunoglobulin-for-chronic-autoimmune-neuropathies-a-meta-analysis
#20
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
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