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Multifocal motor neuropathy

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https://www.readbyqxmd.com/read/28808176/teaching-video-neuroimages-trapezius-muscle-hypertrophy-in-multifocal-motor-neuropathy
#1
Pierre R Bourque, Mahmoud Al-Hajji, Jocelyn Zwicker, Jodi Warman Chardon
No abstract text is available yet for this article.
August 15, 2017: Neurology
https://www.readbyqxmd.com/read/28762574/a-comparative-study-of-brachial-plexus-sonography-and-magnetic-resonance-imaging-in-chronic-inflammatory-demyelinating-neuropathy-and-multifocal-motor-neuropathy
#2
H S Goedee, B A Jongbloed, J-T H van Asseldonk, J Hendrikse, A F J E Vrancken, H Franssen, S Nikolakopoulos, L H Visser, W L van der Pol, L H van den Berg
BACKGROUND AND PURPOSE: To compare the performance of neuroimaging techniques, i.e. high-resolution ultrasound (HRUS) and magnetic resonance imaging (MRI), when applied to the brachial plexus, as part of the diagnostic work-up of chronic inflammatory demyelinating neuropathy (CIDP) and multifocal motor neuropathy (MMN). METHODS: Fifty-one incident, treatment-naive patients with CIDP (n = 23) or MMN (n = 28) underwent imaging of the brachial plexus using (i) a standardized MRI protocol to assess enlargement or T2 hyperintensity and (ii) bilateral HRUS to determine the extent of nerve (root) enlargement...
August 1, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28717088/long-term-effects-of-intravenous-cyclophosphamide-in-combination-with-mesna-provided-intravenously-and-via-bladder-perfusion-in-a-patient-with-severe-multifocal-motor-neuropathy
#3
Eri Iwasawa, Takuya Ohkubo, Tadashi Kanouchi, Takashi Kanda, Hidehiro Mizusawa, Takanori Yokota
A 25-year-old woman presenting with progressive muscle weakness in the distal extremities in the absence of sensory involvement for 2 years was diagnosed with multifocal motor neuropathy (MMN). Her disease was difficult to manage with various immunosuppressants, and the muscle weakness eventually progressed to involve the respiratory muscles, necessitating mechanical ventilation. Intravenous cyclophosphamide (CY) dramatically improved her symptoms, and she has since maintained her ambulatory status for 18 years with intermittent CY therapy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28708133/autoantibodies-in-chronic-inflammatory-neuropathies-diagnostic-and-therapeutic-implications
#4
REVIEW
Luis Querol, Jérôme Devaux, Ricard Rojas-Garcia, Isabel Illa
The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed against paranodal proteins in patients with CIDP...
July 14, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28681489/nerve-ultrasound-as-follow-up-tool-in-treated-multifocal-motor-neuropathy
#5
T W Rattay, N Winter, B F Décard, N M Dammeier, F Härtig, M Ceanga, H Axer, A Grimm
BACKGROUND AND PURPOSE: High-resolution ultrasound is a valuable tool in supporting the diagnosis of multifocal motor neuropathy (MMN) but longitudinal data under therapy are lacking. METHODS: The change in peripheral nerve ultrasound pattern in patients with MMN was assessed over time. Patients with MMN received a thorough initial examination and follow-up over a period of 6-12 months using high-resolution ultrasound of the cervical roots and the nerves of the arms and legs, nerve conduction studies, Medical Research Council Sum Score (MRCSS) and Rotterdam Inflammatory Neuropathy Cause and Treatment Group (INCAT) score to evaluate changes under treatment...
July 5, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28644258/multifocal-motor-neuropathy-associated-with-infliximab-a-case-report-and-a-literature-review
#6
Ayse O Bayrak, Hasan Ulusoy, Necdet Bolat, Baki Doğan, Taner Ozbenli
Multifocal motor neuropathy with conduction block (MMN-CB) is purely a motor neuropathy with progressive weakness that is characteristically caused by conduction blocks. Association with antiganglioside antibodies and a good response to immunomodulating therapies suggest an autoimmune etiology. In rare cases, MMN-CB has been reported as an adverse effect of infliximab, a tumor necrosis factor-α blocker. We present a case of MMN-CB due to infliximab in a 45-year-old man with psoriatic arthritis who was exposed to the drug for 2 years because of a delayed diagnosis...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28601286/autoantibody-responses-to-nodal-and-paranodal-antigens-in-chronic-inflammatory-neuropathies
#7
E K Mathey, N Garg, S B Park, T Nguyen, S Baker, N Yuki, C Yiannikas, C S Lin, J M Spies, R Ghaoui, M H Barnett, S Vucic, J D Pollard, M C Kiernan
Autoantibodies to nodal/paranodal proteins have been reported in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). To determine the frequency of anti-paranodal antibodies in our cohort of CIDP patients and to validate the presence anti-nodal antibodies in MMN, sera were screened for IgG against human neurofascin 155, contactin-1, neurofascin 186 and gliomedin using ELISA. In CIDP patients, 7% were anti-NF155 IgG4 positive and 7% were anti-CNTN1 IgG4 positive...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28597710/comparing-treatment-options-for-chronic-inflammatory-neuropathies-and-choosing-the-right-treatment-plan
#8
Eduardo Nobile-Orazio, Francesca Gallia, Fabrizia Terenghi, Mariangela Bianco
Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled. Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies...
June 26, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28566163/subcutaneous-immunoglobulin-treatment-in-cidp-and-mmn-efficacy-treatment-satisfaction-and-costs
#9
REVIEW
Lars H Markvardsen, Thomas Harbo
Subcutaneous administration of immunoglobulin (SCIG) in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) has been reported in several case reports and in a few randomized trials during the last decade. In this review we present the studies on SCIG in CIDP and MMN with special focus on the clinical effects. Moreover, the effect on quality of life, side effects to SCIG and the health economic perspectives are reviewed. Nine case studies, three randomized trials and six long-term, follow-up studies were identified...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28523213/brachial-plexus-magnetic-resonance-imaging-differentiates-between-inflammatory-neuropathies-and-does-not-predict-disease-course
#10
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
#11
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...
May 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28368901/fortuitous-diagnosis-of-preexisting-neuropathy-during-ultrasound-guided-regional-anesthesia-performance-a-case-report
#12
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...
June 15, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28320129/immunoglobulin-therapy-in-the-treatment-of-multifocal-motor-neuropathy
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
June 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28000233/multifocal-sensory-demyelinating-neuropathy-report-of-a-case
#19
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
#20
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
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