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Multifocal motor neuropathy

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https://www.readbyqxmd.com/read/29748118/diaphragmatic-dysfunction-as-the-presenting-symptom-in-neuromuscular-disorders-a-retrospective-longitudinal-study-of-etiology-and-outcome-in-30-german-patients
#1
Matthias Türk, Irina Weber, Gernot Vogt-Ladner, Rolf Schröder, Martin Winterholler
Diaphragmatic dysfunction is well-known in advanced stages of neuromuscular disorders. However, data on its presence as the presenting symptom in neuromuscular disorders is scarce. The goal of this retrospective longitudinal study was to evaluate the etiology and clinical outcome in patients, in whom uni- or bilateral diaphragmatic dysfunction was primarily diagnosed, before a specific neuromuscular disease was found. Patients with critical illness neuropathy/myopathy were excluded from this study. Analysis of the medical records of two tertiary referral centers for patients with neuromuscular diseases identified 30 corresponding patients with diaphragmatic dysfunction (17 unilateral; 13 bilateral)...
April 9, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29742802/a-case-of-acute-onset-multifocal-motor-neuropathy-after-mycoplasma-infection
#2
Daisuke Nakashima, Yu-Ichi Noto, Yukiko Tsuji, Chihiro Fujii, Akihiro Tanaka, Tomoyuki Ohara, Masanori Nakagawa, Toshiki Mizuno
No abstract text is available yet for this article.
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29720545/multifocal-demyelinating-motor-neuropathy-and-hamartoma-syndrome-associated-with-a-de-novo-pten-mutation
#3
Boglarka Bansagi, Vietxuan Phan, Mark R Baker, Julia O'Sullivan, Matthew J Jennings, Roger G Whittaker, Juliane S Müller, Jennifer Duff, Helen Griffin, James A L Miller, Grainne S Gorman, Hanns Lochmüller, Patrick F Chinnery, Andreas Roos, Laura E Swan, Rita Horvath
OBJECTIVE: To describe a patient with a multifocal demyelinating motor neuropathy with onset in childhood and a mutation in phosphatase and tensin homolog ( PTEN ), a tumor suppressor gene associated with inherited tumor susceptibility conditions, macrocephaly, autism, ataxia, tremor, and epilepsy. Functional implications of this protein have been investigated in Parkinson and Alzheimer diseases. METHODS: We performed whole-exome sequencing in the patient's genomic DNA validated by Sanger sequencing...
May 2, 2018: Neurology
https://www.readbyqxmd.com/read/29610592/predominant-upper-limb-chronic-demyelinating-polyneuropathy-associated-with-hbv-infection
#4
Ioan-Cristian Lupescu, Adriana Octaviana Dulamea
Chronic inflammatory demyelinating polyneuropathy is an acquired, presumably immune-mediated peripheral neuropathy, characterized by symmetric sensory-motor involvement. Although most often idiopathic, it has been described in association with several disorders, sometimes improving under treatment. We present the case of a 57-year-old male who was admitted to hospital for paresthesias and muscle weakness affecting both upper limbs, initially only the hands, but with worsening and ascending progression during the last three years...
December 2017: Mædica
https://www.readbyqxmd.com/read/29582519/peripheral-neuropathy-in-tangier-disease-a-literature-review-and-assessment
#5
Metin Mercan, Vildan Yayla, Serdar Altinay, Serhat Seyhan
Tangier disease (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high-density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with Tangier disease with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in Tangier disease...
March 26, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29484273/home-versus-hospital-immunoglobulin-treatment-for-autoimmune-neuropathies-a-cost-minimization-analysis
#6
Gwendal Le Masson, Guilhem Solé, Claude Desnuelle, Emilien Delmont, Marc Gauthier-Darnis, Sophie Puget, Isabelle Durand-Zaleski
Background: Prior clinical trials have suggested that home-based Ig treatment in multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variant Lewis-Sumner syndrome (LSS) is safe and effective and is less costly than hospital-administered intravenous immunoglobulin (IVIg). Methods: A French prospective, dual-center, cost minimization analysis was carried out to evaluate IVIg administration (5% concentrated) at home versus in hospital with regard to costs, patients' autonomy, and patients' quality of life...
February 2018: Brain and Behavior
https://www.readbyqxmd.com/read/29329103/analysis-of-anti-ganglioside-antibodies-by-a-line-immunoassay-in-patients-with-chronic-inflammatory-demyelinating-polyneuropathies-cidp
#7
Juliane Klehmet, Stefanie Märschenz, Klemens Ruprecht, Benjamin Wunderlich, Thomas Büttner, Rico Hiemann, Dirk Roggenbuck, Andreas Meisel
BACKGROUND: Unlike for acute immune-mediated neuropathies (IN), anti-ganglioside autoantibody (aGAAb) testing has been recommended for only a minority of chronic IN yet. Thus, we used a multiplex semi-quantitative line immunoassay (LIA) to search for aGAAb in chronic-inflammatory demyelinating polyneuropathy (CIDP) and its clinical variants. METHODS: Anti-GAAb to 11 gangliosides and sulfatide (SF) were investigated by LIA in 61 patients with IN (27 typical CIDP, 12 distal-acquired demyelinating polyneuropathy, 6 multifocal-acquired demyelinating sensory/motor polyneuropathy, 10 sensory CIDP, 1 focal CIDP and 5 multifocal-motoric neuropathy), 40 with other neuromuscular disorders (OND) (15 non-immune polyneuropathies, 25 myasthenia gravis), 29 with multiple sclerosis (MS) and 54 healthy controls (HC)...
January 12, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29305208/vestibulo-cochlear-function-in-inflammatory-neuropathies
#8
Marisa Blanquet, Jens A Petersen, Antonella Palla, Dorothe Veraguth, Konrad P Weber, Dominik Straumann, Alexander A Tarnutzer, Hans H Jung
OBJECTIVE: We aimed to quantify peripheral-vestibular deficits that may contribute to imbalanced stance/gait in patients with inflammatory neuropathies. METHODS: Twenty-one patients (58 ± 15 y [mean age ± 1SD]; chronic-inflammatory-demyelinating-polyneuropathy = 10, Guillain-Barré Syndrome = 5, Anti-MAG peripheral neuropathy = 2, multifocal-motor-neuropathy = 4) were compared with 26 healthy controls. All subjects received video-head-impulse testing (vHIT), caloric irrigation and cervical/ocular vestibular-evoked myogenic-potentials (VEMPs)...
April 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29217500/peripheral-neuropathy-as-a-complication-of-diabetic-ketoacidosis-in-a-child-with-newly-diagnosed-diabetes-type-1-case-report
#9
Marta Baszyńska-Wilk, Marta Wysocka-Mincewicz, Anna Świercz, Jolanta Świderska, Magdalena Marszał, Mieczysław Szalecki
BACKGROUND: Neurological complications of diabetic ketoacidosis are considered to be very serious clinical problem. The most common complication is cerebral edema. However this group includes also less common syndromes such as ischemic or hemorrhagic stroke, cerebral venous and sinus thrombosis or very rare peripheral neuropathy. CASE REPORT: We present a case of 9-year old girl with new onset type 1 diabetes, diabetic ketoacidosis, cerebral edema, multifocal vasogenic brain lesions and lower limbs peripheral paresis...
December 8, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/29190522/ultrasound-and-mri-of-nerves-for-monitoring-disease-activity-and-treatment-effects-in-chronic-dysimmune-neuropathies-current-concepts-and-future-directions
#10
REVIEW
Bernhard F Décard, Mirko Pham, Alexander Grimm
New imaging modalities like high-resolution-ultrasound (HRUS) and MR-Neurography (MRN) are increasingly used for the evaluation of the peripheral nervous system. The increasing knowledge on morphological changes observed in different neuropathies has led to a better understanding of underlying pathophysiological processes. The diagnosis of acquired chronic dysimmune neuropathies (CDN) like chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner Syndrome (LSS) or multifocal motor neuropathy (MMN) can be challenging...
January 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29184351/clinico-electrophysiological-and-genetic-overlaps-and-magnetic-resonance-imaging-findings-in-charcot-marie-tooth-disease-a-pilot-study-from-western-india
#11
Satish Vasant Khadilkar, Nahush D Patil, Nikhil Dhananjay Kadam, Khushnuma A Mansukhani, Bhagyadhan A Patel
Background: Charcot-Marie-Tooth (CMT) disease is clinically and genetically heterogeneous. There are no published series describing clinical, electrophysiological, and genetic information on CMT from the Indian subcontinent. Magnetic resonance imaging (MRI) neurography technique provides useful information about the plexus and roots and can be employed in patients with CMT. Settings and Design: A prospective, observational study carried out at a tertiary care hospital in Western India...
October 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29136715/-analysis-of-the-clinical-and-electrophysiological-characteristics-of-multifocal-motor-neuropathy
#12
H F Wang, F Yang, F Cui, Z H Chen, L Ling, X S Huang
Ten patients diagnosed with multifocal motor neuropathy (MMN) were recruited in the Department of Neurology at Chinese PLA General Hospital from January 1, 2009 to August 31, 2015. The clinical and electrophysiological features were analyzed retrospectively. All patients complained of progressive asymmetric limb weakness, which was more severe in distal than in proximal. Five presented muscle atrophy. None had sensory disturbances. All suffered diminished or disappeared tendon reflex, whereas Babinski signs were negative...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29112586/the-role-of-immunoglobulin-in-the-treatment-of-immune-mediated-peripheral-neuropathies
#13
REVIEW
Alejandro Tobon
Immune-mediated neuropathies are a group of peripheral nerve disorders characterized by motor or sensory deficits caused by inflammation leading to demyelination or axonal injury. Intravenous immunoglobulin (IVIg) has been demonstrated to be an effective therapy for the 3 most common immune-mediated neuropathies: Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. This review summarizes current data on the rationale for the use of IVIg in immune-mediated neuropathies, addressing mechanism of action, clinical evidence, and practical considerations for its use...
November 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/28986470/quality-of-life-in-inflammatory-neuropathies-the-in-qol
#14
Thomas H P Draak, Catharina G Faber, Ingemar S J Merkies
BACKGROUND: No consensus exists which quality of life (QoL) measure should be used in patients with inflammatory neuropathies. Moreover, most QoL measures are ordinal-based scales with their known deficiencies. OBJECTIVES: To establish a new disease-specific interval-based QoL questionnaire in inflammatory neuropathies (IN-QoL) using the Rasch model and evaluate its scientific properties (validity, reliability and responsiveness). METHODS: 264 patients with inflammatory neuropathies completed six commonly used QoL questionnaires...
March 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28928949/plasmapheresis-in-neurological-disorders-six-years-experience-from-university-clinical-center-tuzla
#15
Osman Sinanović, Sanela Zukić, Adnan Burina, Nermina Pirić, Renata Hodžić, Mirza Atić, Mirna Alečković-Halilović, Enisa Mešić
BACKGROUND: Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique that is designed to remove substances with a large molecular weight. The TPE procedure includes removal of antibodies, alloantibodies, immune complexes, monoclonal protein, toxins or cytokines, and involves the replenishment of a specific plasma factor. The aim of the study was to describe the clinical response to TPE in various neurological patients, and to assess the clinical response to this therapy...
2017: F1000Research
https://www.readbyqxmd.com/read/28885479/high-dose-subcutaneous-immunoglobulin-in-patients-with-multifocal-motor-neuropathy-a-nursing-perspective
#16
RANDOMIZED CONTROLLED TRIAL
Vilija M Rasutis, Hans D Katzberg, Vera Bril
Multifocal motor neuropathy (MMN), an immune neuromuscular condition causing progressive weakness, usually responds to immune-mediated treatments, including intravenous immunoglobulin (IVIG). Fifteen patients with MMN receiving IVIG were enrolled in an open-label, single-center trial and switched to 20% subcutaneous immunoglobulin (SCIG) using a smooth transition protocol (ie, changing the therapy without interruption or impact on the intended outcome of the therapy). Patients received individualized training and support based on motivation and ability to learn, follow directions, and maintain compliance...
September 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/28881389/comparisons-in-fluctuation-of-muscle-strength-and-function-in-patients-with-immune-mediated-neuropathy-treated-with-intravenous-versus-subcutaneous-immunoglobulin
#17
Ingelise Christiansen, Lars H Markvardsen, Johannes Jakobsen
INTRODUCTION: Variation of muscle strength and function have not been studied in patients with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy whose treatment regimen has been changed from intravenous to subcutaneous immunoglobulin (IVIG to SCIG). METHODS: In a prospective open label study, patients were changed from monthly IVIG to weekly SCIG. The primary endpoint was the variation of isokinetic muscle strength (cIKS). Secondary endpoints were variation of Medical Research Council (MRC) score, grip strength (GS), 9-hole-peg test (9-HPT), 40-meter-walk test (40-MWT)...
September 7, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28837908/motor-unit-remodelling-in-multifocal-motor-neuropathy-the-importance-of-axonal-loss
#18
Nidhi Garg, James Howells, Con Yiannikas, Steve Vucic, Arun V Krishnan, Judith Spies, Hugh Bostock, Emily K Mathey, John D Pollard, Susanna B Park, Matthew C Kiernan
OBJECTIVE: To estimate the degree of axonal loss in patients diagnosed with multifocal motor neuropathy (MMN) using a novel assessment of motor unit numbers and size. METHODS: Automated motor unit number estimation using a compound muscle action potential (CMAP) scan was undertaken in median nerves with conduction block. Results were compared with 30 age-matched healthy controls. RESULTS: Compared with healthy controls, MMN patients had fewer motor units (MMN: 33±11vs HC: 93±36 [mean±SD]; p<0...
October 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28808176/teaching-video-neuroimages-trapezius-muscle-hypertrophy-in-multifocal-motor-neuropathy
#19
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
#20
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...
October 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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