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Muscle & Nerve

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https://read.qxmd.com/read/30767274/long-term-safety-and-efficacy-of-eculizumab-in-generalized-myasthenia-gravis
#1
Srikanth Muppidi, Kimiaki Utsugisawa, Michael Benatar, Hiroyuki Murai, Richard J Barohn, Isabel Illa, Saiju Jacob, John Vissing, Ted M Burns, John T Kissel, Richard J Nowak, Henning Andersen, Carlos Casasnovas, Jan L De Bleecker, Tuan H Vu, Renato Mantegazza, Fanny L O'Brien, Jing Jing Wang, Kenji P Fujita, James F Howard
INTRODUCTION: Eculizumab is effective and well tolerated in patients with anti-acetylcholine receptor antibody-positive refractory generalized myasthenia gravis (gMG; REGAIN; NCT01997229). We report an interim analysis of an open-label extension of REGAIN evaluating eculizumab's long-term safety and efficacy. METHODS: 117 patients received eculizumab (1,200 mg every 2 weeks) for 22.7 months (median). RESULTS: The safety profile of eculizumab was consistent with REGAIN; no cases of meningococcal infection were reported during the interim analysis period...
February 14, 2019: Muscle & Nerve
https://read.qxmd.com/read/30758837/cladribine-in-myasthenia-gravis-a-case-urging-for-prudence
#2
LETTER
Guy Laureys, Jan L De Bleecker
No abstract text is available yet for this article.
February 13, 2019: Muscle & Nerve
https://read.qxmd.com/read/30734335/treatment-outcome-in-juvenile-onset-myasthenia-gravis
#3
Aleksandra Jastrzębska, Miłosz Jastrzębski, Barbara Ryniewicz, Anna Kostera-Pruszczyk
INTRODUCTION: Myasthenia Gravis (MG) is an autoimmune disease of the neuromuscular junction. Approximately 10-15% of MG patients have juvenile (<18 years of age) onset. We aimed to assess the clinical course, outcome and subjectively perceived health status of a cohort of juvenile MG patients. METHODS: This was a retrospective analysis of medical records of 101 patients followed by a cross-sectional questionnaire study. RESULT: The mean age was 12...
February 7, 2019: Muscle & Nerve
https://read.qxmd.com/read/30734323/motor-amplitudes-may-predict-emg-confirmed-radiculopathy-in-patients-referred-for-radiating-limb-pain
#4
Brendan McNeish, Sandra Hearn, Anita Craig, Ann Laidlaw, Mark Ziadeh, James K Richardson
INTRODUCTION: Radiculopathy is diagnosed by needle electromyography, with nerve conduction studies excluding alternative diagnoses. METHODS: In patients referred for electrodiagnostic evaluation of radiating limb pain, we compared ulnar motor amplitudes between those with and without electromyographically-confirmed C8 radiculopathy, as well as fibular motor amplitudes between those with and without electromyographically-confirmed L5 radiculopathy. RESULTS: Patients with electromyographically-confirmed C8 or L5 radiculopathy demonstrated decreased ulnar or fibular motor amplitudes, respectively, as compared to patients without...
February 7, 2019: Muscle & Nerve
https://read.qxmd.com/read/30734322/towards-in-vivo-determination-of-peripheral-nervous-system-immune-activity-in-als
#5
Stefanie Schreiber, Frank Schreiber, Cornelia Garz, Grazyna Debska-Vielhaber, Anne Assmann, Valentina Perosa, Susanne Petri, Reinhard Dengler, Peter Nestor, Stefan Vielhaber
INTRODUCTION: We aimed to identify ALS patients displaying suspected peripheral nervous system (PNS) inflammation, to compare them to those with suspected PNS degeneration. METHODS: We measured sonographic median and ulnar nerve cross-sectional area (CSA) and CSF albumin/serum albumin ratio (Qalb ) in ALS patients, to classify them as having suspected PNS degeneration (small CSA/low Qalb ) or inflammation (larger CSA/high Qalb ). RESULTS: 57% of patients had suspected PNS degeneration, 21% had suspected PNS inflammation, and 21% displayed suspected "normal PNS state"...
February 7, 2019: Muscle & Nerve
https://read.qxmd.com/read/30734317/expanding-the-central-nervous-system-disease-spectrum-associated-with-flnc-mutation
#6
Stefano C Previtali, Marina Scarlato, Paolo Vezzulli, Alessandra Ruggieri, Daniele Velardo, Sara Benedetti, Giacomo Torini, Bruno Colombo, Lorenzo Maggi, Daniela Di Bella, Cinzia Gellera, Grazia D'Angelo, Marina Mora
No abstract text is available yet for this article.
February 7, 2019: Muscle & Nerve
https://read.qxmd.com/read/30706490/twice-weekly-glucocorticosteroids-in-infants-and-young-boys-with-duchenne-muscular-dystrophy
#7
Anne M Connolly, Craig M Zaidman, Paul T Golumbek, Mary M Cradock, Kevin M Flanigan, Nancy L Kuntz, Richard S Finkel, Craig M McDonald, Susan T Iannaccone, Pallavi Anand, Catherine A Siener, Julaine M Florence, Linda P Lowes, Lindsay N Alfano, Linda B Johnson, Alina Nicorici, Leslie L Nelson, Jerry R Mendell
INTRODUCTION: Glucocorticosteroids (GC) are effective in slowing weakness in boys with Duchenne muscular dystrophy (DMD). METHODS: This is a multisite, one-year, open-label trial of twice-weekly prednisolone (5mg/kg/dose) in infants/young boys (0.4-2.4 years) with DMD. We compared changes in Bayley III Scales of Infant Development (Bayley-III) to untreated boys followed for one year (historical control cohort-HCC). Twenty-three of 25 completed the study. RESULTS: Treated boys gained an average of 0...
February 1, 2019: Muscle & Nerve
https://read.qxmd.com/read/30702153/vasculitic-neuropathy-comparison-of-clinical-predictors-with-histopathological-outcome
#8
Dev Nathani, Michael H Barnett, Judith Spies, John Pollard, Min-Xia Wang, Matthew C Kiernan
INTRODUCTION: To improve diagnostic accuracy, this study compared pre-biopsy clinical parameters with subsequent pathological confirmation of peripheral nerve vasculitis. METHODS: Clinical, laboratory and neurophysiological parameters were analysed for consecutive patients referred for nerve biopsy with suspected vasculitis. Patients were assigned pathologic categories of definite, probable, possible or absent vasculitis using validated guidelines. Patients with definite or probable vasculitis were considered to have pathologically confirmed vasculitis...
January 31, 2019: Muscle & Nerve
https://read.qxmd.com/read/30702151/a-case-of-ultrasound-diagnosis-of-supraorbital-neuralgia
#9
Francesco Barbato, Andrea Di Paolantonio, Giuseppe Granata
No abstract text is available yet for this article.
January 31, 2019: Muscle & Nerve
https://read.qxmd.com/read/30701589/the-evidence-is-stacked-against-thymectomy-in-musk-myasthenia-gravis
#10
EDITORIAL
Hani Kushlaf, Yuebing Li
No abstract text is available yet for this article.
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30698289/inhibition-of-microrna-92a-increases-blood-vessels-and-satellite-cells-in-skeletal-muscle-but-does-not-improve-dmd-related-phenotype-in-mdx-mice
#11
Mayank Verma, Yoko Asakura, Atsushi Asakura
INTRODUCTION: The vasculature and blood flow in muscle is perturbed in Duchenne muscular dystrophy (DMD) and its mdx mouse model. MicroRNA-92a (miR-92a) is enriched in endothelial cells, especially during ischemic injury. METHODS: Since antagonizing miR-92a was shown to result in increased proliferation and migration of endothelial cells and recovery from ischemia, we assessed the effects of Antagomir-92a in vitro in muscle stem cell culture and in vivo in mdx mice...
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30697788/diffusion-weighted-magnetic-resonance-imaging-is-useful-for-assessing-inflammatory-myopathies
#12
Marie Faruch, Ana Isabel Garcia, Montse Del Amo, Jaume Pomes, Jaime Isern, Sergio Prieto González, Josep María Grau, José César Milisenda, Xavier Tomas
INTRODUCTION: Short tau inversion recovery (STIR) sequences in whole body MRI (WB-MRI) are usually used for detecting muscle edema (ME) in inflammatory myopathies. We evaluated b-value 800 diffusion weighted imaging (b800 DWI). METHODS: Two radiologists independently and a consensus reader retrospectively re-examined 60 patients with inflammatory myopathies and 15 controls. For each subject, 78 muscles were analyzed with 3 sets of imaging acquisitions: T1-weighted (T1) turbo spin echo (TSE) and STIR; T1 and DWI; T1, STIR and DWI...
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30697769/neuromuscular-ultrasound-as-an-initial-evaluation-for-suspected-myopathy-a-case-report
#13
Suzahn E Ebert, Kevin Brenzy, Michael S Cartwright
No abstract text is available yet for this article.
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30697763/decoupled-epineurial-and-axonal-deformation-in-mouse-median-and-ulnar-nerves
#14
Jaemyoung Sung, Jakub Sikora-Klak, Stephanie Adachi, Elisabeth Orozco, Sameer B Shah
INTRODUCTION: Peripheral nerves accommodate mechanical loads during joint movement. Hypothesized protective features include increased nerve compliance near joints and axonal undulation. How axons perceive nerve deformation is poorly understood. We tested whether nerves increase local axonal undulation in regions of high epineurial strain, to protect nerve fibers from strain-induced damage. METHODS: Regional epineurial strain was measured near the elbow in median and ulnar nerves of mice expressing axonal fluorescence, pre- and post-decompression...
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30697760/chronic-immune-sensorimotor-polyradiculopathy-cismp-report-of-a-case-series
#15
Thananan Thammongkolchai, Omer Suhaib, Pichet Termsarasab, Yuebing Li, Bashar Katirji
INTRODUCTION: Chronic immune sensorimotor polyradiculopathy (CISMP) is a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant affecting both sensory and motor nerve roots without evidence of peripheral nerve demyelination. METHOD: We report a case series of nine patients with CISMP, identified from two tertiary centers. Clinical, electrodiagnostic, and neuroradiologic features, and treatment responses, were retrospectively reviewed. RESULTS: Patients presented with sensorimotor deficits and hypo-/areflexia, predominantly involving lower extremities...
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30697753/translation-and-validation-of-the-arabic-version-of-the-myasthenia-gravis-activities-of-daily-living-scale
#16
Mohammed H Alanazy, Ahmad R Abuzinadah, Taim Muayqil
INTRODUCTION: We translated the myasthenia gravis (MG)-specific activities of daily living (MG-ADL) scale into Arabic (MG-ADL-A) and assessed its psychometric properties. METHODS: We assessed reliability using Cronbach's α, reproducibility using the intraclass correlation coefficient (ICC), and validity using Spearman's correlations with MG composite (MGC) score, MG-specific manual muscle test (MG-MMT), and MG quality-of-life revised Arabic version (MGQOL15R-A)...
January 30, 2019: Muscle & Nerve
https://read.qxmd.com/read/30681175/a-study-supporting-possible-expression-of-kir2-1-channels-in-peripheral-nerve-in-a-patient-with-andersen-tawil-syndrome
#17
Kazumoto Shibuya, Atsuko Tsuneyama, Minako Beppu, Sonoko Misawa, Yukari Sekiguchi, Hiroshi Amino, Yo-Ichi Suzuki, Tomoki Suichi, Keigo Nakamura, Satoshi Kuwabara
No abstract text is available yet for this article.
January 25, 2019: Muscle & Nerve
https://read.qxmd.com/read/30681174/centronuclear-myopathy-with-cardiomyopathy-due-to-recessive-titinopathy
#18
Jennifer M Martinez-Thompson, Thomas L Winder, Teerin Liewluck
No abstract text is available yet for this article.
January 25, 2019: Muscle & Nerve
https://read.qxmd.com/read/30681163/adipose-derived-stem-cells-delay-muscle-atrophy-after-peripheral-nerve-injury-in-the-rodent-model
#19
Benjamin K Schilling, M Asher Schusterman, Deok-Yeol Kim, Alex Repko, Katarina Klett, George J Christ, Kacey G Marra
INTRODUCTION: Injuries to peripheral nerves cause distal muscle atrophy. The effects of adipose-derived stem cell (ASC) injections into a muscle after injury were examined. METHODS: A 1.5cm defect in the rat sciatic nerve was created, resulting in gastrocnemius muscle atrophy. The nerve defect was repaired with autograft; DiR-labeled ASCs were injected into the gastrocnemius immediately postoperatively. Quantitation of gross musculature and muscle fiber area; cell survival, fibrosis, lipid deposition, inflammation, and reconstructive responses were investigated...
January 25, 2019: Muscle & Nerve
https://read.qxmd.com/read/30681158/the-use-of-collision-tests-to-identify-physiological-differences-between-the-median-and-ulnar-nerves
#20
Magdalena Koszewicz, Mariusz Szydlo, Jerzy Gosk, Malgorzata Wieczorek, Slawomir Budrewicz
INTRODUCTION: Median and ulnar nerves are used in comparative electrophysiological studies. We analyzed the conduction parameters in these hand nerves in healthy volunteers in order to find any physiological differences between them. METHODS: We performed standard conduction studies, and conduction velocity distribution (CVD) tests with estimation of three quartiles in 31 healthy, right-handed volunteers: 17 women, 14 men, mean age 44.8±15.5 years old. RESULTS: The conduction velocities in all quartiles of CVD tests were statistically faster in the ulnar nerve (p<0...
January 25, 2019: Muscle & Nerve
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