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Muscle & Nerve

Thomas McCaffrey, Michela Guglieri, Alexander P Murphy, Katherine Md Bushby, Anna Johnson, John P Bourke
Introduction The significance of abnormal cardiac measures in asymptomatic females who harbor dystrophin gene mutations is controversial. Methods Echo-measures of ventricular function were compared with published norms in a cross-sectional study of 130 (age 39 ± 15.7 years) 'carriers' of Duchenne or Becker muscular dystrophy (DBMD). Correlations between cardiomyopathy (CM) and mutation, CK levels, age, and muscle symptoms were investigated. Results Depending on definition, CM prevalence was 3-33%. Ejection fraction (Simpson) was < 55% in 9 (13%) and < 40% in 2 (2...
October 19, 2016: Muscle & Nerve
Donald B Sanders, Janice M Massey
OBJECTIVE: To determine if single-fiber EMG (SFEMG) jitter accurately reflects change in severity in myasthenia gravis (MG). METHODS: We reviewed jitter and outcome data from all MG patients in our clinic who had at least 2 jitter measurements in the extensor digitorum (ED) or frontalis muscle. RESULTS: Change in all parameters of jitter measured with SFEMG electrodes predicted clinical change with acceptable accuracy. Absolute and percentage change in mean MCD were equally accurate in predicting clinical change and were more accurate than change in the proportion of fiber pairs with blocking or normal jitter...
October 19, 2016: Muscle & Nerve
Alan Pestronk, Richard Keeling, Rati Choksi
OBJECTIVE: We studied mitochondrial impairment as a factor in the pathologic equivalent of sarcopenia, muscle fiber atrophy associated with increased age. METHODS: Mitochondrial oxidative enzyme activities and coenzyme Q10 levels were measured in frozen human proximal limb muscles with combined age and atrophy, age alone, atrophy alone, denervation, immune myopathies, and mitochondrial disorders with ophthalmoplegia. RESULTS: Sarcopenia (age and atrophy) had reduced mean activities of mitochondrial Complexes I, II, and II+III, with severe reduction of Complex I activity in 54% of patients...
October 19, 2016: Muscle & Nerve
Sarah Youssof, Carol Romero-Clark, Teddy Warner, Emily Plowman
INTRODUCTION: The Swallowing Quality of Life instrument (SWAL-QOL) is a patient-reported outcome (PRO) measure of swallowing-related quality of life (SR-QoL). Its psychometric properties in oculopharyngeal muscular dystrophy (OPMD) are not known. METHODS: We administered the SWAL-QOL to U.S. OPMD Registry participants. We described SR-QoL profiles and assessed reliability and validity. RESULTS: Mean composite score in 113 individuals with OPMD was 54...
October 19, 2016: Muscle & Nerve
Suma Babu, Erik P Pioro, Jianbo Li, Yuebing Li
INTRODUCTION: We compared the yield of limb and thoracic paraspinal muscles for revealing lower motor neuron (LMN) dysfunction on electromyography (EMG) in amyotrophic lateral sclerosis (ALS). METHODS: A retrospective review of 354 patients with clinically definite or probable ALS was performed. Seventeen limb muscles and thoracic paraspinal muscles were evaluated for the presence of both active and chronic denervation. RESULTS: Distal limb muscles showed the highest electrodiagnostic sensitivities of LMN dysfunction in ALS regardless of onset region and diagnostic certainty at the time of diagnosis...
October 19, 2016: Muscle & Nerve
Imene Dalichaouche, Yamina Sifi, Carinne Roudaut, Karima Sifi, Abdelmadjid Hamri, Leila Rouabah, Noureddine Abadi, Isabelle Richard
INTRODUCTION: We report the genetic analysis of a large series of 76 Algerian patients from 65 unrelated families that presented with early onset severe muscular dystrophy and a clinical phenotype resembling Limb-girdle muscular dystrophy type 2C (LGMD2C). METHODS: To define the genetic basis of the diseases in these families, we undertook a series of analysis of the γ-sarcoglycan (SGCG) and DMD genes. RESULTS: Fifteen families were shown to carry SGCG variants...
October 19, 2016: Muscle & Nerve
Dustin Anderson, Grayson Beecher, Trevor Steve, Ho Jen, Richard Camicioli, Douglas W Zochodne
INTRODUCTION: Hodgkin lymphoma (HL) is a common lymphoid malignancy rarely associated with Guillain-Barré Syndrome (GBS). In most cases, GBS does not precede HL. METHODS: We describe a patient with acute inflammatory demyelinating polyneuropathy (AIDP) who fulfilled criteria for GBS that heralded undiagnosed HL. RESULTS: Cerebrospinal fluid (CSF) studies revealed albuminocytologic dissociation with significant protein elevation (250 mg/dL)...
October 18, 2016: Muscle & Nerve
Ted M Burns, Gordon A Smith
No abstract text is available yet for this article.
October 18, 2016: Muscle & Nerve
Patrick J McEnany
No abstract text is available yet for this article.
October 18, 2016: Muscle & Nerve
Michael H Rivner, Siyang Liu, Brandy Quarles, Brandi Fleenor, Chengyong Shen, Jinxiu Pan, Lin Mei
INTRODUCTION: The prevalence and characteristics of Agrin and LRP4 antibody positive amyotrophic lateral sclerosis (ALS) patients were studied. METHODS: We tested 82 ALS patients and 59 controls for Agrin and LRP4 antibodies using ELISA. RESULTS: We found that 13.8% of ALS patients had Agrin antibodies, and 9.8% had LRP4 antibodies. Women ALS patients are twice as likely as men to have antibodies. Agrin-positive ALS patients are younger than Agrin-negative ALS patients...
October 18, 2016: Muscle & Nerve
Yusuf A Rajabally, Fu Liong Hiew
INTRODUCTION: The most efficient method of performing electrophysiology for Guillain-Barré syndrome (GBS) is unknown. METHODS: We retrospectively analyzed electrophysiological data of 97 consecutive GBS patients from Birmingham, U.K. (2001-12) studied ≤3 weeks post-onset. RESULTS: The sensitivity of electrophysiology for each GBS subtype was dependent on the tested upper and lower limb nerves. In acute inflammatory demyelinating polyneuropathy (AIDP), abnormalities were predominant in the arms, whereas leg abnormalities predominated in axonal GBS...
October 17, 2016: Muscle & Nerve
Jingzi Zhong, Tiantian Xu, Gang Chen, Haixia Liao, Jiapeng Zhang, Dan Lan
Introduction Duchenne and Becker muscular dystrophies (DMD and BMD) are X-linked myopathies caused by mutations of the dystrophin gene. Methods Multiplex ligation-dependent probe amplification (MLPA) combined with next-generation sequencing (NGS) of the exons of the dystrophin gene were performed in 92 suspected DMD/BMD patients. Patients with negative results were subjected to additional muscle diseases panel tests. Results DNA rearrangements were detected in 65(70.65%) patients using MLPA. The deletions primarily clustered at exons 45-55, followed by exons 2-19...
October 17, 2016: Muscle & Nerve
Yaacov Anziska, Afsana Rahman
No abstract text is available yet for this article.
October 17, 2016: Muscle & Nerve
Anna Petryk, Lynda E Polgreen, Molly Grames, Dawn A Lowe, James S Hodges, Peter Karachunski
INTRODUCTION: Dystrophinopathies are X-linked muscle degenerative disorders that result in progressive muscle weakness complicated by bone loss. This study's goal was to evaluate feasibility and tolerability of whole body low intensity vibration (WBLIV) and its potential effects on muscle and bone in patients with Duchenne or Becker muscular dystrophy. METHODS: This 12-month pilot study included 5 patients (age 5.9-21.7 years) who used a low-intensity Marodyne LivMD plate vibrating at 30-90 Hz for 10 minutes/day for the first 6 months...
October 8, 2016: Muscle & Nerve
Amelia A Miramonti, Nathaniel D M Jenkins, Preeti D Oza, Joseph P Weir, Joel T Cramer
INTRODUCTION: This study examined relationships among mechanomyographic (MMG), electromyographic (EMG), and peak twitch torque (PTT) responses as well as test-retest reliability when recorded during recruitment curves in the soleus muscle. METHODS: PTT, EMG (M-wave, H-reflex), and MMG responses were recorded during recruitment curves in 16 subjects (age=24±2y) on 2 separate days. The sum of the M-wave and H-reflex (M+H) was calculated. Correlations among variables and test-retest reliability were determined...
October 8, 2016: Muscle & Nerve
Samuel L Buckner, Scott J Dankel, Kevin T Mattocks, Matthew B Jessee, J Grant Mouser, Brittany R Counts, Jeremy P Loenneke
In this paper we revisit a topic originally discussed in 1955, namely the lack of direct evidence that muscle hypertrophy from exercise plays an important role in increasing strength. To this day, long-term adaptations in strength are thought to be primarily contingent on changes in muscle size. Given this assumption, there has been considerable attention placed on programs designed to allow for maximization of both muscle size and strength. However, the conclusion that a change in muscle size affects a change in strength is surprisingly based on little evidence...
October 7, 2016: Muscle & Nerve
Lawrence R Robinson, Michael Christie, Sanjeev Nandedkar
No abstract text is available yet for this article.
October 6, 2016: Muscle & Nerve
Khosro Farhad, Thomas H Brannagan
No abstract text is available yet for this article.
October 4, 2016: Muscle & Nerve
Elena S Mazzone, Anna Mayhew, Jacqueline Montes, Danielle Ramsey, Lavinia Fanelli, Sally Dunaway Young, Rachel Salazar, Roberto De Sanctis, Amy Pasternak, Allan Glanzman, Giorgia Coratti, Matthew Civitello, Nicola Forcina, Richard Gee, Tina Duong, Marika Pane, Mariacristina Scoto, Maria Carmela Pera, Sonia Messina, Gihan Tennekoon, John W Day, Basil T Darras, Darryl C De Vivo, Richard Finkel, Francesco Muntoni, Eugenio Mercuri
INTRODUCTION: There is a growing need for a robust clinical measure to assess upper limb motor function in spinal muscular atrophy (SMA), as the available scales lack sensitivity at the extremes of the clinical spectrum. We report the development of the Revised Upper Limb Module (RULM), an assessment specifically designed for upper limb function in SMA patients. METHODS: An international panel with specific neuromuscular expertise performed a thorough review of scales currently available to assess upper limb function in SMA...
October 4, 2016: Muscle & Nerve
Cullen M O'Gorman, Travis G O'Brien, Andrea J Boon
INTRODUCTION: Ultrasound (US) evaluation of diaphragm thickness and contractility is an effective tool in neurogenic diaphragm dysfunction. There are limited data about the value of this technique in patients with myopathy. METHODS: We performed a retrospective chart review of cases with EMG-confirmed myopathy and real-time US evaluation of the diaphragm. Diaphragm thickness and thickening ratio (maximal inspiration/expiration) were measured. Demographic, imaging, pathology, and genetic data were reviewed, and the clinical diagnosis was recorded...
October 4, 2016: Muscle & Nerve
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