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Muscle & Nerve

Anna Bystrup Jacobsen, Hugh Bostock, Hatice Tankisi
INTRODUCTION: The objective of this study was to evaluate a recently developed motor unit number estimation (MUNE) method, MScanFit MUNE (MScan), as a measure of disease progression in amyotrophic lateral sclerosis (ALS) compared to compound muscle action potential (CMAP) amplitude and two traditional MUNE methods. METHODS: ALS patients were evaluated clinically by ALS Functional Rating Scale-Revised (ALSFRS-R). MScan, multiple point stimulation MUNE (MPS) and motor unit number index (MUNIX) were performed in the abductor pollicis brevis (APB) muscle at baseline (27 patients), 4 months (23 patients) and 8 months (16 patients)...
July 19, 2018: Muscle & Nerve
Miguel M Pinto, Soledad Monges, Edoardo Malfatti, Fabiana Lubieniecki, Xavière Lornage, Laura Alias, Clémence Labasse, Angéline Madelaine, Michel Fardeau, Jocelyn Laporte, Eduardo F Tizzano, Norma B Romero
INTRODUCTION: Mutations in the EXOSC3 gene are responsible for type 1 pontocerebellar hypoplasia, an autosomal recessive congenital disorder characterized by cerebellar atrophy, developmental delay and anterior horn motor neuron degeneration. Muscle biopsies of these patients often show characteristics resembling classic spinal muscle atrophy, but to date no distinct features have been identified. METHODS: Clinical data and muscle biopsy findings of three unrelated patients with EXOSC3 mutations are described...
July 19, 2018: Muscle & Nerve
Nelly Béchir, Émilie Pecchi, Christophe Vilmen, Monique Bernard, David Bendahan, Benoît Giannesini
INTRODUCTION: Postnatal activin/myostatin type IIB receptor (ActRIIB) blockade increases skeletal muscle mass and strength, but also increases muscle fatigability and impairs oxidative metabolism. The aim of this study was to determine in vivo whether this increased fatigability is due to energy supply limitation. METHODS: The impact of 8-week ActRIIB blockade with soluble receptor (sActRIIB-Fc) upon muscle function and ATP fluxes was investigated noninvasively using multimodal magnetic resonance and indirect calorimetry measurements in wild-type mice...
July 19, 2018: Muscle & Nerve
Hyunjin Kim, Young-Min Lim, Eun-Jae Lee, Yeo Jin Oh, Kwang-Kuk Kim
INTRODUCTION: Although thymectomy is an important therapeutic option for myasthenia gravis (MG), factors predicting remission after thymectomy are not well known. METHODS: We retrospectively reviewed acetylcholine receptor antibody (AChR-Ab)-positive MG patients who had undergone thymectomy. Prognostic factors predicting remission were investigated. Changes in AChR-Ab titer before and after thymectomy were also evaluated. RESULTS: Among the 179 patients, 52...
July 18, 2018: Muscle & Nerve
Maxwell Greene, Catherine Lomen-Hoerth
No abstract text is available yet for this article.
July 18, 2018: Muscle & Nerve
Mana Higashihara, Masahiro Sonoo, Akihiko Ishiyama, Yu Nagashima, Kohji Matsumoto, Haruo Uesugi, Madoka Mori-Yoshimura, Miho Murata, Shigeo Murayama, Hirofumi Komaki
INTRODUCTION: Needle EMG has been an important diagnostic tool, although discomfort may limit its use in some children. We investigated the diagnostic utility of the Clustering Index (CI) method, a quantitative analysis for surface EMG (SEMG), in children. METHODS: SEMG was recorded from the tibialis anterior muscle. Discriminant analysis between neurogenic and myopathic patients was performed for whole epochs using the CI and area values. RESULTS: Forty-five children (29 myopathies, 16 neurogenic disorders; age 9...
July 17, 2018: Muscle & Nerve
Yu-Ichi Noto, Nidhi Garg, Tiffany Li, Hannah C Timmins, Susanna B Park, Kazumoto Shibuya, Nortina Shahrizaila, William Huynh, José M Matamala, Thanuja Dharmadasa, Con Yiannikas, Steve Vucic, Matthew C Kiernan
INTRODUCTION: The present study explored potential diagnostic markers of nerve ultrasound in differentiating amyotrophic lateral sclerosis (ALS) from mimic disorders. METHODS: Ultrasound of the median, ulnar and tibial nerves was undertaken in 53 ALS patients, 32 patients with ALS-mimic disorders and 30 controls. Nerve cross-sectional area (CSA) and distal-proximal ratios were calculated. RESULTS: The median nerve CSA in the upper arm was decreased (7...
July 17, 2018: Muscle & Nerve
Pinar Gencpinar, Gamze Çelmeli, Özgür Duman, Şenay Haspolat, Hilmi Uysal
INTRODUCTION: The aim of this study was to determine alterations in axonal excitability in tibial nerve as compared to median nerve axonal excitability in patients with diabetic polyneuropathy. METHODS: Six patients with diabetic polyneuropathy and 10 patients with diabetes mellitus without polyneuropathy were enrolled. RESULTS: In comparison to diabetic patients without polyneuropathy, the tibial nerve strength-duration time constant was significantly longer and supernormality was lower in those with polyneuropathy...
July 17, 2018: Muscle & Nerve
Alberto M Cappellari, Francesca Tiberio, Gianfranco Alicandro, Diego Spagnoli, Nadia Grimoldi
INTRODUCTION: We investigated the possible role of intercostal surgical neurolysis in relieving chronic neuropathic pain refractory to other non-surgical treatments in patients with postsurgical thoracic pain. METHODS: We retrospectively collected clinical data on patients referred to the Neurosurgery Unit of Policlinic Hospital of Milan. Ten patients (age range: 20-68 years) suffering from neuropathic pain for at least two months following thoracic surgery underwent intercostal neurolysis...
July 11, 2018: Muscle & Nerve
Emily K Plowman, Lauren Tabor-Gray, K Michelle Rosado, Terrie Vasilopoulos, Raele Robison, Jennifer L Chapin, Joy Gaziano, Tuan Vu, Clifton Gooch
INTRODUCTION: The purpose of this study was to determine the impact of an in home expiratory muscle strength training (EMST) program on pulmonary, swallow and cough function in individuals with amyotrophic lateral sclerosis (ALS). METHODS: EMST was tested in a prospective, single center, double-blind, randomized, controlled trial in 48 ALS individuals who completed eight weeks of either active-EMST (n=24) or sham-EMST (n=24). The primary outcome to assess treatment efficacy was change in maximum expiratory pressure (MEP)...
July 7, 2018: Muscle & Nerve
Xiaoyan Li, Ya Zong, Cliff S Klein, Ping Zhou
INTRODUCTION: This study performed motor unit number estimation (MUNE) of the abductor hallucis (AH) muscle from 16 healthy control subjects based on the compound muscle action potential (CMAP) scan. METHODS: Muscle responses to electrical stimuli ranging from sub-threshold to supramaximal intensity were recorded and MUNE was determined from a model of the responses (MscanFit program). RESULTS: The average CMAP amplitude and MUNE of the AH for the right and left sides combined were 19...
July 7, 2018: Muscle & Nerve
Jennifer Tinklenberg, Margaret Beatka, James Lw Bain, Emily M Siebers, Hui Meng, R Scott Pearsall, Michael W Lawlor, Danny A Riley
INTRODUCTION: Difficulty in modeling congenital contractures (deformities of muscle-tendon unit development that include shortened muscles and lengthened tendons) has limited research of new treatments. METHODS: Early immobilization of the ankle in prepuberal mice was used to produce deformities similar to congenital contractures. Stretch treatment, electrostimulation, and local intramuscular injection of a follistatin analog (FST-288, Acceleron Pharma) were assessed as therapeutic interventions for these deformities...
July 7, 2018: Muscle & Nerve
Christian A Pitcher, Catherine M Elliott, Jane P Valentine, Katherine Stannage, Sian A Williams, Peter J Shipman, Siobhán L Reid
INTRODUCTION: This study aims to determine the lower limb morphological characteristics of skeletal muscle of ambulant children with spastic cerebral palsy (CP) and typically developing (TD) children. METHODS: Seventeen children with spastic diplegic CP (10 males, 5-12 years, Gross Motor Function Classification System (GMFCS) I & II) and 19 TD children (8 males, 5-11 years) underwent lower limb T1 -weighted MRI. Morphological characteristics of the triceps surae including muscle volume, anatomical cross-sectional area, muscle length and subcutaneous adipose tissue were digitally quantified, and the proportional distribution calculated...
July 7, 2018: Muscle & Nerve
Mohammad Alrajeh, David C Preston
INTRODUCTION: The aim of this study was to evaluate the value of high resolution ultrasound (HRUS) in patients with ulnar neuropathy whose electrophysiology displayed an axonal, non-localizing pattern. METHODS: A prospective study of patients referred to an EMG laboratory for ulnar neuropathy was performed. Of 56 patients with clinical and electrodiagnostic (EDX) evidence of ulnar neuropathy, twelve patients were identified with non-localizing electrophysiology who subsequently underwent HRUS of the ulnar nerve...
July 7, 2018: Muscle & Nerve
Angela Bowman, Stephan Rudolfer, Peter Weller, Jeremy D P Bland
INTRODUCTION: Many prognostic factors have been studied in carpal tunnel decompression but most studies consider only a subset of variables. METHODS: 3332 operations were used to develop prognostic models and 885 operations for validation. Outcome recorded on a Likert scale was dichotomized into success or failure. Modeling was performed with both logistic regression and artificial neural networks using 87 candidate variables. RESULTS: Both approaches produced predictive multivariate models for outcome with areas under a receiver operating characteristic curve of 0·7 in the validation dataset...
July 7, 2018: Muscle & Nerve
Marlena Wosiski-Kuhn, Miles S Lyon, James Caress, Carol Milligan
With the emerging popularity of immune-modulatory therapies to treat human diseases there is a need to step back from hypotheses aimed at assessing a condition in a single-system context and instead take into account the disease pathology as a whole. In complex diseases, such as amyotrophic lateral sclerosis (ALS), the use of these therapies to treat patients has been largely unsuccessful and likely premature given our lack of understanding of how the immune system influences disease progression and initiation...
July 6, 2018: Muscle & Nerve
Miles Lyon, Marlena Wosiski-Kuhn, Rachel Gillespie, James Caress, Carol Milligan
Amyotrophic lateral sclerosis (ALS) is a severely debilitating disease characterized by progressive degeneration of motor neurons. Charcot first described ALS in 18691 ; however, its pathogenesis remains unknown, and effective treatments remain elusive. It is apparent that new paradigms must be investigated to understand the effectors of ALS, including inflammation, immune responses, and the body's response to stress and injury. Here, we discuss the potential role of the immune system in ALS pathogenesis and critically review evidence from patient and animal studies...
July 6, 2018: Muscle & Nerve
Jordan Kamel, Rory Knight-Sadler, Mark Cook, Leslie Roberts
INTRODUCTION: Single fiber F-waves (SFF-waves) is a technique that assesses the entire length of single motor fibers using a concentric needle. We investigate its utility in detecting early diabetes-related neuropathy, and compare this to conventional surface F-waves (CF-waves). METHODS: 16 patients with diabetes and either no neuropathy or mild neuropathy were assessed and compared to 16 age and height matched control participants. RESULTS: Both CF-waves and SFF-waves were abnormal in all five patients who had mild neuropathy...
July 5, 2018: Muscle & Nerve
Chafic Karam, Diana Dimitrova
No abstract text is available yet for this article.
June 26, 2018: Muscle & Nerve
Khosro Farhad, Anne Louise Oaklander
No abstract text is available yet for this article.
June 25, 2018: Muscle & Nerve
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