Functional significance of upper and lower motor neuron impairment in amyotrophic lateral sclerosis.

abstract：:The degree of upper extremity active range of motion provided by an admittance control robot compared with a commercially available passive arm support for individuals with DMD who have limited arm function was investigated in this study. The reachable workspace evaluation was used to assess active range of motion pro...

journal_title：Muscle & nerve

authors： Corrigan MC,Foulds RA

更新日期：2020-06-01 00:00:00

abstract：:We describe a consanguineous family that had progressive myopathy with rimmed vacuole (RV) formation and amyloid deposition. Patient 1 is a 71-year-old woman with muscle atrophy in the lumbar girdle and lower extremities. Patient 2 is a 40-year-old man (the son of Patient 1) with fatty changes in the biceps femoris mu...

journal_title：Muscle & nerve

authors： Kojima Y,Sakai K,Ishida C,Asaka T,Hamaguchi T,Nozaki I,Fukushima K,Tsuchiya A,Kametani F,Yazaki M,Okino S,Yamada M

更新日期：2009-09-01 00:00:00

abstract：INTRODUCTION:Lower limb strength has never been characterized separately for late-onset and adult myotonic dystrophy type 1 (DM1) phenotypes. METHODS:The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 asse...

journal_title：Muscle & nerve

authors： Petitclerc É,Hébert LJ,Mathieu J,Desrosiers J,Gagnon C

更新日期：2017-07-01 00:00:00

abstract：:Comparison was made between simultaneous recordings of the electromyogram (EMG) and acoustic myogram (AMG) signals and the torque produced by the lumbar erectores spinae muscles of three healthy subjects while they performed isometric contractions. Repeatability of each signal was studied. The AMG had a relationship t...

journal_title：Muscle & nerve

authors： Stokes IA,Moffroid MS,Rush S,Haugh LD

更新日期：1988-04-01 00:00:00

abstract：INTRODUCTION:In this study we investigated the interaction between adipose tissue-derived stem cells (ASCs) and myoblasts in co-culture experiments. METHODS:Specific inductive media were used to differentiate ASCs in vitro into a Schwann cell-like phenotype (differentiated adipose tissue-derived stem cells, or dASCs) ...

journal_title：Muscle & nerve

authors： El-Habta R,Kingham PJ,Backman LJ

更新日期：2018-02-01 00:00:00

abstract：:We reviewed results of immunotherapy in patients with demyelinating motor neuropathy (DMN), and found that patients over 50 years of age at onset responded poorly, and younger patients responded variably to intervention. We suggest that patients with DMN be given a guarded prognosis, particularly if >50 years of age a...

journal_title：Muscle & nerve

authors： Markson L,Janzen D,Bril V

更新日期：1998-12-01 00:00:00

abstract：INTRODUCTION:In mitochondrial disease, it is likely that energy substrate depletion leads to paralysis of ATPase-dependent pumps, resulting in membrane depolarization. Axonal depolarization has been demonstrated in a crisis, but not in the resting state. We, therefore, stressed axons using ischemia to see if this would...

journal_title：Muscle & nerve

authors： Ng K,Kumar KR,Sue C,Burke D

更新日期：2013-05-01 00:00:00

abstract：:We observed a marked prolongation of the transcranially evoked silent period during continuous intrathecal administration of high doses of the gamma-aminobutyric acid (GABA)B receptor agonist baclofen in a patient with generalized dystonia. Size of motor evoked potentials and central conduction time remained unchanged...

journal_title：Muscle & nerve

authors： Siebner HR,Dressnandt J,Auer C,Conrad B

更新日期：1998-09-01 00:00:00

abstract：:Somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) to transcranial and spinal stimulation from upper and lower limb muscles were elicited in 13 patients with syringomyelia. Seven had an associated Chiari type I anomaly. Diagnosis was confirmed by MRI. In 5 cases, SEPs and MEPs were performed bef...

journal_title：Muscle & nerve

authors： Nogués MA,Pardal AM,Merello M,Miguel MA

更新日期：1992-09-01 00:00:00

abstract：:We examined and compared a mechanosensitive human sural neuroma and a contralateral sural nerve collected simultaneously from a patient involved in a diabetic neuropathy research protocol. Using indirect immunofluorescence staining. we identified a striking difference in the content within axons of two neuropeptides, ...

journal_title：Muscle & nerve

authors： Zochodne DW,Theriault M,Sharkey KA,Cheng C,Sutherland G

更新日期：1997-07-01 00:00:00

abstract：INTRODUCTION:The minimal clinically important difference (MCID) is the smallest outcome change that has clinical significance. Its use has not been established in the study of myasthenia gravis (MG). METHODS:Patients from a published intravenous immunoglobulin (IVIg) vs. placebo study were studied. One anchor-based an...

journal_title：Muscle & nerve

authors： Katzberg HD,Barnett C,Merkies IS,Bril V

更新日期：2014-05-01 00:00:00

abstract：INTRODUCTION:Amyloidosis derived from transthyretin (TTR) molecules is typically caused by mutations of the TTR gene. METHODS:We describe an elderly patient with a severe length-dependent polyneuropathy that unexpectedly proved to be caused by wild-type transthyretin amyloidosis. RESULTS:The diagnosis was made by mus...

journal_title：Muscle & nerve

authors： Lam L,Margeta M,Layzer R

更新日期：2015-07-01 00:00:00

abstract：INTRODUCTION:Duration of training to reliably measure nerve cross-sectional area with ultrasound is unknown. METHODS:A retrospective review was performed of ultrasound data, acquired and recorded by 2 examiners-an expert and either a trainee with 2 months (novice) or a trainee with 12 months (experienced) of experienc...

journal_title：Muscle & nerve

authors： Garcia-Santibanez R,Dietz AR,Bucelli RC,Zaidman CM

更新日期：2018-02-01 00:00:00

abstract：:Multifocal motor neuropathy (MMN) is a chronic, immune-mediate, peripheral myelinopathy. Inherent in its name, MMN implies involvement of two or more motor nerves. We report three patients with weakness and partial motor conduction block restricted to a single nerve and localized to sites that are not at risk for entr...

journal_title：Muscle & nerve

authors： Felice KJ,Goldstein JM

更新日期：2002-05-01 00:00:00

abstract：:Beta-chemokines attract and activate T cells and monocytes and have a key role in chronic inflammation. Certain beta-chemokines, such as monocyte chemoattractant protein-1 (MCP-1), have been reported to be upregulated in the idiopathic inflammatory myopathies (IIM). We studied the distribution of beta-chemokine recept...

journal_title：Muscle & nerve

authors： De Paepe B,De Bleecker JL

更新日期：2005-05-01 00:00:00

abstract：:Motoneurons can be activated both reflexly and antidromically following electrical stimulation of peripheral nerves. These H reflexes and F waves are clinically useful responses which interface at the level of the peripheral nerves and the spinal cord. Because these responses are commonly employed in the electrodiagno...

journal_title：Muscle & nerve

authors： Fisher MA

更新日期：1992-11-01 00:00:00

abstract：:The axonal distribution of voltage-dependent Na+ channels was determined during inflammatory demyelinating disease of the peripheral nervous system. Experimental allergic neuritis was induced in Lewis rats by active immunization. In diseased spinal roots Na+ channel immunofluorescence at many nodes of Ranvier changed ...

journal_title：Muscle & nerve

authors： Novakovic SD,Levinson SR,Schachner M,Shrager P

更新日期：1998-08-01 00:00:00

abstract：:Motor unit (MU) number and size estimates were obtained from the human flexor digiti minimi muscle using histological methods. Ten adult fresh cadavers (33-74 years old) were used. The number of MUs was 130 +/- 15 and the MU size was 108 +/- 10. These values are similar to those reported for other hypothenar muscles, ...

journal_title：Muscle & nerve

authors： Santo Neto H,de Carvalho VC,Marques MJ

更新日期：1998-01-01 00:00:00

abstract：:In this study some particular and puzzling aspects of amyotrophic lateral sclerosis (ALS) are discussed, with an emphasis on the role of neocortical evolution. Other issues explored include the nature of the clinical deficit in ALS; anatomical and evolutionary aspects of the neocortex, motor cortex, and corpus callosu...

journal_title：Muscle & nerve

authors： Eisen A

更新日期：2009-08-01 00:00:00

abstract：:The spectrum of neuromuscular disorders among intensive care unit (ICU) patients has shifted toward disorders acquired within the ICU and away from "traditional" neuromuscular disorders that lead to ICU admission. We sought to assess this spectrum by determining the causes and relative frequencies of neuromuscular dis...

journal_title：Muscle & nerve

authors： Lacomis D,Petrella JT,Giuliani MJ

更新日期：1998-05-01 00:00:00

abstract：INTRODUCTION:The neuropathy in patients with neurofibromatosis type 2 (NF2) is difficult to quantify and follow up. In this study we compared 3 methods that may help assess motor axon pathology in NF2 patients. METHODS:Nerve conduction studies in median nerves were supplemented by deriving motor unit number estimates ...

journal_title：Muscle & nerve

authors： Farschtschi S,Gelderblom M,Buschbaum S,Bostock H,Grafe P,Mautner VF

更新日期：2017-03-01 00:00:00

abstract：INTRODUCTION:Conduction block (CB) has been included in the Rajabally criteria for axonal Guillain-Barré syndrome (GBS). Because the nerve roots may be affected early in GBS, detection of proximal CB by the triple stimulation technique (TST) can be useful. METHODS:We describe TST findings in 2 patients who presented w...

journal_title：Muscle & nerve

authors： Taieb G,Grapperon AM,Duclos Y,Franques J,Labauge P,Renard D,Yuki N,Attarian S

更新日期：2015-12-01 00:00:00

abstract：INTRODUCTION:Cachexia increases morbidity and mortality of cancer patients. The progressive loss of muscle mass negatively affects physical function and quality of life. We previously showed reduced muscle insulin-like growth factor-1 (IGF-1) expression and enhanced myostatin signaling in tumor-bearing animals. This st...

journal_title：Muscle & nerve

authors： Bonetto A,Penna F,Aversa Z,Mercantini P,Baccino FM,Costelli P,Ziparo V,Lucia S,Rossi Fanelli F,Muscaritoli M

更新日期：2013-09-01 00:00:00

abstract：INTRODUCTION:Balance impairment contributes to gait dysfunction, falls, and reduced quality of life in adults with Charcot-Marie-Tooth disease (CMT) but has been minimally examined in pediatric CMT. METHODS:The CMT Pediatric Scale (CMTPedS) was administered to 520 children with CMT. Associations between balance functi...

journal_title：Muscle & nerve

authors： Estilow T,Glanzman AM,Burns J,Harrington A,Cornett K,Menezes MP,Shy R,Moroni I,Pagliano E,Pareyson D,Bhandari T,Muntoni F,Laurá M,Reilly MM,Finkel RS,Eichinger KJ,Herrmann DN,Troutman G,Bray P,Halaki M,Shy ME,Yu

更新日期：2019-09-01 00:00:00

abstract：:We describe a 58-year-old male with a few years history of multifocal weakness in the upper limbs with minimal to absent sensory complaints. He was diagnosed as having multiple compressive neuropathies, which required repeated decompressive surgeries. Electrodiagnostic studies prior to diagnosis were limited to a few ...

journal_title：Muscle & nerve

authors： Beydoun SR

更新日期：1998-06-01 00:00:00

abstract：INTRODUCTION:The 6-minute walk test (6MWT) is a well-established clinical assessment of functional endurance, validated as a measure of walking ability in spinal muscular atrophy (SMA). The current availability of disease-modifying therapies for SMA indicates a growing need for normative reference data to compare SMA p...

journal_title：Muscle & nerve

authors： Goodwin AM,Cornett KMD,McKay MJ,Burns J,Garber CE,De Vivo DC,Montes J

更新日期：2020-03-01 00:00:00

abstract：:Eleven patients with previous polio were studied. The concentration of energy-related metabolites and energy charge was measured from the vastus lateralis muscle, as was isometric muscle strength of knee extension. Cross-sectional area of the quadriceps femoris muscle was calculated from magnetic resonance imaging. Re...

journal_title：Muscle & nerve

authors： Nordgren B,Falck B,Stålberg E,Ronquist G,Waldenström A,Ahlström H,Hemmingson A

更新日期：1997-11-01 00:00:00

abstract：:Muscle cramps are difficult to study scientifically because of their spontaneity and unpredictability. Various laboratory techniques to induce muscle cramps have been explored but the best technique for inducing cramps is unclear. Electrical stimulation appears to be the most reliable, but there is a perception that i...

journal_title：Muscle & nerve

authors： Miller KC,Knight KL

更新日期：2007-11-01 00:00:00

abstract：:Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

journal_title：Muscle & nerve

authors： Kleopa KA,Barchi RL

更新日期：2002-09-01 00:00:00

abstract：INTRODUCTION:Despite the existence of guidelines, painful neuropathy is often inappropriately treated. We sought to determine the effectiveness of a clinical decision support system on guideline-recommended medication use. METHODS:We randomized neurology providers, stratified by subspecialty, to a best practice alert ...

journal_title：Muscle & nerve

authors： Reynolds EL,Burke JF,Banerjee M,Callaghan BC

更新日期：2020-05-01 00:00:00