Congenital cervical spinal atrophy.

abstract：INTRODUCTION:Muscle fiber denervation increases with age, yet studies at the tissue level are sparse due to the challenging nature of establishing the relative role of regeneration and denervation. METHODS:Muscle biopsies were obtained from the vastus lateralis of 70 healthy men (aged 72 ± 6 years; range, 65-94). Mess...

journal_title：Muscle & nerve

authors： Soendenbroe C,Heisterberg MF,Schjerling P,Karlsen A,Kjaer M,Andersen JL,Mackey AL

更新日期：2019-10-01 00:00:00

abstract：:The motor end-plate fine structure and distribution of junctional acetylcholine receptors (AChRs) in patients with amyotrophic lateral sclerosis (ALS) were studied. Morphometric analysis was carried out in 74 end-plates (92 innervated and 47 denuded postsynaptic regions) from 10 patients with ALS. AChR was studied by ...

journal_title：Muscle & nerve

authors： Tsujihata M,Hazama R,Yoshimura T,Satoh A,Mori M,Nagataki S

更新日期：1984-03-01 00:00:00

abstract：:Immunologic syndromes are associated with hepatitis B viral (HBV) infection. However, mononeuropathy multiplex (MM), a syndrome in which immune factors may play a role, is rare in acute HBV infection. Few cases of MM associated with HBV infection have been reported. We report a case of acute HBV infection in which MM ...

journal_title：Muscle & nerve

authors： Cohen JA,Wilborn SL,Rector WG Jr,Golitz LE

更新日期：1990-03-01 00:00:00

abstract：:Muscle fatigue or neuromuscular disease may result in central activation failure during maximal voluntary contractions (MVCs). Superimposition of an electrically stimulated contraction during an MVC has been used to detect central activation failure. To determine the most sensitive means of quantitating central activa...

journal_title：Muscle & nerve

authors： Kent-Braun JA,Le Blanc R

更新日期：1996-07-01 00:00:00

abstract：:The mdx mouse, an animal model used to study Duchenne muscular dystrophy, has a nonsense mutation in exon 23 of the dystrophin gene which should result in a truncated protein that cannot be correctly localized at the sarcolemma of the muscle fibers. Immunohistochemical staining with antidystrophin antibodies has shown...

journal_title：Muscle & nerve

authors： Wilton SD,Dye DE,Laing NG

更新日期：1997-06-01 00:00:00

abstract：:Median neuropathies proximal to the wrist are uncommon and usually result from penetrating injuries, fracture dislocation of the distal humerus, or compression by fibrous bands. A 66-year-old man suffered a comminuted fracture of the proximal humerus after a fall. Electrodiagnostic studies revealed a severe proximal m...

journal_title：Muscle & nerve

authors： Veilleux M,Richardson P

更新日期：2000-03-01 00:00:00

abstract：:The physiological and phenotypic properties of motor units in the cat soleus muscle were studied after 4 months of inactivity induced by spinal cord isolation (SI). The soleus of some SI cats were stimulated for 30 min/day during an isometric (SI-I), shortening (SI-S), or lengthening (SI-L) phase of a simulated step c...

journal_title：Muscle & nerve

authors： Zhong H,Roy RR,Hodgson JA,Talmadge RJ,Grossman EJ,Edgerton VR

更新日期：2002-08-01 00:00:00

abstract：:We have previously demonstrated that patients with mitochondrial myopathies can benefit from short-term aerobic exercise training. In this study, we compared the responses to short-term aerobic training of patients with mitochondrial myopathies, patients with nonmetabolic myopathies, and sedentary normal subjects. Tra...

journal_title：Muscle & nerve

authors： Taivassalo T,De Stefano N,Chen J,Karpati G,Arnold DL,Argov Z

更新日期：1999-09-01 00:00:00

abstract：:Sialorrhea is frequently a socially disabling symptom in patients with bulbar amyotrophic lateral sclerosis (ALS). In this open-label prospective study, we report the effect of botulinum toxin A (Botox) injection into the parotid glands in 10 patients with bulbar ALS and socially disabling sialorrhea. We applied three...

journal_title：Muscle & nerve

authors： Verma A,Steele J

更新日期：2006-08-01 00:00:00

abstract：BACKGROUND:Limited data exist regarding myopathies with early or prominent dysphagia. METHODS:A retrospective study was performed (January 2003 to August 2019) to identify myopathy patients in whom dysphagia was the initial symptom or was disproportionately severe compared with limb weakness. RESULTS:Thirty-two patie...

journal_title：Muscle & nerve

authors： Triplett JD,Pinto MV,Hosfield EA,Milone M,Liewluck T

更新日期：2020-09-01 00:00:00

abstract：INTRODUCTION:C8-root impingement by C7/T1 lesions on neuroimaging studies is not consistently observed in C8 radiculopathy. We hypothesized that C7 or T1 root lesions (with a pre- or postfixed plexus) or cervical myelopathy might explain some "C8 radiculopathies" without C8 root compression. METHODS:Retrospective anal...

journal_title：Muscle & nerve

authors： Hehir MK,Figueroa JJ,Zynda-Weiss AM,Stanton M,Logigian EL

更新日期：2012-06-01 00:00:00

abstract：:The protein compositions of subcellular fractions of muscle obtained from 17 Duchenne dystrophy patients, 15 disease controls (10 different primary myopathies, 5 spinal muscular atrophy patients), and 10 normals were examined by polyacrylamide gel electrophoresis. The gels were stained with Coomassie Brilliant Blue an...

journal_title：Muscle & nerve

authors： Niebroj-Dobosz I,Kornguth S,Schutta H,Siegel FL,Hausmanowa-Petrusewicz I

更新日期：1989-04-01 00:00:00

abstract：:The relationship between motor function and muscle strength in patients with spinal muscular atrophy (SMA) is still controversial. In 120 genetically proven SMA patients, aged 5 years or older, we measured muscle strength in the arms and legs by a hand-held dynamometer, forced vital capacity by a spirometer, and the t...

journal_title：Muscle & nerve

authors： Merlini L,Bertini E,Minetti C,Mongini T,Morandi L,Angelini C,Vita G

更新日期：2004-04-01 00:00:00

abstract：:Muscle of donor origin was formed after implantation of H-2Kb-tsA58 muscle precursor cells (mpc) into irradiated mdx nu/nu mouse muscles. A series of injections of the myotoxin, notexin, which destroys mature muscle fibers but spares muscle precursor cells and other tissues, was made into the mpc-injected muscles, lea...

journal_title：Muscle & nerve

authors： Gross JG,Morgan JE

更新日期：1999-02-01 00:00:00

abstract：:Large muscle genes are often sequenced using complementary DNA (cDNA) made from muscle messenger RNA (mRNA) to reduce the cost and workload associated with sequencing from genomic DNA. Two potential barriers are the availability of a frozen muscle biopsy, and difficulties in detecting nonsense mutations due to nonsens...

journal_title：Muscle & nerve

authors： Waddell LB,Monnier N,Cooper ST,North KN,Clarke NF

更新日期：2011-08-01 00:00:00

abstract：:Electrodiagnostic evaluation of patients with suspected polyneuropathy is useful for detecting and documenting peripheral abnormalities, identifying the predominant pathophysiology, and determining the prognosis for certain disorders. The electrodiagnostic classification of polyneuropathy is associated with morphologi...

journal_title：Muscle & nerve

authors： Donofrio PD,Albers JW

更新日期：1990-10-01 00:00:00

abstract：:We measured acetylcholinesterase (AChE) in the non-endplate region of rat muscle, documenting its intrinsic activity within muscle fibers, as well as the extrinsic level in the capillaries and endomysium. When each muscle was considered as a whole, intrinsic AChE activity detected within the fibers was stronger in the...

journal_title：Muscle & nerve

authors： Nakano S,Akiguchi I,Yasuda Y,Nakamura S,Kameyama M,Kimura J

更新日期：1990-08-01 00:00:00

abstract：:Rhabdomyolysis, a syndrome of skeletal muscle breakdown with leakage of muscle contents, is frequently accompanied by myoglobinuria, and if sufficiently severe, acute renal failure with potentially life-threatening metabolic derangements may ensue. A diverse spectrum of inherited and acquired disorders affecting muscl...

journal_title：Muscle & nerve

authors： Warren JD,Blumbergs PC,Thompson PD

更新日期：2002-03-01 00:00:00

abstract：INTRODUCTION:In this study we investigated the influence of gender and obesity on electrical current thresholds in an attempt to optimize the application of skeletal muscle electrical stimulation (ES) in clinical practice. METHODS:Thirty-two obese and 35 age-matched, non-obese men and women received graded ES to the q...

journal_title：Muscle & nerve

authors： Maffiuletti NA,Morelli A,Martin A,Duclay J,Billot M,Jubeau M,Agosti F,Sartorio A

更新日期：2011-08-01 00:00:00

abstract：:Abnormalities of GM2 ganglioside metabolism owing to hexosaminidase A (Hex A) deficiency have been associated with ALS phenotypes. The clinical features described in these ALS patients with Hex A deficiency include early onset, positive family history, and/or long disease duration. In an attempt to determine prospecti...

journal_title：Muscle & nerve

authors： Gudesblatt M,Ludman MD,Cohen JA,Desnick RJ,Chester S,Grabowski GA,Caroscio JT

更新日期：1988-03-01 00:00:00

abstract：:EDL muscles from normal and dystrophic (dy2j) mice of various ages were examined. Muscles were divided into three groups according to age: 7 to 14 days postnatal, 16 to 21 days postnatal, and 6 months old, to assess age and/or phenotype related differences in the muscle response to caffeine or high K+. The response of...

journal_title：Muscle & nerve

authors： Dangain J,Neering IR

更新日期：1993-01-01 00:00:00

abstract：:Patients with prior poliomyelitis may experience muscle function deterioration decades after onset of disease. The present study is aimed at describing electromyographic and morphometric evidence of muscular compensation and of on-going muscular instability. Ten subjects 42-62 years of age with onset of polio 25-52 ye...

journal_title：Muscle & nerve

authors： Einarsson G,Grimby G,Stålberg E

更新日期：1990-02-01 00:00:00

abstract：INTRODUCTION:The involvement of small myelinated fibers in the early stages of diabetic neuropathy (DN) has been suggested, but few objective methods can identify minimal neuropathy. The purpose of this study was to determine whether there are differences in contact heat evoked potential (CHEP) parameters between healt...

journal_title：Muscle & nerve

authors： Wong MC,Chung JW

更新日期：2011-12-01 00:00:00

abstract：:To define numerically the clinical severity of facioscapulohumeral muscular dystrophy (FSHD), we developed a protocol that quantifies muscle weakness by combining the functional evaluation of six muscle groups affected in this disease. To validate reproducibility of the protocol, 69 patients were recruited. Each patie...

journal_title：Muscle & nerve

authors： Lamperti C,Fabbri G,Vercelli L,D'Amico R,Frusciante R,Bonifazi E,Fiorillo C,Borsato C,Cao M,Servida M,Greco F,Di Leo R,Volpi L,Manzoli C,Cudia P,Pastorello E,Ricciardi L,Siciliano G,Galluzzi G,Rodolico C,Santoro L

更新日期：2010-08-01 00:00:00

abstract：INTRODUCTION:Mutations in gap junction protein beta 1 (GJB1) on the X chromosome represent one of the most common causes of hereditary neuropathy. We assessed manifestations associated with a rare 3' untranslated region mutation (UTR) of GJB1 in a large family with X-linked Charcot-Marie-Tooth disease (CMTX). METHODS:...

journal_title：Muscle & nerve

authors： Chen DH,Ma M,Scavina M,Blue E,Wolff J,Karna P,Dorschner MO,Raskind WH,Bird TD

更新日期：2018-05-01 00:00:00

abstract：:The intramembrane particle (IMP) profile of control and dystrophic (Bio 14.6) hamster cardiac muscle plasma membrane was assessed in freeze-fracture replicas to determine whether this animal model of muscular dystrophy exhibits the same membrane characteristics found in skeletal muscle from other more thoroughly studi...

journal_title：Muscle & nerve

authors： Graham KA,Shivers RR,Atkinson BG

更新日期：1984-09-01 00:00:00

abstract：:We report a family with familial amyloid polyneuropathy (FAP), showing an early-onset and a fatal outcome before age 30. Transthyretin (TTR) gene analysis showed one point mutation (T-->C change) in the second base of codon 55, and the corresponding amino acid substitution of proline (Pro) for leucine (Leu) was confir...

journal_title：Muscle & nerve

authors： Yamamoto K,Hsu SP,Yoshida K,Ikeda S,Nakazato M,Shiomi K,Cheng SY,Furihata K,Ueno I,Yanagisawa N

更新日期：1994-06-01 00:00:00

abstract：:Chronic and acute administration of epinephrine or related sympathomimetic agents are typically prescribed for the treatment of clinical disorders such as hypotension, anaphylactic and allergic reactions, and bronchial asthma. In addition to its effects on these infirmaties and on carbohydrate metabolism, epinephrine ...

journal_title：Muscle & nerve

authors： Williams JH,Barnes WS

更新日期：1989-12-01 00:00:00

abstract：INTRODUCTION:We sought to determine the specificity of compound muscle action potential (CMAP) durations and amplitudes in a large critical illness neuromyopathy (CINM) cohort relative to controls with other neuromuscular conditions. METHODS:Fifty-eight patients with CINM who had been seen over a 17-year period were r...

journal_title：Muscle & nerve

authors： Kramer CL,Boon AJ,Harper CM,Goodman BP

更新日期：2018-03-01 00:00:00

abstract：:Single muscle fibers were prepared from biopsy specimens of Duchenne muscular dystrophy (DMD), normal, and neuromuscular disease controls. Single muscle cells were classified as type 1, type 2, or intermediate by the skinned fiber method. The intermediate fiber was most abundant in DMD, comprising 29% of fibers examin...

journal_title：Muscle & nerve

authors： Takagi A,Ishiura S,Nonaka I,Sugita H

更新日期：1982-05-01 00:00:00