bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nonviral intranuclear inclusions in a nerve biopsy: An ultrastructural study
Authors: Vallat J. M. , Leboutet M. J. , Loubet A. , Corvisier N. , Dumas M. 
Published: January 2005

AbstractDuring the ultrastructural study of a nerve biopsy from a diabetic patient suffering from chronic polyradiculoneuritis, we observed that a significant number of endoneural fibroblasts had abnormal nuclei. They contained a round, centrally located inclusion consisting of numerous irregularly arranged filaments approximately 15 nm in diameter. No such lesion in peripheral nerve has previously been reported. Interpretation of these abnormalities is difficult.
Treatment of “permanent” muscle weakness in familial hypokalemic periodic paralysis
Authors: Dalakas Marinos C. , Engel W. King 
Published: January 2005

AbstractThree patients with Hypokalemic Periodic Paralysis (HOPP)‐associated progressive interattack muscle weakness, who became unresponsive or worsened by acetazolamide, responded favorably to dichloro‐phenamide, a more potent carbonic anhydrase inhibitor. Dichloro‐phenamide in single‐blind placebo‐controlled trials, considerably improved functional strength in two of the patients and had a moderate but definite effect in the third. Muscle groups graded 4/5 (MRC scale) returned to normal; very weak (0–3/5) atrophic muscles, improved to a minor degree. In one patient with acetazolamide‐resistant paralytic attacks, dichlorophenamide also diminished the frequency and severity of the acute attacks. Dichlorophenamide had, in the present study, less effect than acetazolamide in reducing serum HCO and elevating Cl−. Its effectiveness may be related to the degree of sensitivity of certain HOPP patients to alterations of Cl− and/or HCO serum levels or to a different action of the drug unrelated to carbonic anhydrase inhibition or acidosis. Dichlorophenamide should be considered as an alternate to acetazolamide in the treatment of patients with HOPP‐associated interattack muscle weakness who have become unresponsive or worsened by acetazolamide.
Hypoxanthine and McArdle disease: A clue to metabolic stress in the working forearm
Authors: Brooke Michael H. , Patterson Victor H. , Kaiser Kenneth K. 
Published: January 2005

AbstractForearm exercise in a patient with myophosphorylase deficiency resulted in abnormally high levels of hypoxanthine in the venous blood. The post‐exercise hypoxanthine response may reflect ATP depletion in the muscle and provides a useful screening test in muscle diseases with abnormal energy metabolism.
Prolonged electromechanical coupling time intervals in skeletal muscle of pigs susceptible to malignant hyperthermia
Authors: Nelson Thomas E. , Flewellen Eugene H. , Arnett David W. 
Published: January 2005

AbstractElectromechanical properties of skeletal muscle were compared between malignant hyperthermia (MH)‐susceptible and control pigs. These muscle properties were studied both in vivo and in vitro. The in vivo time interval from nerve stimulation to onset of mechanical tension was longer in muscle of pigs susceptible to MH. Kinetics of formation of the in vivo compound muscle action potential did not differ between pig groups, whereas the time from peak of the muscle action potential to tension onset was longer for MH muscle. In vitro the time from direct stimulation to tension onset was also longer for MH muscle. These results suggest that the mechanism causing longer electromechanical time intervals in MH skeletal muscle is distal to the myoneural junction. Data comparing contraction kinetics between muscle from MH and control pigs indicates that the mechanism contributing to the longer time interval involves the transfer of signal from the transverse tubule to the sarcoplasmic reticulum in the pigs studied.
Effects of graded duration of stretch on normal and dystrophic skeletal muscle
Authors: Frankeny John R. , Holly Robert G. , Ashmore C. Robert 
Published: January 2005

AbstractDaily passive stretch for six weeks ranging from 30 minutes to 24 hours per day was studied in the patagialis (PAT) muscle of normal and dystrophic chickens. Significant increases in wet weight, cross‐sectional area, and mean fiber cross‐sectional area occurred in both normal and dystrophic PAT in response to stretch of all daily durations tested. More than 50% of the growth occurring in response to continuous stretch was elicited by as little as 30 minutes of stretch per day. Oxidative enzyme capacity increased proportionately with increasing durations of stretch in the normal PAT. Similarly, increasing duration of stretch progressively retarded the onset of histopathological signs in the dystrophic PAT. We conclude that daily stretching for as little as 30 minutes per day is a powerful inducer of growth in normal and dystrophic muscle and that the progress of the histopathology in dystrophic muscle is delayed in proportion to the daily duration of stretch.
A benign form of reducing body myopathy
Authors: Oh Shin J. , Meyers Gary J. , Wilson Edward R. , Alexander C. Bruce 
Published: January 2005

AbstractA 4‐year‐old boy with nonprogressive myopathy since 2 years of age had “reducing bodies” in muscle biopsy specimens. Clinical and morphological findings are presented, which support the existence of a benign form of reducing body myopathy.
The electrodiagnostic findings in peripheral neuropathy associated with monoclonal gammopathy
Authors: Kelly John J. 
Published: January 2005

AbstractThe electrodiagnostic findings in 51 patients with monoclonal serum proteins of different etiologies and peripheral neuropathies are analyzed and the findings in an additional 10 patients with multiple myeloma are discussed. The nine patients with monoclonal gammopathy of undetermined significance had electrodiagnostic patterns consistent with either predominant axonal degeneration or demyelination. Almost all 15 patients with osteosclerotic myeloma had evidence of a demyelinating neuropathy. The 27 patients with primary systemic amyloidosis almost always displayed evidence of an axonal neuropathy with superimposed carpal tunnel syndrome in some cases. The 10 patients with multiple myeloma had heterogeneous findings. Electrodiagnostic studies can aid in classifying these patients and suggest the likelihood of a specific etiology.
Age‐related differences in absolute numbers of skeletal muscle satellite cells
Authors: Gibson Marcia C. , Schultz Edward 
Published: January 2005

AbstractThe absolute number of satellite cells (SC) in young and adult rat skeletal muscle was estimated by correlating data from light and electron microscopic quantitation of myofiber nuclei (myonuclei and satellite cell nuclei) with biochemical assays for determining total muscle DNA content. Expressed both as a proportion of total myofiber nuclei and as absolute numbers, satellite cells are more numerous in the predominantly oxidative soleus muscle than in the mixed glycolytic/oxidative extensor digitorum longus (EDL) muscle at 1, 12, and 24 months of age. Satellite cell proportions decline gradually in both muscles with advancing age, but absolute numbers increase significantly in the soleus between 1 and 12 months, while EDL exhibits a continuous significant decline in satellite cells between each of the ages examined.
Distribution of different fiber types in human skeletal muscles: Effects of aging studied in whole muscle cross sections
Authors: Lexell Jan , Henriksson‐Larsén Karin , Winblad Bengt , Sjöström Michael 
Published: January 2005

AbstractThe effects of aging on the total number and size of fibers, and the proportion and distribution of type 1 (slow twitch) and type 2 (fast twitch) fibers were studied in cross sections (15 μ thick) of autopsied whole m. vastus lateralis from two age groups. Each group consisted of six, previously physically healthy males (mean age 72 ± 1 years and 30 ± 6 years, respectively). The size of the muscles of the older individuals was 18% smaller (P < 0.01) and the total number of fibers was 25% lower (P < 0.01) than those of the young individuals (mean number 364,000 ± 50,000 vs 478,000 ± 56,000). There was, however, no significant difference in the mean fiber size (indirectly determined) orthe proportion of the two fiber types, though a preferential reduction in type 2 fiber number in the aged individuals was seen. The relative occurrence of the fiber types at various depths in the aged muscles was found to be more even than in muscles from the young individuals. The results suggest that the aging atrophy in m. vastus lateralis, at least up to the age of 70, is primarily the result of a loss of fibers.
Quantitation of metabolites in human skeletal muscle during rest and exercise: A comparison of methods
Authors: Sabina Richard L. , Swain Judith L. , Bradley Walter G. , Holmes Edward W. 
Published: January 2005

AbstractQuantitation of muscle metabolites in vastus lateralis needle biopsies from human subjects at rest and following exercise is evaluated using three different methods of normalization of the data, i.e., total protein, total creatine, and NAD+. Coefficients of variation between patient analyses are similar when the metabolite data are normalized by any of the descriptors in resting muscle and in muscle samples obtained following vigorous exercise. When multiple samples are taken from individual patients the intrapatient coefficient of variation is less than that for interpatient comparisons. Normalization to total creatine or NAD+ yields significantly smaller coefficients of variation than does normalization to total protein in these intrapatient comparisons (P < 0.05). Based on these comparisons, we conclude that any of these methods evaluated for normalizing metabolite content is adequate for studying human subjects, but the total creatine and NAD+ methods may offer some advantage because of the lower variability obtained with these analyses.
Effects of acrylamide and some other sulfhydryl reagents on spontaneous and pathologically induced terminal sprouting from motor end‐plates
Authors: Kemplay Sheila , Cavanagh John B. 
Published: January 2005

AbstractThe innervation of the normal rat sternocostalis muscle exhibits a constant low level of short spontaneous terminal sprouts visible in zinc iodide‐osmium (ZIO) and in methylene blue‐stained preparations. Acrylamide inhibits these spontaneous sprouts in a dose‐dependent manner. This inhibition is mimicked by N‐ethylmaleimide (a sulfhydryl group blocking agent) and can be nullified when acrylamide is given after the sulfhydryl group protecting agent, dithiothreitol (DTT). This could not be reversed by giving DTT 3 hours after acrylamide. Further more when given alone DTT increases the level of spontaneous terminal sprouting seen in ZIO and in methylene blue‐stained preparations. These findings suggest that the binding of acrylamide to sulfhydryl groups is involved in the inhibitory process. Acrylamide also reduces the number and length of the reactionary terminal sprouts, seen in ZIO and in methylene blue‐stained preparations, that follow partial denervation or local injection of botulinum toxin. These inhibitory effects are long‐lasting; recovery still has not fully occurred 4 weeks after a single dose of acrylamide (50 mg/kg). The roles of glutathione and other sulfhydryl components of axons are discussed in relation to the mechanism of acrylamide neurotoxicity.
Neurons induce contractions in myotubes containing only muscular dysgenic nuclei
Authors: Powell Jeanne A. , Peterson Alan , Paul Carol V. 
Published: January 2005

AbstractMuscular dysgenic (mdg/mdg) myotubes cultured alone do not contract. Glucosephosphate isomerase (GPI‐1) isozymes were analyzed to determine the final genotype of cultured dysgenic (mdg/mdg, gpi‐1a/a) myotubes to which normal embryonic spinal cord and limb cells (CBA/J +/+, gpi‐1b/b) had been added. Although both myoblast and spinal cord cell additions caused induction of spontaneous contractions, only myoblasts fused with the myotubes to create functional heterokaryons. Spinal cord cells did not fuse with dysgenic myotubes, but formed functional synapses.
The treatment of scoliosis in duchenne muscular dystrophy
Authors: Rideau Y. , Glorion B. , Delaubier A. , Tarlé O. , Bach J. 
Published: January 2005

AbstractThere are not, as yet, clear indications for the surgical management of scoliosis in Duchenne muscular dystrophy (DMD), taking into account the varying severity of the clinical course. Monitoring the vital capacity can be most important for the indication and timing of surgery. In some cases, delaying surgical intervention with conservative management using spinal braces and wheelchair inserts can permit the restrictive lung syndrome to advance to the point that surgery will be contraidicated. Ten such patients conservatively treated for an average of 5 years exhibited perhaps a slower progression but ultimately an advanced deformity. From a second group of five carefully selected and surgically treated patients, indications for spinal surgery were reviewed. Surgical intervention should be prophylatically undertaken when there is high risk of a rapidly evolving curve with a severe restrictive lung syndrome.
Topographical localization of motor endplates in cryosections of whole human muscles
Authors: Aquilonius Sten‐Magnus , Askmark Håkan , Gillberg Per‐Göuran , Nandedkar Sanjeev , Olsson Yngve , Stårlberg Erik 
Published: January 2005

AbstractThe distribution of motor endplates in biceps brachii, tibialis anterior, and sartorius muscles from human adults was studied by staining longitudinal cryosections of whole muscle for cholinesterase. A special freezing technique was used to prevent the muscle from cracking before sectioning on a heavy cryostat microtome. The results from a large number of cryosections from biceps brachii and tibialis anterior muscles were analyzed by a computer and the topographical distributions of endplates in different views of the muscles were reconstructed. In the biceps brachii muscle, the endplates formed a fairly distinct, slightly V‐shaped band through the middle of the two heads. In the tibialis anterior muscle, the majority of the endplates were superficially distributed along the whole muscle. In the longitudinal sections from the middle part of the muscle, they gave the pattern of a parabola with its apex at the proximal end of the muscle. In the sartorius muscle, the endplates were scattered throughout the muscle and no endplate band was observed. The findings are in accordance with results obtained 30 years ago in investigations of muscle from small children and stillborn infants.
Myosin components of the latissimus dorsi and the pectoralis major muscles in the dystrophic chicken
Authors: Bandman Everett 
Published: January 2005

AbstractThe myosin composition of the anterior latissimus dorsi, the posterior latissimus dorsi, and the pectoralis major muscles was examined in the inbred White Leghorn dystrophic chicken and its isogenic normal line at different ages during development and maturation. Using the biochemical methods of native gel electrophoresis, one‐ and two‐dimensional sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS PAGE), and peptide mapping, it was found that myosin isozyme changes occurred normally in the anterior latissimus dorsi muscle. However, in the posterior latissimus dorsi muscle, slow myosin components which were not present in the adult normal muscle were present in the adult dystrophic muscle. In addition, the pectoralis major muscle of the dystrophic chicken failed to undergo the neonatal to adult fast myosin isozyme transition. Our data also showed that muscle cell cultures derived from the pectoralis major muscle of dystrophic chickens expressed identical myosin components to cultures derived from normal embryos. However, since these cultures only produced embryonic myosins even after 1 month in culture, it implied that cells in tissue culture were phenotypically normal because present cell culture conditions were insufficient to induce the fetal to adult isozyme changes.
Characterization of rabbit masseter muscle fibers
Authors: Mabuchi K. , Pinter K. , Mabuchi Y. , Sreter F. , Gergely J. 
Published: January 2005

AbstractMyosins of histochemically distinguishable single fibers of rabbit masseter muscle—type 1, 2A, 2B, and slow fibers—have been characterized by gel electrophoresis under dissociating (sodium dodecyl sulfate) and nondissociating (inorganic pyrophosphate) conditions, and by analysis of peptide maps of the heavy chains following limited proteolytic degradation. Type 2B fibers contain more LC3 homodimer than type 2A fibers; peptide maps of their heavy chain are different although the two myosins comigrate on pyrophosphate gel electrophoresis. Slow fiber myosin migrates more slowly than fast myosin and has a distinct peptide map. Differences were also found among fibers of the same histochemical type but originating in different muscles. In adductor magnus 2B myosin the LC1 + LC3 heterodimer band is the strongest, while in masseter 2B myosin the heterodimer band is the strongest, while in masseter 2B myosin the heterodimer is the weakest. Statistical considerations suggest that in masseter there is a mechanism preferentially forming the homodimers. More work is needed to determine the mechanism by which phenotypical differences occur among various fiber types in the same muscle and between corresponding fiber types in different muscles.
Brominated vegetable oil myopathy: Inhibition at multiple sites
Authors: Carroll James E. , Norris Beverly J. , Villadiego Aida , Wheeler Steve D. 
Published: January 2005

AbstractSkeletal muscle lipid storage was induced by feeding rats brominated vegetable oil (BVO). The defect was examined by measuring radioactive substrate oxidation, intermediates of fatty acid oxidation, and activities of oxidative enzymes. One‐ and U‐[14C] palmitate and 1‐[14C] pyruvate oxidation were reduced in muscle after four doses of BVO. Inhibition of U‐[14C] palmitate oxidation occurred after two doses. Short chain acylcoenzyme A(CoA) derivatives accumulated in the muscle. Several enzymes of β‐oxidation were significantly reduced, with the greatest reduction in 3‐ketoacyl‐CoA thiolase. The inhibition probably affected multiple sites of CoA and CoA‐derivative metabolism.
Evolution of nerve conduction velocity in later childhood and adolescence
Authors: Lang Heikki A. , Puusa Asko , Hynninen Pirkko , Kuusela Vesa , Jäntti Ville , Sillanpää Matti 
Published: January 2005

AbstractSensory and/or motor nerve conduction velocities (NCVs) were measured in median, radial, sural, and peroneal nerves of 54 healthy girls and 75 healthy boys aged 3–19 years. Independent of the nerve and sex, both motor and sensory NCVs increased in the upper extremities and decreased in the lower ones as a function of age/growth in length. NCV increased in the upper limbs on an average 3.17 m/sec in the boys and in the girls 1.47 m/sec per 10 years of life. As for the nerves of the lower extremities, NCV slowed down on the average 1.81 m/sec in the boys and in the girls 3.62 m/sec per 10 years of age. The change varied in different nerves. A highly significant one (P <0.001) was found in both the motor and sensory NCVs of peroneal nerve in the girls and in the sensory NCV of radial nerve in boys. On the average NCV was in most nerves faster in the girls than in boys of the same age. According to these results, changes occur in the function—and thus probably also in the morphology—of peripheral nerves even in later childhood and adolescence. These changes pertain to general maturation, growth in length of the limbs, and possibly also sex.
Alcoholic polyneuropathy
Authors: Shields Robert W. 
Published: January 2005

AbstractAlcoholic polyneuropathy is a common disorder, characterized by nonspecific clinical, electromyographic, and pathologic features. Nerve conduction studies and needle electromyography disclose findings consistent with a generalized, sensorimotor, axonal degenerative, polyneuropathy. Alcoholic polyneuropathy may be suspected when such findings occur in the setting of long‐standing alcoholism and malnutrition. The lack of specific diagnostic criteria for alcoholic polyneuropathy requires that other potential etiologies be excluded before that diagnosis is made. The institution of a nutritionally‐balanced diet constitutes the principal therapy for alcoholic polyneuropathy, however, significant improvement following such therapy is not frequently seen.
Rat diaphragm: Changes in muscle fiber type frequency with age
Authors: Maltin Charlotte A. , Duncan Linda , Wilson Andrew B. 
Published: January 2005

AbstractThis study demonstrates age‐related changes in fiber type, distribution, and diameter that suggest that the diaphragm does not attain a static pattern of fiber type in adults. Furthermore, the progressive increase in the FG fiber diameters may be of importance in the interpretation of physiologic and biochemical parameters measured in the diaphragm muscle of animals of different age groups.
Causes of the increased fiber density in muscular dystrophies studied with single fiber EMG during electrical stimulation
Authors: Hilton‐Brown P. , Stålberg E. , Trontelj J. , Mihelin M. 
Published: January 2005

AbstractThe impulse transmission in motor end‐plates and along muscle fibers was studied with single fiber EMG in 13 patients with muscular dystrophy during intramuscular nerve stimulation and direct muscle fiber stimulation. For comparison, three patients with spinal muscular atrophy (SMA) and five normal subjects were studied. At nerve stimulation, 25% of the recordings in the patients with muscular dystrophy showed “increased” jitter (>55 m̈sec), 48% “normal” (10–55 m̈sec), and 27% “abnormally low” jitter (<5 m̈sec). At direct muscle fiber stimulation, the jitter was “abnormally low” in 91% of the recordings; 9% of the recordings had normal jitter. In SMA, no abnormally low jitter values were found. Increased jitter is a sign of impaired impulse transmission in immature intramuscular nerves and motor end‐plates. “Abnormally low jitter” suggests the presence of branched muscle fibers and ephaptic transmission between hyperexcitable fibers.
Stiffness and contractile properties of avian normal and dystrophic muscle bundles as measured by sinusoidal length perturbations
Authors: Feit Howard , Kawai Masataka , Schulman Michael I. 
Published: January 2005

AbstractBoth tension and stiffness as a function of muscle length were measured under relaxing conditions on isolated small bundles of chemically skinned myofibers from normal and dystrophic chicken pectoral muscles. It was shown that the dystrophic muscle was stiffer than normal muscle and developed more tension for the same amount of stretch. A fraction of stiffness was not removed by extraction with either 0.6 M Kl or with 5 M guanidine HCl mixed with 1% mercaptoethanol. The stiffness of dystrophic muscle was also unaffected by treatment with bacterial collagenase under conditions that destroyed the stiffness of tendon. Nyquist plots of normal and dystrophic muscles during calcium‐activated isometric contraction were very similar and were characteristic of fast‐twitch muscle, as evidenced by three clear exponential processes. The normal appearance of the Nyquist plot of dystrophic muscle demonstrates that cross‐bridge function is not altered, and the characteristic slowing of contraction and relaxation is not a consequence of a fast‐to‐slow transformation of muscle types. The increased stiffness of dystrophic muscle may be a very fundamental change in the biomechanics of dystrophy. We postulate that the stiffness is mediated by an altered form of collagen, which is collagenase‐resistant by virtue of excessive crosslinking.