bluuebunny/crossref_metadata_2025_split huggingface dataset:
Changes in membrane structure following tenotomy of the rat soleus muscle
Authors: Baker James H. , Baldwin Kate M. 
Published: January 2005

AbstractMany of the histological characteristics of neuromuscular disorders, including central core lesions and nemaline rods, can be produced experimentally by tenotomy of the soleus muscle in the rat. Using the freeze fracture technique, a decrease in the number of intramembrane particles of both the P and E faces of the plasma membranes of tenotomized soleus muscle fibers was observed. While many of the degenerative changes which occur after tenotomy are known, the causes of the degeneration are still unknown. The decrease in intramembrane particles may reflect some significant change in the function of the plasma membrane resulting in the appearance of the degenerative changes which follow tenotomy.
X‐ray diffraction from striated muscles and nerves in normal and dystrophic mice
Authors: Kurg Teorin , Stinson Robert H. , Millman Barry M. 
Published: January 2005

AbstractThe structure of striated muscle (thick and thin filaments, filament lattice, and collagen), peripheral nerve myelin, and tendon collagen were studied in tissues from dystrophic and normal mice using small‐angle x‐ray diffraction. There were increases in the amount of disorganized tissue in the dystrophic mice, and the time course of the changes was monitored over the first 42 weeks of life. As the dystrophic mice became older, the contractile apparatus of the muscles appeared to atrophy, while the amount of collagen increased. In general, the molecular structure and packing appeared to remain unchanged as the disease progressed, although changes in the relative amounts and the organization of proteins were noted. In both normal and dystrophic mice, the collagen periodicity (65.7 nm) was 2% smaller when detected in muscle tissue compared with that detected in tendon tissue.
Isolated proximal median neuropathy
Authors: Roth G. , Ludy J.‐P. , Egloff‐Baer S. 
Published: January 2005

AbstractFour cases are described in which the patients developed an isolated lesion of the median nerve in the proximal part of the arm during sleep. In the three patients that were available for follow‐up the course was favorable with complete recovery in several months. The causes of the lesions are unknown; several factors that could have played roles in causing the lesions are discussed.
Endogenous cathepsin B Inhibitor activity in normal and myopathic red and white skeletal muscle
Authors: Spanier Arthur M. , Bird John W. C. 
Published: January 2005

AbstractDespite extensive biochemical and morphological studies on the degenerative muscle diseases, the primary chemical lesions are still obscure, both in humans and animals. In this report we examine the activities of the lysosomal endoproteinase cathepsin B and its endogenous inhibitor(s) in the red and white skeletal muscles of guinea pigs with a nutritional muscular myopathy induced by vitamin E deficiency. We observed a twofold increase (P < 0.005) in the activity of cathepsin B in the white skeletal muscles of the vitamin E‐deficient (E−) animals over that of the normal (N) and control (E+) groups. Assessment of the activity of endogenous cathepsin B inhibitor revealed a one and a half times greater amount of inhibitor in N when compared to E−; this difference in inhibitor activity applied to both red (masseter) and white (medial head, gastrocnemius) muscle. When the specific activity of cathepsin B in the E−tissue was corrected for inhibitor activity, the corrected value was not significantly different from either the E+ or the N tissue.
Drug evaluation in muscular dystrophy of the chicken
Authors: Entrikin Richard K. , Patterson Gary T. , Wilson Barry W. 
Published: January 2005

AbstractA high‐capacity, step‐wise system for drug evaluation in chickens with inherited muscular dystrophy has been developed. This report details the protocol and presents the first results from a rapid evaluation of the ameliorative effects of drugs on the impaired righting ability of dystrophic chicks. Of 31 compounds evaluated, only methysergide and corticosterone significantly increased righting ability.
Single fiber electromyography in chronic renal failure
Authors: Konishi Tetsuro , Nishitani Hiroshi , Motomura Shunji 
Published: January 2005

AbstractNineteen patients with chronic renal failure receiving intermittent hemodialysis had single fiber electromyography studies of their extensor digitorum communis muscles. Jitter values were abnormal, more so in men than in women. The jitter abnormalities tended to improve after intermittent hemodialysis treatments lasting longer than 1 year.
Paucity and disorganization of presynaptic membrane active zones in the lambert‐eaton myasthenic syndrome
Authors: Fukunaga Hidetoshi , Engel Andrew G. , Osame Mitsuhiro , Lambert Edward H. 
Published: January 2005

AbstractPresynaptic membrane active zones are related to synaptic vesicle exocytosis, and the large intramembrane particles in these zones may represent voltage‐sensitive calcium channels. We tested the hypothesis that an abnormality of the zones is associated with the low probability of quantal release in the Lambert‐Eaton myasthenic syndrome (LEMS). Freeze‐fractured presynaptic membranes were studied in nine patients with LEMS (227 end‐plates) and in 14 controls (148 end‐plates). Satisfactory replicas of 94 LEMS and 83 control presynaptic membrane P‐faces were obtained. Presynaptic membrane areas were estimated by stereometric analysis. The LEMS samples showed a marked decrease in active zones and active zone particles per unit area. The average number of particles per active zone was also reduced. Clusters of large particles were observed with increased frequency in the LEMS samples. These may have arisen by aggregation of active zone particles. There was no decrease in the overall density of intramembrane particles not associated with active zones or clusters. The distribution of these particles according to size resembled the control distribution except for a small decrease in the frequency of 5.3–5.8 nm particles. The findings can explain the reduced quantal release in LEMS, and strongly suggest that active zone particles are targets of the pathogenic autoantibodies recently demonstrated in this disease.
The misdiagnosis of unilateral presentations of amyotrophic lateral sclerosis
Authors: O'Reilly Daniel F. , Brazis Paul W. , Rubino Frank A. 
Published: January 2005

AbstractFifty‐eight patients with definite amyotrophic lateral sclerosis (ALS) were investigated retrosectivley. Variables investigated included the age of onset of motor symptoms, the sex of the patient, the presenting signs and symptoms, and the initial clinical diagnosis. The patients were separated by mode of onset into five clinical categories: I. Conventional or Classical (31%); II Predominantly bulbar (19%); III. Pseudopolyneuritic (5.2%); IV. Paraparetic (13.8%); and V. Hemiparetic or Mills' Variant (31%) presentations. The average age of onset of symptoms was 55.3 years, with little difference among the clinical groups except for a later age of onset in the bulbar form. The initial diagnosis of ALS was correct in 95% of the patients with a predominantly bilateral mode of onset of symptoms (the conventional, bulbar, and paraparetic groups) but was correct in only 38% of patients with predominantly unilateral onset (hemiparetic) and pseudopolyneutric forms. We suggest that the clinician be aware of these atypical, unilateral forms of ALS in order that an accurate diagnosis may be made.
F response latency determination
Authors: Fisher Morris A. 
Published: January 2005

AbstractF response latencies were analyzed. Data were obtained from normal subject as well as in sequential studies recorded from the author. Based on series of 10 F responses, mean in comparision to minimal latency values are more reproducible and provide a statistically consistent measure of F wave conduction. The data also indicate an inherent variability in F wave latencies between groups of 10 responses. The results are consistent with F waves in normal subjects discharging from subpopulations of a motoneuron pool.
Membrane alterations in skeletal muscle fibers of dystrophic mice
Authors: Kerr Lynne M. , Sperelakis Nick 
Published: January 2005

AbstractSkeletal muscle fibers from dystrophic mice and littermate controls (ReJ‐129) were characterized electrically and then injected with an intracellular marker. In this way they could be identified for examination with an electron microscope to correlate the relative time course of electrical and ultrastructural aherations resulting from the dystrophic process. On the average, dystrophic muscle fibers displayed decreased membrane potentials (−59 ± 1.2 vs −79 ± 0.7 mV for normals), decreased specific membrane resistivity (517 ± 27 vs 642 ± 34 Ω‐cm2 for normals), and depressed action potential (AP) maximum rates of rise (+Vmax) (352 ± 9 vs 417 ± 9 V/s for normals) and amplitudes (92 ± 1.2 vs 102 ± 1.0 mV for normals) at an experimentally polarized membrane potential of −90 mV. Membrane resistivity and AP +Vmax were decreased even in those fibers from dystrophic muscles that displayed normal ultrastructure (classified visually and by ratio of sarcoplasmic reticulum to total cell volume). These findings support the membrane hypothesis of muscular dystrophy that membrane lesions are the primary lesion in the disease process.
Clinical investigation in duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history
Authors: Brooke Michael H. , Fenichel Gerald M. , Griggs Robert C. , Mendell Jerry R. , Moxley Richard , Miller J. Philip , Province Michael A. 
Published: January 2005

AbstractA prospective study of 114 patients with DMD provided data for “power” calculations for future therapeutic trials. There was a decline in strength of 0.4 units per year (on a 0‐10 scale). Contractures of the iliotibial bands, hip flexors, and heel cords developed before 6 years. Contractures of other joints accompanied the increased use of wheelchairs. All children walked until 8 years with functional “improvement” between 3–6 years. Children of the same age varied widely in their strength, degree of contracture, and functional abilities. Fifteen percent of the patients appear to have a milder variety of the disease and are termed “outliers.” To test a drug which might slow the disease to 25% of its original rate of progression, two groups (placebo and treatment) of 40 patients each would have to be followed for one year.
Increase of muscle peroxisomal enzymes and myotonia induced by nafenopin, a hypolipidemic drug
Authors: Behrens María Isabel , Soza Marco Antonio , Inestrosa Nibaldo C. 
Published: January 2005

AbstractThe chronic administration of nafenopin, a hypolipidemic drug, induced an increase in catalase and acyl‐CoA oxidase activities in various skeletal muscles, including the gracilis, diaphragm, soles, and extensor digitorum longus. The magnitude of the increase was around 100% for both enzymes in each of the muscles studied in spite of the different basal level. These changes seem to be specific of the peroxisomal enzymes because acetylcholinesterase, which is not peroxisomal, did not follow the same pattern in all the muscles. Concomitant with the increase in muscle peroxisomal enzymes, the skeletal muscles presented an altered electromyogram with prolonged insertional activity, repetitive firing of action potentials, and myotonic runs characteristic of myotonia. Our results suggest a role for peroxisomes in the myotonic disorder.
Multiple sclerosis and hypertrophic demyelinating peripheral neuropathy
Authors: Il Ro Young , Alexander C. Bruce , Oh Shin J. 
Published: January 2005

AbstractA combination of multiple sclerosis (MS) and hypertrophic demyelinating neuropathy has been reported in a few autopsy studies. We are reporting a unique case of such a combination, which was proved by the sural nerve biopsy. A patient with classical MS on history and findings had areflexia and sensory abnormalities in stocking distribution. The nerve conduction study showed marked abnormalities indicative of demyelinating neuropathy, and sural nerve biopsy was typical of hypertrophic demyelinating neuropathy.
The dual connective tissue system of rat soleus muscle
Authors: Moore M. J. 
Published: January 2005

AbstractRat soleus muscle has two types of connective tissue associated with it which do not overlap except at the myotendinous junction. One, an areolar type, is restricted mainly to the spaces between muscle fibers and the other, a regular dense type, is located only beyond the ends of muscle fibers in structures called microtendons. The definitive tendon of a muscle thus represents the accumulation of all the microtendons, one of which is contributed by each muscle fiber. The diameter distributions were the same for collagen fibrils from areolar tissue and microtendons but different from tendon collagen. Marked changes occur in the diameter distribution of the microtendon collagen as it passes into and blends with that of the tendon.
Clinical electrophysiology of infantile botulism
Authors: Cornblath David R. , Sladky John T. , Sumner Austin J. 
Published: January 2005

AbstractInfantile botulism is a recently recognized cause of acute hypotonic paresis and respiratory failure in young infants. Electrophysiological testing has proven useful in early diagnosis in suspected cases by demonstrating abnormal neuromuscular transmission as is known to occur in botulism. Twenty‐five infants with bacteriologically proven botulism were studied by uniform methods in our laboratory and characteristic electrophysiological abnormalities were found. Repetitive stimulation at 20 and 50 Hz was the most specific single test; 23 patients (92%) showed incremental responses. Stimulation at low rates was less specific. Concentric needle electromyography provided useful supplemental information. Short‐duration, low‐amplitude motor unit potentials were prominent in 22 patients (92%) accompanied by abnormal spontaneous activity in 13 patients (54%). Compound muscle action potential amplitudes were usually reduced, but motor and sensory conduction studies were otherwise normal. Electrodiagnostic testing demonstrated one or more characteristic abnormalities in all cases of infantile botulism. This constellation of electrophysiological abnormalities, combined with an appropriate clinical picture, was so distinctive as to allow early presumptive diagnosis of infant botulism, before the results of bacteriological testing were available.
Severe childhood muscular dystrophy affecting both sexes and frequent in tunisia
Authors: Ben Hamida Mongi , Fardeau Michel , Attia Neïla 
Published: January 2005

AbstractThe authors reported a large study of 93 children presenting a severe form of progressive muscular dystrophy. The first clinical symptoms were noticed between 3 to 12 years. The atrophy affects, predominantly, the girdle and truncal muscles. The hypertrophy of the calves is almost consistent. The progression of the disease is severe, often like that of the Duchenne type. In most of the cases, inability to walk occurs between 10 and 20 years. The serum creatine kinase activity is markedly high in the first stages of the disease. There is a necrotic regenerative pattern at muscle biopsy, associated with a marked type 1 predominance. The disease appears to be inherited as an autosomal recessive trait, with equal distribution among the two sexes. There is a marked variability in the intensity of symptoms and in the severity of the course of the disease from one sibling to another, and from one family to another. This disease is frequent in Tunisia and seems to be related to the high degree of consanguinity in this country.
The in vitro effects of D‐penicillamine upon anti‐achr production by thymic and peripheral blood lymphocytes from patients with myasthenia gravis
Authors: Scadding Glenis K. , Calder Linda , Newsom‐Davis John 
Published: January 2005

AbstractD‐Penicillamine added to lymphocyte cultures from myasthenia gravis patients infrequently and inconsistently stimulated anti‐AChR production. This usually occurred in the presence of pokeweed mitogen (PWM), and often only at a single concentration of D‐penicillamine which varied among patients. The change was typically paralleled by an increase in total IgG production of similar magnitude, suggesting that the effect was nonspecific. D‐penicillamine did not affect AChR expression in cultured thymic adherent cells. These data provide no evidence that penicillamine induces myasthenia gravis by direct stimulation of anti‐AChR antibody‐producing cells.
Immunohistochemical demonstration of β‐enolase in human skeletal muscle
Authors: Ibi Tohru , Sahashi Ko , Kato Kanefusa , Takahashi Akira , Sobue Itsuro 
Published: January 2005

AbstractWe have localized β‐enolase activity in human skeletal muscle fiber using the immunohistochemical method (two‐step method). The first immunoreagent was rabbit anti‐human β‐enolase serum raised in New Zealand white rabbit, and the second was peroxidase conjugated staphylococcal protein A. The immunohistochemical reaction for β‐enolase was noted higher in type 2 fiber, which demonstrates the low oxidative and high glycolytic enzyme activity, than in type 1 fiber.
Double staining of the muscle fibers and interstitial tissue components for automatic analysis of muscle biopsies
Authors: Hantaï Daniel , Albe Xavier , Fardeau Michel 
Published: January 2005

AbstractA staining technique is presented in order to facilitate the automatic analysis of muscle biopsies. It renders possible the visualization on the same section of the myofibrillary adenosine‐triphosphatase (ATPase) activity under the microscope with transmitted light, and of the fluorescent staining of fibronectin which marks the outlines of the muscle fibers by a simple switch to ultraviolet (UV) light.
Peripheral neuropathy in osteosclerotic myeloma: Clinical and electrodiagnostic improvement with chemotherapy
Authors: Donofrio P. D. , Albers J. W. , Greenberg H. S. , Mitchell B. S. 
Published: January 2005

AbstractA patient with a severe remote‐effect polyneuropathy and other paraneoplastic features of osteosclerotic myeloma improved dramatically with melphalan and prednisone treatment. Serial electrodiagnostic studies provided an objective means of following the response to therapy and documented the improvement. We believe this represents the first reported patient with multifocal osteosclerotic myeloma and a myelomatous polyneuropathy responding to melphalan and prednisone.
Relationship of genotype and in vitro contractility in mdg/mdg ↔ +/+ “mosaic” myotubes
Authors: Peterson Alan , Pena Sergio 
Published: January 2005

AbstractMuscular dysgenesis (mdg) in the mouse is an autosomal recessive lethal disorder that is manifested by a gross failure of skeletal muscle development. In vitro mdg/mdg myoblasts proliferate normally and fuse successfully into myotubes, but these myotubes fail to contract either spontaneously or in response to physiological stimuli despite the presence of effective contractile elements and an ability to propagate action potentials normally. We have determined that mdg/mdg and +/+ myoblasts are capable of fusing in vitro to form “mosaic”myotubes which typically express an apparently normal contractile phenotype. Electrophoretic analysis of the relative activities of myotube glucosephosphate isomerase (GPI‐1) isozymes provided a means of estimating the proportions of myonuclei of each genotype within individual myotubes. Only a very small proportion of genotypically normal myonuclei were required for expression of an apparently normal contractile phenotype.
The size of the myofibers in mature grafts of the mouse extensor digitorum longus muscle
Authors: Thomas Denise , Klueber Kathleen , Bourke Dianna , Ontell Marcia 
Published: January 2005

AbstractThe histochemical profile of stabilized orthotopically grafted mouse extensor digitorurn longus muscles (EDL) and fiber type diameter distribution in the graft was compared with control muscles. Histochemical fiber typing, based on myofibrillar ATPase reactions, indicated that type 1 fiber accounted for < 1%, type 2a fibers for 30%, and type 2b fibers for 69% of the total fiber population of the graft. In control muscles of 56‐day‐old and 156‐day‐old mice, type 1 fibers accounted for <1%, type 2a fibers for 36%, and type 2b fibers for 63% of the total fiber population of normal muscles. The regenerating myofibers in stabilized grafts failed to achieve the normal fiber diameter. The type 2b fibers exhibited greater growth inhibition than did type 2a fibers.
Pre‐ and postnatal growth and protein turnover in four muscles of the rat
Authors: Kelly Frank J. , Lewis Sheena E. M. , Anderson Paul , Goldspink David F. 
Published: January 2005

AbstractDevelopmental growth and associated changes in protein turnover and nucleic acid concentrations have been studied in four individual skeletal muscles. They have also been related to changes in the whole animal. The growth rates of both fast and slow muscle types progressively diminished from the fetus to old age. Similarly, the fractional rates of protein synthesis (measured in vivo) and breakdown in each muscle type declined with age; the changes in the former correlating with decreases in the ribosomal capacities of the muscles. Throughout, fast muscles possessed lower turnover rates. The mean half‐lives of mixed proteins were 12.0, 14.4, 13.5, and 7.2 days in the extensor digitorum longus (EDL), gastrocnemius, diaphragm, and soleus muscles, respectively, 310 days postpartum. Muscle atrophy was found at 735 days, at which stage the decreased protein synthetic rate in the soleus was due to a fall in the ribosomal capacity, while that in the EDL was attributable to a decreased synthetic rate per ribosome.
Is the F wave elicited in a select group of motoneurons?
Authors: Kimura Jun , Yanagisawa Hideo , Yamada Thoru , Mitsudome Akihisa , Sasaki Hiroyuki , Kimura Akio 
Published: January 2005

AbstractThe F wave represents only a small percentage of the motoneuron pool invaded antidromically by any single impulse. We studied 22 median nerves from 11 healthy subjects to determine whether the recurrent discharges are preferntially generated in a select group of motoneurons with certain physiologic characteristics. Partial excitation of the nerve elicited the F wave with no consistent bias toward either the lower or higher threshold motor fibers. When the fast conducting axons were progressively blocked using a collision technique, the F wave continued to appear in the remaining slow conducting axons which escaped the collision. We conclude that the recurrent discharges occur in approximately 1% of antidromically activated motoneurons irrespective of their peripheral excitability or conduction characteristics.