bluuebunny/crossref_metadata_2025_split huggingface dataset:
Creatine kinase activity in normal and duchenne muscular dystrophy fibroblasts
Authors: Davis Michael H. , Cappel Roseann , Vester John W. , Samaha Frederick J. , Gruenstein Eric 
Published: January 2005

AbstractCultured human skin fibroblasts from 9 patients with Duchenne muscular dystrophy (DMD) and 8 normal age‐ and sex‐matched controls were examined for creatine kinase (CK) activity. Both the normal and the DMD fibroblasts were found to have significant levels of CK activity (approximately 10 × 10−3 IU per milligram of fibroblast protein). The control cells had slightly higher CK activity than the DMD lines, but this difference was not significant (0.2 < P < 0.1). The MM (muscle) isozyme, the BB (brain) isozyme, and the MB (hybrid) isozyme of CK were found to be present in fibroblasts. The isozymes were separated by electrophoresis and the relative amount of each was determined for both normal and DMD cells. In normal fibroblasts, approximately 48% of the total CK activity was of the MM type, 40% was of the BB type, and 12% was of the MB type with no significant differences apparent between normal and DMD groups. The presence in human fibroblasts of significant levels of CK activity with a characteristic isozyme profile is an important consideration for studies of this “marker” enzyme in the pseudohypertrophic muscle of DMD.
Sources of error in the diagnosis of guillain‐barre syndrome
Authors: Feit Howard , Tindall Richard S. A. , Glasberg Mark 
Published: January 2005

AbstractA careful monitoring of the accuracy of diagnosis in six cases of Guillain‐Barre syndrome has shown that a substantial proportion of these patients initially diagnosed as having Guillain‐Barre syndrome on the basis of characteristic clinical findings and an elevated level of protein in the spinal fluid had a neuropathy caused by another etiology. The pitfalls in the laboratory and clinical diagnosis of disorders that were confused with Guillain‐Barre syndrome were several: the pattern of neurological dysfunction in the Guillain‐Barre syndrome was not unique to that disorder; no specific laboratory test existed to confirm the diagnosis of Guillain‐Barre syndrome; and the laboratory diagnosis of other causes of similar neurological disorders (especially heavy metal intoxication) depended upon tests that are very unreliable.
Membrane myopathy: Morphological similarities to duchenne muscular dystrophy
Authors: Pestronk Alan , Parhad Irma M. , Drachman Daniel B. , Price Donald L. 
Published: January 2005

AbstractFocal lesions in the plasma membrane overlying wedge‐shaped defects in muscle fibers (“delta lesions”) are an early pathological change in Duchenne muscular dystrophy (DMD). Abnormalities in the plasma membrane have been suggested as a cause of these lesions and of the degeneration of muscle fibers in DMD. We investigated the role of plasma membrane defects in the production of delta lesions by examining the effects of a series of membrane‐active agents—lysolecithin, deoxycholate, Triton X‐100, and melittin—on the muscles of rats in vivo. Within minutes after treatment with these agents, the muscle fibers developed typical delta lesions. Identical morphological changes were produced by the calcium ionophore A23187, suggesting that calcium entry may play an important role in this process. We conclude that damage to the plasma membrane or calcium entry can reproduce characteristic features of the muscle pathology seen in DMD. This model should prove useful in elucidating the mechanisms of muscle fiber damage and degeneration in DMD.
Protein synthesis in bupivacaine (Marcaine)‐treated, regenerating skeletal muscle
Authors: Jones George H. 
Published: January 2005

AbstractSkeletal muscle regeneration has been induced by injection of the myotoxic drug bupivacaine (Marcaine) into the rat tibialis anterior muscle. Doses of 1.5 and 1.0% wt/vol produce significant levels of muscle regeneration, but these doses also produce large regions of ischemic muscle. Doses of 0.75 and 0.5% bupivacaine are also effective in inducing regeneration and produce little or no ischemia. Regenerating muscle is significantly more active in the incorporation of 35S‐methionine into protein than is control muscle, and the activity increase is directly proportional to the bupivacaine dose injected. Polyribosomes were isolated in greater yield from bupivacaine‐treated muscles, as compared with control muscles, 5 days postinjection, and were also more active in cell‐free protein synthesis than control polysomes. Again, the yield and activity of the muscle polysomes was directly proportional to the bupivacaine concentration used for injection. Polyacrylamide gel electrophoresis of polysomal cell‐free reaction mixtures demonstrated the synthesis of a number of myofibrillar proteins.
Quantitation of muscle function in children: A prospective study in duchenne muscular dystrophy
Authors: Scott O. M. , Hyde S. A. , Goddard C. , Dubowitz V. 
Published: January 2005

AbstractA protocol has been developed for the quantitative assessment of muscle function in children with muscle disease. It includes total muscle strength (% MRC) based on a clinical assessment of strength of 32 groups using the 6‐point MRC grading; the force of 8 selected muscle groups measured with a specially designed electromyometer; a motor ability score based on 20 consecutive motor activities; walking times over 28 and 150 feet, and recording of muscle contractures. A 3‐year sequential study of 61 boys with Duchenne dystrophy showed progressive decline of muscle strength with age, a close correlation of total strength and the motor ability score (r = 0.89), and a curvilinear relationship of muscle strength with walking times over 28 and 150 feet (r = 0.78 and 0.79, respectively). A profile of the natural progression of Duchenne dystrophy has been established which could serve as a reference base for the assessment of cases at varying ages and their response to therapy and management.
Preparation of cell‐free extracellular matrix from human peripheral nerve
Authors: Johnson P. C. , Duhamel R. C. , Meezan E. , Brendel K. 
Published: January 2005

AbstractThe extracellular matrix of human peripheral nerve, which is mainly basement membrane and fibrillar collagen, has been prepared by a procedure involving extensive detergent extraction of isolated endoneurium and perineurium obtained from various nerves. The ultrastructure of the isolated nerve extracellular matrix was indistinguishable from that seen in sections of intact nerve, indicating that the extraction procedure preserved the morphological integrity of these connective tissue components. The amino acid and carbohydrate compositions of the nerve extracellular matrix preparation were typically collagenous in nature containing a high content of glycine, proline, 4‐hydroxyproline, and alanine and significant amounts of lysine and hydroxylysine. The preparations contained virtually no 3‐hydroxyproline and a low content of glucose and galactose compared to pure basement membranes, indicating that interstitial rather than basement membrane collagens predominated. This preparation appears well
hyphen;suited to both the ultrastructural and biochemical study of the extracellular matrix of peripheral nerve.
Electromyographic study of diabetic and alcoholic polyneuropathic patients treated with gangliosides
Authors: Bassi Sirio , Albizzati Maria Grazia , Calloni Elio , Frattola Lodovico 
Published: January 2005

AbstractA double‐blind, randomized electromyographic investigation was conducted of the effects of cerebral ganglioside treatment on patients suffering from diabetic or alcoholic polyneuropathy. Cerebral gangliosides (50 mg once a day) administered to 15 diabetic and to 15 alcoholic neuropathic patients for 40 days, facilitated the reappearance of sensory potentials and significantly increased the MAP amplitude in median, ulnar, and peroneal nerves. In relation to ganglioside treatment, there was no significant change in the conduction velocities or in the distal latencies of these nerves, nor was there a change in the duration of the MAPs. On the basis of these results, it is suggested that the cerebral gangliosides are capable of inducing an improvement in the excitability of nerve fibers and of facilitating the processes of reinnervation, probably by means of an enhancement of fiber sprouting.
Altered supernormality in multiple sclerosis peripheral nerve
Authors: Eisen Andrew , Paty Donald , Hoirch Maureen 
Published: January 2005

AbstractSupernormality is a regular part of the recovery cycle of nerve. Supernormality was expressed in terms of amplitude, and in 20 control subjects the mean (S2 conditioned/S2 unconditioned) ratio of the median sensory nerve action potential amplitude recorded at the elbow measured 285 ± 174 (range 119 to 783). A supramaximal conditioning shock was delivered 6 msec in advance of the test pulse. Seventeen (42.5%) of 40 patients with multiple sclerosis (MS) failed to demonstrate supernormality, the (S2 conditioned/S2 unconditioned) ratio being less than 100. This abnormality probably reflects a delay in recovery cycle and is additional evidence implicating peripheral nervous system involvement in multiple sclerosis.
Clinical spectrum of fascial inflammation
Authors: Simon David B. , Ringel Steven P. , Sufit Robert L. 
Published: January 2005

AbstractThe diverse clinical manifestation of fascial inflammation are reviewed including the entities of eosinophilic fasciitis, fascial inflammation in association with connective tissue diseases, fibrositis, nodular fasciitis, and necrotizing fasciitis. The neuromuscular clinician is encouraged to consider the possibility of fascial inflammation when evaluating patients with painful, aching extremities. A simple technique for including a fascial specimen when performing a routine muscle biopsy is described.
Axonal degeneration and focal muscle fiber necrosis in human thallotoxicosis: Histopathological studies of nerve and muscle
Authors: Limos Leonore C. , Ohnishi Akio , Suzuki Nozomi , Kojima Noriaki , Yoshimura Takeo , Goto Ikuo , Kuroiwa Yoshigoro 
Published: January 2005

AbstractTwo cases of proven thallium poisoning were studied with special reference to the neuromuscular changes. These cases showed a consistent pattern of symptomatology of peripheral sensorimotor neuropathy, encephalopathy, and alopecia. Light and electron microscopic studies of the sural nerve biopsies exhibited mainly axonal degeneration without concomitant segmental demyelination. The densities of both large and small myelinated fibers were mildly decreased. Biopsied peroneus brevis muscles showed distinct features of focal muscle fiber necrosis which has not been reported before. Muscle fibers with prominent central nucleation and fiber splitting were frequently seen. These findings are compatible with myopathic changes. In spite of the existence of severe axonal degeneration and muscle atrophy on clinical observation, no neurogenic muscle changes were found.
Congenital nemaline myopathy. II. Quantitative changes in α‐actinin and myosin in skeletal muscle
Authors: Stuhlfauth Ingo , Jennekens Frans G. I. , Willemse Jacobus , Jockusch Brigitte M. 
Published: January 2005

AbstractSkeletal muscle obtained from 2 patients with congenital nemaline myopathy (CNM) and from a healthy control was analyzed by 1‐ and 2‐ dimensional gel electrophoresis. In total extracts, an increase of α‐actinin by a factor of 2:3 was found for CNM muscle as compared with the control. One‐ and two‐dimensional gels revealed the presence of LCF3, the smallest light chain associated with type 2 (fast) myosin in total extracts of normal control of mixed fiber type. Both CNM samples showed the absence of this polypeptide. This result is consistent with the finding that muscle of the 2 patients exhibited nearly exclusively the ATPase activity indicative of type 1 (slow) myosin.
Skeletal muscle lysosomes: Comparison of lysosomes from normal and dystrophic avian pectoralis muscle as a function of age
Authors: Baxter Jeffrey H. , Suelter Clarence H. 
Published: January 2005

AbstractThe properties of skeletal muscle lysosomes from normal and dystrophic chickens were studied to assess their involvement in the dystrophic process. A method is described for isolation of a three‐to‐sevenfold purified lysosome fraction with 29–33% yield. Lysosomal enzymes in crude homogenates and isolated lysosome‐enriched fractions from dystrophic muscle exhibit decreased latency for N‐acetyl β‐D‐glucosaminidase, acid phosphatase, and cathepsin D. However, no differences in the fragility of lysosomes in isolated lysosome‐enriched fractions from normal and dystrophic muscle were observed using shear, sonication and detergent stress. Lower percent recovery, enrichment factor and percent latency of acid phosphatase compared to N‐acetyl‐β‐D‐glucosaminidase and cathepsin D were observed from both normal and dystrophic muscle. These results are consistent with the presence of a significant amount of nonlysosomal acid phosphatase activity in skeletal muscle.
Prospective study of X‐linked progressive muscular dystrophy in campania
Authors: Nigro Giovanni , Comi Lucia I. , Limongelli Francesco M. , Giugliano Maria Antonietta M. , Politano Luisa , Petretta Vito , Passamano Luigia , Stefanelli Severo 
Published: January 2005

AbstractWithin the Campania region of southern Italy a prospective study on X‐linked progressive muscular dystrophy was conducted over a period of 12 years from 1969 to 1980, inclusive. The mean incidence rate was 21.7 per 100,000 male livebirths for Duchenne muscular dystrophy (DMD) cases and 3.2 per 100,000 male livebirths for Becker muscular dystrophy (BMD) cases. The familial cases were 38.5% among the DMD patients and 50% among the BMD patients. Myocardial involvement appeared in DMD patients at about 6 years of age in a high percentage of cases and increased progressively until the last years of life, when cardiac damage occurred in 95% of cases. The percentage of myocardial involvement in BMD patients was very low before 13 years of age, but increased progressively until 20 years, when cardiac damage occurred in 80% of cases studied; severe cardiomyopathy did not occur before the age of 21. The data reported also include the effects of age on physical performance, serum creatine kinase activity and serum myoglobin levels, the types of cardiac damage, and the causes of death.
Neuropathy in the hypereosinophilic syndrome
Authors: Dorfman Leslie J. , Ransom Bruce R. , Forno Lysia S. , Kelts Alan 
Published: January 2005

AbstractPeripheral nerve dysfunction was a prominent clinical feature in each of three patients with the hypereosinophilic syndrome (HES). The neuropathy, occurring at the onset of marked eosinophilia or at a time of its worsening, had a painful onset, evolved over 1–4 weeks, and affected both sensory and motor function. Electrodiagnostic studies demonstrated both multifocal and generalized nerve involvement, with the former predominating (multiple mononeuropathy). Nerve and muscle biopsies from two patients revealed severe axonal degeneration with neurogenic atrophy of muscle. The eosinophilia decreased dramatically with corticosteroid treatment and the neuropathy gradually improved, but all three patients manifested residual neurologic disability 17–22 months after onset. The neuropathy may be on an ischemic basis or may be related to neurotoxicity of eosinophil products.
Paradoxical effect of isoproterenol on hamster hereditary polymyopathy
Authors: Jasmin Gaëtan , Proschek Libuse 
Published: January 2005

AbstractIsoproterenol (ISO), a potent β‐adrenoreceptor agonist, was found to interfere with the development and progression of hamster hereditary polymyopathy. Cytoprotection involved both skeletal and heart muscles with reduced myofibrillar degeneration, phagocytosis, and an unusual scarring process rarely seen at this stage of the disease. A decrease in the Ca content of heart and hemidiaphragm homogenates corroborated these findings. The significant drop of serum creatine kinase with restoration of alkaline phosphatase activity towards normal values provided additional support to the therapeutic effect of ISO. Except for an increase in magnesium, there were no changes in serum electrolytes. The modifications in plasma membrane permeability together with improvement in microcirculation are some of the features whereby ISO can ameliorate muscle cell energy metabolism. It is inferred that the alleged primary role of calcium in the development of this inherited myopathy should be further scrutinized.
Acetylcholinesterase and ATPases in motor neuron degenerative diseases
Authors: Rasool C. G. , Chad D. , Bradley W. G. , Connolly B. , Baruah J. K. 
Published: January 2005

AbstractAcetylcholinesterase (AChE) activity was measured in the presence of the specific inhibitor of pseudocholinesterase, iso‐OMPA, in plasma from patients with amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), neuromuscular disease controls, and normal controls. Both AChE and Na‐K ATPase activities were measured in erythrocyte ghost membranes from ALS and normal controls. Activities of erythrocyte ghost AChE and Na‐K ATPase did not differ between ALS and control patients, suggesting that erythrocyte membranes were normal in ALS. However, the activity of plasma AChE in patients with ALS and PMA was increased significantly over plasma activity in disease controls and normal controls. In addition, in an animal model of human PMA, the Wobbler mouse, plasma AChE activity was increased significantly over littermate controls. The explanation for the increase in plasma acetylcholinesterase was not clear; however, a number of potentially useful clinical points followed from this study. First, there was no relationship between a specific subtype of motor neuron disease and the level of AChE activity. Second, AChE activity appeared to vary directly with the duration of PMA but not with the severity of PMA. This did not correlate with either the duration or severity of ALS. Last, plasma AChE activity was normal in about 30% of patients who had motor neuron disease; therefore, AChE assay had limited use in the diagnosis of ALS or PMA.
Saphenous nerve evoked potentials and the assessment of intraabdominal lesions of the femoral nerve
Authors: Synek V. M. , Cowan J. C. 
Published: January 2005

AbstractA technique for recording somatosensory evoked potentials after stimulation of the patellar branch of the saphenous nerve is described and the results from 11 normal subjects and from two patients with intraabdominal femoral nerve injuries are presented. One patient developed a nerve lesion after an abdominal surgical procedure and the second suffered a gunshot wound causing femoral nerve paralysis. Both patients recovered spontaneously. Evoked potentials from saphenous nerves showed a significant discrepancy between faster motor recovery and delay in reinstating sensory axonal conduction.
Evolution of nerve conduction abnormalities in children with dominant hypertrophic neuropathy of the charcot‐marie‐tooth type
Authors: Gutmann Ludwig , Fakadej Alexander , Riggs Jack E. 
Published: January 2005

AbstractSerial motor conduction velocities and distal motor latencies were determined in two pairs of dizygotic twins, each born to a parent with dominant hypertrophic neuropathy of the Charcot‐Marie‐Tooth type (HMSN‐I). Motor nerve conduction velocities could not distinguish between the normal and affected twin of the first pair studied at birth. Distal motor latency in the affected twin at birth, however, was border‐line prolonged. The affected twin of the second pair had slowed motor velocities at age 17 months, but the extent of conduction slowing had not yet fully developed. Studies of these patients and the affected family members showed that maximal slowing of motor nerve conduction velocities evolved over the first 3–5 years of life in HMSN‐I. Prolongation of distal motor latency may be the earliest abnormality observed in HMSN‐I and this abnormality evolves over 10 or more years.
AAEE minimonograph #20: Macro EMG
Authors: Stålberg Erik 
Published: January 2005

AbstractA new EMG recording technique called macro EMG is described. The recording electrode is the cannula of the modified single fiber electromyography (SFEMG) electrode. By means of spike triggered averaging, the contribution from all muscle fibers in a motor unit is extracted. The resulting signal reflects the number and size of muscle fibers in one motor unit. This paper presents the details of the technique, the neurophysiological basis for generation of the macro EMG signal, and the typical findings in normal and diseased muscles. Finally, examples are given of the additional information that can be obtained by combining investigations with SFEMG, conventional EMG, and macro EMG in the same muscles.
The distribution of conduction velocities (DCV) in peripheral nerves: A review
Authors: Dorfman Leslie J. 
Published: January 2005

AbstractRecent advances in digital signal processing have permitted the development of clinically relevant, noninvasive, computer‐based methods for estimating the distribution of conduction velocities (DCV) in motor, sensory, and mixed populations of large myelinated nerve fibers. All currently available methods incorporate explicit and implicit assumptions about the nature of the unit impulses under study. Preliminary investigations using DCV methods have clarified some issues concerning conduction of impulses in the different fiber subpopulations of normal and diseased human nerves. In the presence of severe nerve disease, DCV analysis is usually either impractical or superfluous; additional studies are needed to define its range of clinical applicability. Extension of this technology to clinical analysis of small myelinated and unmyelinated fiber populations will require improvements in the techniques of nerve stimulation and recording.
Hypokalemic periodic paralysis: In vitro investigation of muscle fiber membrane parameters
Authors: Rüdel Reinhardt , Lehmann‐Horn Frank , Ricker Kenneth , Küther Gerald 
Published: January 2005

AbstractTo study the mechanism of attacks in familial hypokalemic paralysis, we recorded resting membrane potentials, action potentials, current–voltage relationships, and isometric forces in intercostal muscle fibers from three patients. In normal extracellular medium, the resting potential was reduced, but membrane conductance was not different from control. Excitability was reduced and the action potentials had no overshoot. On exposure to a 1‐mM potassium solution, with or without insulin, the cells depolarized to about −50 mV, and became inexcitable. Over the tested membrane potential range from −120 to −40 mV, the slope conductance in the 1‐mM potassium solution was not different from that of control fibers in a 1‐mM potassium solution. In particular, the potassium component conductance was not reduced. Depolarized fibers could not be completely repolarized by returning to a 3.5‐mM potassium solution. An experimentally induced transient shift of the chloride equilibrium potential to a highly negative value caused stable repolarization. Paralysis could also be induced by replacement of extracellular chloride with an impermanent anion, a treatment which causes myotonia in healthy fibers. It was concluded that the basic defects are a reduced excitability and an increased sodium conductance, and that these defects are aggravated on reduction of the extracellular potassium concentration.
A new approach to intramuscular placement of horseradish peroxidase
Authors: Klueber Kathleen M. , Ontell Marcia 
Published: January 2005

AbstractA new method of intramuscular placement of a semisolid paste of horseradish peroxidase (HRP) using a root canal file is described. This method has been demonstrated to produce consistent and reproducible numbers of labeled motoneurons in the mouse extensor digitorim longus muscle (EDL). While the number of labeled cells is similar to that found with optimal intramuscular injection of liquid HRP, this new technique provides more reproducible results by eliminating the reflux often associated with intramuscular injection of liquid HRP.