bluuebunny/crossref_metadata_2025_split huggingface dataset:
Spinal fusion in duchenne muscular dystrophy: A multidisciplinary approach
Authors: Shapiro Frederic , Sethna Navil , Colan Steven , Wohl Mary Ellen , Specht Linda 
Published: January 2005

AbstractWe studied 27 Duchenne muscular dystophy patients having spinal fusion for scoliosis. One patient died intraoperatively of cardiac arrest; all others have done well with no instances of malignant hyperthermia, postoperative ventilatory system dependence, pneumothorax, persisting infection, neurologic damage, nonunion, or pain. The anesthetic management included primarily intravenous general anesthetics with minimal myocardial depressant effects, avoiding succinylcholine and inhalation agents. Preoperative cardiac studies aided anesthetic management intra‐operatively. There was analmost universal sinus tachycardia. Holter monitoring defined 4 fo 16 with ventricular premature beats, 4 of 16 with atrial premature beats, and no ventricular tachycardia or atrial flutter or fibrillation. Echocardiogram demonstrated mitral prolapse in 2 of 22, frequent abnormal systolic performance with abnormal shortening fraction < 28% in 7 of 16, and reduced rate‐corrected velocity of fiber shortening in 9 of 15. Afterload was elevated in 7 of 15. The mean forced vital capacity (FVC) preoperatively was 45.3 ± 15.9% with continuing diminution to 28.7 ± 14.9% at 3.3 ± 2.2 years after surgery. The main benefit of surgical stabilization is the relative ease and comfort of wheelchair seating compared with those nonoperated patients who develop progressive deformity. We have not seen lasting improvement or stabilization in FVC following surgery as decreasing function is related primarily to muscle weakness.
Neuromuscular transmission as a function of motor unit size in patients with prior polimyelitis
Authors: Maselli Ricardo A. , Cashman Neil R. , Wollman Robert L. , Salazar‐Grueso Edgar F. , Roos Raymond 
Published: January 2005

AbstractWe studied neuromuscular transmission in 16 patients with prior poliomyelitis by measuring single fiber electromyographic (SFEMG) jitter. This was compared with 3 indirect methods of assessing reinnervation: SFEMG fiber density, macro EMG, and the presence of fibre type grouping on muscle biopsy. In patients with acute poliomyelitis before the age of 10, there was a positive correlation between the extent of neuromuscular transmission impairment, demonstrated by increased SFEMG jitter, and the enlargement of the motor unit, as indicated by increased fiber density, increased macro EMG signals, and fiber type grouping on muscle biopsy. However, there was no correlation between any of these parameters and the presence or absence of new symptoms of weakness. These findings suggest that impaired neuromuscular transmission is most common in patients with prior poliomyelitis whose motor units have been maximally enlarged by axonal sprouting, but is independent of the presence or absence of new symptoms of weakness.
Beneficial effects of training on developing dystrophic muscle
Authors: Elder Geoffrey C. B. 
Published: January 2005

AbstractThe objective of this study was to determine whether increased contractile activity is beneficial or detrimental to developing dystrophic muscle. Hamsters (20‐days‐old) were gradually introdued to running at a speed of 14m/min at 10% grade for 2 (T2) or 4 (T4) h/d for 4 weeks, 5 d/wk. Histological and fiber type properties were determined in the soleus (SOL), plantaris (PL), and extensor digitorum longus and contractile properties in SOL and PL from 5 animals/group, including 5 controls. Experimental animals had normal body and muscle mass. Training for 2 h/d had little effect on SOL contractile properties, whereas 4 h/d resulted in significant increases in force, percentage of type I fibers, and type I hypertrophy. Force also increased in PL. Muscle necrosis was reduced in SOL (T2 and T4) and unchanged in PL. In conclusion, endurance training generally had a beneficial or, at least, no detrimental effect on developing dystrophic muscles.
Needle electromyography of the diaphragm
Authors: Bolton Charles F. , Grand'maison Francois , Parkes Anthony , Shkrum Michael 
Published: January 2005

AbstractA method is described for performing needle electromyography of the diaphram which is safe and causes little discomfort. It provides valuable information concerning neuropathies and myopathies which may affect the diaphragm, and complements information derived form phrenic nerve conduction studies. Firing patterns of motor unit potentials during spontaneous respiration identify upper motor neurone disorders causing respiratory insufficiency.
Cutaneous reflexes in Parkinson's disease
Authors: Fuhr Peter , Zeffiro Thomas , Hallett Mark 
Published: January 2005

AbstractThe physiology of rigidity in Parkinson's disease (PD) can be investigated by the study of reflexes. Cutaneous reflexes (CR) were measured in 10 patients with PD and in 10 age‐ and sex‐matched normal volunteers. EMG activity was recorded from the first dorsal interosseous muscle with surface electrodes, rectified and averaged. The index finger was stimulated with an intensity four times the sensory threshold. The subjects abducted the index finger with 20% of maximal force. While the latencies of the different reflex components and the amplitudes of the excitatory peaks were not different in the two groups, the first inhibitory components was less pronounced in patients with PD as compared with normals. This effect is partially reversed with dopaminergic drug treatment. The results are compatible with the loss of an inhibitory spinal mechanism elicited by cutaneous afferents, and can be a partial explanation for increased tone in PD.
Quantitation of axon loss and conduction block in acute radial nerve palsies
Authors: Watson Bradley V. , Brown William F. 
Published: January 2005

AbstractEleven acute radial nerve palsies were examined between 3 days and 14 weeks following the onset of the neuropathy. Our objective was to quantify the relative extent of axon loss and conduction block in radial motor fibers supplying the extensor and abductor pollicis longus (EPL/APL) muscles. In 10 of 11 cases, conduction block exceeded axon loss. Maximum motor and sensory conduction velocities were normal distal to the spiral groove, suggesting that the larger myelinated fibers were not selectively involved in this acute neuropathy. The localization of the conduction block and slowing was, in every case, across the spiral groove. This method provides a relatively simple way of assessing the approximate contributions of axon loss and conduction block, and fits well with the early and usually complete clinical recovery in these cases.
Myotrophic effects of an anabolic steroid in rabbit limb muscles
Authors: Salmons Stanley 
Published: January 2005

AbstractThere has been no reliable evidence that the actions of anabolic steroids extend to limb muscles. In this study, female rabbits were treated with anabolic steroid (nandrolone decanoate) or arachis oil placebo for 4 weeks or 12 weeks. After 12 weeks, tibialis anterior muscles of treated animals showed highly significant increases in wet weight (38%), twitch tension (66%), maximum isometric tetanic tension (48%), maximum cross‐sectional area (27%), and specific tension (17%). Fiber type composition showed a significant trend toward a less oxidative metabolic character. The experiments provided clear physiological and morphological evidence of a steroid‐induced hypertrophy that was not attributable to fluid retention or changes in body weight. Of the muscles examined, the myotrophic effect was confined to the tibialis anterior muscle; extensor digitorum longus, plantaris, and soleus muscles showed no significant response. The work establishes an experimental model for the response of limb muscles to anabolic compounds.
The influence of the stimulus on normal sural nerve conduction velocity: A study of the latency of activation
Authors: Krarup Christian , Horowitz Steven H. , Dahl Kristian 
Published: January 2005

AbstractConduction along the sural nerve was studied in 64 normal subjects using near‐nerve electrodes. Conduction velocities over the same nerve segments were calculated: (1) from the latency recorded from a site of stimulation to a site of recording (1R‐method); and (2) from the difference in latency between 2 recording sites, the site of stimulation being situated elsewhere along the nerve (2R‐method). Consistently faster velocities were seen with the 2R‐method and could best be explained by a fixed delay of about 0.15 ms at the stimulus site (latency of activation, utilization time). This delay was markedly prolonged when a ramp rather than a rectangular stimulus was applied, though fast fibers were excited with both types of stimuli. The delay is thought to be dependent on the relationship between the density of current at the stimulus site and the threshold of responding fibers. © 1992 John Wiley & Sons, Inc.
Case‐of‐the‐month: Painful thigh mass in a young woman: Diabetic muscle infarction
Authors: Barohn Richard J. , Kissel John T. 
Published: January 2005

AbstractA 29‐year‐old female with type I diabetes mellitus developed pain, focal tenderness, and swelling in the posterior left thigh. Subsequent evaluation included a muscle biopsy, which revealed large confluent areas of necrosis and edema, compatible with a diagnosis of diabetic muscle infarction (DMI). Diabetic muscle infarction (DMI) is an unusual neuromuscular complication of diabetes mellitus. DMI begins with the acute onset of focal pain and swelling in the thigh. The anterior compartment (quadriceps muscle group) or posterior compartment (hamstring muscle group) are most frequently involved. The focal region of muscle damage can be noninvasively viewed by magnetic resonance imaging and radionuclide scans. Muscle biopsy demonstrates large confluent regions of muscle necrosis and edema. DMI needs to be differentiated from other processes that can cause leg pain in a diabetic patient.
A PCR‐based assay for the wild‐type dystrophin gene transferred into mdx mouse
Authors: Shrager Joseph B. , Naji Ali , Kelly Alan M. , Stedman Hansell H. 
Published: January 2005

AbstractMyoblast transfer has emerged as a promising treatment for inherited myopathies such as Duchenne muscular dystrophy (DMD). Further development of the technique's therapeutic potential requires an experimental system in which issues of graft rejection can be clearly discriminated from those related to myoblast biology. Here we report the development and initial application of a quantitative assay for myogenic cells bearing a wild‐type dystrophin gene following transfer into the mdx mouse. The technique relies upon the ability of a mutagenizing polymerase chain reaction (PCR) primer to create a new restriction site in the amplification production of the wild‐type, but not the mdx dystrophin gene. The ratio of host to donor cells can be determined from muscle biopsies as small as 1 mg, regardless of donor H‐2 background. This simple technique should allow a number of basic questions related to myoblast and direct gene transfer to be addressed using the mdx mouse model. © 1992 John Wiley & Sons, Inc.
Abnormal distribution of desmin and vimentin in myofibers in adult onset myotubular myopathy
Authors: Misra Ajay K. , Menon Nirmala K. , Mishra Shri K. 
Published: January 2005

AbstractWe report, for the first time, muscle immunocytochemical studies in sporadic, adult onset myotubular myopathy (SAOMM), which show intramyofibrillar central, perinuclear desmin and vimentin. This pattern was absent in a normal control and in myofibers with increased internal nuclei associated with denervation and myotonic muscular dystrophy (MyD). These findings resemble those reported in 8‐ to 15‐week‐old human fetal myotubes and myofibers of infantile MM, implying a possible regression of intermediate filaments of adult myofibers to an early developmental phase in SAOMM. © 1992 John Wiley & Sons, Inc.
Turns‐amplitude analysis of the electromyographic recruitment pattern disregarding force measurement. II. Findings in patients with neuromuscular disorders
Authors: Liguori Rocco , Dahl Kristian , Fuglsang‐Frederiksen Anders , Trojaborg Werner 
Published: January 2005

AbstractTurns‐amplitude analysis of the electromyographic recruitment pattern was performed on‐line in the brachial biceps muscle of 46 patients with neuromuscular disorders using the mean amplitude as an indicator of force. The parameters, peak‐ratio (PR) and number of time intervals (TI) from 0 to 1.5 ms, were increased in patients with myopathy. In patients with neurogenic involvement, the characteristic pattern was a decreased PR and a decreased incidence of TI between 0 and 1.5 ms. The results indicate that the two parameters supplement each other as some of the patients were identified only by one or the other. In patients with myopathy, the method had a higher diagnostic yield than the individual motor unit action potential analysis. The method is objective, fast, and reliable. © 1992 John Wiley & Sons, Inc.
Evidence for new isoform of fast myosin heavy chain in rat skeletal muscle
Authors: Sawchak Judith A. , Leung Betty , Shafiq Sayid A. 
Published: January 2005

AbstractThe purpose of this study was to investigate whether the previously demonstrated heterogeneity of myosin heavy chain (MHC) in type 2B fibers of the superficial portion of the lateral gastrocnemius (SLG) muscle of the rat may be due to presence of type 2D/X fibers. Immunohistochemical identification of MHC heterogeneity, histochemical identification of appendicular 2D/X muscle fibers, and immunoblotting of myosins were used. It was found that some,but not all, of the heterogeneity of rat SLG is correlated with the presence of type 2D/X fibers. Immunoblots with MHC from several muscles revealed the presence of at least two antigenically distinct isoforms of MHC with the same electrophoretic mobility as the “2B” band. These results show that a previously undetected type of MHC is expressed in rat skeletal muscle and raise the possibility that there may be as yet undetected MHC isoforms in human muscle which may be clinically important. © 1992 John Wiley & Sons, Inc.
The quadriceps strength of healthy elderly people remeasured after eight years
Authors: Greig Carolyn A. , Botella Jose , Young Archie 
Published: January 2005

AbstractIsometric quadriceps strength was remeasured in 14 healthy survivors of a group of elderly people first studied 8 years previously. There were 4 men (median age 81 years, range 79 to 84) and 10 women (82 years, range 79 to 89). They were selected for their health, not their levels of physical activity. Nevertheless, they were active when first studied and, with 1 exception, had maintained or increased their levels of physical activity. Isometric quadriceps strength was well preserved; the median change in the strength of the stronger quadriceps was only −0.3% per annum (95% confidence interval = −1.4 to +0.8). © 1993 John Wiley & Sons, Inc.
“All‐or‐none” cytochrome C oxidase positivity in mitochondria in chronic progressive external ophthalmoplegia: An ultrastructural—cytochemical study
Authors: Matsuoka Taro , Goto Yu‐Ichi , Nonaka Ikuya 
Published: January 2005

AbstractTeased single muscle fibers from 6 patients with chronic progressive external ophthalmoplegia (CPEO) showed a segmental defect in cytochrome c oxidase (COX) activity. On ultrastructural–cytochemical examination, the majority of mitochondria in COX‐positive segments were COX‐positive, whereas all mitochondria in COX‐negative segments were COX‐negative. This “all‐or‐none” COX positivity in mitochondria in CPEO with deleted mitochondrial DNA can be explained by the “threshold effect,” which induces the tissue‐specific involvement and clinical heterogeneity. © 1993 John Wiley & Sons, Inc.
Frequency of acoustic myography during isometric contraction of fresh and fatigued muscle and during dynamic contractions
Authors: Dalton Philip A. , Stokes Maria J. 
Published: January 2005

AbstractThe frequency of the acoustic myographic (AMG) signal was examined during fresh and fatigued isometric contractions of quadriceps and during dynamic contractions of biceps brachii (BB) in healthy subjects. Recordings were obtained from quadriceps over a range of forces between 10% and 100% maximal voluntary contraction prior to, and 15 minutes after, a fatiguing exercise. Recordings from BB were obtained over a range of submaximal forces (0–8.5 kg) during concentric and eccentric contractions. The mean power frequency (MPF) of the AMG signal was analyzed during each of these contractions by fast‐Fourier transform (FFT). The MPF was not significantly different (P > 0.05) during fresh and fatigued contractions of quadriceps and increased quadratically with force in both states (r = 0.81, fresh; r = 0.77, fatiqued). During concentric contractions of BB the MPF initially with force, but then decreased at the heavier loads (>5.5 kg). The MPF of eccentric contractions did not significantly (P > 0.05) alter with force. The AMG MPF was within a similar low frequency range for both muscles, during different types of contraction, and was unaltered with fatigue. © 1993 John Wiley & Sons, Inc.
Innervation patterns in the cat tibialis anterior six months after self‐reinnervation
Authors: Bodine‐Fowler Sue C. , Unguez Graciela A. , Roy Roland R. , Armstrong Alison N. , Edgerton V. Reggie 
Published: January 2005

AbstractThe spatial distribution of fibers belonging to a single motor unit was analyzed in 10 motor units from the tibialis anterior of the cat 6 months after denervation and self‐reinnervation of the anterior (superficial) compartment of the muscle. After self‐reinnervation, the distribution patterns of the fibers in the fast motor units were significantly different than control, whereas the fiber distribution in the slow unit was similar to control. Reinnervated fast units had a significant increase in the number of adjacencies among motor unit fibers, and there were often distinct “clusters or groups” of fibers distributed within the motor unit territory. Clustering or grouping of fibers was evident within the motor unit, even though fiber type grouping was not evident within the muscle. The differences in distribution patterns between control and reinnervated units may be related to variations in the branching pattern of axons during reinnervation compared to the process that occurs during development. © 1993 John Wiley & Sons, Inc.
AAEM case report #26: Seventh cranial neuropathy
Authors: Gilchrist James M. 
Published: January 2005

AbstractAAEM CASE REPORT #26. A 25‐year‐old man with acute, bilateral facial palsies is presented. He had a lymphocytic meningitis, history of tick bites, and lived in an area endemic for Lyme disease, which was ultimately confirmed by serology. Electrodiagnostic investigation included facial motor nerve study, blink reflex and electromyography of facial muscles, which were indicative of a neurapraxic lesion on the right and an axonopathic lesion on the left. The clinical course was consistent with these findings as the right side fully recovered and the left remained plegic. The clinical features of Lyme associated facial neuritis are reviewed, as is the electrodiagnostic evaluation of facial palsy.
Motor unit estimation: Reproducibility of the spike‐triggered averaging technique in normal and ALS subjects
Authors: Bromberg Mark B. 
Published: January 2005

AbstractReproducibility of the spike‐triggered averaging technique of motor unit estimation (MUE) was assessed in biceps‐brachialis muscle in 10 normal subjects and 15 subjects with amyotrophic lateral sclerosis (ALS). MUE was calculated by dividing the compound muscle action potential by the mean amplitude of 15 surface motor unit potentials (S‐MUPs) of low recruitment threshold. Averaged MUE values in normal subjects were higher than in ALS subjects, with few values overlapping. Differences between test and retest MUE values were not significant for either subject group. The relative differences between test‐retest values were 45.3% for normal subjects and 32.6% for ALS subjects. Correlation coefficients between test and retest values were low (r = 0.07) for normal subjects when influential outlying points were removed, and higher (r = 0.65) for ALS subjects when individuals with MUE values within the normal range were removed. The higher correlation of testretest MUE values in ALS subjects compared to normal subjects may be due to a greater probability of resampling among the smaller number of motor units in ALS subjects. In summary, the reproducibility and technical aspects of the spike‐triggered averaging technique are similar to those reported for other MUE techniques. © 1993 John Wiley & Soncs, Inc.
Single channel properties of synaptic acetylcholine receptors in dystrophic fibers
Authors: Brennan Christine , Henderson Leslie P. 
Published: January 2005

AbstractNeuromuscular transmission in dystrophic mice has been extensively studied through analysis of nerve‐evoked endplate potentials. In the present study, single channel ACh‐induced activity recorded from endplates of fast twitch muscle fibers from mdx and dy dystrophic mice, was compared with activity recorded from wild‐type fiber endplates to ascertain whether expression of these phenotypes leads to changes in ACh receptor properties and synaptic transmission. An 89 pS class of ACh‐induced events predominated in recordings from wild‐type and both strains of dystrophic mice. The mean open times for this class of events was well described by two exponential components, one with a voltage‐independent time constant of ∼ 0.3 ms and the other with a time constant of 2–5 ms which increased e‐fold with ∼ 120 mV of hyperpolarization. The expression of the mdx or the dy phenotype was not associated with significant differences in the conductance, distribution of open durations, or the voltage‐dependence of the mean open time for this class of ACh‐induced events. © 1993 John Wiley & Soncs, Inc.
Acute conduction block in vitro following exposure to antiganglioside sera
Authors: Arasaki Keisuke , Kusunoki Susumu , Kudo Norio , Kanazawa Ichiro 
Published: January 2005

AbstractWe studied the role of antiganglioside antibodies in the pathogenesis of autoimmune neuropathies using an in vitro preparation of a rat sciatic nerve. Human and rabbit sera with high titers of the antibodies were applied to a restricted segment of the sciatic nerve mounted in a recording chamber, and the compound nerve action potentials of the myelinated and unmyelinated fibers were observed. Myelinated fiber conduction became blocked at the segment within a few hours, whereas the unmyelinated fiber conduction remained unchanged. These results suggest that antiganglioside sera directly produce an acute conduction block only in myelinated nerve fibers and that this in vitro model is useful for studying the ionic mechanism by which the acute conduction block occurs. © 1993 John Wiley & Sons, Inc.