bluuebunny/crossref_metadata_2025_split huggingface dataset:
AAEM minimonograph #43: Neuromuscular problems in the performing arts
Authors: Lederman Richard J. 
Published: January 2005

AbstractOver the past 10–15 years, there has been increasing interest in the health problems of performing artists. In this review, I will discuss the major playing‐related disorders seen in instrumental musicians. Among the 672 instrumentalists evaluated, the major diagnoses identified included musculoskeletal disorders in 64%, peripheral nerve problems in 22.5%. and focal dystonia in 7%. Sixty percent of instrumentalists were female although males predominate in the group with focal dystonia. The average age of those evaluated was 32 years. Among musculoskeletal disorders overuse syndrome is the most common. Frequent peripheral nerve disorders include thoracic outlet syndrome, carpal tunnel syndrome, and ulnar neuropathy. A characteristic distribution of symptoms and signs is identified for each instrument group. Electrodiagnostic studies are an important part of the evaluation of these disorders. With carefully designed treatment, the majority of instrumental musicians can be returned to full and pain‐free playing activities. The success rate is highest in some entrapment neuropathies but remains low in focal dystonia. © 1994 John Wiley & Sons, Inc.
Osteomalacic myopathy
Authors: Russell James A. 
Published: January 2005

AbstractA 45‐year‐old woman reported the development of thigh pain followed within a year by proximal muscle weakness. Clinical findings included short stature, prominent kyphoscoliosis, proximal weakness, and brisk reflexes. Recognition of an increased level of serum alkaline phosphatase and hypophosphatemia led to the diagnosis of osteomalacia. Identification of iron deficiency anemia and hypocholesterolemia implicated previously unrecognized gluten‐sensitive enteropathy with associated vitamin D malabsorption as the cause of the osteomalacia. Adherence to a gluten‐free diet and treatment with vitamin D2 resulted in weight gain, resolution of pain, and improvement in strength within 3 months. Painful proximal weakness and hyperreflexia may be the initial and primary manifestations of osteomalacia, a readily treatable cause of muscle and bone disease. © 1994 John Wiley & Sons, Inc.
Pathogenesis of axonal Guillain–Barré syndrome: Hypothesis
Authors: Yuki Nobuhiro 
Published: January 2005

AbstractPathologic studies of acute motor axonal neuropathy show strong evidence of the presence of primary axonal Guillain–Barré syndrome (GBS). The pathogenesis of axonaf GBS is speculated to be as follows: (1) Infection by an organism induces the high production of a crossreactive antibody between an infectious agent and the motor nerve axon in patients with a particular immunogenetic background. (2) The antineural antibody binds to the motor nerve terminals, thereby inhibiting motoneuron excitability and causing muscular weakness. (3) Binding of the antineural antibody, or subsequent functional impairment of the motoneurons, causes the motor axon to degenerate from the terminals. (4) In severe cases, extensive axonal loss and central chromatolysis of the motoneurons occur. These inhibit recovery and lead to a poor functional prognosis. © 1994 John Wiley & Sons, Inc.
Muscle histopathology in myotonic dystrophy in relation to age and muscular weakness
Authors: Tohgi Hideo , Kawamorita Atsushi , Utsugisawa Kimiaki , Yamagata Munehisa , Sano Mitsuhiko 
Published: January 2005

AbstractWe studied histopathological changes in the biceps brachii muscle in relation to age and the degree of muscle weakness in 64 patients (aged 11–59 years) with myotonic dystrophy. The proportion of type 1 fibers was unaltered in the adolescent patients compared with control values, but increased with age. The average diameters of all the fiber types were smaller than control values in the adolescents, suggesting immature development; however, there was an increase in diameter with age that was associated with an increase of hypertrophic type 2 fibers. At all ages, type 1 fibers were smaller than type 2 ones. Small angular fibers and small group atrophy consisted mainly of type 1 fibers, their incidences decreasing with age. The severity of muscular weakness was related to the predominance of type 1 fibers, the reduction in the number of hypertrophic type 2 fibers, and the accumulation of adipose cells, but not to the presence of small angular fibers or to small group atrophy. © 1994 John Wiley & Sons, Inc.
Fetus‐like dystrophin expression and other cytoskeletal protein abnormalities in centronuclear myopathies
Authors: Mora Marina , Morandi Lucia , Merlini Luciano , Vita Giuseppe , Baradello Alice , Barresi Rita , Di Blasi Claudia , Blasevich Flavia , Gebbia Marinella , Daniel Sergio , Cornelio Ferdinando 
Published: January 2005

AbstractWe have investigated supposed maturational arrest of muscle in ceatronuclear myopathies (CNMs) by characterizing the expression of dystrophin, other cytoskeletal proteins, and fetal myosin in the muscle fibers of 9 CNM patients (4 sporadic, 3 familial, 2 adult sporadic). Dystrophin and β‐spectrin localized intracytoplasmically in centrally nucleated fibers. Talin and vinculin were normally expressed. Desmin was radially organized in several fibers in all patients. Scattered vimentin positive fibers were found in 3 cases. Six myotonic dystrophy cases and 4 inflammatory myopathy cases with regenerating fibers were also studied: dystrophin and the membrane cytoskeletal proteins were normally expressed in the former; and dystrophin, spectrin, and vinculin were reduced in the latter. Intracytoplasmic dystrophin is further evidence of maturational arrest in CNMs. Spectrin and dystrophin codistnbute in these pathological conditions as in normal muscle. We conclude that the altered cytoskeletal network found in CNMs likely plays a pathogenetic role in these conditions. © 1994 John Wiley & Sons, Inc.
Assessment of motor pathways to masticatory muscles: An examination technique using electrical and magnetic stimulation
Authors: Türk Ülkü , Rösler Kai M. , Mathis Johannes , Müllbacher Wolf , Hess Christian W. 
Published: January 2005

AbstractTo study motor pathways to masticatory muscles, a new recording technique using surface electrodes was developed. The recording electrode was mounted on a spatula and inserted enorally into the pterygomandibular plica over the belly of m. masseter. Using this technique, mean latencies/amplitudes of the compound action potentials (CMAPs) in 18 healthy subjects were 1.2 ms/4.9 mV after electrical stimulation of the trigeminal nerve below the zygomatic arch, and 5.5 ms/1.1 mV after magnetic stimulation of the cortex. In 15 patients with unilateral lesions of the facial nerve, masticatory CMAPs had virtually symmetrical configuration, latency, and amplitude, excluding a major contribution of volume conducted activity from other cranial muscles. The technique was evaluated in patients after surgical treatment for trigeminal neuralgia. Patients with with retrogasserian thermocoagulation and central demyelinating lesions were consistently identified. © 1994 John Wiley & Sons, Inc.
Sixth annual stuart reiner memorial lecture: Embryonic development of nerve and muscle
Authors: Sohal G. S. 
Published: January 2005

AbstractThis article provides a basic scheme of sequential anatomic and some physiologic events occurring during the course of embryonic development of motor neurons and muscles, leading to the establishment of mature nerve‐muscle relationships. Motor neurons and muscles begin their development independently and during embryogenesis they become dependent on each other for further development and survival. Aspects of development which occur independently and those requiring mutual interactions are identified. The development of motor neurons is discussed with respect to their production, projection, neuromuscular transmission, myelination, sprouting, survival, and death. The development of muscles is discussed with respect to the origin, differentiation, and muscle fiber types. Discussion on the development of neuromascular junction includes differentiation of presynaptic nerve terrninal, postsynaptic components, and elimination of multiple axons.© 1995 John Wiley &Sons, Inc.
CMAP variability as a function of electrode site and size
Authors: Van Dijk J. Gert , van der Kamp W. , Tjon‐A‐Tsien Aimee 
Published: January 2005

AbstractThe site of the recording electrode influences the amplitude of the compound muscle action potential (CMAP) and its variation over a length of nerve. The effects of large electrodes on this source of intra‐individual variability were assessed. Right median nerves of 20 healthy subjects were studied, and recordings made at three sites (at 1‐cm intervals) using five electrode sizes (0.01, 1, 2, 4, and 10 cm2). Site‐induced variability was defined as the standard deviation (SDi) and coefficient of variation (CVi) of the measurements of the three sites. Site induced variability of all parameters (latency, duration, amplitude, area, MNCV, and the percentile changes of duration, amplitude, and area over the forearm) decreased significantly with electrode size. Decreases were most pronounced for amplitude and area: CVi fell from 29% and 30% (0.01‐cm2 electrode) to 10% and 8% (10 cm2). It is argued that large electrodes record activity of more motor units than small electrodes, and that their measurement fields overlap to a greater extent. The use of large electrodes is recommended in order to reduce site‐induced CMAP variability.© 1995 John Wiley &Sons, Inc.
Monopolar needle evaluation of paraspinal musculature in the cervical, thoracic, and lumbar regions and the effects of aging
Authors: Travlos Andrew , St Trueman Sandra , Eisen Andrew 
Published: January 2005

AbstractMotor unit characteristics (Phases, turns, amplitude, duration, and area) were measured using a monopolar needle and narrowed bandpass (500 Hz to 10 kHz) in cervical, thoracic, and lumbar paraspinal muscles in 66 subjects (36 women and 30 men) aged 22‐81 years (mean age 45.7 years). Cervical motor units were of lower amplitude and area than thoracic and lumbar motor units (P = 0.0001), respectively. The durations of lumbar motor units were longer and larger than cervical and thoracic motor units (P = 0.0004). Analysis of covariance of the measured characteristics against age showed no significance in the cervical and thoracic regions. In lumbar paraspinal motor units, phases and turns increased significantly with age (P = 0.044 and P = 0.016, respectively). The increase was < 1.0 phase over 30 years. Motor unit amplitude increased with aging (P = 0.055) showing an increase of 360 μV over a 30‐year time period. It is appropriate to assess the number of phases and turns, as well as the duration of the motor units in the cervical and thoracic (but not lumbar) spines in addition to seeking evidence of spontaneous, single fiber discharges.© 1995 John Wiley &Sons, Inc.
Late motor involvement in cases presenting as “chronic sensory demyelinating polyneuropathy”
Authors: Berger Alan R. , Herskovitz Steven , Kaplan Jerry 
Published: January 2005

AbstractChronic inflammatory demyelinating polyneuropathy (CIDP) is usually characterized by prominent motor deficits. A pure sensory presentation, labeled chronic sensory demyelinating neuropathy (CSDN), has been reported, but it is unclear if this neuropathy is a distinct clinical and immunologic entity or merely the sensory presentation of the more usual sensorimotor CIDP. We describe 5 patients with what initially appeared to be CSDN; 3 subsequently developed substantial weakness coincident with the electrophysiologic appearance of multifocal motor conduction block. These cases indicate that, in some cases, CSDN may be a transitional clinical stage of CIDP in which the more usual sensorimotor deficits develop later. Immune‐based therapy, including intravenous immunoglobulin, was found to be effective in both the pure sensory and sensorimotor types. © 1995 John Wiley & Sons, Inc.
Issues & opinion. Sporadic ALS and chromosome 22: Evidence for a possible neurofilament gene defect
Authors: Meyer Michael A. , Potter Nicholas T. 
Published: January 2005

AbstractALS is associated with the P2 blood group phenotype. Molecular evidence now shows the gene encoding this antigen to be on the long arm of human chromosome 22 near the newly discovered gene for heavy neurofilament (NF‐H). Since an ALS‐type condition can be generated in transgenic mice expressing the human NF‐H gene, and since the gene for the CNTF‐related cytokine leukemia inhibitory factor (LIF) is located adjacent to this gene, it is hypothesized that a defect on the chromosome 22 band region q12 is involved in the pathogenesis of sporadic ALS. © 1995 John Wiley & Sons, Inc.
Issues & opinion. Neurofilaments, free radicals, excitotoxins, and amyotrophic lateral sclerosis
Authors: Al‐Chalabi Ammar , Powell John F. , Leigh P. Nigel 
Published: January 2005

AbstractThere is increasing evidence implicating abnormalities of neurofilament function in the pathogenesis of amyotrophic lateral sclerosis (ALS). The observation that the P2 blood protein phenotype is overrepresented in patients with ALS is potentially important, but needs confirmation. It should be shown that this segregation is selective for ALS. If it is, the implications outlined in Meyer's hypothesis will need to be explored, bearing in mind that much of the evidence implicating excitotoins, free redicals, and neurofilaments in familial and sporadic ALS is still circumstantial. Thus the identification of candidate genes, the pursuit of large segregation studies, and identification of specific point mutations, remain key goals in ALS research. © 1995 John Wiley & Sons, Inc.
AAEM minimonograph #16: Instrumentation and measurement in electrodiagnostic medicine–part I
Authors: Gitter Andrew J. , Stolov Walter C 
Published: January 2005

AbstractTechnical and instrumentation factors play an important role in obtaining reliable information during electrodiagnostic studies. With contemporary electrodiagnostic equipment, neurophysiologic potentials are detected using a variety of electrodes and undergo differential amplification, filtering, conversion to digital form, and finally, analysis and display. Understanding the signal processing principles, limitations, and sources of errors that can occur during this multistep process can improve the technical quality of studies, minimize preventable errors, and improve clinical interpretation. Part I of this minimonograph reviews the basic principles of action potential generation and overviews electrodiagnostic instrumentation. The concept of waveform frequency content is related to the role of filters in suppressing noise while preserving waveform latency, amplitude, and morphology. The electrical characteristics of various surface and needle electrodes influence instrument design and the nature of the potentials recorded. This is especially important in understanding the differences in motor unit characteristics obtained from monopolar and concentric needle electrodes. © 1995 John Wiley & Sons, Inc.
Childhood‐onset oculopharyngodistal myopathy with chronic intestinal pseudo‐obstruction
Authors: Amato Anthony A. , Jackson Carlayne E. , Ridings Larry W. , Barohn Richard J. 
Published: January 2005

AbstractOculopharyngodistal myopathy is characterized by the adult onset of ptosis, external ophthalmoplegia, dysphagia, and distal weakness. Although dysphagia is common, other gastrointestinal involvement has not been described. We report a case with childhood onset who developed chronic intestinal pseudo‐obstruction. Other myopathies associated with ophthalmoplegia and intestinal pseudo‐obstruction such as mitochondrial cytopathies were excluded. Whether oculopharyngodistal myopathy is a variant of oculopharyngeal muscular dystrophy or a distinct neuromusclar disorder is unknown and requires further study. © 1995 John Wiley & Sons, Inc.
Ca2+ Release channels of pigs heterozygous for malignant hyperthermia
Authors: Shomer Nirah H. , Mickelson James R. , Louis Charles F. 
Published: January 2005

AbstractPorcine malignant hyperthermia (MH) is an autosomal recessive disorder resulting from a mutation in the skeletal muscle sarcoplasmic reticulum (SR) Ca2+ release channel. The Ca2+ release properties of SR vesicles isolated from pigs heterozygous for the MH gene have been demonstrated previously to be intermediate to those of vesicles isolated from MH‐susceptible (MHS) and normal pigs. The Ca2+ release channel is tetrameric, so the intermediate Ca2+ release properties of heterozygous pig SR preparations could result either from populations of MHS and normal homotetramers, or populations of heterotetrameric Ca2+ release channels with properties unique from those of the two types of homozygous channels. To discriminate between these possibilities, the single channel percent open time (Po) and channel dwell time distributions of SR Ca2+ release channels were analyzed. These data suggest that the heterozygous porcine Ca2+ release channel population must contain heterotetramers with properties distinct from those of either MHS or normal channels. The data also imply that the Ca2+ release channel population in MHS humans who are heterozygous for a dominant mutation in this protein also contains heterotetrameric channels.© 1995 John Wiley & Sons, Inc.
Immunolocalization of triadin, DHP receptors, and ryanodine receptors in adult and developing skeletal muscle of rats
Authors: Carl S. Lewis , Felix K. , Caswell A. H. , Brandt N. R. , Brunschwig J.‐P. , Meissner G. , Ferguson D. G. 
Published: January 2005

AbstractThe dihydropyridine receptors (DHPR) and the ryanodine receptors (RyR) are well‐characterized proteins of the triad junctions of skeletal muscle fibers. Recently, a newly discovered 95‐kDa protein, triadin, has been purified from rabbit skeletal muscle heavy sarcoplasmic reticulum (SR) vesicles. WE have used indirect immunogold EM to localize triadin to the junctional face of the SR in isolated triads. In addition, we have used indirect immunofluorescence to localize triadin in relation to the DHPR and the RyR in adult and developing rat skeletal muscle. In double immunolabeling experiments of longitudinally oriented adult rat skeletal muscle tissue, triadin‐specific and RyR‐specific antibodies resulted in a characteristic striated staining pattern. The staining arising from these antibodies completely overlapped when examined by computer analysis of digitized laser scanning confocal microscopy images. A similar result was obtained in double staining experiments using antibodies raised against the DHPR and the RyR suggesting that all three proteins are located in the triads in situ. The developmental expression of the three triad proteins was examined using double labeling of skeletal muscle tissue from several fetal and early postnatal ages. The staining patterns of triadin, RyR, and DHPR antibodies were overlapping throughout development, suggesting that from their earliest appearance the three proteins are components of the triads. © John Wiley & Sons, Inc.
Immunocytochemical localization of vinculin in muscle and nerve
Authors: Massa Roberto , Silvestri Giulia , Sancesario Giuseppe , Bernardi Giorgio 
Published: January 2005

AbstractA complete survey of the immunofluorescence distribution of the cytoskeletal protein vinculin in the normal skeletal muscle and peripheral nerve of humans and of different rodent species was performed. Our results enable us to localize vinculin in different types of adhesion plaques such as sarcolemmal costameres and neuromuscular and myotendinous junctions, but also in a fine intermyofibrillar lattice, possibly associated with intermediate filaments and/or with the triads. Moreover, we describe the presence of vinculin in junctional domains of several, previously unrecognized, specialized cells such as: the outer sheath of the muscle spindle capsule, the multilayered flat cells of the perineurium, the smooth muscle cells of epineurial blood vessels, and the endothelial cells in the endoneurium. These data call for a major role of vinculin in mechanisms of adhesion between cells, between cell and substrate and between intermyofibrillar components in the neuromuscular system. Such knowledge provides an anatomical background for studies on the possible pathological effects induced by an impairment in vinculin function. © John Wiley & Sons, Inc.
Desmin‐related neuromuscular disorders
Authors: Goebel Hans H. 
Published: January 2005

AbstractDesmin, the intermediate filament protein of skeletal muscle fibers, cardiac myocytes, and certain smooth muscle cells, is a member of the cytoskeleton linking Z‐bands with the plasmalemma and the nucleus. The pathology of desmin in human neuromuscular disorders is always marked by increased amounts, diffusely or focally. Desmin is highly expressed in immature muscle fibers, both during fetal life and regeneration as well as in certain congenital myopathies, together with vimentin. Desmin is also enriched in neonatal myotonic dystrophy and small fibers in infantile spinal muscular atrophy. Focal accretion of desmin may be twofold, in conjunction with certain inclusion bodies, cytoplasmic and spheroid bodies, and in a more patchy fashion, granulofilamentous material. Both lesions have been found in certain families, affected by a myopathy and/or cardiomyopathy. Other proteins, e.g., dystrophin, vimentin, actin, ubiquitin, and α‐B crystallin, may also be overexpressed. Desmin pathology may be genetically regulated or may merely reflect profoundly impaired metabolism of several proteins within myofibers. © John Wiley & Sons, Inc.
Electrophysiological and urodynamic studies to monitor surgical outcome in children with tethered spinal cords
Authors: Kothari Milind J. , Bauer Stuart B. , Logigian Eric L. , Shefner Jeremy M. 
Published: January 2005

AbstractChildren with myelodysplasia often develop tethering of their spinal cord within 2–3 years after correction of their initial birth defect, leading to either neurological or urological dysfunction. Surveillance of these children includes electrophysiologic/urodynamic (EMG/UDS) studies to monitor improvement or deterioration of their neurourological function. We reviewed 39 patients who underwent surgical untethering from 1979 to 1994 to determine whether postoperative EMG/UDS studies were useful in assessing outcome after surgery. All patients had a complete neurologic examination and an EMG/UDS prior to and 3 months after surgery. Twenty patients (51%) showed improvement in their EMG/UDS study after untethering, 15 (39%) demonstrated no significant change, and 4 (10%) showed deterioration. Improvement consisted of either return of previously absent motor units, return of sacral reflexes, or improved bladder dynamics. In a further 3 patients, subsequent deterioration on serial postoperative EMG/UDS testing was the first indication of recurrent tethering. We conclude that serial EMG/UDS evaluation in patients at risk for tethering of the spinal cord may serve as a useful adjunct to close clinical observation. © 1995 John Wiley & Sons, Inc.