bluuebunny/crossref_metadata_2025_split huggingface dataset:
Immunotherapy of idiopathic inflammatory neuropathies
Authors: Donofrio Peter D. 
Published: August 2003

AbstractEvaluation of peripheral neuropathy is a common reason for referral to a neurologist. Recent advances in immunology have identified an inflammatory component in many neuropathies and have led to treatment trials using agents that attenuate this response. This article reviews the clinical presentation and treatment of the most common subacute inflammatory neuropathies, Guillain–Barré syndrome (GBS) and Fisher syndrome, and describes the lack of response to corticosteroids and the efficacy of treatment with plasma exchange and intravenous immunoglobulin (IVIG). Chronic inflammatory demyelinating polyneuropathy, although sharing some clinical, electrodiagnostic, and pathologic similarities to GBS, improves after treatment with plasma exchange and IVIG and numerous immunomodulatory agents. Controlled trials in multifocal motor neuropathy have shown benefit after treatment with IVIG and cyclophosphamide. Also discussed is the treatment of less common inflammatory neuropathies whose pathophysiology involves monoclonal proteins or antibodies directed against myelin‐associated glycoprotein or sulfatide. Little treatment data exist to direct the clinician to proper management of rare inflammatory neuropathies resulting from osteosclerotic myeloma; POEMS syndrome; vasculitis; Sjögren's syndrome; and neoplasia (paraneoplastic neuropathy). Muscle Nerve 28: 273–292, 2003
Inflammatory demyelination in a patient with CMT1A
Authors: Vital Anne , Vital Claude , Lagueny Alain , Ferrer Xavier , Ribière‐Bachelier Catherine , Latour Philippe , Petry Klaus G. 
Published: August 2003

AbstractWe report a case of Charcot‐Marie‐Tooth disease (CMT), with identified PMP22 gene duplication (CMT type 1A), and with evidence of an inflammatory demyelinating process superimposed on the course of the chronic genetic disease. Macrophage‐associated demyelination was observed on the peripheral nerve biopsy. This observation supports some experimental data from the literature and shows that there may be a genetic susceptibility to inflammatory demyelinating processes in certain CMT kindreds. Muscle Nerve 28: 373–376, 2003
Electromyography of the anal sphincter: Which muscle to examine?
Authors: Podnar Simon 
Published: August 2003

AbstractElectromyographic examination of the deeper external anal sphincter (EAS) muscle is far more uncomfortable than of the subcutaneous muscle, so we tested the need for its examination. We compared the findings in 85 paired examinations of ipsilateral subcutaneous and deeper EAS muscles in 67 patients with a cauda equina lesion. When the other muscle was normal, the deeper or subcutaneous muscle was pathological in 18% or 13% of pairs, respectively. In addition, the subcutaneous EAS muscle was usually more severely affected than the deeper EAS muscle. Thus, examination of the deeper EAS muscle did not increase the sensitivity of MUP analysis significantly, and did not seem clinically necessary. Muscle Nerve 28: 377–379, 2003
Two novel mutations in the muscle glycogen phosphorylase gene in McArdle's disease
Authors: Gámez Josep , Rubio Juan C. , Martín Miguel A. , Fernández‐Cadenas Israel , Garcia‐Arumi Elena , Andreu Antoni L. , Arenas Joaquín 
Published: August 2003

AbstractWe report on a Spanish family with myophosphorylase (EC 2.4.1.1) deficiency (McArdle's disease). The proband and his symptomatic sister were compound heterozygous for two novel mutations: a T‐to‐G transversion in exon 14 (c1722 T>G) that changes a tyrosine to a stop codon (Y573X), and a G‐to‐A transition in exon 15 (c1827 G>A) that disrupts the consensus signal at the donor splicing site. These findings further expand knowledge of the genetic bases of muscle glycogen phosphorylase deficiency. Muscle Nerve 28: 380–382, 2003
Cyclosporin A treatment increases rat soleus muscle oxidative capacities
Authors: Sanchez Hervé , N'Guessan Benoit , Ribera Florence , Ventura‐Clapier Renée , Bigard Xavier 
Published: August 2003

AbstractPrevious studies suggested that administration of cyclosporin A (CsA), an immunosuppressive agent, contributes to the increased fatigability of heart transplant recipients. The aim of this study was to investigate whether CsA itself, without vehicle, affects the function of mitochondria maintained in situ, in rats treated with CsA (25mg/kg/day) dissolved in ethanol and olive oil. Treatment with CsA induced a 16% decrease in slow myosin heavy chain (MHC) associated with a 225% increase in fast MHCIIa. The proportion of fibers expressing type IIa MHC increased as a result of CsA treatment. Soleus from the CsA‐treated animals showed an increase in both basal (+85%) and maximal (+37%) mitochondrial respiration (P < 0.001), consistent with a 24% increase in citrate synthase activity, whereas the apparent Km for adenosine diphosphate was unchanged. By itself, CsA has no deleterious effects on muscle oxidative capacity but induces alterations in energy metabolism in accordance with the increased proportion of fast‐twitch oxidative fibers. Muscle Nerve 28: 324–329, 2003
A prospective evaluation of phrenic nerve conduction in multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy
Authors: Macia Frederic , Le Masson Gwendal , Rouanet‐Larriviere Marie , Habonimana Desire , Ferrer Xavier , Marthan Roger , Lagueny Alain 
Published: August 2003

AbstractThe purpose of the study was to evaluate electrophysiologically phrenic nerve involvement in multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). The response latencies following phrenic nerve stimulation were increased in 11 of 14 (80%) patients in the CIDP group but in only 1 of 14 (8%) patients in the MMN group. The mean diaphragmatic compound muscle action potential (CMAP) was significantly lower in amplitude in the CIDP group compared to the MMN group and to a control group of 8 subjects (P < 0.001). There were no significant differences between the MMN and control groups. Only the reduction in CMAP amplitude correlated with the presence of restrictive lung function. Phrenic nerve conduction measurement should be performed more systematically, especially in CIDP and, when diaphragmatic CMAPs are reduced in amplitude, pulmonary function tests should be performed to look for a restrictive lung syndrome. Muscle Nerve 28: 319–323, 2003
Ventilatory dysfunction in mdx mice: Impact of tumor necrosis factor–alpha deletion
Authors: Gosselin Luc E. , Barkley Jacob E. , Spencer Melissa J. , McCormick Kathleen M. , Farkas Gaspar A. 
Published: August 2003

AbstractMuscular dystrophy is associated with inflammation and fiber necrosis in the diaphragm that may alter ventilatory function. The purpose of this study was to determine to what extent in vivo ventilatory function in dystrophic (mdx) mice was compromised and to assess the impact of deletion of tumor necrosis factor–alpha (TNF‐α), a known proinflammatory cytokine, on ventilatory function, diaphragm contractility, and myosin heavy chain (MHC) distribution in 10–12‐month‐old mdx mice. Although the resting ventilatory pattern did not significantly differ between control and mdx mice, the ventilatory response to hypercapnia in mdx mice was significantly attenuated. Elimination of TNF‐α significantly improved the hypercapnic ventilatory response and diaphragm muscle maximal isometric force. Long‐term TNF‐α deletion also altered the myosin heavy chain isoform profile of the diaphragm. These data indicate that a blunted ventilatory response to hypercapnia exists in mdx mice, and that TNF‐α influences the progressive deterioration of diaphragm muscle in mdx mice. Muscle Nerve 28: 336–343, 2003
Neurophysiological changes in demyelinating and axonal forms of acute experimental autoimmune neuritis in the Lewis rat
Authors: Taylor Jude M. , Pollard John D. 
Published: August 2003

AbstractMyelin‐sensitized T‐ and B‐cells (lymph node cells) induced experimental autoimmune neuritis (EAN) in Lewis rats after passive transfer to naive recipients. After 6 days, all recipient rats developed tail paresis that progressed to limb paresis within 12–72 h. Progressive nerve conduction changes consistent with demyelination in the sciatic nerve (conduction block and prolongation of the distal motor latencies) and lumbar nerve roots (initial low F‐wave frequencies followed by later prolongation in F‐wave latencies) were observed during the disease. For comparison, adoptive transfer experimental autoimmune neuritis (AT‐EAN) of differing disease severity was induced by titrating the dose of P2‐specific T‐cells. In contrast to EAN induced by myelin‐sensitized T‐ and B‐cells, AT‐EAN was predominantly associated with rapid nerve conduction changes consistent with axonal dysfunction and degeneration. These findings demonstrate that distinct forms of EAN with different pathophysiological mechanisms are induced by the passive transfer of P2‐specific T‐cell lines or myelin‐specific T‐cells and B‐cells. The electrophysiological changes in EAN induced by myelin‐specific T‐ and B‐cells are very similar to those seen clinically during acute inflammatory demyelinating polyneuropathy, whereas AT‐EAN has less resemblance to axonal forms of Guillain–Barré syndrome. Muscle Nerve 28: 344–352, 2003
Rapsyn mutations in myasthenic syndrome due to impaired receptor clustering
Authors: Maselli Ricardo A. , Dunne Vanessa , Pascual‐Pascual Samuel Ignacio , Bowe Constance , Agius Mark , Frank Rochelle , Wollmann Robert L. 
Published: August 2003

AbstractRapsyn, a 43‐kDa postsynaptic protein, is essential for anchoring and clustering acetylcholine receptors (AChRs) at the endplate (EP). Mutations in the rapsyn gene have been found to cause a postsynaptic congenital myasthenic syndrome (CMS). We detected six patients with CMS due to mutations in the rapsyn gene (RAPSN). In vitro studies performed in the anconeus muscle biopsies of four patients showed severe reduction of miniature EP potential amplitudes. Electron microscopy revealed various degrees of impaired development of postsynaptic membrane folds. All patients carried the N88K mutation. Three patients were homozygous for N88K and had less severe phenotypes and milder histopathologic abnormalities than the three patients who were heterozygous and carried a second mutation (either L14P, 46insC, or Y269X). Surprisingly, two N88K homozygous patients had one asymptomatic relative each who carried the same genotype, suggesting that additional genetic factors to RAPSN mutations are required for disease expression. Muscle Nerve 28: 293–301, 2003
Improvement of muscle healing through enhancement of muscle regeneration and prevention of fibrosis
Authors: Sato Kenji , Li Yong , Foster William , Fukushima Kazumasa , Badlani Neil , Adachi Nobuo , Usas Arvydas , Fu Freddie H. , Huard Johnny 
Published: August 2003

AbstractSkeletal muscle is able to repair itself through regeneration. However, an injured muscle often does not fully recover its strength because complete muscle regeneration is hindered by the development of fibrosis. Biological approaches to improve muscle healing by enhancing muscle regeneration and reducing the formation of fibrosis are being investigated. Previously, we have determined that insulin‐like growth factor–1 (IGF‐1) can improve muscle regeneration in injured muscle. We also have investigated the use of an antifibrotic agent, decorin, to reduce muscle fibrosis following injury. The aim of this study was to combine these two therapeutic methods in an attempt to develop a new biological approach to promote efficient healing and recovery of strength after muscle injuries. Our findings indicate that further improvement in the healing of muscle lacerations is attained histologically by the combined administration of IGF‐1 to enhance muscle regeneration and decorin to reduce the formation of fibrosis. This improvement was not associated with improved responses to physiological testing, at least at the time‐points tested in this study. Muscle Nerve 28: 365–372, 2003
Spinal accessory neuropathy, droopy shoulder, and thoracic outlet syndrome
Authors: Al‐Shekhlee Amer , Katirji Bashar 
Published: August 2003

AbstractDroopy shoulder has been proposed as a cause of thoracic outlet syndrome. Two patients developed manifestations of neurovascular compression upon arm abduction, associated with unilateral droopy shoulder and trapezius muscle weakness caused by iatrogenic spinal accessory neuropathies following cervical lymph node biopsies. The first patient developed a cold, numb hand with complete axillary artery occlusion when his arm was abducted to 90°. The second patient complained of paresthesias in digits 4 and 5 of the right hand, worsened by elevation of the arm, with nerve conduction findings of right lower trunk plexopathy (low ulnar and medial antebrachial cutaneous sensory nerve action potentials). Spinal accessory nerve grafting (in the first patient) coupled with shoulder strengthening physical exercises in both patients resulted in gradual improvement of symptoms in 2 years. These two cases demonstrate that unilateral droopy shoulder secondary to trapezius muscle weakness may cause compression of the thoracic outlet structures. Muscle Nerve 28: 383–385, 2003
Peripheral neuropathy in scleroderma
Authors: Poncelet Ann N. , Connolly M. Kari 
Published: August 2003

AbstractBecause patients with scleroderma report neuropathic symptoms including numbness, paresthesias, and dysesthesias, we assessed peripheral nerve function in such patients. Fourteen scleroderma patients underwent complete neurologic examination, nerve conduction studies (NCS) and quantitative sensory testing (QST). Neurologic examination revealed reduced vibration (7) or pinprick (4) sensation in the upper or lower extremities, focal atrophy or proximal weakness (2), and decreased deep tendon reflexes (2). NCS showed reduced sensory nerve action potentials (1) and carpal tunnel syndrome (1). QST of the upper and lower extremity revealed increased cold or vibration detection thresholds in 8 of 14 patients. Our findings suggest that peripheral neuropathy occurs in patients with scleroderma at a higher frequency than previously appreciated. These findings cannot be ascribed to compression neuropathies, but rather involve large and small fibers in a non–length‐dependent fashion. Larger, prospective studies using the more sensitive QST as well as pathologic studies of nerve, including cutaneous innervation, are needed to further assess the characteristics and etiology of the neuropathy. Muscle Nerve 28: 330–335, 2003
Clinical spectrum of muscle weakness in human West Nile virus infection
Authors: Leis A. Arturo , Stokic Dobrivoje S. , Webb Risa M. , Slavinski Sally A. , Fratkin Jonathan 
Published: August 2003

AbstractPoliomyelitis has recently been identified as a cause of muscle weakness in patients with West Nile virus (WNV) infection. However, the clinical spectrum of WNV‐associated weakness has not been described. We reviewed data on 13 patients with WNV infection. Patients with muscle weakness were classified into one of three distinct groups based on clinical features. Group 1 comprised five patients who developed acute flaccid paralysis, four with meningoencephalitis and one without fever or other signs of infection. Paralysis was asymmetric, and involved from one to four limbs in individual patients. Electrodiagnostic studies confirmed involvement of anterior horn cells or motor axons. Group 2 involved two patients without meningoencephalitis who developed severe but reversible muscle weakness that recovered completely within weeks. Muscle weakness involved both lower limbs in one patient and one upper limb in the other. Group 3 consisted of two patients who experienced subjective weakness and disabling fatigue, but had no objective muscle weakness on examination. In addition to the three distinct groups, two other patients developed exaggerated weakness in the distribution of preexisting lower motor neuron dysfunction. We conclude that the clinical spectrum of WNV‐associated muscle weakness ranges from acute flaccid paralysis, with or without fever or meningoencephalitis, to disabling fatigue. Also, preexisting dysfunction may predispose anterior horn cells to additional injury from WNV. Awareness of this spectrum will help to avoid erroneous diagnoses and inappropriate treatment. Muscle Nerve 28: 302–308, 2003
Relative contributions of neural mechanisms versus muscle mechanics in promoting finger extension deficits following stroke
Authors: Kamper D.G. , Harvey R.L. , Suresh S. , Rymer W.Z. 
Published: August 2003

AbstractThe origins of impaired finger and hand function were examined in 10 stroke survivors with chronic spastic hemiparesis, with the intent of assessing whether mechanical restraint or altered neurophysiological control mechanisms are responsible for the well‐known impairment of finger extension. Simultaneous extension of all four metacarpophalangeal (MCP) joints of the impaired hand was either externally imposed using a rotary actuator or attempted voluntarily by the subject. Trials were conducted both before and after administration of a local anesthetic, blocking the median and ulnar nerves at the elbow. The anesthetic was administered to reduce the activity of the muscles flexing the MCP joints, in order to distinguish mechanical from neuronal resistance to imposed MCP rotation. We found that the nerve blockade resulted in a reduction in velocity‐dependent torque (P = 0.01), thereby indicating significant joint impedance due to spasticity. Blockade also produced a posture‐dependent reduction in static torque in declaratively relaxed subjects (P = 0.04), suggesting some tonic flexor activity for specific hand postures. No change in either extensor isometric (P = 0.33) or isokinetic (0.53) torque was apparent, but 3 of the 10 subjects did exhibit substantial (>10°) improvement in voluntary MCP extension following the blockade. This improvement seemed largely due to a decrease in inappropriate flexor activity during the movement, rather than an increase in extensor activity. We argue that persistent and inappropriate flexor activation plays a role in limiting voluntary finger extension, and that this activation is potentially a reflection of altered supraspinal control of key spinal pathways. In all cases, this inappropriate activation was compounded by weakness, apparent in both the extensor and flexor muscles. Muscle Nerve 28: 309–318, 2003
Caffeine impairs intramuscular energy balance in patients susceptible to malignant hyperthermia
Authors: Textor Zoran , Beer Meinrad , Anetseder Martin , Köstler Herbert , Kagerbauer Erwin , Kenn Werner , Hahn Dietbert , Roewer Norbert 
Published: August 2003

AbstractMalignant hyperthermia (MH) is a metabolic myopathy with an abnormal release of calcium by the sarcoplasmic reticulum (SR), triggered by volatile anesthetics and succinylcholine. Similarly, caffeine enhances Ca2+release by the SR in vitro. In a prospective, randomized study, high‐energy phosphates were studied by intramuscular 31‐phosphorus magnetic resonance spectroscopy (31P‐MRS) in 10 MH‐susceptible (MHS) and 7 MH‐nonsusceptible (MHN) subjects before and after injection of 0.5 ml caffeine (20 mM). Intramuscular energy balance, measured by the ratios of Pi/PCr and Pi/γ‐ATP, did not differ between MHS and MHN patients before and after intramuscular caffeine injection. However, within each group, Pi/PCr and Pi/γ‐ATP increased significantly only in the MHS group. Intramuscular caffeine injection seemed to impair the metabolic balance in MHS individuals. This may reflect a local calcium overload leading to consumption of high‐energy phosphates and increase of inorganic phosphate. Intramuscular stimulation by caffeine and 31P‐MRS may provide a valuable tool to investigate MH‐related metabolic disturbances. Muscle Nerve 28: 353–358, 2003
Caffeine increases spinal excitability in humans
Authors: Walton C. , Kalmar J. , Cafarelli E. 
Published: August 2003

AbstractThe Hoffman reflex (H reflex) has long been established as a measure of spinal excitability. Caffeine is one of the most widely consumed drugs in the world. Because it is known to increase excitatory neurotransmission, we hypothesized that caffeine would increase spinal excitability and thus alter the H reflex by increasing its amplitude. Seven subjects each attended the laboratory on 2 days. Caffeine (6 mg/kg) was administered on one day and a placebo was administered on the other. The tibial nerve was stimulated at incremental intensities to create an H‐reflex recruitment curve prior to capsule administration (pretest) and 1 h later (posttest) on each day. The slope of H‐reflex recruitment curve normalized to that of the M wave (Hslp/Mslp) was compared (pretest to posttest). Caffeine increased spinal excitability 43 ± 17% (P < 0.05). Thus, caffeine may be used to safely increase spinal excitability in electrophysiological studies of the human neuromuscular system. Our results also suggest that caffeine intake should be controlled when the H reflex is used in diagnostic and experimental situations. Muscle Nerve 28: 359–364, 2003
Broadcasting in generalized chordal rings
Authors: Comellas F. , Hell P. 
Published: August 2003

AbstractBroadcasting is an information dissemination process in which a message originating at one node of a communication network (modeled as an undirected graph) is sent to all other nodes by means of calls involving two nodes at a time, with each node participating in at most one call at any time. We are interested in efficient broadcasting in a class of cubic graphs known as generalized chordal rings. These graphs have been found useful for having a small diameter D, among graphs with a given number of vertices and maximum degree. We show that the minimum broadcast time in any generalized chordal ring is D, D + 1, or D + 2. For the generalized chordal rings of diameter D which have the greatest (or greatest‐known) number of nodes, we then evaluate exactly the minimum broadcast time. It turns out to be D + 1 when D is even and D + 2 when D is odd. For these purposes, we review the construction of these extremal generalized chodal rings. We also review the optimal broadcast schemes for infinite triangular grids, which we use to prove our bounds. Finally, we ask for the maximum number of nodes that can be informed by a broadcast in time t in any generalized chordal ring. We answer this completely for even t and almost completely for odd t. We use a geometric approach, based on plane tessellations. © 2003 Wiley Periodicals, Inc.
Improved preprocessing, labeling and scaling algorithms for the Weight‐Constrained Shortest Path Problem
Authors: Dumitrescu I. , Boland N. 
Published: August 2003

AbstractMuch has been written on shortest path problems with weight, or resource, constraints. However, relatively little of it has provided systematic computational comparisons for a representative selection of algorithms. Furthermore, there has been almost no work showing numerical performance of scaling algorithms, although worst‐case complexity guarantees for these are well known, nor has the effectiveness of simple preprocessing techniques been fully demonstrated. Here, we provide a computational comparison of three scaling techniques and a standard label‐setting method. We also describe preprocessing techniques which take full advantage of cost and upper‐bound information that can be obtained from simple shortest path information. We show that integrating information obtained in preprocessing within the label‐setting method can lead to very substantial improvements in both memory required and run time, in some cases, by orders of magnitude. Finally, we show how the performance of the label‐setting method can be further improved by making use of all Lagrange multiplier information collected in a Lagrangean relaxation first step. © 2003 Wiley Periodicals, Inc.