bluuebunny/crossref_metadata_2025_split huggingface dataset:
Concomitant dermatomyositis and myasthenia gravis presenting with respiratory insufficiency
Authors: Van De Warrenburg Bart P.C. , Hengstman Gerald J.D. , Vos Pieter E. , Boerman Rudolph H. , Ter Laak Henk J. , Van Engelen Baziel G.M. 
Published: October 2004

AbstractWe report a 28‐year‐old woman with a history of chronic immune‐mediated hepatitis, in whom the simultaneous manifestation of dermatomyositis and myasthenia gravis resulted in severe neck extensor weakness and subacute respiratory insufficiency, followed by proximal muscle weakness and external ophthalmoplegia. Radiological signs of a thymoma were absent. The distinguishing clinical, electrophysiological, and biopsy findings are discussed. We suggest that an underlying immunoregulatory disorder was present, explaining the occurrence of three rare immune‐mediated diseases in one patient. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 293–296, 2002 DOI 10.1002/mus.10022
Mycophenolate mofetil in the therapy of polymyositis associated with a polyautoimmune syndrome
Authors: Schneider C. , Gold R. , Schäfers M. , Toyka K.V. 
Published: October 2004

AbstractMycophenolate mofetil 1.5 g daily (30 mg/kg body weight) was given to a patient with ankylosing spondylitis, ulcerative colitis, and severe refractory polymyositis after conventional treatment regimes had failed. No severe side effects occurred. Considerable improvement of clinical symptoms and electromyographic findings were seen within 6 months after the initiation of mycophenolate mofetil, allowing for tapering and discontinuation of methylprednisolone. Mycophenolate mofetil may be considered as an useful alternative in the treatment of polymyositis when standard therapeutic regimens fail. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 286–288, 2002 DOI 10.1002/mus.10026
Electrical stimulation factors in potentiation of human quadriceps femoris
Authors: Binder‐Macleod Stuart A. , Dean Jesse C. , Ding Jun 
Published: October 2004

AbstractPotentiation is the enhancement of force seen after repetitive activation of skeletal muscle. The frequency and duration of stimulation, total number of pulses delivered to the muscle, and the peak forces or force‐time integrals produced by the stimulation all have been suggested to affect the degree of potentiation. The purpose of this study was to determine the effect of the electrical stimulation characteristics on the development of post‐activation potentiation. Eleven subjects were tested with five potentiating trains, including 12‐pulse 100‐HZ, 31‐HZ, 14‐HZ, and 5‐HZ, and 6‐pulse 14‐HZ trains. The potentiating trains differed in stimulation frequency, train duration, and total number of pulses. They also produced different peak forces and force time integrals from the activated muscles. Our results showed that the 12‐pulse 5‐HZ train produced about 50% less potentiation than the other four potentiating trains. At stimulation frequencies of 14 HZ or higher, the total number of pulses delivered to the muscle was the primary factor in potentiation development. Furthermore, peak force and force‐time integral had no effects on the rate or amount of potentiation. These results should help clinicians and researchers to design protocols that control for the effects of muscle potentiation. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 271–279, 2002 DOI 10.1002/mus.10027
Acceleration by MS‐818 of early muscle regeneration and enhanced muscle recovery after surgical transection
Authors: Sugiyama Noriyuki , Yoshimura Atsushi , Fujitsuka Chie , Iwata Hisashi , Awaya Akira , Mori Satoru , Yoshizato Hideo , Fujitsuka Noriaki 
Published: October 2004

AbstractThe synthesized pyrimidine compound MS‐818 has neurotrophic effects in several kinds of neuronal cells, but its effect with respect to muscle cells remains unknown. We therefore examined the effects of MS‐818 on regeneration for 12 weeks in a wounded area (damaged and gap areas) of cut muscle in adult rats. The right semitendinosus muscles of reated and control groups were severed and sutured at the belly and the left semitendinosus muscles were left intact. MS‐818 was administered intraperitoneally to the treated group at a dose of 5 mg/kg once daily. Control rats received an equal volume of physiological saline. A reference group underwent no surgical procedure. MS‐818 significantly increased the maximal isometric twitch tension (Tmax) compared to control and reference rats after week 4 (approximately 1.4‐fold control value; 0.6‐fold reference value). Northern blotting showed that MS‐818 enhanced myogenin mRNA expression to about 1.5‐fold above the control level at 2, 4, and 7 days after surgery. Immunohistochemical and histochemical studies showed significant enhancement in the treated group since myogenic cells expressed desmin and were positive for neonatal myosin, and the fiber diameters and numbers of premature myofibers and end plates were increased when compared with those in the control group. These results show that MS‐818 accelerated the proliferation and differentiation of activated satellite cells and the fusion of myotubes to form immature myofibers. At week 12, Tmax, fiber diameter, and number of end plates in the treatment group recovered 60, 85, and more than 100%, respectively, compared to the reference group. The mechanism of MS‐818 effects on the accelerated regeneration of cut muscle is discussed. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 218–229, 2002 DOI 10.1002/mus.10028
Analysis of motor pathway involvement in konzo using transcranial electrical and magnetic stimulation
Authors: Tshala‐Katumbay Desire , Eeg‐Olofsson Karin Edebol , Kazadi‐Kayembe Theodore , Tylleskär Thorkild , Fällmar Peo 
Published: October 2004

AbstractTo elucidate the involvement of motor pathways in konzo, 21 konzo subjects (mean age 22 years) underwent transcranial electrical stimulation (TES) in 1998. Fourteen konzo subjects (mean age 21 years) underwent transcranial magnetic stimulation (TMS) in 2000. Three subjects underwent both TES and TMS. Motor evoked potentials (MEPs) were recorded in the abductor pollicis brevis (APB) muscle with TES, and in the abductor digiti minimi (ADM) and tibialis anterior (TA) muscles with TMS. APB‐MEPs were normal in 2 of 21 subjects and absent in 9; central conduction time (CCT) was prolonged in 10. Resting ADM‐MEPs were absent in 9 of 14 subjects with clinically preserved upper limbs. Among these nine, seven subjects responded after facilitation. Most subjects (13 of 14) failed to show TA‐MEPs. Of the subjects who underwent both types of stimulation, one had normal TES‐MEP but abnormal ADM‐MEP with TMS. These findings suggest involvement of both corticomotoneurons and motor descending pathways in konzo. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 230–235, 2002 DOI 10.1002/mus.10029
Effects of voluntary activity on the excitability of motor axons in the peroneal nerve
Authors: Kuwabara Satoshi , Cappelen‐Smith Cecilia , Lin Cindy S.‐Y. , Mogyoros Ilona , Burke David 
Published: October 2004

AbstractTo investigate whether there are inter‐nerve differences in the extent and pattern of axonal excitability changes produced by voluntary contractions of tibialis anterior (TA) and abductor pollicis brevis (APB), threshold tracking was used to measure axonal excitability parameters [threshold, supernormality and strength‐duration time constant (τSD)] of peroneal and median motor axons in 11 healthy subjects. Maximal contractions for 1 min resulted in an increase in threshold, an increase in supernormality, a decrease in τSD and an increase in latency, all of which indicate axonal hyperpolarization. The increase in threshold was less in peroneal axons (18 ± 4%) than median axons (37 ± 6%, mean ± SEM, P < 0.001), and was accompanied by smaller absolute changes in latency, supernormality, and τSD. Peroneal axons had less supernormality at rest but a greater change in supernormality for the change in threshold. There were major contraction‐induced changes in the compound muscle action potential of TA but not that of APB. Voluntary contractions depress axonal excitability, but the changes are quantitatively different for motor axons innervating different muscles. There are three clinical implications. First, weakness and fatigue due to activity‐dependent conduction block may vary for different muscles, independent of disease severity, and therapeutic strategies to overcome activity‐dependent conduction block may not be equally effective for different muscles. Second, in motor control studies using the H reflex to document motoneuron excitability, a constant stimulus will not produce a constant neural volley if the stimulated axons have been activated by, for example, a voluntary contraction. Third, TA is probably not optimal for testing for activity‐dependent conduction block. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 176–184, 2002 DOI 10.1002/mus.10030
Assessment of contractile and noncontractile components in human skeletal muscle by magnetic resonance imaging
Authors: Holmbäck Anna Maria , Askaner Krister , Holtås Stig , Downham David , Lexell Jan 
Published: October 2004

AbstractA magnetic resonance imaging (MRI) technique for the assessment of contractile and noncontractile components of human skeletal muscle is described, and the inter‐rater and intra‐rater test–retest reliability for repeated measurements from the same MR image are examined. Twenty cross‐sectional MR images from the right lower leg were obtained from 30 healthy young men and women (mean age 24.1 years, SD 3.3). The anatomical cross‐sectional area (aCSA; cm2), the cross‐sectional area of noncontractile components (Noncon; cm2), the contractile cross‐sectional area (cCSA = aCSA minus Noncon; cm2), and the relative amount of Noncon (%), of the ankle dorsiflexor muscle compartment were determined for each slice using a computer‐based image analysis system. Reliability for repeated measurements of the slice with the largest aCSA for the 30 subjects was analyzed by two raters on two different occasions. Inter‐rater reliability on both occasions, assessed by the intraclass correlation coefficient (ICC), was excellent for cCSA (ICC3.1 = 0.99) and Noncon (ICC3.1 > 0.82). Intra‐rater (between occasions) reliability was excellent for the two raters for measurements of cCSA (ICC1.1 = 0.99) and Noncon (ICC1.1 > 0.94). Bland and Altman analyses did not identify any clinically relevant bias in the measurements. Method errors were acceptable: within subjects coefficients of variation (CV) was less than 1.8% for cCSA and less than 16.3% for Noncon. It is concluded that repeated measurements of contractile and noncontractile components of the ankle dorsiflexor muscle compartment, obtained from the same MR image, are highly reliable. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 251–258, 2002 DOI 10.1002/mus.10031
Rapidly progressive amyloid polyneuropathy associated with a novel variant transthyretin serine 25
Authors: Yazaki Masahide , Yamashita Taro , Kincaid John C. , Scott John R. , Auger Raymond G. , Dyck Peter J. , Benson Merrill D. 
Published: October 2004

AbstractWe report a 52‐year‐old woman with a novel transthyretin (TTR) variant serine replacing alanine at residue 25 [Ala25Ser (Serine 25)], who showed a unique clinical picture with a relatively acute onset neuropathy within a few days of an influenza vaccination, progressing to a severe degree within 2 years. Sural nerve biopsy revealed amyloid deposition in the endoneurium. Sequencing of the proband's DNA revealed a G to T transversion at the first position of codon 25 of TTR gene. DNA analysis of this family showed the same mutation in her older sister and a niece, but her parents did not have the mutation. Haplotype analysis revealed the mutation to be clearly linked to haplotype III allele inherited from the proband's father. These results indicate this novel Serine 25 mutation originated in the paternal germline mosaicism. It is possible that the vaccination had an influence on the unique clinical picture, but this remains uncertain. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 244–250, 2002 DOI 10.1002/mus.10032
Spasticity causes a fundamental rearrangement of muscle–joint interaction
Authors: Lieber Richard L. , Fridén Jan 
Published: October 2004

AbstractSarcomere length was measured in flexor carpi ulnaris (FCU) muscles from patients with severely spastic wrist flexion contractures (n = 6), as well as from patients with radial nerve injury and a normally innervated FCU (n = 12). Spastic FCU muscles had extremely long sarcomere lengths with the wrist fully flexed (3.48 ± 0.44 μm) compared to the FCU muscles of patients with radial nerve injury (2.41 ± 0.31 μm). In three of the patients with spastic wrist flexion contractures, the slope of the FCU sarcomere length–joint angle relationship was measured and found to be, essentially, normal (0.017 ± 0.005 μm/°, n = 3) suggesting that serial sarcomere number (and therefore muscle fiber length) was unchanged in spite of the dramatic absolute sarcomere length change. These results indicate that spasticity results in a major alteration of normal muscle–joint anatomical relationships that has not previously been recognized to our knowledge. We hypothesize that the results are explained either by the inability of muscle fibers to add serial sarcomeres in response to growth, or the selective loss of FCU muscle length secondary to the central nervous system lesion. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 265–270, 2002 DOI 10.1002/mus.10036
Titin and nebulin content in human skeletal muscle following eccentric resistance exercise
Authors: Trappe Todd A. , Carrithers John A. , White Faber , Lambert Charles P. , Evans William J. , Dennis Richard A. 
Published: October 2004

AbstractWe measured titin and nebulin content in muscle biopsies from the vastus lateralis before and 24 h after one bout of high‐intensity eccentric knee extensor resistance exercise in seven men (26 ± 3 years). Titin and nebulin content were significantly (P < 0.05) reduced after exercise by 30 and 15%, respectively. These results suggest that the structural components of the myofibrillar apparatus are degraded following high‐intensity eccentric resistance exercise in humans. Loss of these proteins may have important implications for the mechanisms regulating the adaptive response of skeletal muscle to resistance exercise. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 289–292, 2002 DOI 10.1002/mus. 10037
Magnetic stimulation of the central and peripheral nervous systems
Authors: Weber Markus , Eisen Andrew A. ,   AAEM
Published: October 2004

AbstractSince 1985, when the technique of transcranial magnetic stimulation (TMS) was first developed, a wide range of applications in healthy and diseased subjects has been described. Comprehension of the physiological basis of motor control and cortical function has been improved. Modifications of the basic technique of measuring central motor conduction time (CMCT) have included measurement of the cortical silent period, paired stimulation in a conditioning test paradigm, repetitive transcranial magnetic stimulation (rTMS), and peristimulus time histograms (PSTH). These methods allow dissection of central motor excitatory versus inhibitory interplay on the cortical motor neuron and its presynaptic connections at the spinal cord, and have proven to be powerful investigational techniques. TMS can be used to assess upper and lower motor neuron dysfunction, monitor the effects of many pharmacological agents, predict stroke outcome, document the plasticity of the motor system, and assess its maturation and the effects of aging, as well as perform intraoperative monitoring. The recent use of rTMS in the treatment of depression and movement disorders is novel, and opens the way for other potential therapeutic applications. © 2002 American Association of Electrodiagnostic Medicine. Muscle Nerve 25: 160–175, 2002 DOI 10.1002/mus.10038
Unilateral cranial and phrenic nerve involvement in axonal Guillain–Barré syndrome
Authors: Sakakibara Yumi , Mori Masahiro , Kuwabara Satoshi , Katayama Kaoru , Hattori Takamichi , Koga Michiaki , Yuki Nobuhiro 
Published: October 2004

AbstractA 49‐year‐old woman developed acute left facial, hypoglossal, and phrenic nerve palsies, as well as dysphagia and weakness in the neck and arms. Electrophysiologic studies showed an acute motor axonal neuropathy. Serum anti‐GM1 IgG antibody was positive. Intavenous immunoglobulin treatment resulted in good clinical recovery. The present report indicates that the cranial and phrenic nerves may be affected unilaterally in Guillain–Barré syndrome, and that there is clinical variability in the axonal subtype of this syndrome. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 297–299, 2002 DOI 10.1002/mus.10041
Congenital inflammatory myopathy: A demonstrative case and proposed diagnostic classification
Authors: McNeil Stephen M. , Woulfe John , Ross Catherine , Tarnopolsky Mark A. 
Published: October 2004

AbstractThere have been few reports of congenital inflammatory myopathy in the literature, and most of these have been associated with congenital muscular dystrophy. We review the literature and present a case with electromyographic and muscle biopsy evidence of congenital inflammatory myopathy with onset in the perinatal period and no evidence of a congenital muscular dystrophy. There was evidence of subjective improvement following corticosteroid administration (∼ mg/kg per day) with a concomitant normalization of the serum creatine kinase activity. Of particular interest in the case was the history of maternal infection, suggesting a possible postinfectious molecular mimicry as the etiology of the muscle inflammation. This case suggests that a rare form of congenital inflammatory myopathy does exist that is not associated with a congenital muscular dystrophy. A preliminary classification scheme is proposed to separate true congenital cases from those acquired after birth and those cases associated with congenital dystrophy. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 259–264, 2002 DOI 10.1002/mus.10043
Psychometric evaluation of a new handicap scale in immune‐mediated polyneuropathies
Authors: Merkies Ingemar S.J. , Schmitz Paul I.M. , Van Der Meché Frans G.A. , Samijn Johnny P.A. , Van Doorn Pieter A. 
Published: October 2004

AbstractA new handicap measure, the Rotterdam nine‐item handicap scale, was developed and its validity, reliability, and responsiveness evaluated in patients with immune‐mediated polyneuropathies. We evaluated 113 stable patients, of whom 83 had Guillain–Barré syndrome (GBS), 22 had chronic inflammatory demyelinating polyneuropathy (CIDP), and 8 had a gammopathy‐related polyneuropathy. We also studied 20 patients with recently diagnosed GBS (n = 7) or CIDP (n = 13) and changing clinical conditions (longitudinal group). Significant discriminatory validity and correlation with the Rankin scale were demonstrated for the Rotterdam nine‐item handicap scale (stable group: Spearman's test, r = −.76 to −.78; longitudinal group: intraclass correlation coefficient, r = .83; P < .0001). Also, good reliability (r = .89–.98; P < .0001) and high responsiveness values (standardized response mean values > .8) were obtained for the Rotterdam nine‐item handicap scale. In contrast to the Rankin scale, the Rotterdam scale not only provided information regarding mobility but also highlighted physical independence, occupation, and social integration. These results illustrate the clinical usefulness of the Rotterdam nine‐item handicap scale under these conditions. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002
Hypokalemic periodic paralysis associated with malignant hyperthermia
Authors: Rajabally Yusuf A. , El Lahawi Mohammed 
Published: October 2004

AbstractHypokalemic periodic paralysis is in most cases related to mutations within the dihydropyridine receptor gene. Susceptibility to malignant hyperthermia has been linked to a different part of the same gene, but is more frequently caused by mutations within the ryanodine receptor gene. We report the association of the two disorders in a patient for whom the most frequent mutations for hypokalemic periodic paralysis were not found. This suggests further genetic heterogeneity of this condition, the interest of this case residing in the known coupling between dihydropyridine and ryanodine receptors. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002
Effects of temperature on neuromuscular electrophysiology
Authors: Rutkove Seward B. 
Published: October 2004

AbstractLike nearly all biologic structures, the peripheral nervous system is remarkably temperature sensitive. Clinical neurophysiologists are most aware of the untoward effects of cooling on nerve conduction studies, including reduced conduction velocity, prolonged distal latency, and increased response amplitude and duration. However, familiarity with the effects of temperature variation on the peripheral nervous system can also provide a deeper understanding of the physiological mechanisms underlying the function of nerve, muscle, and neuromuscular junction in health and disease. Intentional temperature alteration can also improve the diagnostic accuracy of certain electrophysiologic tests, such as the use of heat when performing repetitive nerve stimulation in myasthenia gravis or the use of cold during needle electromyography in some of the myotonic disorders. Finally, extremes of temperature have long been known to produce permanent neuronal dysfunction; recent investigations are beginning to elucidate the mechanisms of such injury. © 2001 American Association of Electrodiagnostic Medicine Muscle Nerve 24: 867–882, 2001
Stimulated single fiber electromyography in the mouse: Techniques and normative data
Authors: Gooch Clifton L. , Mosier Dennis R. 
Published: October 2004

AbstractAs the number of new transgenic mouse models of human neuromuscular disease continues to increase, the development of sophisticated electrophysiologic techniques for assessing the peripheral nervous system in these models has become important. Neuromuscular junction (NMJ) dysfunction, in particular, is often not detectable by morphologic or other techniques. To enable sensitive testing of murine NMJ function, we developed and tested a method for stimulated single fiber electromyography (S‐SFEMG) in the gastrocnemius muscles of anesthetized mice. Jitter was assessed by measuring the mean consecutive latency difference (MCD) of single fiber responses to sciatic nerve stimulation at 2 HZ. Mean MCD values in normothermic mice were in the range of 6–8 μs for different strains, with no MCD values exceeding 25 μs. Reduced core temperature (to 29°–30°C) resulted in increased jitter, whereas intubation and mechanical ventilation of mice did not alter these values. Intraperitoneal and intravenous injection of vecuronium, however, resulted in progressively increased jitter followed by blocking in continuously monitored fibers. These observations validate the utility of S‐SFEMG in mice as an index of NMJ function under a variety of physiologic conditions, and suggest that a high safety factor for neuromuscular transmission exists at mouse NMJs. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 941–945, 2001
Transcarpal motor conduction velocity in carpal tunnel syndrome
Authors: Walters R. Jon L. , Murray Nicholas M.F. 
Published: October 2004

AbstractTranscarpal motor conduction to abductor pollicis brevis (APB) was evaluated in 43 patients (70 hands) with suspected carpal tunnel syndrome (CTS). Transcarpal motor conduction was abnormal in 80% of hands compared with 11.5% with prolongated distal motor latency from wrist stimulation. Transcarpal motor conduction was comparable in sensitivity with transcarpal sensory conduction and 2nd lumbrical–interosseous latency difference. Transcarpal motor conduction is a sensitive test for diagnosis of CTS. Sensory fibers were no more susceptible than motor fibers to compression in the carpal tunnel, and fibers to APB were as susceptible as those to the 2nd lumbrical muscle. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 966–968, 2001