bluuebunny/crossref_metadata_2025_split huggingface dataset:
Duchenne de boulogne: Electrodiagnosis of poliomyelitis
Authors: Reincke Hanna , Nelson Kevin R. 
Published: January 2005

AbstractDuchenne de Boulogne was among the first to investigate neuromuscular diseases. He stimulated muscle and nerve with moistened surface electrodes, thereby avoiding tissue necrosis. Technique and self‐designed equipment are discussed in his first major work de I'electrisation localisee. During his 30 years of practice, he examined several hundred patients with poliomyelitis. With electrodiagnostic evaluation, he focused on “electrocontractility,” the intensity of muscle contraction elicited by electrical stimulation. Based on his electrophysiologic findings, Duchenne suggested that the responsible lesion resided within the spinal cord. He used electrical stimulation for treatment and recognized prognostic features.
Autonomic function and unmyelinated fibers in chronic inflammatory demyelinating polyradiculonuropathy
Authors: Ingall Timothy J. , McLeod James G. , Tamura Naotoshi 
Published: January 2005

AbstractBecause autonomic function may be abnormal in some demyelinating peripheral neuropathies, it has been studied with a number of noninvasive and invasive tests in 14 patients with chronic inflammatory demyelinating polyradiculoneuropathy. There were abnormalities of the 30:15 ratio in 3 patients and an abnormal thermoregulatory sweat test in 5 patients. These findings are indicative of mild parasympathetic and sympathetic dysfunction. Minor pathological changes in unmyelinated fibers were demonstrated on morphometric examination of sural nerves of 10 patients.
Impairment of retrograde axonal transport in wobbler mouse motor neuron disease
Authors: Mitsumoto Hiroshi , Ferut Anita L. , Kurahashi Kozo , McQuarrie Irvine G. 
Published: January 2005

AbstractThe earliest horseardish peroxidase (HRP) neuronal labeling (the fastest retrograde transport) was determined by histochemical techniques at various intervals after intramuscular HRP injection in wobbler mice and normal littermates. In the clinically impaired forelimb system, the retrograde transport rate was 150–170 mm/day in wobbler mice and 170–230 mm/day in controls. However, there was no statistical difference between the two groups. The neuronal HRP accumulation at the early intervals was significantly less in wobbler mice than controls, suggesting that the amount of HRP transport was dminished in each axon. For the clinically intact hindlimb nerves, the rate was normal in wobbler mice, but the amount of neuronal HRP was significantly increased. Retrograde axonal transport appeared to be affected in a differential fashion, depending on the extent of disease.
Electrophysiological monitoring during lipomyelomeningocele resection
Authors: Phillips Lawrence H. , Park T. S. 
Published: January 2005

AbstractSurgical resection of a lipomyelomeningocele carries a risk of injury to nerve roots in the cauda equina. We have devised a technique for combined spinal evoked potential and peripheral nerve compound action potential recording for intraoperative monitoring of this surgical procedure. We report the results of six cases where this technique was used. In each case, the monitoring prevented resection of viable neural tissue, and all six patients were neurologically unchanged postoperatively.
Mononeuropathy multiplex associated with acute hepatitis B infection
Authors: Cohen Jeffrey A. , Wilborn Sandra L. , Rector William G. , Golitz Loren E. 
Published: January 2005

AbstractImmunologic syndromes are associated with hepatitis B viral (HBV) infection. However, mononeuropathy multiplex (MM), a syndrome in which immune factors may play a role, is rare in acute HBV infection. Few cases of MM associated with HBV infection have been reported. We report a case of acute HBV infection in which MM involving the median, ulnar and lateral femoral cutaneous nerves complicated the course of the acute illness. Skin biopsy demonstrated vasculitic changes which may accompany the immunologic factors in HBV infection.
Acute sensory neuronopathy with preserved SEPs and long‐latency reflexes
Authors: Knazan Morton , Bohlega Saeed , Berry Kenneth , Eisen Andrew 
Published: January 2005

AbstractA case of spontaneous acute sensory neuronopathy is described in a previously healthy 19‐year‐old male. He had marked sensory ataxia with relative sparing of cutaneous sensation. There was no recovery over 4 years. Sural nerve biopsy showed modest axonal loss. Sensory evoked potentials were mildly abnormal in the face of absent sensory nerve action potentials. The long‐latency (R2) reflex was preserved whereas the short‐latency (R1) reflex was absent. The findings indicate that loss of large‐diameter (groupla) ganglion cells are primarily responsible for this syndrome.
M wave changes with temperature in amyotrophic lateral sclerosis and disorders of neuromuscular transmission
Authors: Denys Eric H. 
Published: January 2005

AbstractLocal cooling of the muscle resulted in significant increases in M wave surface areas in patients with ALS, myasthenia gravis, the Lambert‐Eaton myasthenic syndrome, and also in controls. The most striking increases were seen in patients with early ALS who had minimal lower motor neuron involvement and/or little defect on neuromuscular transmission and patients with the myasthenic syndrome. Patients with myasthenia gravis had intermediate increases between these groups and the controls; there was a large increases in M wave surface area in myasthenia gravis compared with controls but this could be accounted for by improvement in neuromuscular transmission. The large increase in M wave surface area in early ALS could be the result of temporal summation of individual muscle fibers in motor units with increased fiber densities. The increase in both ALS and the myasthenic syncrome could result also from the loss of a trophic factor causing changes at the muscle membrane.
Glucose infusion abolishes the excessive ATP degradation in working muscles of a patient with McArdle's disease
Authors: Mineo Ikuo , Kono Norio , Yamada Yuya , Hara Naoko , Kiyokawa Hiroaki , Hamaguchi Tomoya , Kawachi Masanori , Yamasaki Tomoyuki , Nakajima Hiromu , Kuwajima Masamichi , Tarui Seiichiro 
Published: January 2005

AbstractA 23‐year‐old woman with McArdle' disease performed mild leg exercise on a bicycle ergometer. Saline or 10% glucose solution was infused throughout exercise. After exercise with saline infusion, her plasma concentration of ammonia, hypoxathine and creatine kinase increased greatly. Conversely, after exercise with glucose infusion, there were no appreciable changes in these plasma substances. In addition, she noticed that glucose infusion relieved her from muscle symptoms during exercise. These findings seggest that glucose infusion to patiets with McArdle' desease ameliorates excessive ATP degradation in exercising muscles.
Significance of magnetic coil position in peripheral motor nerve stimulation
Authors: Ravnborg Mads , Blinkenberg Morten , Dahl Kristian 
Published: January 2005

AbstractThe influences of coil position and coil–nerve distance on compound muscle action potentials (CMAPs), recorded from the first dorsalinterosseus muscle during magnetic stimulation of the brachial segment of the ulnar nerve, were studied in 10 healthy volunteers. A 14‐cm coil was held tangentially to the skin with the center overlying the nerve. Mapping of the CMAP latencies and amplitudes was made as the coil was displaced laterally in steps of 1 cm and in planes 0–3 cm from the skin surface. Stimulation with the coil center positioned 3 cm laterally to the nerve with the coil current directed proximally yielded the largest amplitudes with minimal variability and the most constant relationship to electrically evoked CMAPs. In this position the interindividual and intraindividual reproducibility of the magnetically evoked latencies were at least as good as those of electric stimulation when coil–skin distance was ⩽ 2 cm.
Regenerated EDL muscle of rats requires innervation to maintain AChE molecular forms
Authors: Melone Mariarosa A. B. , De Lucia Domenico , Fratta Mario , Cotrufo Roberto 
Published: January 2005

AbstractExtensores digitorum longi of rats, infarcted and denervated by different surgical procedures, were used to analyze by biochemical and cytochemical methods the acetylcholinesterase (AChE) changes during muscle degeneration, regeneration, and early or delayed reinnervation. Biochemical tests showed that the regenerating muscle produces globular AChE forms (36% of controls) and small amounts of A12 (16S) asymmetric from (5% of controls); at the end of the regeneration, innervation and electromechanical function are required for the complete recovery of globular forms, and are absolutely critical to prevent A12 (16S) disappearance. Cytochemical observations showed that, unlike nicotinic receptor, AChE deposited at the neuromuscular junction before ischemic necrosis is protected from breakdown, as is the basal lamina of muscle fibers. Taken together, these observations contribute to the understanding of the factors that play a critical role in muscle repair and are, therefore, of clinical relevance.
The anatomy and physiology of nerve injury
Authors: Sunderland Sir Sydney 
Published: January 2005

AbstractNerves have a structure of considerable complexity with features of special relevance to nerve injury and nerve regeneration. These include variations in the cross‐sectional areas devoted to fascicular and epineurial tissue, the fascicular redistribution and mixing of different branch fibers brought about by fascicular plexuses, and the numbers of nerve fibers representing individual branches. The elasticity and tensile strength of nerve trunks and their capacity to resist traction deformation reside in the fascicular tissue, while the epineurium provides a protective cushion against compression. The microstructure of nerve trunks provides the basis for a classification of nerve injuries into five degrees of severity with partial and mixed types—each with a clearly defined pathology and distinguishing clinical features. Following a transection injury, changes occur in the severed axons, endoneurial tubes, fasciculi, and nerve trunk. The type of injury and the nature of these changes determine the outcome of axon regeneration.
Soleus‐specific myopathy induced by passive stretching under local tetanus
Authors: Mizuno Yoshihiko , Chou Samuel M. 
Published: January 2005

AbstractTwenty‐four adult albino rats were injected with tetanus toxin into the right gastrocnemius muscle and then subjected to sustained dorsiflexion of the right ankle joint for 2 to 14 days. Histologic examinations of the soleus after this procedure showed myopathic changes, characterized by variations in fiber diameters, myonecrosis with opaque fibers, interstitial fibrosis, and small groups of regenerated fibers. Electron microscopy revealed derangement of T‐tubules immediately adjacent to the sarcolemma in the early degenerative stage. The size and wet weight of soleus increased compared to that of the control side between 2 and 5 days post‐tetanus. Serum GOT, LDH, and creatine kinase (CK) levels were elevated especially in the early degenerative stages. Peri‐ and endomysial fibrosis developed gradually from about 3 days post‐tetanus. Pathomechanisms inducing these changes were discussed.
Central changes in hypothyroid myopathy: A case report
Authors: Evans Rebecca M. , Watanabe Itaru , Singer Philip A. 
Published: January 2005

AbstractA muscle biopsy from a patient with hypothyroid myopathy showed striking central changes. “Cores” were seen in most type 1 fibers with oxidative enzyme prepartions. Electron microscopy of these areas revealed Z disc streaming, myofilament disruption, and absent mitochondria. The myopathy rapidly improved with thyroxine treatment. This biopsy is of interest, as numerous “cores” have not previously been reported in hypothyroid myopathy.
Time course of sprouting during muscle reinnervation in vitamin E‐deficient rats
Authors: Cuppini Riccardo , Cecchini Tiziana , Cuppini Carla , Ciaroni Sandra , Del Grande Paolo 
Published: January 2005

AbstractA typical aspect of motoneuron plasticity is the sprouting which occurs during muscle reinnervation, resulting in a transitory multiple innervation of the muscle cells. In order to verify the effect of a decreased protection from free radical attack on the sprouting, the multiple innervation in the extensor digitorum longus muscle, following sciatic nerve crush and regeneration, was studied in vitamin E‐deficient rats. Thus, the innervated end‐plates and the end‐plates with multiple innervation were studied with histochemical and electrophysiological techniques. The percentage of innervated end‐plates was similar in both groups at 30 as well as at 60 days after nerve crush. Nevertheless, multiple innervation was found in a larger part of the muscle and it lasted longer in the deficient rats. This finding is discussed in relation to some of the major hypotheses of sprouting; it may be relevant in the treatment of some lesions of peripheral nerve.
Effects of low calcium and calcium antagonists on skeletal muscle staircase and fatigue
Authors: Williams Jay H. 
Published: January 2005

AbstractThe purpose of this investigation was to determine the effects of low extracellular calcium and calcium antagonists on skeletal muscle staircase and fatigue. Initial experiments revealed that, brief exposure (10 minutes) of single frog sartorius muscle to diltiazem, D‐600 (5 and 30 m̈M) and low calcium Ringer's solution (LCR, calcium replaced by magnesium and EGTA) had little effect on isometric twitches evoked every 30 seconds. However, when stimulated at 1 per second for 15 minutes, the calcium antagonists significantly decreased the magnitude and time course of the staircase, whereas LCR decreased only the time course. Each experimental condition significantly increased the rate of fatigue while diltiazem and D‐600 both increased the magnitude of fatigue. Following the stimulation period, caffeine (10 mM) elicited contractures from all muscles whereas high potassium (180 mM) elicited contractures from control muscles only. These results indicate that calcium channel antagonists depress the skeletal muscle staircase response. They also indicate that these compounds as well as LCR enhance the fatigue process. Extracellular calcium influx may therefore have some influence on skeletal muscle twitches during prolonged repetitive activity.
Polyglucosan body disease
Authors: Cafferty Maureen S. , Lovelace Robert E. , Hays Arthur P. , Servidel Serenella , Dimauro Salvatore , Rowland Lewis P. 
Published: January 2005

AbstractAdult polyglucosan disease has been described in 15 cases. All had signs of peripheral neuropathy, upper motor neuron signs, and 12 of the 15 had sphincter problems. Dementia was prominent in 8 of 15 cases. We reported 2 cases that contained these clinical features. Electrophysiological studies showed axonal neuropathy. Somatosensory evoked potentials on the second patient were abnormal. Sural nerve biopsy showed clusters of polyglucosan bodies. Although the presence of polyglucosan bodies in biopsy is nonspecific, the number as well as the clinical features are necessary to make the diagnosis. Branching enzyme activity in muscle extracts of the muscles were normal. Hence, a specific enzyme abnormality is not yet known.
Synchronous positive and negative myoclonus due to pontine hemorrhage
Authors: Palmer Jeffrey B. , Tippett Donna C. , Wolf James S. 
Published: January 2005

AbstractWe report a case of synchronous positive and negative myoclonus following pontine hemorrhage. Constant synchronous jerking of the eyes, tongue, face, mandible, larynx, pharynx, and diaphragms persisted during sleep. Jerking of limb muscles occurred during volitional activities, but not at rest. Inability to sustain glottic adduction during phonation contributed to severe dysarthria. Electromyography (EMG) revealed positive myoclonus of the branchial musculature with synchronous negative myoclonus in a generalized distribution Treatment with trazodone reduced the ocular myoclonus but worsened the dysphagia. We suggest that a single neural rhythm generator may produce both positive and negative myoclonus.
Dystrophin expression in myotubes formed by the fusion of normal and dystrophic myoblasts
Authors: Huard Johnny , Labrecque Claude , Dansereau Guy , Robitaille Lynda , Tremblay Jacques P. 
Published: January 2005

AbstractMdx mouse dystrophy is characterized by the absence in the muscle cytoplasmic membrane of a high molecular weight protein called dystrophin. A possible avenue for treatment of muscular dystrophies is to inject normal myoblasts in a dystrophic muscle to form hybrid muscle fibers. Hybrid myotubes were formed in vitro by the fusion of normal rat and dystrophic mouse (mdx) myoblasts. Staining with Hoechst dye 33258 permitted the clear distinction of mouse and rat nuclei. Immunostaining demonstrated that dystrophin was present over the entire membrane of all hydrid myotubes even when nuclei ratio normal/dystrophic was low.
Intraoperative recordings of spinal somatosensory evoked potentials to tibial nerve and sural nerve stimulation
Authors: Pelosi Luciana , Caruso Giuseppe , Cracco Roger Q. , Cracco Joan B. , Balbi Piero 
Published: January 2005

AbstractSomatosensory evoked potentials (SSEPs) to stimulation of the tibial nerve at the knee (TN‐K) and ankle (TN‐A), and the sural nerve at the ankle (SN‐A), were recorded from 3 or 4 spinal levels during surgery for scoliosis in 11 neurologically normal subjects. With stimulation of all 3 nerves, the propagation velocity along the spine was nonlinear: it was faster over cauda equina and midthoracic cord than over caudal spinal cord. Over the midthoracic cord, TN‐K SSEP propagation was faster than that of TN‐A and SN‐A SSEPs, whereas over the caudal spinal cord these values were similar on stimulation of all 3 nerves. These data suggest that fast conducting second order afferent fiber systems contribute to spinal cord SSEPs evoked by stimulating both mixed and cutaneous peripheral nerves.
Tocainide therapy in muscle cramps and spasms due to neuromuscular disease
Authors: Puniani Trilok S. , Bertorini Tulio E. 
Published: January 2005

AbstractSignificant improvement in symptoms of disabling cramps and muscle spasms was obtained in 9 patients with motor neuron diseases, tetany, and myotonic disorders who were treated with tocainide, a lidocaine analog. No significant side effects were observed except for light‐headedness and fatigue in 1 patient, who also showed slight prolongation of intraventricular conduction time. Tocainide is useful in treating disabling muscle spasms and cramps associated with conditions characterized by neuromuscular irritability. This effect is probably based on stabilization of the membrane potential at various levels (motor neurons, peripheral nerve, or muscle fiber membrane).
Invited review: Electrodiagnostic assessment and monitoring of motor unit changes in disease
Authors: Stålberg Erik 
Published: January 2005

AbstractThe motor unit characteristics change dynamically with disease. This is the basis for the use of neurophysiologic methods for diagnostic purposes and for long‐term monitoring. A great variety of nerve conduction and EMG parameters are available to study the motor unit. They reflect different aspects of motor unit function. Therefore, the electromyographer must choose his technique and optimal parameters depending on the situation, type of condition, whether the purpose is diagnosis or monitoring, and so on. In this presentation the relationship between EMG parameters and physiologic and morphologic counterparts in some pathologic conditions will be discussed.
Muscle regeneration following segmental necrosis in tenotomized muscle fibers
Authors: Baker James H. , Poindextor Christine E. 
Published: January 2005

AbstractThe aim of this study was to determine how the new myotendinous junctions were re‐established at the proximal and distal ends of the soleus muscle after tenotomy. Both proximal and distal tendons of the soleus muscle in mature female rats were severed. The animals were killed and the soleus muscles were removed and prepared for light and electron microscopic examination 1, 3, 5, 7, 14, 21, 28, and 42 days after the operation. It was found that segmental fiber destruction followed by removal by macrophages occurred at the ends of the soleus muscle fibers. This resulted in the liberation and myogenic activation of satellite cells. By 3 days after tenotomy the fusion of myoblasts to form myotubes could be seen. The myotubes developed within the original basal lamina and reattached to the surviving non‐necrotic segments and grew in both length and width so that by 6 weeks postoperation, normal myotendinous junctions had been reformed. This study is the first to show that re‐establishment of the myotendinous junction following tenotomy is accomplished by regeneration of the necrotic ends of the tenotomized fibers.
The natural history of minicore‐multicore myopathy
Authors: Penegyres Peter K. , Kakulas Byron A. 
Published: January 2005

AbstractTo determine the natural history of minicore‐multicore myopathy (MMM) we studied 5 patients who were found to have this disorder from 6 to 16 years ago. Four of these patients had improved muscle strength since their original assessment and one patient had deteriorated. There were no clinical electrophysiological or pathological features that distinguished this last patient from the others. We conclude, therefore, that most patients with MMM may be expected to improve as time passes but, for unknown reasons, a minority may deteriorate. This information should be helpful to clinicians in their prognostic advice to new patients.
Infantile Pompe's disease, lipid storage, and partial carnitine deficiency
Authors: Verity M. Anthony 
Published: January 2005

AbstractA diagnosis of infantile Pompe's disease (glycogenosis type II) was made by muscle biopsy on a 6‐month‐old infant boy seen with hypotonia, weakness, and developmental regression. Histochemistry and electron microscopy revealed a vacuolar myopathy with massive glycoge accumulation associated with increased neutral lipid as demonstrated on Oil Red O reactions. Pleomorphic, hypertrophic mitochondria with distortion of cristae and electron‐dense deposits within the matrix were identified. Acid α−1,4‐glucosidase activity was absent but associated with increased neutral maltase activity and a variable compensatory rise in activity of other lysosomal enzymes. Biochemical studies demonstrated low free carnitine, normal acylcarnitine, increased activity of carnitine palmityl and acyl transferases, and other enzymes of β‐oxidation with the notable exception of low normal β‐hydroxyacyl‐CoA dehydrogenase activity. The explanation for the lipid accumulation is uncertain but is likely related to the combination of low carnitine concentration in muscle, low β‐hydroxyacyl CoA dehydrogenase, representing a rate limiting enzyme of β‐oxidation, and nonspecific defective mitochondrial function.