bluuebunny/crossref_metadata_2025 huggingface dataset:
Quantitation of muscle function in children: A prospective study in duchenne muscular dystrophy
Authors: Scott O. M. , Hyde S. A. , Goddard C. , Dubowitz V.
Published: January 2005

A protocol has been developed for the quantitative assessment of muscle function in children with muscle disease. It includes total muscle strength (% MRC) based on a clinical assessment of strength of 32 groups using the 6‐point MRC grading; the force of 8 selected muscle groups measured with a specially designed electromyometer; a motor ability score based on 20 consecutive motor activities; walking times over 28 and 150 feet, and recording of muscle contractures. A 3‐year sequential study of 61 boys with Duchenne dystrophy showed progressive decline of muscle strength with age, a close correlation of total strength and the motor ability score (r = 0.89), and a curvilinear relationship of muscle strength with walking times over 28 and 150 feet (r = 0.78 and 0.79, respectively). A profile of the natural progression of Duchenne dystrophy has been established which could serve as a reference base for the assessment of cases at varying ages and their response to therapy and management.
Preparation of cell‐free extracellular matrix from human peripheral nerve
Authors: Johnson P. C. , Duhamel R. C. , Meezan E. , Brendel K.
Published: January 2005

The extracellular matrix of human peripheral nerve, which is mainly basement membrane and fibrillar collagen, has been prepared by a procedure involving extensive detergent extraction of isolated endoneurium and perineurium obtained from various nerves. The ultrastructure of the isolated nerve extracellular matrix was indistinguishable from that seen in sections of intact nerve, indicating that the extraction procedure preserved the morphological integrity of these connective tissue components. The amino acid and carbohydrate compositions of the nerve extracellular matrix preparation were typically collagenous in nature containing a high content of glycine, proline, 4‐hydroxyproline, and alanine and significant amounts of lysine and hydroxylysine. The preparations contained virtually no 3‐hydroxyproline and a low content of glucose and galactose compared to pure basement membranes, indicating that interstitial rather than basement membrane collagens predominated. This preparation appears well
hyphen;suited to both the ultrastructural and biochemical study of the extracellular matrix of peripheral nerve.
Electromyographic study of diabetic and alcoholic polyneuropathic patients treated with gangliosides
Authors: Bassi Sirio , Albizzati Maria Grazia , Calloni Elio , Frattola Lodovico
Published: January 2005

A double‐blind, randomized electromyographic investigation was conducted of the effects of cerebral ganglioside treatment on patients suffering from diabetic or alcoholic polyneuropathy. Cerebral gangliosides (50 mg once a day) administered to 15 diabetic and to 15 alcoholic neuropathic patients for 40 days, facilitated the reappearance of sensory potentials and significantly increased the MAP amplitude in median, ulnar, and peroneal nerves. In relation to ganglioside treatment, there was no significant change in the conduction velocities or in the distal latencies of these nerves, nor was there a change in the duration of the MAPs. On the basis of these results, it is suggested that the cerebral gangliosides are capable of inducing an improvement in the excitability of nerve fibers and of facilitating the processes of reinnervation, probably by means of an enhancement of fiber sprouting.
Altered supernormality in multiple sclerosis peripheral nerve
Authors: Eisen Andrew , Paty Donald , Hoirch Maureen
Published: January 2005

Supernormality is a regular part of the recovery cycle of nerve. Supernormality was expressed in terms of amplitude, and in 20 control subjects the mean (S2 conditioned/S2 unconditioned) ratio of the median sensory nerve action potential amplitude recorded at the elbow measured 285 ± 174 (range 119 to 783). A supramaximal conditioning shock was delivered 6 msec in advance of the test pulse. Seventeen (42.5%) of 40 patients with multiple sclerosis (MS) failed to demonstrate supernormality, the (S2 conditioned/S2 unconditioned) ratio being less than 100. This abnormality probably reflects a delay in recovery cycle and is additional evidence implicating peripheral nervous system involvement in multiple sclerosis.
Clinical spectrum of fascial inflammation
Authors: Simon David B. , Ringel Steven P. , Sufit Robert L.
Published: January 2005

The diverse clinical manifestation of fascial inflammation are reviewed including the entities of eosinophilic fasciitis, fascial inflammation in association with connective tissue diseases, fibrositis, nodular fasciitis, and necrotizing fasciitis. The neuromuscular clinician is encouraged to consider the possibility of fascial inflammation when evaluating patients with painful, aching extremities. A simple technique for including a fascial specimen when performing a routine muscle biopsy is described.
Axonal degeneration and focal muscle fiber necrosis in human thallotoxicosis: Histopathological studies of nerve and muscle
Authors: Limos Leonore C. , Ohnishi Akio , Suzuki Nozomi , Kojima Noriaki , Yoshimura Takeo , Goto Ikuo , Kuroiwa Yoshigoro
Published: January 2005

Two cases of proven thallium poisoning were studied with special reference to the neuromuscular changes. These cases showed a consistent pattern of symptomatology of peripheral sensorimotor neuropathy, encephalopathy, and alopecia. Light and electron microscopic studies of the sural nerve biopsies exhibited mainly axonal degeneration without concomitant segmental demyelination. The densities of both large and small myelinated fibers were mildly decreased. Biopsied peroneus brevis muscles showed distinct features of focal muscle fiber necrosis which has not been reported before. Muscle fibers with prominent central nucleation and fiber splitting were frequently seen. These findings are compatible with myopathic changes. In spite of the existence of severe axonal degeneration and muscle atrophy on clinical observation, no neurogenic muscle changes were found.
Congenital nemaline myopathy. II. Quantitative changes in α‐actinin and myosin in skeletal muscle
Authors: Stuhlfauth Ingo , Jennekens Frans G. I. , Willemse Jacobus , Jockusch Brigitte M.
Published: January 2005

Skeletal muscle obtained from 2 patients with congenital nemaline myopathy (CNM) and from a healthy control was analyzed by 1‐ and 2‐ dimensional gel electrophoresis. In total extracts, an increase of α‐actinin by a factor of 2:3 was found for CNM muscle as compared with the control. One‐ and two‐dimensional gels revealed the presence of LCF3, the smallest light chain associated with type 2 (fast) myosin in total extracts of normal control of mixed fiber type. Both CNM samples showed the absence of this polypeptide. This result is consistent with the finding that muscle of the 2 patients exhibited nearly exclusively the ATPase activity indicative of type 1 (slow) myosin.
Skeletal muscle lysosomes: Comparison of lysosomes from normal and dystrophic avian pectoralis muscle as a function of age
Authors: Baxter Jeffrey H. , Suelter Clarence H.
Published: January 2005

The properties of skeletal muscle lysosomes from normal and dystrophic chickens were studied to assess their involvement in the dystrophic process. A method is described for isolation of a three‐to‐sevenfold purified lysosome fraction with 29–33% yield. Lysosomal enzymes in crude homogenates and isolated lysosome‐enriched fractions from dystrophic muscle exhibit decreased latency for N‐acetyl β‐D‐glucosaminidase, acid phosphatase, and cathepsin D. However, no differences in the fragility of lysosomes in isolated lysosome‐enriched fractions from normal and dystrophic muscle were observed using shear, sonication and detergent stress. Lower percent recovery, enrichment factor and percent latency of acid phosphatase compared to N‐acetyl‐β‐D‐glucosaminidase and cathepsin D were observed from both normal and dystrophic muscle. These results are consistent with the presence of a significant amount of nonlysosomal acid phosphatase activity in skeletal muscle.
Prospective study of X‐linked progressive muscular dystrophy in campania
Authors: Nigro Giovanni , Comi Lucia I. , Limongelli Francesco M. , Giugliano Maria Antonietta M. , Politano Luisa , Petretta Vito , Passamano Luigia , Stefanelli Severo
Published: January 2005

Within the Campania region of southern Italy a prospective study on X‐linked progressive muscular dystrophy was conducted over a period of 12 years from 1969 to 1980, inclusive. The mean incidence rate was 21.7 per 100,000 male livebirths for Duchenne muscular dystrophy (DMD) cases and 3.2 per 100,000 male livebirths for Becker muscular dystrophy (BMD) cases. The familial cases were 38.5% among the DMD patients and 50% among the BMD patients. Myocardial involvement appeared in DMD patients at about 6 years of age in a high percentage of cases and increased progressively until the last years of life, when cardiac damage occurred in 95% of cases. The percentage of myocardial involvement in BMD patients was very low before 13 years of age, but increased progressively until 20 years, when cardiac damage occurred in 80% of cases studied; severe cardiomyopathy did not occur before the age of 21. The data reported also include the effects of age on physical performance, serum creatine kinase activity and serum myoglobin levels, the types of cardiac damage, and the causes of death.
Neuropathy in the hypereosinophilic syndrome
Authors: Dorfman Leslie J. , Ransom Bruce R. , Forno Lysia S. , Kelts Alan
Published: January 2005

Peripheral nerve dysfunction was a prominent clinical feature in each of three patients with the hypereosinophilic syndrome (HES). The neuropathy, occurring at the onset of marked eosinophilia or at a time of its worsening, had a painful onset, evolved over 1–4 weeks, and affected both sensory and motor function. Electrodiagnostic studies demonstrated both multifocal and generalized nerve involvement, with the former predominating (multiple mononeuropathy). Nerve and muscle biopsies from two patients revealed severe axonal degeneration with neurogenic atrophy of muscle. The eosinophilia decreased dramatically with corticosteroid treatment and the neuropathy gradually improved, but all three patients manifested residual neurologic disability 17–22 months after onset. The neuropathy may be on an ischemic basis or may be related to neurotoxicity of eosinophil products.
Paradoxical effect of isoproterenol on hamster hereditary polymyopathy
Authors: Jasmin Gaëtan , Proschek Libuse
Published: January 2005

Isoproterenol (ISO), a potent β‐adrenoreceptor agonist, was found to interfere with the development and progression of hamster hereditary polymyopathy. Cytoprotection involved both skeletal and heart muscles with reduced myofibrillar degeneration, phagocytosis, and an unusual scarring process rarely seen at this stage of the disease. A decrease in the Ca content of heart and hemidiaphragm homogenates corroborated these findings. The significant drop of serum creatine kinase with restoration of alkaline phosphatase activity towards normal values provided additional support to the therapeutic effect of ISO. Except for an increase in magnesium, there were no changes in serum electrolytes. The modifications in plasma membrane permeability together with improvement in microcirculation are some of the features whereby ISO can ameliorate muscle cell energy metabolism. It is inferred that the alleged primary role of calcium in the development of this inherited myopathy should be further scrutinized.
Acetylcholinesterase and ATPases in motor neuron degenerative diseases
Authors: Rasool C. G. , Chad D. , Bradley W. G. , Connolly B. , Baruah J. K.
Published: January 2005

Acetylcholinesterase (AChE) activity was measured in the presence of the specific inhibitor of pseudocholinesterase, iso‐OMPA, in plasma from patients with amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), neuromuscular disease controls, and normal controls. Both AChE and Na‐K ATPase activities were measured in erythrocyte ghost membranes from ALS and normal controls. Activities of erythrocyte ghost AChE and Na‐K ATPase did not differ between ALS and control patients, suggesting that erythrocyte membranes were normal in ALS. However, the activity of plasma AChE in patients with ALS and PMA was increased significantly over plasma activity in disease controls and normal controls. In addition, in an animal model of human PMA, the Wobbler mouse, plasma AChE activity was increased significantly over littermate controls. The explanation for the increase in plasma acetylcholinesterase was not clear; however, a number of potentially useful clinical points followed from this study. First, there was no relationship between a specific subtype of motor neuron disease and the level of AChE activity. Second, AChE activity appeared to vary directly with the duration of PMA but not with the severity of PMA. This did not correlate with either the duration or severity of ALS. Last, plasma AChE activity was normal in about 30% of patients who had motor neuron disease; therefore, AChE assay had limited use in the diagnosis of ALS or PMA.
Saphenous nerve evoked potentials and the assessment of intraabdominal lesions of the femoral nerve
Authors: Synek V. M. , Cowan J. C.
Published: January 2005

A technique for recording somatosensory evoked potentials after stimulation of the patellar branch of the saphenous nerve is described and the results from 11 normal subjects and from two patients with intraabdominal femoral nerve injuries are presented. One patient developed a nerve lesion after an abdominal surgical procedure and the second suffered a gunshot wound causing femoral nerve paralysis. Both patients recovered spontaneously. Evoked potentials from saphenous nerves showed a significant discrepancy between faster motor recovery and delay in reinstating sensory axonal conduction.
Evolution of nerve conduction abnormalities in children with dominant hypertrophic neuropathy of the charcot‐marie‐tooth type
Authors: Gutmann Ludwig , Fakadej Alexander , Riggs Jack E.
Published: January 2005

Serial motor conduction velocities and distal motor latencies were determined in two pairs of dizygotic twins, each born to a parent with dominant hypertrophic neuropathy of the Charcot‐Marie‐Tooth type (HMSN‐I). Motor nerve conduction velocities could not distinguish between the normal and affected twin of the first pair studied at birth. Distal motor latency in the affected twin at birth, however, was border‐line prolonged. The affected twin of the second pair had slowed motor velocities at age 17 months, but the extent of conduction slowing had not yet fully developed. Studies of these patients and the affected family members showed that maximal slowing of motor nerve conduction velocities evolved over the first 3–5 years of life in HMSN‐I. Prolongation of distal motor latency may be the earliest abnormality observed in HMSN‐I and this abnormality evolves over 10 or more years.
AAEE minimonograph #20: Macro EMG
Authors: Stålberg Erik
Published: January 2005

A new EMG recording technique called macro EMG is described. The recording electrode is the cannula of the modified single fiber electromyography (SFEMG) electrode. By means of spike triggered averaging, the contribution from all muscle fibers in a motor unit is extracted. The resulting signal reflects the number and size of muscle fibers in one motor unit. This paper presents the details of the technique, the neurophysiological basis for generation of the macro EMG signal, and the typical findings in normal and diseased muscles. Finally, examples are given of the additional information that can be obtained by combining investigations with SFEMG, conventional EMG, and macro EMG in the same muscles.
The distribution of conduction velocities (DCV) in peripheral nerves: A review
Authors: Dorfman Leslie J.
Published: January 2005

Recent advances in digital signal processing have permitted the development of clinically relevant, noninvasive, computer‐based methods for estimating the distribution of conduction velocities (DCV) in motor, sensory, and mixed populations of large myelinated nerve fibers. All currently available methods incorporate explicit and implicit assumptions about the nature of the unit impulses under study. Preliminary investigations using DCV methods have clarified some issues concerning conduction of impulses in the different fiber subpopulations of normal and diseased human nerves. In the presence of severe nerve disease, DCV analysis is usually either impractical or superfluous; additional studies are needed to define its range of clinical applicability. Extension of this technology to clinical analysis of small myelinated and unmyelinated fiber populations will require improvements in the techniques of nerve stimulation and recording.
Hypokalemic periodic paralysis: In vitro investigation of muscle fiber membrane parameters
Authors: Rüdel Reinhardt , Lehmann‐Horn Frank , Ricker Kenneth , Küther Gerald
Published: January 2005

To study the mechanism of attacks in familial hypokalemic paralysis, we recorded resting membrane potentials, action potentials, current–voltage relationships, and isometric forces in intercostal muscle fibers from three patients. In normal extracellular medium, the resting potential was reduced, but membrane conductance was not different from control. Excitability was reduced and the action potentials had no overshoot. On exposure to a 1‐mM potassium solution, with or without insulin, the cells depolarized to about −50 mV, and became inexcitable. Over the tested membrane potential range from −120 to −40 mV, the slope conductance in the 1‐mM potassium solution was not different from that of control fibers in a 1‐mM potassium solution. In particular, the potassium component conductance was not reduced. Depolarized fibers could not be completely repolarized by returning to a 3.5‐mM potassium solution. An experimentally induced transient shift of the chloride equilibrium potential to a highly negative value caused stable repolarization. Paralysis could also be induced by replacement of extracellular chloride with an impermanent anion, a treatment which causes myotonia in healthy fibers. It was concluded that the basic defects are a reduced excitability and an increased sodium conductance, and that these defects are aggravated on reduction of the extracellular potassium concentration.
A new approach to intramuscular placement of horseradish peroxidase
Authors: Klueber Kathleen M. , Ontell Marcia
Published: January 2005

A new method of intramuscular placement of a semisolid paste of horseradish peroxidase (HRP) using a root canal file is described. This method has been demonstrated to produce consistent and reproducible numbers of labeled motoneurons in the mouse extensor digitorim longus muscle (EDL). While the number of labeled cells is similar to that found with optimal intramuscular injection of liquid HRP, this new technique provides more reproducible results by eliminating the reflux often associated with intramuscular injection of liquid HRP.
Neuronotrophic effects of skeletal muscle fractions on neurite development
Authors: Hsu L. , Natyzak D. , Trupin G. L.
Published: January 2005

Soluble fractions of chick embryonic skeletal muscle stimulated radial outgrowth of long neurites from peripheral ganglia. Dorsal root ganglia were more responsive to the growth stimulus of muscle fractions than sympathetic ganglia. Muscle fractions from chicks immobilized with d‐tubocurarine chloride (dtc) were significantly more effective in stimulating growth than normal muscle fractions. Neuritic outgrowth stimulated by muscle fractions was not blocked by antisera to the Nerve Growth Factor (NGF) indicating that these neuronotrophic effects were not due to NGF.
The motor end‐plate fine structure and ultrastructural localization of acetylcholine receptors in amyotrophic lateral sclerosis
Authors: Tsujihata Mitsuhiro , Hazama Ryuji , Yoshimura Toshiro , Satoh Akira , Mori Masataka , Nagataki Shigenobu
Published: January 2005

The motor end‐plate fine structure and distribution of junctional acetylcholine receptors (AChRs) in patients with amyotrophic lateral sclerosis (ALS) were studied. Morphometric analysis was carried out in 74 end‐plates (92 innervated and 47 denuded postsynaptic regions) from 10 patients with ALS. AChR was studied by means of peroxidaselabeled α‐bungarotoxin binding in 45 end‐plates (86 innervated and 36 denuded postsynaptic regions) from 6 patients with ALS. The postsynaptic regions were denuded of their nerve terminals in 33.8% (9.8% in controls). However, the postsynaptic folds in denuded regions were fairly well preserved, as were junctional AChRs. No highly simplified postsynaptic regions were observed. These results suggest that reinnervation of the skeletal muscle by nerve sprouting in ALS may occur in the previously denuded postsynaptic regions and the preserved postsynaptic regions including AChRs, the basement membrane, and Schwann cells may play an important role in this process.
Acute vs. chronic compressive neuropathy
Authors: Miller Robert G.
Published: January 2005

Studies of the management of acute and chronic compressive peripheral neuropathies have not kept pace with advances in our understanding of the pathophsiology of these disorders. In this review, the pathophysiological differences between acute and chronic compressive neuropathy are reviewed from experimental models as well as from clinical examples in humans. It is concluded that surgical intervention is unlikely to alter significantly the course of recovery in acute compressive neuropathies. Similarly, in chronic compressive neuropathies without progressive weakness, conservative treatment is indicated because of the high incidence of spontaneous improvement. However, in patients with progressive weakness, especially in conjunction with electrophysiological evidence of partial denervation or conduction block (but not simply slowing of nerve conduction), surgical treatment is recommended.
Unclassified familial myopathy resembling steinert disease, without myotonia
Authors: Telerman‐Toppet Nicole , Khoubesserian Patrick , Bacq Michèle , Durdu Jacques , Lambelin Dominique , Lousberg Geneviève , Coërs Christian
Published: January 2005

A familial myopathy with predominantly proximal muscle atrophy is described. Although several clinical features such as sternomastoid and brachioradialis muscle involvement suggested Steinert disease, myotonia was not demonstrated by clinical examination or EMG. Histological data were consistent with myotonic dystrophy.
Endoneurial ATPase activity in tangier disease and other peripheral neuropathies
Authors: Sutherland Wayne H. F. , Pollock Martin
Published: January 2005

Endoneurial sodium, potassium adenosine triphosphatase (Na+, K+‐ATPase) and Mg2+‐ATPase activities were determined in routine sural nerve biopsies from patients being evaluated for peripheral neuropathy. A significant reduction of endoneurial Na+, K+‐ATPase and Mg2+‐ATPase activities was shown in six sural nerve biopsies from patients with Tangier disease complicated by mononeuopathy multiplex or progressive axonal neuropathy. Peripheral nerve ATPase activities did not correlate with myelinated or unmyelinated nerve fiber densities in these biopsies. Other peripheral neuronal disorders with reduced endoneurial Na+, K+‐ATPase and Mg2+‐ATPase activities included severe vasculitic neuropathy, diabetic neuropathy, tomaculous neuropathy, and motoneuron disease. Such reduced levels of ATPase activity in peripheral nerve may relate to altered endoneurial lipid metabolism and impaired axoplasmic flow.
Membrane currents in human intercostal muscle at varied extracellular potassium
Authors: Kwiecinski Hubert , Lehmann‐Horn Frank , Rüdel Reinhardt
Published: January 2005

Hyperpolarizing and depolarizing square steps were imposed on the membrane potential of excised human intercostal muscle fibers by means of a 3‐microelectrode voltage clamp. The steady‐state amplitudes of the membrane currents inducing such steps were investigated as a function of the membrane potential, while the muscle was bathed in solutions varying in potassium content (Ke = 1, 3.5, 7, 20, and 60 mM). At all potassium concentrations, the membrane acted as a rectifier, both in the inward‐ and outward‐going directions. Inward currents were much reduced when Ke was lowered from 3.5 to 1 mM, and were increased when Ke was raised beyond 3.5 mM. The delayed outward current was reduced when Ke was increased from 3.5 mM to 7 mM and higher potassium concentration. The results were qualitatively similar to those reported for rat skeletal muscle.
Distal muscular dystrophy with autosomal recessive inheritance
Authors: Scoppetta Ciriaco , Vaccario Maria Luigia , Casali Carlo , di Trapani Girolamo , Mennuni Gioacchino
Published: January 2005

Two sisters presented with distal weakness and their muscle biopsy was dystrophic. This distal muscular dystrophy has an autosomal recessive inheritance and its features are somewhat different from the more common autosomal dominant distal muscular dystrophy and include: (a) onset in early adult life: (b) involvement of distal leg muscles and especially peroneal muscles; (c) marked early elevation of serum creatine kinase (CK); (d) brief duration, small amplitude motor units and fibrillation on electromyography; and (e) histologic features of a dystrophic myopathy.
A comparative study on glucagon effect between mcardle disease and tarui disease
Authors: Mineo Ikuo , Kono Norio , Shimizu Takao , Sumi Seiichi , Nonaka Kyohei , Tarui Seiichiro
Published: January 2005

Pretreatment with glucagon relieved patients with McArdle disease from muscular symptoms during exercise and enhanced exercise performance, though it did not produce any improvement in patients with Tarui disease. The difference in glucagon effect between the two diseases was clearly demonstrated in the bicycle ergometer exercise tests. In addition, the semi‐ischemic forearm exercise tests performed after glucagon injection showed that increased lactate production was significantly incluced by exercise in McArdle disease, but it was not the case in Tarui disease. In McArdle disese, the augmentation in exercise‐induced lactate production was also observed after administration of glucose, or glucose plus insulin, but it was neither observed after administration of insulin alone nor after arginine or epinephrine administration. These findings suggest that the beneficial effect of glucagon in McArdle disease is due to the enhanced utilization of circulating glucose through the muscular glycolytic pathway realized in the coexistence of hyperglycemia and hyperinsulinemia.
New techniques for studying human muscle function, metabolism, and fatigue
Authors: Edwards Richard H. T.
Published: January 2005

To analyze human muscle function, metabolism, and fatigue in both health and disease a variety of techniques may be employed. The most fundamental approach is the measurement of force, which may be generated by either voluntary or electrically stimulated contractions. More detailed information concerning the mechanisms of both weakness and fatigue can be obtained when the electromyogram is recorded simultaneously with force. The needle biopsy technique is recommended for the histological and histochemical examination of skeletal muscle. It has the advantage of being rapid and repeatable and has proved suitable for both diagnostic and follow‐up purposes. Recently developed techniques allow the study of mitochondrial function and protein synthesis. Topical nuclear magnetic resonance spectroscopy is a new and exciting technique for the nonivasive study of muscle metabolism. The opportunities offered by this technique are largely complementary to others available and unlikely to replace them as diagnostic and monitoring tools.
Altered rat skeletal muscle carnitine with age and after denervation
Authors: Czyzewski Krzysztof , Stern Lawrence Z. , Sadeh Menachem , Bahl Joseph J.
Published: January 2005

We investigated the effect of denervation on the concentration of total, free, and acyl carnitine in the soleus (S), extensor digitorum longus (EDL), and anterior tibial (AT) muscles of the rat. Soon after denervation there was a marked decrease in total carnitine in all three muscles. Acyl carnitine levels decreased concomitantly with changes in total carnitine. In control but not in denervated muscle, the carnitine concentration was age‐dependent during the period of rapid growth of the animals. The highest carnitine concentration was observed at 14 weeks of age. The results of this study suggest that normal innervation significantly influences carnitine‐mediated lipid metabolism in skeletal muscle.
Changes in intramuscular collagen and fibronectin in denervation atrophy
Authors: Salonen Vesa , Lehto Matti , Kalimo Hannu , Penttinen Risto , Aro Hannu
Published: January 2005

The distribution of collagen types I, III, and V and fibronectin was investigated by means of immunofluorescent techniques in denervated and normal rat skeletal muscle. During a period of 28 days, a distinct atrophy developed in the denervated gastrocnemius muscle and was accompanied by an increase in types I and III collagen in the endomysium and perimysium. The amount of type V collagen showed little change, whereas fibronectin increase closely parallelled types I and III collagen. The results indicate that denervation atrophy in muscles is accompanied by striking fibrotic changes due to mesenchymal types I and III collagen.
Peripheral neuropathies associated with monoclonal proteins: A clinical review
Authors: Kelly John J.
Published: January 2005

Over the last decade, the increasing use of serum and urine protein electrophoretic screening of patients with idiopathic peripheral neuropathy has led to greater recognition of peripheral neuropathy syndromes that are associated with monoclonal proteins and plasma cell dycrasias. After careful evaluation, most of these patients have benign monoclonal gammopathy, followed in frequency by primary systemic amyloidosis and osteosclerotic myeloma, with occasional cases associated with osteolytic multiple myeloma, Waldenstrom's macroglobulinemia, gamma heavy chain disease, and other rare disorders. Several of these syndromes have distinctive presentations and are recognizable clinically, whereas others (especially multiple myeloma neuropathy) are diverse clinically and are not clearly distinguishable from other chronic neuropathies. The discovery of lgM‐kappa monoclonal proteins directed at myelin antigens in some patients with benign monoclonal gammopathy and the delineation of the syndrome of neuropathy and multiorgan involvement in osteosclerotic myeloma are important developments which may shed light on the mechanism of the remote effects of malignancies on the nervous system.
Experimental acute alcoholic myopathy—a histochemical study
Authors: Haller Ronald G.
Published: January 2005

The histochemical features of muscle injury and repair were examined in an experimental form of acute alcoholic myopathy in the rat. Typical features of rhabclomyolysis were found, which were indicated by scattered muscle fiber necrosis followed by sequential phases of fiber degeneration, phagocytosis, and regeneration. Nests of small, regenerated, or split fibers remained 2 months after the episode of acute myopathy. Vulnerability of type 1 fibers to alcohol‐induced muscle injury was evident by selective involvement of muscles with type 1 fiber predominance and by the fact that regenerating fibers were virtually exclusively type 1. The histologic features of myopathy in this model closely parallel those in human acute alcoholic myopathy.
Assessing sensory involvement in lower limb nerve lesions using somatosensory evoked potential techniques
Authors: Synek Vladimir M.
Published: January 2005

Somatosensory evoked potential (SEP) investigations in 18 patients with lower limb lesions are summarized. Seven patients had problems involving the lateral femoral cutaneous nerve, four the femoral nerve, and seven the sciatic nerve. All lesions were unilateral. SEP techniques were accurate in meralgia paresthetica, and all five patients with demonstrable SEP abnormalities were successfully treated surgically. Delayed recovery of sensory function was shown in two patients with femoral nerve lesions and two patients with sciatic nerve lesions. In patients with sensory symptoms, only an SEP abnormality confined to the nerve distribution provides evidence of a lesion in the sensory pathways and helps to exclude psychologic causes. In a case of peripheral nerve Schwannoma, the presence of a small delay in a case of peripheral SEP supported the preoperative assessment that the tumor was not intrinsic in nature, and this was confirmed at surgery and on subsequent full recovery. The SEP technique allows quantitative assessment of sensory nerve conduction through the peripheral neuron and centripetal pathways.
Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy
Authors: Albers James W. , Donofrio Peter D. , McGonagle Timothy K.
Published: January 2005

We reviewed 180 electroneuromyographic (EMG) studies from patients with acute inflammatory demyelinating polyradiculoneuropathy. EMG criteria suggestive of demyelination were met during the first 5 weeks in 87% of patients; an additional 10% had indeterminate electrodiagnostic evaluations, and 3% demonstrated axonal degeneration only. Motor nerve conduction abnormalities initially predominated, with the nadir of abnormality occurring at week 3. Sensory nerve conduction abnormalities peaked during week 4 and were atypical for polyneuropathy, with 52% of patients having normal sural but abnormal median sensory studies, perhaps reflecting distal nerve involvement. Delayed sensory abnormalities may reflect, in part, secondary involvement related to increased intraneural edema accentuated by compression at sites of anatomic vulnerability. Fibrillation potentials and increased polyphasia appeared between weeks 2 and 5 in proximal and distal muscles simultaneously, which is consistent with either random axonal degeneration at any point along the axon or distal involvement. Resolution of conduction abnormalities began between weeks 6 and 10, with increased mean motor‐evoked amplitude best reflecting functional clinical recovery.
Electrophysiologic study in benign human botulism type B
Authors: Martínez A. Cruz , Anciones B. , Ferrer M. T. , Tejedor E. Díez , Conde M. C. Pérez , Bescansa E.
Published: January 2005

Conventional electromyography (EMG) and single fiber EMG (SFEMG) were performed in a 64‐year‐old diabetic woman with mild type B botulism. The main clinical signs were autonomic dysfunction and cranial nerves paresis. Conventional EMG was normal, except for small changes that were consistent with mild mixed peripheral neuropathy in the lower limbs and were related to diabetes. Repetitive stimulation and results of single stimulus before and after full effort were normal. SFEMG showed increased jitter and impulse blocking in clinically normal muscles. The jitter was frequency dependent and improved at a higher innervation rate. Impulse blocking in potentials with only slightly increased jitter was found. The follow‐up showed improvement of the jitter in agreement with clinical recovery. Jitter abnormalities were recorded after 16 weeks, when clinical signs of botulism had been reversed to normal. Motor unit fiber density increased progressively, and on examination at 8 weeks, some potentials showed very high jitter values. Both findings might suggest new endplate formation, perhaps due to ultraterminal sprouting development.
Effects of long‐term physical training and detraining on enzyme histochemical and functional skeletal muscle characteristics in man
Authors: Larsson Lars , Ansved Tor
Published: January 2005

The adaptability of human skeletal muscle to increased (training) and decreased (detraining) usage was studied in 11 athletes over a 42‐month‐long observation period. Biopsies were taken from the deltoid and the quadriceps muscle, together with measurements of maximum torque output during voluntary knee extensions at high relative to slow speeds of movement. A 16% and 14% decrease in the proportion of type I fibers was seen in the proximal arm and leg muscles, respectively, in the detraining subjects. This conformed with the changes in muscle function. On the other hand, in the training subjects, who increased their activity level through systematic daily physical training over an almost 4‐year‐long period, there were no significant changes seen in fiber type proportions of either arm or leg muscles. This was presumably due to the smaller net change in physical activity level caused by training as compared to detraining in the subjects of this study. Thus, the results show that fiber type proportions in intact human skeletal muscle are not exclusively determined by heredity, but may also be influenced by environmental factors, such as physical activity level.
Effect of denervation on glucose uptake in rat soleus and extensor digitorum longus muscles
Authors: Shoji Shin'ichi
Published: January 2005

From 1 to 14 days after denervation, glucose uptake in the fast extensor digitorum longus and slow soleus muscles in rats was investigated and compared with that of the corresponding intact contralateral muscles. Denervation‐induced atrophy in soleus was greater than that in extensor digitorum longus muscle. Glucose uptake in extensor digitorum longus muscle increased significantly, but that in soleus decreased significantly.
Caveolae preservation in the characterization of human neuromuscular disease
Authors: Lee Robert Edward , Poulos Ann C. , Mayer Richard F. , Rash John E.
Published: January 2005

We have examined freeze‐fracture replicas and conventional thinsection images of rat myofibers prepared by perfusion and by conventional immersion fixation protocols, and myofibers of normal and dystrophic human myofibers prepared by similar immersion fixation methods. In both rat and human myofibers, the size and distribution of caveolae was found to differ substantially according to (1) the method of glutaraldehyde exposure, (2) the depth of the myofiber from the surface exposed to the fixative, and (3) if surgically bisected, the distance from the cut end of the myofiber. Conventional immersion fixation resulted in unavoidable but predictable alterations in sarcolemmal caveolae. These reproducible artifacts of fixation technique substantially complicate the use of caveolae as reliable markers for the characterization of human neuromuscular disease.
Sural nerve biopsy studies in leigh's subacute necrotizing encephalomyelopathy
Authors: Goebel Hans H. , Bardosi Attila , Friede Reinhard L. , Kohlschütter Alfried , Albani Michael , Siemes Hartmut
Published: January 2005

Peripheral neuropathy marked by reduced nerve conduction velocities was found in four unrelated children, betwen the ages of 15 months and 9 years, whose autopsies revealed Leigh's subacute necrotizing encephalomyelopathy. Sural nerve biopsies disclosed primary demyelination and remyelination, as well as loss of myelinated and unmyelinated axons. The use of morphometric and electron microscopic studies shows that these techniques may reveal peripheral neuropathy in Leigh's disease more often than light microscopic methods alone.
Automatic analysis of the electromyographic interference pattern. Part I: Development of quantitative features
Authors: Nandedkar Sanjeev D. , Sanders Donald B. , Stålberg Erik V.
Published: January 2005

We have developed three new features of the electromyographic interference pattern (IP), based on the turns and amplitude of the signal, to quantitate some of the features of the IP that are usually assessed subjectively by an electromyographer. The activity measures the fullness of the IP. The upper centile amplitude (UCA) defines the upper limit of the maximum peak‐to‐peak amplitude of the motor unit action potentials (MUAPs) contained in the IP. The number of small segments (NSS) measures the complexity of the IP, which is a reflection of the polyphasicity of the component MUAPs. The activity and the logarithm of the UCA correlate strongly with the force of muscle contraction at which the IP is measured. The NSS initially increases with the force of contraction and becomes relatively constant at higher force levels. The normal values of these features and the interpretation of their relationships are described in companion papers.
Distribution of capillaries in normal and diseased human skeletal muscle
Authors: Carry Michael R. , Ringel Steven P. , Starcevich Jill M.
Published: January 2005

The distribution of capillaries was studied in muscle biopsies in a large number of normal subjects and in patients with disuse atrophy, inflammatory myopathies, and other chronic myopathic and neurogenic disorders. The cryostat retrieval technique was used, along with capillary and muscle fiber morphometry, to quantitate capillary distribution around specific fiber types. In all diseased and normal muscle, the number of capillaries per fiber (C/F) and the number of capillaries surrounding a fiber (CAF) increased with fiber diameter. More capillaries typically surrounded type 1 than type 2 fibers within a given group. When all inflammatory myopathy patients (polymyositis, dermatomyositis, and polymyositis plus second connective tissue disease) were compared with normal adults, their muscle biopsies had fewer capillaries per fiber over the same diameter range. The numerous variables influencing capillary number in normal and diseased muscle are discussed.
Muscle regeneration and satellite cells in fukuyama type congenital muscular dystrophy
Authors: Terasawa Kenjiro
Published: January 2005

To determine the regenerative capability of muscle fibers in Fukuyama type congenital muscular dystrophy (FCMD), muscle biopsies from nine patients were subjected to morphological examination by histochemical and electron microscopic methods. These results were compared with those from six patients with Duchenne muscular dystrophy (DMD) and nine controls. Degenerating and regenerating fibers were not as frequent in FCMD as in DMD, whereas the number of type 2C fibers in the former was about twice that in the latter. On electron microscopy, numerous immature small fibers with disorganized myofibrils, an increased amount of intermediate filaments, large vesicular nuclei, and prominent nucleoli were observed in FCMD. Satellite cells (SCs), which are known to proliferate and contribute to muscle regeneration after myonecrosis, were not increased in number in FCMD. Their incidence, 7.7% ± 1.8%, was significantly lower than the 16.2% ± 4.0% in DMD (P < 0.005). Moreover, the ratio of area of SC nucleus to the overall area of the SC in FCMD, 64.1% ± 9.7%, was larger than that in DMD, 58.9% ± 9.3% (P < 0.005), suggesting that SCs of FCMD were not in an active state. These findings lead to the conclusion that in FCMD the regenerative process is delayed due to either intrinsic or extrinsic factors, such as dense interstitial fibrosis.
Emery—dreifuss muscular dystrophy: Report of five cases in a family and review of the literature
Authors: Merlini Luciano , Granata Claudia , Dominici Paola , Bonfiglioli Silvia
Published: January 2005

Clinical and laboratory data of five cases of Emery—Dreifuss muscular dystrophy in three generations of a family are reported. Severity of the myopathic picture varied in the five patients considerably from almost noninvolvement to severe manifestations. In contrast, cardiomyopathy was severe not only in three adults but also in a 13‐year‐old boy. A review is given of 73 cases from the 6 major families reported in the literature. Most striking is the high incidence of sudden death. A wide variability in the severity of the myopathic picture was also evident.
Nemaline myopathy of cats
Authors: Cooper Barry J. , De Lahunta Alexander , Gallagher Eleene A. , Valentine Beth A.
Published: January 2005

An apparently inherited myopathy, characterized by the presence of large numbers of nemaline rods in skeletal muscle fibers, was investigated in five cats. Onset of signs varied from 6 months to 1.5 years of age and consisted of reluctance to move, jerky gait and muscle twitching, hyporeflexia, and muscle wasting, which was most prominent in the proximal muscles of the forelimbs. All of the cats, three males and two females, were from the same dam. In addition to the presence of rods, the myopathy was characterized by marked fiber size variation, with atrophy of type 1 and type 2a muscle fibers. In addition, there was infolding of the sarcolemma and fiber splitting. Ultrastructurally, the rods closely resembled those described in human nemaline myopathy.
Motor endplate position of rat gastrocnemius muscle
Authors: Dekhuijzen Aarnoud J. , van Koetsveld Petrus A. , Baan Guustaaf C. , Woittiez Reinout D. , Huijing Peter A.
Published: January 2005

In this study, the relative endplate position of fibers of rat gastrocnemius caput mediale (GM) muscle was determined by counting numbers of sarcomeres. Isolated fibers were teased from the proximal, intermediate, and distal regions of the muscle. Endplates of distal fibers were located on the proximal third of their lengths. Endplates of intermediate fibers were located at half fiber length, and for proximal fibers, a variable endplate position was obtained: in half of the muscles studied, endplates occurred around the proximal one‐third and in the other half near the midpoint of the fiber. Endplate position relative to fiber length was thus found to be dependent on the region of the muscle. Changes in the orientation of endplate zone relative to the muscle belly is likely to take place with changes in muscle length, as shown by a planimetric muscle model. It is argued that architecture of pennate muscles may highly affect characteristics of motor unit potentials.
The effect of aerobic exercise on serum creatine kinase activities
Authors: Nicholson Garth A. , Morgan Graeme J. , Meerkin Matthew , Strauss Eric R. , McLeod James G.
Published: January 2005

Aerobic exercise is now a common form of recreational exercise among young women. In a previous study, more than a third of a group of young mothers volunteering blood samples to establish a creatine kinase (CK) reference range for Duchenne muscular dystrophy (DMD) carrier detection regularly‐participated in aerobic exercise programs. Aerobic exercise programs include eccentric exercises. As eccentric exercise is known to produce a delayed CK peak, this study was carried out to determine the effect of aerobic exercise on serum CK activities. The postexercise serum CK activity peak was monitored in 15 young women (age range 20–23 years) following aerobic exercise classes (45 minutes on 3 consecutive days). Peak values at 24–48 hours following the last class ranged from 90 to 3473 U/liter, or 1.55 to 34.71 times resting values. It is concluded that aerobic exercise programs should be excluded in order to obtain accurate resting serum CK values for muscle disease diagnosis.