bluuebunny/crossref_metadata_2025_split huggingface dataset:
The epidemiology of motor neuron disease in Scotland
Authors: Holloway Susan M. , Emery Alan E. H. 
Published: January 2005

AbstractA study was made of the frequency of motor neuron disease in Scotland. The frequency of the disease was about the same as in most other countries of the world, but it may be increasing in Scotland. Individuals resident in the north and east of the country and those engaged in agricultural work were found to have an increased risk of developing the disease. It is suggested that motor neuron disease might be related to the use of insecticides or may result from a persistent viral infection.
Variability in nerve biopsy findings in a kinship with dominantly inherited charcot‐marie‐tooth disease
Authors: van Weerden Tiemen W. , Houthoff Hendrik Jan , Sie Onggie , Minderhoud Jan M. 
Published: January 2005

AbstractA large kinship with an autosomal hereditary‐motor‐and‐sensory‐neuropathy (HMSN) form of Charcot‐Marie‐Tooth disease (CMTD) is described. The affected members have the clinical features and reduced motor nerve conduction velocities of the hypertrophic type of CMTD, or HMSN I. Biopsies of the sural nerves of five affected members showed a large variability of demyelination and remyelination and onion bulb formation, independent of axonal atrophy and the severity of the disease. These findings are discussed in relation to recent literature.
The influence of testosterone on neuromuscular transmission in hormone sensitive mammalian skeletal muscles
Authors: Souccar Caden , Lapa Antonio José , do Valle José Ribeiro 
Published: January 2005

AbstractThe influence of testosterone on neuromuscular transmission was studied in levator ani (LA) and extensor digitorum longus (EDL) muscles taken from normal rats, castrated rats, and castrated rats treated with testosterone. Thirty days after castration LA muscle weights were reduced by 60%, but the frequency and amplitude of the miniature end‐plate potentials (mepps) were increased by 40% and 50%, respectively. The weights and mepp frequencies of the EDL muscles were not altered after castration, but the mepp amplitudes increased by 30%. The quantal content of the endplate potentials was not affected in either muscle. Administration of testosterone to the castrated rats prevented such changes in the LA muscles. The results indicate that castration of adult rats affects the spontaneous transmitter release in both muscles, but the changes are more pronounced in the levator ani, which is a target muscle for testosterone.
The diagnostic yield of quantified electromyography and quantified muscle biopsy in neuromuscular disorders
Authors: Buchthal Fritz , Kamieniecka Zofia 
Published: January 2005

AbstractElectromyography (EMG), histology, and histochemistry were related in 264 patients with neuromuscular disorders classified according to history and clinical and other laboratory findings. Electromyography and histological and histochemical abnormalities were divided in specific and nonspecific criteria. Specific histochemical criteria alone identified 28% of neurogenic lesions. Criteria of myopathy, obtained from the pattern of electrical activity during 30% of maximal effort, helped to delineate a myopathy when the only abnormality was an increased incidence of polyphasic potentials together with a pattern of full recuitment during maximal effort. Histology, histochemistry, or both, and EMG were concordant with clinical findings in 77% of 188 patients with myopathy and in 91% of 64 patients with neurogenic lesions. The electromyogram was concordant with the clinical classification in 87% of patients with myopathy and in 91% of patients with neurogenic impairment. The biopsy was in agreement with or contributed to the classification in 79% of patients with myopathy and in 92% of patients with neuropathy.
Prevention of skeletal muscle fiber necrosis in hamster dystrophy
Authors: Karpati George , Carpenter Stirling , Prescott Steven 
Published: January 2005

AbstractNecrosis and widespread central nucleation of skeletal muscle cells, which are the main features of skeletal muscle pathology in genetically dystrophic hamsters (UMX‐7.1 strain), were not present in muscles of 45‐ to 65‐day‐old animals which had been surgically denervated at 15 to 18 days of age. Necrosis and widespread central nucleation were also absent in hind leg muscles at 30 to 60 days of age after transection of the high thoracic spinal cord at 15 to 18 days. Normal excitation or contraction of the skeletal muscle fibers in early age appears necessary for the microscopic pathological expression of the dystrophic gene in skeletal muscles in the UMX‐7.1 strain hamsters. These experiments constitute a rare and clear example of consistent prevention of skeletal muscle cell destruction in vivo in an inherited myopathy.
Decreased chemiluminescence in thymocytes of dystrophic hamsters
Authors: Con Andrew R. , Wrogemann Klaus 
Published: January 2005

AbstractWhen thymocytes are stimulated they generate reactive oxygen species, which under appropriate conditions produce chemiluminescence (CL). The reactions occur near the cell surface. Since genetically determined muscular dystrophies are currently considered “membrane diseases”, we tested the CL of thymocytes from dystrophic hamsters, strain BIO 14.6, in comparison with control animals of the Rb‐strain. CL of 33 × 106 thymocytes each was monitored in a liquid scintillation counter at 32 C. Dystrophic cells stimulated with concanavalin A (Con A) reached only 60% of the normal peak CL. When stimulated with the calcium ionophore A23187, dystrophic cells exhibited only 40% of the peak CL of control cells. The thymus weight of dystrophic hamsters was significantly reduced. It is not known yet whether these alterations of the thymus are secondary to extrathymic factors or whether they are intrinsic to the thymocyte. If the latter is true, it would be an indication that the genetic defect of dystrophic hamsters is also expressed in the thymus.
A freeze‐fracture study of normal and dystrophic C57BL mouse muscle
Authors: Dulhunty Angela , Aaist Angelo Valois 
Published: January 2005

AbstractFreeze‐fracture replicas of normal and dystrophic C57BL mouse muscle and kidney were examined to see whether there was a deficit in plasmalemmal particles which others suggest is a feature of dystrophies. When compared with normal membranes there was an increase in the particle density in dystrophic extensor digitorum longus muscle, a decrease in dystrophic soleus muscle, and no change in dystrophic kidney. Therefore there was not a general deficit in intramembrane particles in this dystrophic tissue. Indirect evidence supported the hypothesis that abnormalities in dystrophic mouse muscles are caused by abnormal motor input. The density of indentations, parallel to the T‐tubule, on the flat surface of the terminal cisternae can be modulated by the motor nerve. Changes were found in identation density in dystrophic muscle which were similar to changes seen after transection of the spinal cord in the mid‐thoracic region. There were parallel changes in contractile properties and identation density in dystrophic fibers.
Parenteral branched‐chain amino acid treatment and avian dystrophy
Authors: Hudecki Michael S. , Pollina Catherine M. , Heffner Reid R. 
Published: January 2005

AbstractGenetically homozygous line 413 dystrophic chickens were given twice‐daily intraperitoneal injections of solutions containing branched‐chain amino acids (BCAA‐leucine, valine, isoleucine) either alone or in combination; and their α‐ketoacid analogs (α‐ketoisocaproic and α‐ketoisovaleric acids). Another trial consisted of an amino acid mixture containing BCAA. Amino acid supplementation in each case significantly prolonged righting ability measured regularly by a standardized flip‐test procedure. Enhanced functional ability was not generally accompanied by a decrease in plasma creatine kinase activity. However, a measurable increase in the affected pectoralis major muscle mass and protein content (female chickens in particular) was found with BCAA therapy. Moreover, the increase in muscle bulk was attended in some cases by a reduction in the relative number of degenerating fibers quantitated microscopically. Contrariwise, the amino acid mixture caused a reduction in pectoralis muscle mass. It is concluded that parenteral BCAA therapy offers limited benefit in retarding dystrophic symptoms in the chicken.
Changes in muscle esterases in genetically dystrophic and control littermate mice
Authors: Bianchi Roberto , Biolscid Maurizio Paro , Ghidotti Giacomo , Mussini Emilio 
Published: January 2005

AbstractThe hypothesis that the existence of a family of isoenzymes is at the basis of the heterogeneity of esterase activity among skeletal muscles from dystrophic Re 129J and control littermate mice was tested using isoelectrofocusing (IEF) in polyacrylamide gel. All muscles considered showed not only quantitative heterogeneity, as previously observed in this laboratory, but also qualitative differences with regard to their esterase isoenzymes. Differences in the number and the relative amounts of isoenzymatic bands were found between different muscles from both control and dystrophic animals. In most dystrophic muscles a new isoenzymatic band appeared at pH 6.6. Another band, identified at pH 5.12, was more pronounced in dystrophic than in control muscles. The specificity of these observations is indirectly supported by the fact that other tissue, such as heart, liver, and kidney, did not show the quantitative or qualitative abnormality present in the dystrophic skeletal muscles.
Translocation of the temporalis muscle for treatment of facial paralysis
Authors: White Timothy P. , Faulkner John A. , Markley John M. , Maxwell Leo C. 
Published: January 2005

AbstractOur purpose was to characterize in the rhesus monkey the structure and function of vascularized temporalis muscle flaps innervated by the facial nerve after translocation into the site of the denervated zygomaticus muscle. Animals were killed at 28 to 120 days following translocation. Control data were obtained from the contralateral side. Twenty‐eight days after translocation, the time to reach peak twitch tension and one‐half relaxation time were 170% of control zygomaticus muscle. Contraction times decreased with time and reached control values by 100 days. Absolute isometric tetanic tension was not different between the flap (4.29 ± 1.28 newtons; X ± SEM) and control zygomaticus (3.95 ± 0.80 newtons). Succinate oxidase activity of the flap decreased from 279 ± 18 nl O2/mg protein/min to control zygomaticus values (98 ± 18) by 110 days. The type 1 fiber cross‐sectional area of the flap was 52% of control temporalis muscle and 150% of control zygomaticus muscle (P < 0.05). The temporalis flap demonstrated viable structure and function and appeared useful in facial movements.
Two major allelic forms of myosin light chain‐1 in strains of normal and dystrophic chickens
Authors: Rushbrook Julie Ivory , Yuan Anna I , Stracher Alfred 
Published: January 2005

AbstractEvidence is presented from electrophoresis and peptide‐mapping for the existence of two major allelic forms of myosin light chain‐1 in the fast white muscle fibers of domestic chickens. One form predominates in birds of White Leghorn stock, the other in birds of New Hampshire Red stock. The two light chain‐1 forms were invariant during development. Variability was not detected in light chains‐2 or ‐3. The distribution of the two forms in two strains homozygous for the am gene for muscular dystrophy—Connecticut dystrophic and line 413—and their controls, White Leghorn and line 412, respectively, while clearly unrelated to avian dystrophy, emphasizes the heterogeneity in background genes of these non‐inbred lines and indicates caution in their use in studies of avian dystrophy.
Neuromuscular reaction to paired stimuli
Authors: Reitter Bernd F. , Johannsen Swantje 
Published: January 2005

AbstractA modification of the twin pulse method is described, by which the nerve tested is stimulated at one proximal site percutaneously and the neuromuscular response to it is derived from a dependent muscle. An electronic subtraction procedure provides separation of the conditioned muscle action potential for measurement by means of a microcomputer. Several parameters were determined in the conditioned action potential and compared to the same criteria in an unconditioned single action potential. Changes of the conditioned action potential were measured at fixed stimulus intervals between 2 and 6 msec. Preliminary normal ranges interindividually and intraindividually were established. Their dependency on skin surface temperature was determined. In polyradiculoneuropathy, the data for motor nerve conduction velocity were characteristically pathologic, and neuromuscular reaction to paired stimuli data deviated minimally from normal. In vincristine neuropathy, motor nerve conduction velocity was normal, but neuromuscular reaction to paired stimuli data fell outside the normal range early in the clinical course. Since polyradiculoneuropathy is a primary demyelinating and remyelinating process and vincristine neuropathy is an example of axonal degeneration, neuromuscular reaction to paired stimuli can facilitate the differential diagnosis in polyneuropathy. Furthermore, this method seems apt to quantify axonal degeneration. In a mixed form of polyneuropathy, peroneal muscular atrophy, motor nerve conduction velocity, as well as neuromuscular reaction to paired stimuli, were abnormal, the latter resulting in abnormally high relative amplitudes. In muscle disorders such as Duchenne muscular dystrophy, neuromuscular reaction to paired stimuli data was abnormal, contrasting experiments using double stimulation of the diseased muscle itself.
Genuine myotubular myopathy
Authors: Edström Lars , Wróblewski Romuald , Mair Williamg. P. 
Published: January 2005

AbstractTwo patients, a father and his 14‐year‐old son, were suffering from a facioperoneal syndrome, and muscle biopsy findings were consistent with a myotubular myopathy. The father exhibited central nuclei in most muscle fibers, but his son had typical changes exclusively in hypotrophic type I fibers. The cytochemical and ultrastructural analysis revealed a spectrum of pathological changes typical of myotubular myopathy. Energy‐dispersive electron probe x‐ray microanalysis was performed on 6‐to 12‐μm thick freeze‐dried cryosections visualized in the scanning or scanning transmission mode of electron microscopy. We found a high intracellular sodium and chlorine concentration and a low potassium concentration in comparison with control muscles. These changes pointed in the direction similar to results from human fetal muscle. The changes in the intracellular elemental composition may indicate a membrane pump dysfunction, which might be caused by a partial arrest in muscle fiber maturation.
Sodium currents in human skeletal muscle fibers
Authors: Decoursey T. E. , Bryant S. H. , Lipicky R. J. 
Published: January 2005

AbstractSodium currents from human external intercostal muscle fibers were recorded using the Hille‐Campbell voltage clamp method. Sodium currents are analyzed in terms of the m and h parameters of the Hodgkin‐Huxley model. The results indicate that the kinetics and voltage dependence of sodium currents in human skeletal muscle fibers are very similar to those reported in rat fibers.
Bridging junctional processes in couplings of skeletal, cardiac, and smooth muscle
Authors: Forbes Michael S. , Sperelakis Nick 
Published: January 2005

AbstractJunctional processes are found in the junctional gap of couplings in skeletal, cardiac, and vascular smooth muscle cells. Visualization of the structure of junctional processes is greatly facilitated by the use of a tilting specimen stage and stereoscopic analysis. Relatively small changes of tilt angle create a spectrum of images for the same junctional process which can range from that of an amorphous electron‐opaque body to a distinct linear structure (“pillar”). In both striated and smooth muscles, pillars connect the sarcolemma with the limiting membrane of junctional sarcoplasmic reticulum (J‐SR). In addition, in vascular smooth muscle cells, pillars join the J‐SR with surface‐connected caveolae.
Tropomyosin and troponin C from human fetal, adult, and dystrophic skeletal muscle
Authors: Romero‐Herrera Alejo E. , Lieska Norman G. 
Published: January 2005

AbstractHuman skeletal muscle tropomyosin and troponin C were assessed for potential changes in quantity and subunit composition during development and in Becker muscular dystrophy. The proteins were purified or separated into subunits by ion‐exchange chromatography. The fetal and dystrophic sources yielded less of these proteins than the adult and also contained additonal types of tropomyosin chains. The findings may have implications for the disease process and emphasize the feasibility of direct preparative study of human muscle.
Heterogeneity of adult human striated muscle tropomyosin
Authors: Romero‐Herrera Alejo E. , Nasser Sam , Lieska Norman G. 
Published: January 2005

AbstractThe amino acid sequences of the human skeletal muscle tropomyosins were elucidated. Of the three α‐types identified, the major fraction (identical to the single myocardial species) differed by 23 and 25 amino acid residues from the two minor α‐forms and by 42 residues from β‐tropomyosin. The two β‐components differed only by deletion of the C‐terminal residue in the minor fraction. These sequences will facilitate analysis of tropomyosin from developmental and diseased states.
Prolongation of post‐tetanic facilitation in infant botulism
Authors: Fakadej Alexander V. , Gutmann Ludwing 
Published: January 2005

AbstractThe electrophysiologic studies in a case of infant botulism show a variety of abnormalities including tetanic and post‐tetanic facilitation (PTF) of the small single evoked muscle action potential. The enlarged amplitude of the single evoked muscle action potential following a 10‐second tetanic nerve stimulation persisted up to 21 minutes. The prolongation of PTF is a unique feature of botulism.
Nuclear inclusions in oculopharyngeal muscular dystrophy
Authors: Martin Jean‐Jacques R. , Ceuterick Chantal M. , Mercelis Rudolf J. 
Published: January 2005

AbstractThe ultrastructural study of a skeletal muscle biopsy in a case of familial oculopharyngeal muscular dystrophy confirms the presence of 7–8 nm wide tubular filaments within muscle fiber nuclei.
In vivo administration of a thiol protease inhibitor, E‐64‐C, to hereditary dystrophic chicken
Authors: Sugita Hideo , Kimura Masaaki , Tarumoto Yasuo , Tamai Masaharu , Hanada Kazunori , Ishiura Shoichi , Nonaka Ikuya , Ohzeki Masahiro , Imahori Kazutomo 
Published: January 2005

AbstractLine 413 hereditary muscular dystrophic chickens were given twice daily subcutaneous injections of 150 mg or 300 mg/kg body weight of E‐64‐c, a potent thiol protease inhibitor, from 10 days ex ovo to 89 days of age. The administration of high doses of E‐64‐c did not influence the growth curve, the course of the “flip number” the chronological changes of serum CK, PK, and LDH, and the number of the necrotic fibers and also the histogram of the fiber diameters. The activity of Ca2+‐activated neutral protease (CANP) in superficial pectoralis muscle was decreased by E‐64‐c in female chickens, but still remained higher than normal. In males, however, the CANP activity was not decreased at all. The male dystrophic chickens showed heavier body weights, lower flip number, and higher serum enzymes during the course of the experiment and higher CANP activities in superficial pectoralis muscles than females.
Study of neurotrophism in normal/dystrophic parabiotic mice
Authors: Saito Akitsugu , Law Peter K. , Fleischer Sidney 
Published: January 2005

AbstractThe trophic influences of nerve and muscle on one another were studied in normal and dystrophic littermates of C57BL/6J dy2J mice parabiosed at 20 to 23 days after birth. Each parabiont had a soleus muscle cross‐reinnervated by a tibial nerve of its partner. Ultrastructural abnormalities of muscle and endplate were quantified and compared 6 to 7 months postoperatively. The dystrophic nerve degenerated despite reinnervation to a normal muscle. The normal muscle did not prevent the dystrophic nerve from degenerating, and the dystrophic nerve induced degenerative changes in the reinnervated normal muscle. Normal nerve did not retard the genetically programmed degeneration of the dystrophic muscle. The dystrophic muscle, however, did not appear to cause normal nerve terminals to degenerate. We conclude that both nerve and muscle cells in dystrophic mice express characteristics of muscular dystrophy. Muscle fibers of a few motor units further suffer from abnormal neurotrophic influence because of the degeneration of the motor neurons. Myotrophic influence on nerve was not observed.
Serum creatine kinase isoenzymes: A review
Authors: Nanji Amin A. 
Published: January 2005

AbstractIn the last few years, several conditions giving rise to increases of the MB and BB isoenzymes of creatine kinase, and several electrophoretic variants of creatine kinase, and several electrophoretic variants of creatine kinase have been described. This review summarizes the nonmyocardial causes of increases of both CK‐MB and CK‐BB and also discusses the various electrophoretic variants.
Contraction time, histochemical type, and terminal cisternae volume of rat motor units
Authors: Kugelberg Eric , Thornell Lars‐Eric 
Published: January 2005

AbstractIsometric contraction time (Tc) of 19 fast and slow rat motor units in the soleus and the tibialis anterior muscles was measured. The motor unit fibers subsequently marked by glycogen depletion were histochemically fiber typed as well as analyzed ultrastructurally with respect to volume of the sarcoplasmic reticulum terminal cisternae. The volume density of terminal cisternae (VvTC) was inversely related to Tc over the whole range of motor units, irrespective of type of muscle. The continuous variations in VvTC are concluded to match the extrinsic control of time course of contraction, and fusion frequency of the motor unit to match the frequency characteristics of the individual motoneurons. The volume density dependence of terminal cisternae function would result in an indirect coupling between amount and rate of calcium release and rate of calcium recaptured. VvTC and Tc were the same for some tibialis anterior and soleus motor units in spite of the different types of myosin, indicating that the type 1 and type 2 myosins have specific structural differences in fast as compared with slow muscles.
Axonal transport in neuropathy
Authors: Jakobsen Johannes , Brimijoin Stephen , Sidenius Per 
Published: January 2005

AbstractRecent studies on the distribution of labeled endogenous proteins in the experimental neuropathies induced by streoptozotocin diabetes, galactose feeding, zinc pyridinethione, 2,5‐hexanedione, acrylamide, and p‐bromophenylacetylurea (BPAU) have demonstrated an impaired build up of retrogradely transported material derived from the more distal parts of peripheral nerve. In BPAU neuropathy the transport abnormality is strongly related to the degree of muscle weakness; following treatment with acrylamide the extent of the impairment is dose related. In both toxic conditions the transport abnormality is present well in advance of the first clinical signs of neuropathy. We therefore suggest that changes in retrograde axonal transport play an initial and important role in the development of many axonopathies.