bluuebunny/crossref_metadata_2025_split huggingface dataset:
A quantitative comparison of satellite cell ultrastructure in duchenne muscular dystrophy, polymyositis, and normal controls
Authors: Watkins Simon C. , Cullen Michael J. 
Published: January 2005

AbstractFailure of the muscle to regenerate successfully is an important feature of the pathology of Duchenne muscular dystrophy (DMD). It is known that this is not due to a numerical reduction in the population of satellite cells. We have therefore examined the ultrastructural composition of the satellite cells in 25 DMD cases and compared them with satellite cells from normal subjects and from polymyositis patients to try to identify morphological features that might be associated with an impaired developmental competence. Profiles of randomly and serially sectioned satellite cells were analyzed stereolgically to obtain nuclear and cytoplasmic areas. Within the cytoplasm, the areas occupied by mitochondria, rough endoplasmic reticulum (RER), and Golgi apparatus were measured. Micropinocytotic vesicles (MPVs) at the perïphery of the cells were counted. The nucleus to cytoplasm ratios and mitochondrial, RER, and Golgi volume fractions were not significantly different in the three satellite cell samples. The cells in the DMD sample contained more MPVs than those in the normal subjects. This is attributed to a generalized cell response to a physiologically altered environment in the diseased muscle.
Practical analysis of variability of muscle function measurements in duchenne muscular dystrophy
Authors: Edwards R. H. T. , Chapman S. J. , Newham D. J. , Jones D. A. BSc, PhD 
Published: January 2005

AbstractTo determine the possible sources of variation in performance indicators used in therapeutic trials, electrical stimulation techniques were used to measure contractile properties of the adductor pollicis and quadriceps muscles in boys with Duchenne muscular dystrophy. As no therapeutic effects were observed, longitudinal data obtained are taken to indicate changes in disease progress. Variance in voluntary contractions was found to be similar to that with electrically stimulated contractions; thus, variation could not be attributed to motivational changes, but rather to physiologic changes. Dystrophic muscle was slower to relax and less fatiguable than normal. However, such changes are of less significance to the overall disability compared to the loss of muscle bulk (cross‐sectional area). Important variations in the function of individual muscles essential to complex performance, such as walking or getting up from the floor, could be masked by combining results from several muscle groups.
Calcium‐activated protease activity in tenotomized muscle
Authors: Baker James H. , Margolis Ronald N. 
Published: January 2005

AbstractThe purpose of this study was to investigate the possible role of calcium‐activated neutral protease in the disorganization and dissolution of the myofibrils of the rat soleus that occurs following tenotomy. Rats were killed 3, 5, 7, 14, 21, and 42 days after tenotomy of the soleus, and the muscles were removed and assayed for calcium‐activated protease activity. Maximal protease activity occurred 1 week after tenotomy, at the time when myofibril organization is completely disrupted. Activity was still high 2 and 3 weeks after the operation, but returned to normal levels by 6 weeks, when muscle histology had returned to normal. The time course of the calcium‐activated protease activity corresponded closely to the time course of the morphological changes. Thus, calcium‐activated neutral protease may play a major role in myofibrillar proteolysis following tenotomy and in making the myofibril susceptible to proteolytic attack by other, less specific proteases.
Prevention of recurrent exertional rhabdomyolysis by dantrolene sodium
Authors: Haverkort‐Poels Petra J. E. , Joosten ED M. G. MD, PhD , Ruitenbeek Wim 
Published: January 2005

AbstractA patient with recurrent attacks of exertional rhabdomyolysis and easy fatiguability was treated with dantrolene sodium. She experienced a complete prevention of myoglobinuric attacks and gained a better exercise tolerance. Biochemical findings in this patient suggest a loosely coupled oxidative phosphorylation in mitochondria. The question is raised as to whether dantrolene sodium exerts an effect on the mitochondrial calcium uptake and influences the state of coupling of the mitochondria.
Morphometric studies of sural nerve in childhood
Authors: Ouvrier Robert A. , McLeod J. G. , Conchin Therese 
Published: January 2005

AbstractSural nerve myelinated fiber density and myelinated fiber diameter distribution have been examined in 27 control subjects, ranging in age from 1 day to 59 years. Total transverse fascicular area was measured in 10 of the subjects. There is an exponential decline in myelinated fiber density from birth to adult life. The predicted normal density (D) at any age may be derived from the formula D = (1 × 103)/(0.0699 + 0.00725 √t). The distribution of myelinated fiber diameters is unimodal in the first 4 months of life, and there is a denfinite bimodal distribution by 2 years of age. Total transverse fascicular area of sural nerve increases progressively from values of about 0.25 mm2 in the first week to about 0.82 mm2 at 9 years. Control values for sural nerve morphometry in childhood are essential for accurate interpretation of biopsies in patients with peripheral neuropathy.
Localization of myoglobin in human muscle cells by immunoelectron microscopy
Authors: Kawai Hisaomi , Nishino Hiroshi , Nishida Yoshihiko , Masuda Kenjiro , Saito Shiro 
Published: January 2005

AbstractThe localization of myoglobin in human skeletal muscle cell was studied by immunoelectron microscopy. The Fab'‐horseradish peroxidase (HRP) conjugate was prepared by the maleimide method recently developed by Ishikawa et al. This method gave better recovery and less polymerization of HRP than the periodate method. By the direct immunoperoxidase method using this conjugate, myoglobin was shown to be localized in the I‐band region of skeletal muscle cells. The outer membrane of mitochondria and triads stained intensely, but A‐bands were not stained. Myonuclei and intermyofibrillar spaces were also not stained. The localization of myoglobin in the I‐band implies its function in energy generation.
Relative refractory period: A measure to detect early neuropathy in alcoholics
Authors: Alderson M. Kathryn , Petajan Jack H. 
Published: January 2005

AbstractThe absolute (ARP) and relative refractory period (RRP) of the median sensory nerve was determined in 26 control subjects and 24 alcoholics, nine of whom had symptoms of peripheral neuropathy. Recovery of latency to normal in response to the second stimulus was used to define RRP. A true RRP was calculated by subtracting ARP from measured RRP. Mean ARP for all subjects ranged from 0.75 to 0.80 msec; normal = 0.8 ± 0.2 msec. The true RRP of control subjects was 2.1 ± 0.5 msec, and for all alcoholic subjects it was 3.1 ± 0.5 msec. True RRP for the nine symptomatic alcoholic subjects was 3.6 ± 0.5 msec and 2.9 ± 0.4 msec for those who were asymptomatic. Symptomatic and asymptomatic alcoholics differed significantly from one another, as well as from control subjects (P < 0.001). Routine nerve conduction studies were normal in asymptomatic subjects. Three out of nine symptomatic alcoholics had increases in distal median motor or sensory latency, and three had slight slowing of median nerve conduction velocity. True RRP is more sensitive than routine measures of nerve conduction in the detection of axonal disorders influencing nerve conduction.
T‐Lymphocyte subsets, functional deficits, and morphology in sciatic nerves during experimental allergic neuritis
Authors: Strigård Karin , Olsson Tomas , Brismar Tom , Kristensson Krister , Klareskog Lars 
Published: January 2005

AbstractConduction velocities, demyelination, “macrophage/dendritic” cells, different sets of T‐lymphocytes, and immunoglobulins were estimated in sciatic nerves during various phases of experimental allergic neuritis in Lewis rats. Demyelination was minimal day 15 postimmunization (p.i) when conduction velocity already was reduced, somewhat more pronounced day 17 p.i. when nerve conduction was blocked, and most pronounced day 23 p.i. when nerve conduction partially had recovered. This suggests a dissociation between the degree of demyelination and the functional deficits. Decrease of sciatic nerve conduction velocities coincided with endoneurial appearance of T‐lymphocytes and “macrophage/dendritic” cells, as well as endoneurial immunoglobulins, day 15 p.i. Later partial functional recovery occurred in parallel with the disappearance of T‐cells. The degree of functional deficits thus correlated with the number of endoneurial T‐lymphocytes. T‐cells may, directly or indirectly, initiate several of the disease components in experimental allergic neuritis, including the nerve conduction deficit.
Benign focal amyotrophy: Metrizamide ct evidence of cord atrophy. Case report
Authors: Metcalf James C. , Wood James B. , Bertorini Tulio E. 
Published: January 2005

AbstractBenign focal amyotrophy is an uncommon variant of neuron disease characterized by the gradual development of atrophy usually involving one upper extremity with a subsequent nonprogressive course. Previous descriptive reports have included normal radiological studies. We present a case of benign focal amyotrophy with a metrizamide CT myelographic finding of segmental cord atrophy in a distribution consistent with the patient's clinical findings.
Comparison of magnetic resonance imaging with somatosensory testing in ms suspects
Authors: Eisen Andrew , Odusote Kayode , Li David , Robertson William , Purvis Sherry , Eisen Kathleen , Paty Donald 
Published: January 2005

AbstractTwo hundred patients suspected of having multiple sclerosis (MS), including 42 with progressive myelopathy and 11 with optic neuritis, were investigated with somatosensory evoked potentials (SEPs) and magnetic resonance imaging (MRI). Most had minimal neurological deficit, the mean Kurtzke scale being 2.65. There were 117 patients had two or more MRI lesions suggestive of MS, with a total of 527 lesions identified; 290 (55%) involved the somatosensory pathways, most commonly lying in the mid‐periventricular region (thalamo‐cortical radiations). There was good correlation between positive and negative MRls and SEPs. The MRI was abnormal more frequently than the SEP, except in progressive myelopathy in when both were abnormal with equal frequency. It is proposed that some cases of myelopathy in MS may be due to periventricular rather than spinal pathology. The morphology of the MRI lesion would favor conduction block not slowing of the SEP as being the prime abnormality. This appeared to be true of leg SEPs.
Diagonal z‐lines: An unexplained electron microscopic finding
Authors: Isaacs Hyam , Whistler Toni 
Published: January 2005

AbstractA patient suffering with myasthenia gravis was subjected to muscle biopsy of the deltoid and subsequently of the pectoralis muscle. Electron microscopic examination of the muscles revealed the scattered presence of Zlines that were curiously orientated diagonally across the I band. This appears to be a coincidental finding, the significance of which is unknown and unreported at this time.
Hemifacial spasm and facial myokymia: Electrophysiological findings
Authors: Horowitz Steven H. 
Published: January 2005

AbstractIn two patients, one with clinical hemifacial spasm (HFS) and one with clinical facial myokymia, the spontaneous electromyographic findings consisted of regular burst activity: each burst composed of high frequency repetitive motor unit potentials. These bursts fired synchronously in multiple ipsilateral facial muscles in both patients and from a contralateral facial muscle in the patient with HFS. In addition, the patient with HFS exhibited crossed evoked responses on the affected side with stimulation of the facial and supraorbital nerves on the unaffected side. It is concluded that the current electrophysiological distinctions between HFS and facial myokymia may be unsupportable and that facial nuclear hyperactivity with activation of bilateral facial nuclear connections plays a role in the pathophysiology of HFS.
Friedreich's ataxia: Electrophysiologic and histologic findings in patients and relatives
Authors: Caruso G. , Santoro L. , Perretti A. , Massini R. , Pelosi L. , Crisci C. , Ragno M. , Campanella G. , Filla A. 
Published: January 2005

AbstractPeripheral nerve conduction velocity and cortical evoked potentials were investigated in 48 patients with Friedreich's disease and in 35 relatives. There were 14 patients and 2 relatives who underwent sural nerve biopsy.In the patients sensory conduction velocity was moderately slowed, whereas sensory responses were markedly reduced. Nerve biopsy showed a severe loss of large myelinated fibers and no demyelination. On teased nerve fiber preparations, most fibers presented uniformly short internodes. No correlation was seen between sensory conduction findings or histologic abnormalities and clinical disability.In patients SSEP changes, which were constant, and VEPs, which were frequently involved, were unrelated to the severity or duration of clinical disability.The were 14 relatives who showed clinical signs of Friedreich's disease. Slightly decreased distal conduction velocity along sensory fibers was observed in more than half of the relatives. Nerve biopsy was noncontributory. In conclusion, we could not determine whether the abnormalities observed in the siblings were an expression of a heterozygotic condition, or whether they were early signs of the disease.
Hypogravity‐induced atrophy of rat soleus and extensor digitorum longus muscles
Authors: Riley Danny A. , Ellis Stanley , Slocom Glenn R. , Satyanarayana Tanjore , Bain James L. W. , Sedlak Frank R. 
Published: January 2005

AbstractProlonged exposure of humans to hypogravity causes weakening of their skeletal muscles. This problem was studied in rats exposed to hypogravity for 7 days aboard Spacelab 3. Hindlimb muscles were harvested 12–16 hours postflight for histochemical, biochemical, and ultrastructural analyses. The majority of the soleus and extensor digitorum longus fibers exhibited simple cell shrinkage. However, approximately 1% of the fibers in flight soleus muscles appeared necrotic. Flight muscle fibers showed increased glycogen, lower subsarcolemmal staining for mitochondrial enzymes, and fewer subsarcolemmal mitochondria. During atrophy, myofibrils were eroded by multiple focal losses of myofilaments; lysosomal autophagy was not evident. Tripeptidylaminopeptidase and calcium‐activated protease activities of flight soleus fibers were significantly increased, implying a role in myofibril breakdown. Simple fiber atrophy appears to account for muscle weakening during spaceflight, but fiber necrosis is also a contributing factor.
Characterization of a prostaglandin dysfunction myopathy
Authors: McLennan Ian S. 
Published: January 2005

AbstractInhibition of prostaglandin synthesis in chicken embryos generated a myopathy characterized by ringbinden, loss of Z disks and thick and thin filaments from the myofibrillar apparatus. During the early stages of myogenesis there was also abnormal clustering and contacts between the developing myotubes. This disruption of myofibrillar apparatus occurred in both primary and secondary myotubes. There was also a slight increase in the proportion of type 1 fibers, which resulted from a selective absence of type 2 fibers. The number of motoneurons in the lumbar spinal cord was normal. This, along with the nature of the disruption of the myofibrillar apparatus, was taken as an indication that the generation of this myopathy was not secondary to abnormal motoneuron development.
Effects of fatiguing exercise on high‐energy phosphates, force, and EMG: Evidence for three phases of recovery
Authors: Miller Robert G. , Giannini Don , Milner‐Brown H. S. , Layzer Robert B. , Koretsky Alan P. , Hooper Dana , Weiner Michael W. 
Published: January 2005

AbstractExperiments were designed to evaluate the relative contribution of impulse propagation failure, high‐energy phosphate depletion, lowered pH, and impaired excitation‐contraction coupling to human muscle fatigue and recovery. 31P nuclear magnetic resonance spectroscopy measurements were made on adductor pollicis muscle, together with simultaneous measurements of M‐wave, force, and rectified integrated EMG (RIEMG). During fatigue, maximum voluntary contraction force (MVC) fell by 90%, pH fell from 7.1 to 6.4, and phosphocreatine was almost totally depleted. Neuromuscular efficiency (NME = force/RIEMG) was reduced to 40% of control at the end of the fatiguing contraction, and the M wave was reduced in amplitude and prolonged in duration. Following exercise, the M wave returned to normal within 4 minutes. pH, high‐energy phosphates, and MVC recovered within 20 minutes. By contrast, neuromuscular efficiency did not recover within 60 minutes. These findings indicate three different components of fatigue. The first is reflected by the altered M wave and indicates impaired muscle membrane excitation and impulse propagation. The second, associated with reduced MVC, correlates with the metabolic state of the muscle (PCr and pH). The third, indicated by reduced NME, is independent of changes in high‐energy phosphates and pH and is probably due to impaired excitation‐contraction coupling.
Trial of ganglioside therapy for diabetic neuropathy
Authors: Hallet Mark , Flood Thomas , Slater Norma , Dambrosia James 
Published: January 2005

AbstractThe efficacy of daily intramuscular injection of mixed gangliosides (Cronassial) for highly symptomatic diabetic neuropathy was investigated in a randomized double‐blind trial. There were 37 patients treated with 40 mg of gangliosides or placebo for 12 weeks and studied with physical examination, symptom scales, and electrophysiologic parameters. There was no demonstrated benefit of therapy. Given the spontaneous variation in the measurements employed, larger numbers of patients would have been needed to identify a significant change of 10% owing to therapy.
Thoracic outlet syndrome surgery causing severe brachial plexopathy
Authors: Wilbourn Asa J. 
Published: January 2005

AbstractHundreds of first rib resections are performed yearly in the United States to treat a controversial type of neurogenic thoracic outlet syndrome (NTOS). This surgery was thought to be devoid of serious neurological complications until 1982. However, that year Dale unearthed the rather astounding fact that the members of a single surgical society were aware of nearly 300 brachial plexus injuries resulting in motor deficits that had occurred during such operations, although almost none had been reported in the literature. In this report the features of the two main types of NTOS (“true” and “disputed”) are discussed. The history of brachial plexus injury occurring during TOS surgery is traced back to the late nineteenth century. The clinical and electrophysiological features of eight patients who sustained such injuries are described in detail, the literature on this topic is reviewed, and the location and causes for these injuries are discussed.
Axilla to elbow radial nerve conduction
Authors: Kalantri Ananthlal , Visser Bryan D. , Dumitru Daniel , Grant Arthur E. 
Published: January 2005

AbstractNumerous techniques that evaluate radial nerve conduction from the axilla or supraclavicular fossa to the elbow have been reported. A shortcoming of most protocols is determining the precise radial nerve length as it proceeds along the spiral groove. The present study dissected out and measured directly eight cadaver radial nerves from the axilla to the elbow. These values were compared with a new surface tape measurement technique from axilla to elbow across the bicep muscle, obstetrical calipers over this region, and a surface determination approximating the course of the radial nerve posteriorly in the spiral groove. The anterior surface tape‐measuring procedure compared most favorably with the actual anatomic length. Nerve conduction velocities were then calculated in 20 volunteers using all 3 techniques and compared with the median nerve in the arm. The anterior and posterior tape measurements yielded a conduction velocity of 72.5 ± 4.7 and 86.6 ± 7.0 m/s, respectively, whereas the caliper resulted in 65.7 ± 3.9 m/s. We conclude that proximal radial nerve length assessment employing an anterior tape measurement from axilla to elbow across the bicep musculature is precise and compares favorably with the actual anatomic length of the radial nerve.
Stretch‐induced spinal accessory nerve palsy
Authors: Logigian Eric L. , McInnes Janice M. , Berger Alan R. , Busis Neil A. , Lehrich James R. , Shahani Bhagwan T. 
Published: January 2005

AbstractLeft spinal accessory nerve palsy occurred in a young man when he quickly turned his head to the right while his shoulders were pulled down by heavy hand‐held objects. Electrophysiologic studies demonstrated partial axonotmesis of the spinal accessory nerve branches innervating the sternocleidomastoid and upper and middle trapezius and complete axonotmesis of spinal accessory branches to the lower trapezius. There was a separate, although functionally minor, cervical plexus innervation of the lower trapezius.
Morphometry of intramuscular nerves in amyotrophic lateral sclerosis
Authors: Rosales Raymond L. , Osame Mitsuhiro , Madriaga Evelyn P. , Navarro Jose C. , Igata Akihiro 
Published: January 2005

AbstractMorphometric analysis of 90 intramuscular nerves from the biopsied biceps muscles of 16 cases of amyotrophic lateral sclerosis (ALS) were done and compared with controls. In all fascicles (large and small) of ALS, the total number and the numbers of large and small myelinated fibers were significantly reduced to 52, 16, and 64% of controls, respectively. A histogram of the largest fascicles revealed a unimodal distribution and a shift to the left, showing a single peak at 1 μm diameter. The results indicate proximal and distal involvement of the most remote portions of the nerves to the muscle in ALS and support previous suggestions of a neuronopathy.
Myoadenylate deaminase deficiency: A clinical, genetic, and biochemical study in nine families
Authors: Sinkeler Sietze P. T. , Joosten M. G. , Wevers Ron A. , Oei T. Lian , Jacobs Alga E. M. , Veerkamp Jacques H. , Hamel Ben C. J. 
Published: January 2005

AbstractThe clinical significance of myoadenylate deaminase (MAD) deficiency and its mode of inheritance is still questioned. There were 36 relatives of 9 unrelated MAD deficient patients who were examined with the aid of a standardized ischemic forearm test: 8 new cases of MAD deficiency were detected, 5 of which were confirmed histochemically and biochemically. Obligate heterozygotes showed a normal ammonia production and MAD staining, but the mean activity of the enzyme was significantly less than in a group of controls. The results obtained from the family study strongly suggest an autosomal recessive mode of inheritance. However, only 2 of the 8 newly found MAD deficient individuals complained of exertional myalgia, whereas the remaining 6 were without any symptoms or complaints. The finding casts doubt on the clinical significance of MAD deficiency and the relationship of the deficiency state with exertional myalgia.