bluuebunny/crossref_metadata_2025_split huggingface dataset:
There are no neurophysiologic features characteristic of “axonal” Guillain–Barré syndrome
Authors: Cros Didier , Triggs William J. 
Published: January 2005

AbstractClassical views hold Guillain–Barré syndrome (GBS) as a primary inflammatory‐demyelinating neuropathy in which secondary axonal degeneration may occur, particularly when inflammatory lesions are severe. Feasby and colleagues proposed that primary axonal degeneration can also cause GBS characterized by inexcitable motor nerves and poor outcome. This hypothesis rests largely on the results of a single autopsy in which no inflammation or demyelination were found. Using an illustrative case report confirming earlier studies, we point out that inexcitable motor nerves (or low amplitude compound muscle action potentials [CMAPs]) are of ambiguous significance and may reflect distal demyelination, causing conduction block between distal stimulation sites and target muscles, a pattern not uncommon in GBS. Recovery from such lesions may occur within weeks with restoration of CMAP amplitudes. The recognition of a yet unproven axonal variant of GBS cannot be based solely on documentation of inexcitable motor nerves in the context of rapidly developing weakness. © 1994 John Wiley & Sons, Inc.
Skeletal muscle involvement in tropical spastic paraparesis/HTLV‐1–associated myelopathy
Authors: Gabbai Alberto Alain , Wiley Clayton A. , Oliveira Acary S. B. , Smith Ricardo , Schmidt Beny , Nóbrega Joao A. M. , Bordin Jose O. , Román Gustavo C. 
Published: January 2005

AbstractThe frequency of muscle involvement in TSP/HAM is not known, nor is the precise role that HTLV‐1 and the diverse cytokines play in the genesis of HTLV‐1–associated diseases. In order to better define the frequency and characteristics of the skeletal muscle involvement in TSP/ HAM, we studied 11 affected patients. EMG was performed in 9 patients and muscle biopsy was performed in all 11. Muscle tissue was analyzed using: reverse transcriptase PCR for interleukin‐1 in 8; PCR for HTLV‐1 proviral DNA in 5; and electron microscopy for viral particles in 3. We found pathologic alterations in all 11 patients. Four patients (36%) had a neurogenic process, while a primary muscle involvement was observed in the rest (64%). Four patients (36%) had polymyositis, and 3 (27%) had a noninflammatory myopathy. Muscle weakness in the upper limbs was significantly associated with inflammation in the muscle biopsy. EMG was abnormal in only 2 of 9 patients. Reverse transcriptase PCR did not demonstrate message for interleukin‐1 in any sample examined. PCR did identify HTLV‐1 proviral DNA in the muscle of 3 patients. Retroviral‐like particles were found, by EM, in only one biopsy. HTLV‐1 may play an important role in the pathogenesis of the frequent myopathies associated with HAM/TSP. © 1994 John Wiley & Sons, Inc.
AAEM minimonograph #44: Diseases associated with motor unit activity
Authors: Auger Raymond G. 
Published: January 2005

AbstractStiff‐man syndrome is due to hyperexcitability of anterior horn cells, possibly related to interference with the synthesis or action of gammaaminobutyric acid. Unexpected acoustic and exteroceptive stimuli produce exaggerated muscle responses. Needle electrode examination of involved muscles yields nonspecific findings and demonstrates involuntary motor unit activity. The appearance and firing pattern of motor units are normal except that agonist and antagonist muscles may contract concurrently. Continuous muscle fiber activity (Isaacs' syndrome) comprises a heterogeneous group of hereditary and acquired disorders that cause hyperexcitability of peripheral nerves. Some are associated with electrophysiologic evidence of peripheral neuropathy and some are not. Repetitive afterdischarges often follow the M‐, H‐, and F‐waves. Needle electrode examination reveals an abnormal pattern of motor unit firing, consisting of myokymic discharges, doublets and multiplets, neuromyotonic discharges, and fasciculations. These abnormalities may occur alone or in combination.
Primary sensory neurons in x‐linked recessive bulbospinal neuronopathy: Histopathology and androgen receptor gene expression
Authors: Li Mei , Sobue Gen , Doyu Manabu , Mukai Eiichiro , Hashizume Yoshio , Mitsuma Terunori 
Published: January 2005

AbstractPathology of the primary sensory neurons was examined in 7 autopsied patients and 6 biopsied sural nerves from the patients with X‐linked recessive bulbospinal neuronopathy (SBMA). Large myelinated fibers in the central rami (L‐4 posterior root, L‐4, T‐7, and C‐6 segment of the fasciculus gracilis), and in the peripheral rami (sural nerve) were diminished in a distally accentuated manner, while small myelinated and unmyelinated fibers were well preserved in number. Demyelinating process and axonal atrophy was ubiquitous. The diameter frequency histograms of the dorsal root ganglion (DRG) neurons showed a decrease in the number of large diameter neurons and an increase in the number of small diameter neurons without substantial loss of whole number of neurons, which suggested that neuronal size was atrophied. These data suggested central and peripheral distal axonopathy with neuronal atrophy was the process of sensory neuron involvement. Expression of mutant androgen receptor mRNA with elongated CAG repeat in the DRG and sural nerve supported the view that sensory nerve involvement is the primary process in SBMA.© 1995 John Wiley &Sons, Inc.
Case of the month. Thoracic outlet syndrome—A functional disturbance of the thoracic upper aperture?
Authors: Lindgren Karl‐August , Manninen Hannu , Rytkönen Hannu 
Published: January 2005

AbstractWe describe a young woman with typical neurovascular symptoms of thoracic outlet syndrome (TOS). A three‐dimensional computerized scan showed a “dislocation” of the first rib at the costotransverse joint. The patient responded to a conservative approach to treatment. The patient presented here demonstrates the functional compromise of the upper thoracic aperture that is frequently seen in young women. © 1995 John Wiley &Sons, Inc. © 1995 John Wiley & Sons, Inc.
Differential electrophysiological features of neuropathies associated with 17p11.2 deletion and duplication
Authors: Uncini Antonio , Di Guglielmo Giovanni , Di Muzio Antonio , Gambi Domenico , Sabatelli Mario , Mignogna Teresa , Tonali Pietro , Marzella Rosalia , Finelli Palma , Archidiacono Nicoletta , Rocchi Mariano 
Published: January 2005

AbstractHereditary neuropathy with liability to pressure palsies (HNPP) and hereditary motor‐sensory neuropathy type IA (HMSN IA) are quite distinct clinical entities recently associated to deletion and duplication, respectively, of the 17p11.2 segment including the gene for peripheral myelin protein 22 (PMP‐22). We studied the electrophysiological features of 48 HNPP and 62 HMSN IA motor nerves. Conduction velocities (CV) and compound muscle action potential amplitudes were significantly reduced and distal latencies prolonged in HMSN IA compared to HNPP. CV was uniformly slowed in HMSN IA nerves whereas in HNPP it was focally slowed in 80% of ulnar and 12% of peroneal nerves at usual compression sites. Conduction block was present in 6% of HNPP nerves but in none of HMSN IA. In conclusion: (1) HMSN IA with 17p11.2 duplication presents marked, diffuse, and uniform slowing; (2) HNPP with 17p11.2 deletion presents focal electrophysiological abnormalities possibly correlated with the presence of tomaculae; and (3) under‐and overexpression of PMP‐22 in concurrence with environmental factors might be responsible for the distinctive features of HNPP and HMSN IA. © 1995 John Wiley & Sons, Inc.
AAEM minimonograph #45: The early development of electromyography
Authors: Bonner Francis J. , Devleschoward Allen B. 
Published: January 2005

AbstractThe use of electricity for therapeutic purposes began in the first century and became more refined as the properties of electricity became more understood. The works of Franklin, Galvani, Volta, and others contributed to this body of knowledge. Development of the string galvanometer, the advent of the vacuum tube, the introduction of concentric needle electrodes, and the development of the cathode‐ray oscilloscope occurred during the first half of the 20th century. The science of electromyography and electrodiagnosis grew in its sophistication, leading to the formation of the American Association of Electromyography and Electrodiagnosis (now the American Association of Electrodiagnostic Medicine) with James Golseth, MD, as its first president in 1953. © 1995 John Wiley & Sons, Inc.
Quantification of normal dystrophin mRNA following myoblast transplantation in mdx mice
Authors: Asselin Isabelle , Tremblay Martine , Vilquin Jean‐Thomas , Guérette Benoît , Roy R. , Tremblay Jacques P. 
Published: January 2005

AbstractA mutagenesis RT‐PCR method was used to detect normal dystrophin mRNA following the injection of normal myoblasts in mdx mice using two immunosuppressors. A specific sequence of the dystrophin mRNA (257 bp) was amplified by RT‐PCR from the muscle total RNA. Maell digestion of the amplified products allowed us to distinguish the normal messenger of dystrophin from the dystrophic one and to establish the percentage of normal and of dystrophic (mdx) dystrophin mRNA. Normal dystrophin mRNA was detected using this technique in mdx muscles transplanted with histocompatible normal myoblasts. For this type of transplantation, no significant difference in the percentage of normal dystrophin mRNA was observed between immunosuppressed mice and those not immunosuppressed. No normal dystrophin mRNA was, however, observed in mdx mice following histoincompatible normal myoblast transplantation without immunosuppression. When such transplantations were done in mice immunosuppressed with cyclosporine or FK‐506, normal dystrophin mRNA accounted for 31% and 36% of the total dystrophin mRNA, respectively. In fact, one animal immunosuppressed with FK‐506 expressed as much as 57% of normal dystrophin mRNA. These results thus show that FK‐506 makes it possible to restore dystrophin expression to a level comparable to that observed in DMD carriers that are usually asymptomatic. © 1995 John Wiley & Sons, Inc.
Multiple mitochondrial DNA deletions in a patient with mitochondrial myopathy and cardiomyopathy but no ophthalmoplegia
Authors: Takei Yo‐Ichi , Ikeda Shu‐Ichi , Yanagisawa Nobuo , Takahashi Wataru , Sekiguchi Morie , Hayashi Tetsuya 
Published: January 2005

AbstractDeletions of muscle mitochondrial DNA are known in mitochondrial myopathy patients who have chronic progressive external ophthalmoplegia (CPEO). A 41‐year‐old patient with no apparent family history of this condition suffers from hypertrophic cardiomyopathy, slight muscle atrophy, and weakness of the extremities, but not from CPEO. A muscle biopsy showed the presence of ragged‐red fibers, and Southern blot analysis disclosed multiple deletions of muscle mitochondrial DNA. This combination of clinical features in our patient is atypical in mitochondrial myopathy with demonstrable deleted muscle mitochondrial DNA. Pleomorphic clinical expression is suggested. © John Wiley & Sons, Inc.
Effects of temperature on motor unit action potentials during isometric contraction
Authors: Bertram Martin F. , Nishida Takashi , Minieka Michael M. , Janssen Imke , Levy Charles E. 
Published: January 2005

AbstractThe effect of temperature on motor unit action potential (MUAP) configuration and recruitment was studied using automatic decomposition electromyography (ADEMG) recordings from a concentric needle electrode placed in the first dorsal interosseous (FDI) muscle of 10 normal adult subjects during isometric contraction. Focally cooling the FDI resulted in prolonged MUAP duration (P < 0.001, ANOVA), a finding congruent with those of Buchthal. Focal ulnar cooling at the elbow resulted in the increased MUAP frequency. In contrast to previous studies, there were no significant differences in amplitude or turns. Greater understanding of normal motor unit electrophysiology is necessary to improve diagnostic accuracy of EMG testing © 1995 John Wiley & Sons, Inc.
Characteristics of the fast transport system in mammalian nerve fibers
Authors: Ochs S. , Ranish N. 
Published: January 2005

AbstractThe rate of the fast transport system in cat sensory nerve fibers shown by the distal displacement of a peak of isotopic activity in sciatic nerves after L7 ganglion injection with 3H‐leucine, is 401 ± 35 mm/day. A similar rate of fast transport was also found for motor fibers in a smaller number of studies. An intra‐axonic locus of the labelled material was determined by freeze‐block experiments.Fast transport was differentiated from the slow mechanism of axoplasmic flow by injecting 3H‐leucine into one L7 ganglion and 32P‐orthophosphate into the opposite one of the same animals, the labelled components of the latter moving down the nerve at the much slower rate characteristic of slow axoplasmic transport.The mechanism underlying fast transport is locally present all along the nerve fibers; the soma is not responsible for propulsion. This was shown by “pulse” experiments where ligations were made just distal to the injected ganglion at times from 1–3 hrs afterwards and the same rate of outward movement of labelled materials was found in these fibers without somal continuity.
Changes of axosomatic synapses in the visual cortex of monocularly deprived rats
Authors: Fifková E. 
Published: January 2005

AbstractThe effect of unilateral visual deprivation on axosomatic synapses of the visual cortex was studied. The deprivation was achieved by unilateral lid suture in 14 day old rats which were kept for 8 weeks. The size of axosomatic synapses in layers II to V of the visual cortex were measured. The mean size of the axosomatic synaptic contacts of the visual cortex supplied by the deprived eye was smaller (−23%) through all layers studied. In the axosomatic synaptic population contacts with flattened vesicles prevailed. The size of synapses containing round vesicles was more affected by deprivation than that of terminals containing flattened vesicles. The percentage of synapses with round vesicles was greater in the deprived cortex.
On types of interneurons responding to visual stimulation present in the optic nerve of the rock lobster, Panulirus interruptus
Authors: Wiersma C. A. G. , Yanagisawa K. 
Published: January 2005

AbstractThe classes of visually influenced interneurons in the optic peduncle of the rock lobster, Panulirus interruptus, were determined by single unit analysis. Each class has a restricted number of members. Eleven classes were distinguished, as follows. For light intensity discrimination: sustaining and dimming fibers. For recording movement of objects: fast, medium, jittery and light movement fibers. Seeing fibers perceive motions of both black and white objects and react with an afterdischarge when the object is stopped. Space constant fibers have the additional feature of change in location of their visual fields with body position, and each previous class has a member with this property. Unidirectional movement fibers and multimodal fibers form two further classes, of which especially the last is quite heterogeneous and divisible into numerous subclasses. It is discussed in how far the behavior of these interneuron classes may be refleced in the behavior of the animal.
The effect of insulin convulsions on the metabolism of RNA in the rabbit brain
Authors: Barbato Libero M. , Barbato Iris W. M. , Hamanaka Akihiko 
Published: January 2005

AbstractThe effect of insulin induced convulsions on the metabolism of RNA in brain of developing and adult rabbits has been studied. The incorporation of H3‐cytidine into the RNA of subcellular fractions did not show significant differences in the control and convulsed brains. The specific activity ratio CMP/UMP of nuclear and microsomal RNA was determined in terms of the incorporation of H3‐cytidine and C14‐orotic acid into the CMP and UMP moiety of RNA. This ratio was found to be unaltered by convulsions. The total RNA content of isolated Purkinje cells of developing and adult rabbits was not significantly different after convulsions lasting up to 3 hr. The significance of these experiments was discussed.
Relative potencies of L‐Glutamate analogs on excitatory neuromuscular synapses of the grasshopper, Romalea microptera
Authors: McDonald T. J. , O'Brien R. D. 
Published: January 2005

AbstractA series of 10 amino acid and 17 peptide analogs of L‐glutamate were tested for their ability to block the neurally‐evoked contractions of the retractor unguis muscle isolated from the grasshopper, Romalea microptera.The two most active amino acids other than L‐glutamate were L‐cysteine sulfinic acid and L‐cysteic acid. Like L‐glutamate, 3 × 10−3 M L‐cysteine sulfinic acid was able to block completely the indirect contractile response of the muscle and to elicit muscular contractions. During desensitization or reduction of the neurally‐elicited response, the excitatory postsynaptic potential (EPSP) and graded, electrically‐excited potential were almost completely abolished. Of the β‐substituted amino acids, the compounds with the sulfonyl or sulfinyl groups were more active than the compounds with the phosphonyl or phosphoryl groups. Projection drawings of molecular models of the β‐substituents indicated that the size of the β‐substituent was a critical factor for activity.L‐α‐Hydroxyglutaric acid (2 × 10−2M) induced erratic contractions which were considered the result of an action on the postsynaptic membrane and not limited to the subsynaptic areas.Of the 17 peptides tested at 10−2M, 14 reduced the neurally‐evoked contractions by 10% or more. L‐Glutamic acid decarboxylase (10−4 g/ml) and α‐bungarotoxin (2.5 × 10−5 g/ml) did not alter the neurallyevoked contractile response.
Sustaining fibers in the rock lobster
Authors: York B. 
Published: January 2005

AbstractThe properties of sustaining fibers (SuF's), whose firing frequency is related to the ambient light intensity, were studied in the rock lobster. Most of the firing patterns shown under various conditions were demonstrated to be physiological, by using chronic implantation techniques. Unusual activity at high light intensities suggests that the lobster visual system is equipped to function only under dim light conditions. Unlike the crayfish, the lobster SuF's do not always indicate light levels but only changes in light intensity. It is suggested that the input from these fibers has a large influence on locomotor activity.
The effect of different types of axonal trauma on the synthesis and transport of amine storage granules in rat sciatic nerves
Authors: Karlström Lars , Dahlström Annica 
Published: January 2005

AbstractIn crushed sciatic nerves of rat the accumulation of noradrenaline (NA), proximal to a ligation, proceeds approximately linearly up to 48 hr after operation, as was earlier reported. After 48 hr the NA in the Proximal portion ceases to increase. The reasons for this arrest in N.A‐accumulation have been studied in the present investigation. Rat sciatic nerves were cut, ligated, or crushed at a low level 1–23 days prior to sacrifice. Two hours before death, the nerves were crushed at a level of about 2 cm proximal to the initial operations.Using a histochemical fluorescence method, the relative accumulation of NA above the second high crush was studied. In previously cut or ligated nerves, the NA accumulation observed was very low or zero by 7–9 days, and remained low during the observation period. In previously crushed nerves the NA accumulation was also decreased to rather low levels by 7–9 days. At 15 days, however, the NA accumulation was larger again, and histochemically normal accumulation was observed 17–23 days after the initial crush operation.The results may indicate that axonal trauma causes a depression in the synthesis and transport of amine stronge granules in the adrenergic neurons. Furthermore, the degree and duration of depression seemed to vary with the type of trauma performed. Crushing the nerves, which leaves the connective tissues sheaths uninterrupted, facilitating thus regeneration, appeared to cause a less marked and shorter‐lasting depression than cutting or ligating the nerves.
Chemosensitivity of mouse neuroblastoma cells in vitro
Authors: Peacock John H. , Nelson Phillip G. 
Published: January 2005

AbstractMouse neuroblastoma cells have been tested for their responsiveness to iontophoretically applied acetylcholine, norepinephrine, dopamine, and serotonin using intracellularly recorded membrane potential and resis tance as indicators of response. Acetylcholine can elicit either a membrane depolarization (D‐response) or hyperpolarization (H‐response) or a compound D‐H response from different neuroblastoma cells of a given culture. Some 60 to 70% of tested cells gave no measurable response. A change of culture medium (from DMEM to F‐12) increased the incidence of H‐responses 2‐fold. Dopamine elicited H‐responses in about one‐quarter of cells tested, while no responses were elicited by norepinephrine or serotonin in a small sample of cells.
Effects of long‐term organ culture on neurotransmitter metabolism in the ganglia of Aplysia californica
Authors: Dewhurst Susan A. , Weinreich Daniel 
Published: January 2005

AbstractThe abdominal and pedal‐pleural ganglia of Aplysia californica were maintained in organ culture for periods up to 45 days. Neurophysiological examination of abdominal ganglia indicated that synaptic activity could be evoked in ganglia cultured for 21 days and action potentials and spontaneous postsynaptic potentials persisted for 45 days. The effects of long‐term culture on neurotransmitter metabolism were studied by measuring the activities of ChAc, AChE, AAD, and COMT in both cultured abdominal and pedal‐pleural ganglia. G‐6‐PD and CarnAc activities were also measured. In general, the stabilities of the enzymes in abdominal ganglia to in vitro conditions were similar to those in pedal‐pleural ganglia. None of the enzymes studied were markedly affected by culture conditions for at least fourteen days. After this time, enzymes involved in neurotransmitter synthesis, AAD and ChAc, exhibited fairly abrupt decreases in activity. The degradative enzymes studied, AChE and COMT, showed more gradual decreases in activity. The changes occurring in the neurotransmitter metabolizing enzymes after prolonged periods of time in culture are discussed in relation to the neurophysiological changes which were observed.