bluuebunny/crossref_metadata_2025_split huggingface dataset:
Parkinsonism in alcohol withdrawal: A follow‐up study
Authors: Shandling M. , Carlen P. L. , Lang A. E. 
Published: January 2005

AbstractTransient parkinsonism associated with alcohol intake and withdrawal has previously been described. We followed‐up three patients with acute alcohol withdrawal‐induced parkinsonism 9–11 years after their initial presentation. None showed any evidence of parkinsonism at follow‐up. This suggests that withdrawal‐induced parkinsonism is caused by a completely reversible abnormality in nigrostriatal dopamine transmission, which is unaccompanied by underlying nigral degeneration, as we had previously hypothesized.
Diurnal variability in cranial dystonia
Authors: Montagna Pasquale , Procaccianti Gaetano , Lugaresi Alessandra , Zucconi Marco , Lugaresi Elio 
Published: January 2005

AbstractEleven patients with cranial dystonia were investigated for diurnal variations in disability by means of video recordings. Disability increased significantly from morning to evening. The increase was not related to changes in vigilance levels assessed by dynamic electroencephalogram. Cranial movement disorders display diurnal fluctuations that are probably related to endogenous circadian rhythms.
Positron emission tomography demonstrates dopamine D2 receptor supersensitivity in the striatum of patients with early Parkinson's disease
Authors: Rinne U. K. , Laihinen A. , Rinne J. O. , Naågren K. , Bergman J. , Ruotsalainen U. 
Published: January 2005

AbstractStriatal dopamine D2 receptor binding was studied in vivo with positron emission tomography in seven patients with early Parkinson's disease using [11C]–raclopride. The patients had unilateral symptoms and none of them had received levodopa treatment. The accumulation of [11C]–raclopride in the striatum was rapid and reached a steady state at approximately 40 min after injection. The binding of [11C]–raclopride was measured in the striatum and cerebellum: The total striatal radioactivity in both hemispheres was counted and the respective striatum/cerebellum ratios were calculated. The striatum/cerebellum ratio of [11C]–raclopride binding was significantly (p < 0.01) increased in the hemisphere contralateral to the parkinsonian symptoms as compared with the opposite hemisphere. Thus, this study demonstrates that there is denervation supersensitivity in dopamine D2 receptor binding in early Parkinson's disease.
Tremor and idiopathic dystonia
Authors: Jedynak C. P. , Bonnet A. M. , Agid Y. 
Published: January 2005

AbstractThe clinical and electromyographic characteristics of tremor were studied in 45 patients presenting with various forms of idiopathic dystonia. Dystonic tremor was shown to be postural, localized, and irregular in amplitude and periodicity, absent during muscle relaxation, exacerbated by smooth muscle contraction, and associated frequently with myoclonus. Although it resembles essential tremor, dystonic tremor seems to be a distinct entity: it is more irregular witha broader range of frequecies; it is asymmetric and remains localized; myoclonus is sometimes associated. This type of tremor is most often seen in the presence of dystonia, but may be observed without evident dystonic symptoms.
Parkinsonism and extraocular motor abnormalities with unusual neuropathological findings
Authors: Calabrese Vincent P. , Hadfield M. Gary 
Published: January 2005

AbstractParkinsonian patients with ocular motility abnormalities are usually considered to have progressive supranuclear palsy. However, a number of other conditions have been noted to have the combination of parkinsonism and ocular problems. We report a case of rigid akinetic parkinsonism, oculomotor palsy, and eyelid apraxia with postmortem examination. Our findings are unusual in that there was marked gliosis of the substantia nigra with a large amount of free extracellular neuromelanin despite a 3‐year clinical course. Only rare hyaline inclusion bodies and no neurofibrillary tangles were seen in the brainstem. Excessive calcification of the vessels of the globus pallidus were also noted. This case represents another example of the diversity of conditions producing parkinsonism with extraocular motor abnormalities.
Treatment outcomes in cervical dystonia: A clinimetric study
Authors: Lindeboom R. , de Haan R. J. , Aramideh M. , Brans J. W. M. , Speelman J. D. 
Published: January 2005

AbstractWe reviewed 21 studies (8 blinded and 13 open) on the treatment of botulinum toxin type A for cervical dystonia, directed to the health aspects used to evaluate the patients' response to treatment (Medline search 1985–1993, English language literature). The prerequisite for comparing the treatment results was that studies had to evaluate similar aspects of disease. The ICIDH model, outlined by the World Health Organization in 1980, orders the different health outcomes into distinct classes of disease consequences. Our aim was to order the health outcomes according to the model and, thus, to study the comparability of treatment outcomes. Three differences could be identified between the objective and the subjective instruments. (a) The aspects measured by the subjective instruments varied substantially; of the 22 different subjective instruments identified in 18 studies, 8 measured impairments, 5 disability, and 9 could not be classified according to the ICIDH model. The objective instruments measured impairments. (b) All objective instruments were multiitem, whereas only 2 of 22 subjective instruments could be identified as multiitem. (c) The subjective instruments were generally poorly documented with regard to the number of items, score range, or grading. We conclude that the treatment outcomes can only be compared on the objective level of assessment and with regard to the patients with painful dystonia. The subjective instruments, particularly those focusing on disease‐specific disability, deserve further research. The ICIDH model offers a useful framework for selection, improvement, and development of outcome instruments. Because the model clearly demarcates the different consequences of disease, adoption will enhance the comparability of outcomes in cervical dystonia intervention trials.
Brainstem myoclonus in a patient with non‐dopa‐responsive parkinsonism
Authors: Clouston P. D. , Lim C. L. , Fung V. , Yiannikas C. , Morris J. G. L. 
Published: January 2005

AbstractIn the few reports where electrophysiologic techniques have been used to characterise stimulus sensitive myoclonus in the setting of a parkinsonian syndrome, the origin of the myoclonus has usually been found to be cortical. We describe a patient with parkinsonism unresponsive to levodopa who had myoclonus that was both spontaneous and induced by somatosensory stimuli. In addition, autonomic symptoms and a marked sleep disturbance were present early in his illness. Results of electrophysiologic investigations including electromyography (EMG) studies, routine electroencephalography (EEG) recording, jerked locked back‐averaging of EEG, and somatosensory evoked potentials were consistent with a brainstem origin for the myoclonic jerks. Following ipsilateral digital and supraorbital electrical stimulation, the earliest muscle activation occurred in the trapezius. An all night sleep study showed frequent myoclonic jerks during sleep and markedly abnormal sleep architecture. We believe that this patient's myoclonus was related to pathologic changes in brainstem reticular nuclei that occurred as part of his disease process. To our knowledge, brainstem myoclonus has not been described as a feature of parkinsonian syndromes.
Subacute skeletal myopathy induced by 2,4‐dichlorophenoxyacetate in rats and guinea pigs
Authors: Danon Jak M. , Karpati George , Carpenter Stirling 
Published: January 2005

AbstractRats and guinea pigs were injected with 2,4‐dichlorophenoxyacetate (2,4‐D) in amounts sufficient to keep them myotonic for over 12 hr daily. The biceps brachii muscles were studied by histochemistry, epoxy resin histology, and electron microscopy after one to five days. Changes observed include proliferation of the longitudinal component of the sarcoplasmic reticulum, production of new myofilaments on the periphery of cells and in nuclei, activation of satellite cells, accumulation of lipid droplets, myofibrillar damage with Z‐disc streaming, necrosis, and regeneration. The action mechanisms of 2,4‐D are not well understood. Some of the phenomena observed could be classified as proliferative and may be analogous to the auxin action of 2,4‐D in plants. The production of intranuclear filaments is probably related to the diffusion of newly formed cytoplasmic proteins into nuclei.
Sensory (1a) and F‐wave conduction velocity in the proximal segment of the tibial nerve
Authors: Panayiotopoulos Chrysostomos P. , Scarpalezos Spiros , Nastas P. E. 
Published: January 2005

AbstractThe F‐wave was used to estimate the fastest motor nerve conduction velocity (MNCV) along the proximal segment (spinal cord to knee) of the axons of the tibial nerve in 65 control subjects, 8 patients with diabetic polyneuropathy, and 4 patients with Charcot‐Marie‐Tooth disease. Terminal latencies and MNCV along the distal segment (knee to ankle) of the nerve were also estimated with the conventional M‐response technique. This article describes another method for estimating sensory nerve conduction velocity (SNCV) along 1a fibers of the tibial nerve for the spinal cord‐to‐knee segment. Proximal and distal MNCV showed moderate slowing in patients with diabetic polyneuropathy and severe slowing in those with Charcot‐Marie‐Tooth disease. In 17 control subjects, proximal MNCV for the axons supplying the abductor hallucis muscle was compared to that estimated for the axons to the gastrocnemius muscle. MNCV for the proximal is faster than that for the distal small muscle. In 2 patients, M‐ and F‐responses were not obtained from the abductor hallucis; in these patients, effective estimation of the proximal MNCV was made from the gastrocnemius muscle and showed marked reduction.
Amino acid transport by a muscle cell line
Authors: Logan William J. 
Published: January 2005

AbstractAn established rat muscle cell culture, L‐6, was employed for the evaluation of muscle amino acid transport. The initial influx of 16 radiolabeled amino acids into these cells was determined, and an overall uptake profile was constructed. L‐6 cells accumulate amino acids progressively with time by saturable, temperature‐dependent processes. As found in other preparations, several amino acids have sodium‐dependent uptake systems. There is a wide range in the rate of transport of the different amino acids. The overall profile of uptake is fairly reproducible and may be characteristic of muscle tissue; it does not resemble that previously found in other cell types. The profile of amino acid uptake into these cells compares favorably with the pattern of amino acid transfer from bloodstream to muscle found in intact rats. It is concluded that muscle cell transport is basically similar to that of other cell types. L‐6 muscle cells have an amino acid uptake profile which resembles that of intact muscle; they should thus prove useful in the further delineation of muscle amino acid transport mechanisms.
Monomelic muscle hypertrophy following transient ischemic attacks: A case report
Authors: Isaacs Edward R. , Larson Paul 
Published: January 2005

AbstractA 39‐year‐old right‐handed male suffered several episodes of transient right‐sided weakness, sensory loss, and Broca's aphasia at age 31. Diagnostic studies failed to demonstrate any cause for these events. Following a complete recovery, he noted the onset of stiffness and increasing muscle bulk in his right arm, which gradually restricted its use. Simultaneous recordings from contracting biceps and triceps muscles in both arms suggested that the hypertrophy was physiologically induced by an abnormal mechanism providing resistance during phasing activity.
Electrodiagnostic findings in acute porphyric neuropathy
Authors: Albers James W. , Robertson William C. , Daube Jasper R. 
Published: January 2005

AbstractOf 115 patients with acute intermittent porphyria seen during a 20‐year period, 11 had experienced an acute episode of quadriparesis. Nerve conduction studies performed on 8 of these 11 patients showed lowamplitude compound action potentials and normal velocity measurements. Needle electromyography demonstrated prominent fibrillation potentials, especially in proximal muscles. The changes in these findings with time confirm that this disorder is an acute axonal neuropathy.
Histology and ultrastructure of alterations in neuropathy
Authors: Behse Friedrich , Carlsen Frits 
Published: January 2005

AbstractHistologic findings are described in nerves from men exposed to lead, from patients with discrete clinical signs of peripheral neuropathy, and from controls. Every nerve from control subjects showed an abnormality (paranodal remyelination, segmental remyelination, or regeneration) in teased fibers. The only histologic alteration in eight lead‐exposed males without signs or symptoms of neuropathy was a slightly increased incidence of paranodal remyelination. Sixteen patients with discrete neurologic symptoms and signs had a loss of large myelinated fibers and an increased incidence of regenerated fibers among teased fibers. Electron microscopy of unmyelinated fibers showed an increased occurrence of Schwann‐cell processes, of fibers undergoing degeneration, and of Schwann‐cell subunits with many profiles as the earliest signs of abnormality. Clinically mild neuropathies may exhibit advanced regeneration in the case of unmyelinated fibers. The earliest sign of degeneration in myelinated fibers was a diminution in the number of axonal organelles.
Preservation of bulk and strength in muscles affected by neurogenic lesions
Authors: Willison Robin G. 
Published: January 2005

AbstractMechanisms involved in maintaining bulk and strength in partially denervated muscles of patients with neurogenic lesions are discussed. Estimation of muscle fiber hypertrophy by electrophysiological examination is possible, but it depends on the construction of sampling electrodes with precise geometry. The arrangement of muscle fibers of motor units within the muscle is discussed by analogy with the four‐color map problem. An experiment to measure the twitch tensions of individual motor units in man is devised; it requires the use of microprocessor control of the stimulus and recurrent measurements of the twitch forces.
Pattern of electrical activity in normal and diseased muscle
Authors: Fuglsang‐Frederiksen A. 
Published: January 2005

AbstractExamination of the number of spikes (turns) per 5 sec and the mean amplitude of electrical activity, measured both at a force adjusted relative to maximum force (Po) and at a standard force, made it possible to distinguish between (1) a decrease in force arising from loss or inactivity of motor units, and (2) loss of muscle fibers or diminution in their cross‐sectional area. The initial decrease in force of disused quadriceps muscle (following immobilization of the knee joint) resulted mainly from inactivity of motor units. In patients with motoneuron disease or a lesion of the brachial plexus, the number of turns at a force adjusted relative to Po was diminished because of increased potential duration and loss of motor units. Turns at a standard force of 2 kg were normal. In myopathy, the ratio of turns to amplitude was increased in two‐thirds of the patients.
Myosin changes in experimental 2,4‐dichlorophenoxyacetate myopathy
Authors: Muhlrad Andras , Friedman Margalit 
Published: January 2005

AbstractYoung rats treated with 10 to 14 daily injections of 2,4‐dichlorophenoxyacetate (2,4‐D) developed a myopathy mainly involving fast muscles. Myosin isolated from the gastrocnemius muscles of treated and normal control animals differed in several respects. The Ca2+− and Mg2+ ‐mediated ATPases were higher in myopathic muscle myosin than in normals. Alkylation of thiols by N‐ethylmaleimide (NEM) induced an increase of Ca2+ ‐activated ATPase that was higher in normal than in myopathic myosin. Trinitrophenylation of reactive amino groups by 2,4,6‐trinitrobenzene sulfonate (TBS) induced an increase in Mg2+ ‐mediated ATPase in both preparations, but the increase was higher in normals. Although the heavy‐ and light‐chain pattern was identical in normal and myopathic myosin, during storage at 0°C the relative amount of myopathic L2 light chain decreased. Myosins fragmented either by limited proteolysis with trypsin and chymotrypsin or by specific cleavage at tryptophanyl and cysteinyl peptide bonds showed differences on sodium dodecylsulfate (SDS)‐polyacrylamide‐gel electrophoresis. The results indicate that there is a change in the heavy chains of myosin isolated from the gastrocnemius muscle in 2,4‐D‐induced rat myopathy.
Myosin autoantibodies reacting with selective muscle fiber types
Authors: McDonald Brian L. , Dawkins Roger L. , Robinson Jennifer 
Published: January 2005

AbstractMyosin in striated muscle exists in at least two immunologically distinct forms. Human autoantibodies specific for either form show selective muscle fiber reactivity. One group of antibodies reacts with type 2b (white) muscle fibers, the other with type 2a (red) and type 1 (intermediate) fibers. All of these antibodies react with the A band of glycerinated myofibrils. Both groups of antibodies react with cardiac muscle. Muscle‐fiber‐specific antibodies are detected in approximately 1% of sera submitted for diagnostic screening. Sera from eight patients were studied further. The two patients with antibodies against type 2b (white) muscle myosin had rheumatoid arthritis. The six patients with antibodies against type 2a (red) and type 1 (intermediate) myosin had clinical features which included various autoimmune and infectious disorders.
Lack of benefit of allopurinol in duchenne dystrophy
Authors: Mendell Jerry R. , Wiechers David O. 
Published: January 2005

AbstractA double‐blind, controlled study was conducted to assess the effects of allopurinol on Duchenne muscular dystrophy. Six patients—three treated with allopurinol and three given a placebo—were followed up for one year and were evaluated by functional and manual muscle testing as well as by determination of serum creatine kinase (CK) levels. Patients in both the allopurinol‐treated group and the placebo group deteriorated at varying rates without evidence of therapeutic benefit. To exclude the possibility of error attributable to small sample size, all six patients were treated with allopurinol for an additional eight‐week period. Muscle strength continued to deteriorate during this phase of the study.
Delayed functional disability in dystrophic chickens receiving chemotherapy
Authors: Hudecki Michael S. , Pollina Catherine M. , Bhargava Arvind K. , Hudecki Rajmohini S. , Heffner Reid R. 
Published: January 2005

AbstractLine 413 early‐onset, genetically homozygous dystrophic chickens were given twice‐daily intraperitoneal injections of the antiserotoninergic drug cinanserin, alone or in combination with methysergide. Other trials consisted of penicillamine treatment in combination with either methysergide or cyproheptadine. Chemotherapy significantly prolonged the righting ability of treated dystrophic chickens, as measured by a periodic standardized fliptest procedure. Plasma creatine kinase activities were not affected by any of the various drug treatments. However, the blood serotonin levels of the dystrophic chickens (grand mean 1.47 μg serotonin/ml blood) were found to be significantly higher (p<0.001) than those in the corresponding normal chickens (0.99 μg/ml). This finding may partially account for the antiserotoninergic drug enhancement in righting ability that was demonstrated in the drug‐treated dystrophic chickens.
Electrophysiologic properties of intercostal muscle fibers in human neuromuscular diseases
Authors: Gruener Raphael , Stern Lawrence Z. , Markovitz David , Gerdes Clarence 
Published: January 2005

AbstractElectrophysiologic properties of biopsied normal and diseased intercostal muscle fibers were examined using intracellular microelectrode techniques. The resting potentials of all diseased muscle fibers were found to be depolarized. Those from Duchenne dystrophy patients showed the largest depolarization, followed by those from patients with myotonic muscular dystrophy, myotonia congenita, and motor neuron disease. All of the diseased fibers except those from myotonia congenita patients demonstrated an impaired ability to generate action potentials. In the latter fibers, the higher‐than‐normal membrane resistance was associated with hyperexcitability. When the membrane was hyperpolarized to the normal range, however, action potential characteristics in all fibers were near normal, except in motor neuron disease. All action potentials were blocked by tetrodotoxin. These findings—i.e., that all fibers were capable of generating action potentials when hyperpolarized, and that all action potentials were blocked by tetrodotoxin—suggest the relative intactness, in the disease studied here, of the regenerative sodium conductance mechanism.
Antigenic modulation and receptor loss in experimental autoimmune myasthenia gravis
Authors: Lindstrom Jon , Einarson Brett 
Published: January 2005

AbstractImmunization of groups of rats with 0.1–100 μg of acetylcholine receptor (AChR) purified from the electric organ of Torpedo californica resulted in dose‐dependent (1) loss of acetylcholine receptor from the rats' muscles, (2) binding of antibodies to many of the receptors remaining in muscle, and (3) production of antibodies in serum capable of cross‐reacting with receptor solubilized from rat muscle. Addition of antibodies from rats immunized with electric organ acetylcholine receptors to muscle cells in culture caused loss of receptor by accelerating the rate of receptor degradation. Monovalent antibody fragments did not accelerate degradation unless antiantibody was added to cross‐link the monovalent antibody fragments bound to receptors. This indicates that cross‐linking of receptors by antibody molecules triggers accelerated receptor degradation, leading to receptor loss. The rate of increase in receptor destruction due to antigenic modulation observed in vitro appears sufficient to account for the extent of receptor loss observed in vivo. Endocytosis of antibody cross‐linked receptors may be a rate‐limiting step common to antigenic modulation in vitro and in vivo.
Penicillamine‐induced myasthenic responses in the guinea pig
Authors: Burres Steven A. , Richman David P. , Crayton John W. , Arnason Barry G. W. 
Published: January 2005

AbstractTo study the basis for the myasthenic illness that develops in some patients receiving D‐penicillamine (D‐P), we measured neuromuscular transmission in D‐P‐treated guinea pigs. Treated animals manifested edrophonium chloride–repairable decremental responses to 30‐Hz nerve stimulation (8.5% ± 1.5%—mean ± SEM; N = 16) compared with the response in saline‐treated control animals (−0.3% ± 0.85%, N = 10, p <0.005). Posttetanic phenomena were studied in 5 treated animals; 3 revealed posttetanic exhaustion. Miniature endplate potential (MEPP) amplitudes were significantly reduced in 11 treated animals (0.612 ± 0.016 mV) compared with 6 controls (0.713 ± 0.024 mV, p <0.01). The MEPP frequency appeared reduced in treated animals. Both MEPP amplitude and MEPP frequency correlated with the decrement.
Weakness from magnesium‐containing cathartics: Electrophysiologic studies
Authors: Swift Thomas R. 
Published: January 2005

AbstractThe case history of a patient with severe muscular weakness resulting from magnesium intoxication following oral and rectal administration of magnesium citrate cathartics is reported. The findings of the electrophysiologic studies were characteristic of the neuromuscular blockade seen in this disorder, including marked reduction in evoked muscle action potential amplitude which progressively declined on nerve stimulation at low rates, and a striking degree of facilitation of evoked muscle action potential after exercise or on high‐frequency stimulation. When the hypermagnesemia was corrected, strength returned to normal.