bluuebunny/crossref_metadata_2025_split huggingface dataset:
Primary CNS lymphoma presenting as a choreic movement disorder followed by segmental dystonia
Authors: Poewe W. H. , Kleedorfer B. , Willeit J. , Gerstenbrand F. 
Published: January 2005

AbstractClinical presentation of primary CNS lymphoma with an extrapyramidal movement disorder has not been recorded. A 66‐year‐old woman presented with chorea involving her left arm and subsequently developed rightsided segmental dystonia with prominent hemifacial dystonic spasms, milder torticollis and dystonia of the right arm. Investigations revealed primary CNS lymphoma with extensive involvement of the right‐sided basal ganglia as well as lesions confined to the head of the left caudate nucleus and the corpus callosum. Chorea of her left arm subsided with progressing disease while remission of right‐sided segmental dystonia was observed following radiotherapy of the brain. This patient's findings and a review of the literature suggest a possible relation between cranio‐cervical dystonia and pathology affecting the head of the caudate nucleus.
Oxyferriscorbone elevates the total iron content of blood but not brain
Authors: Dexter D. T. , Jenner P. , Marsden C. D. 
Published: January 2005

AbstractFollowing the intravenous infusion of oxyferriscorbone into the tail vein of the rat, the blood and brain metal ion content was measured over the following 72 h period. Administration of oxyferriscorbone increased the total iron content of blood for up to 24 h following intravenous infusion. In contrast, there was no increase in the total iron content of the cerebellum or striatum. Overall, there was no change in total zinc or copper content of the blood or brain following oxyferriscorbone administration. The effect of oxyferriscorbone in Parkinson's disease may not be related to any alteration in total iron content of the brain.
Effects of etybenzatropine and diazepam on levodopa‐induced diphasic dyskinesias in Parkinson's disease
Authors: Pourcher Emmanuelle , Bonnet Anne‐Marie , Kefalos John , Dubois Bruno , Agid Yves 
Published: January 2005

AbstractLevodopa‐induced onset and end‐of‐dose dyskinesia are rare but disabling disorders. Although they can be attenuated by increasing and dividing the daily dose of levodopa, this does not constitute a therapeutic approach. In this pilot study, etybenzatropine, an anticholinergic drug, and diazepam, a selective benzodiazepine, were administered in addition to a single dose of levodopa in nine patients with Parkinson's disease. Both drugs tended to decrease the severity and the duration of onset and end‐of‐dose dyskinesia, and to increase the duration of action of levodopa on parkinsonian symptoms.
Treatment of movement disorders with trihexyphenidyl
Authors: Jabbari Bahman , Scherokman Barbara , Gunderson Carl H. , Rosenberg Michael L. , Miller Joseph 
Published: January 2005

AbstractThe clinical efficacy of the trihexyphenidyl was investigated in 100 patients with movement disorders. The study group consisted of 54 women and 46 men. Their ages ranged from 18 to 70 years, and their duration of illness varied from a few months to 36 years. Each patient had a videotape of the movements and a neurological examination, before administration of the drug, at the time of maximum or effective dosage, and one week after withdrawal from trihexyphenidyl. The drug was administered at an initial total daily dose of 2 mg and gradually increased to a total daily dose of 60 mg over a period of 4–6 weeks. Improvements were rated both clinically and from the videotapes. Three groups of movement disorders demonstrated a significant response to trihexyphenidyl: (1) dystonia 37%; tonic torticollis demonstrated a significantly better response than the clonic variant (80% vs. 22%). (2) rhythmic‐oscillatory movements of brainstem–cerebellar origin (palatal myoclonus, pendular nystagmus, facial myokymia) 90%; (3) cerebellar tremor 75%. Among 32 responders, 17 (56%) continued taking trihexyphenidyl beyond 24 months. Side effects consisted of dryness of the mouth, jitteriness, stomatitis, blurred vision, and forgetfulness.
Cerebral sarcoidosis presenting as supranuclear gaze palsy with hypokinetic rigid syndrome
Authors: Schlegel U. , Clarenbach P. , Cordt Almut , Steudel A. 
Published: January 2005

AbstractA 31‐year‐old man with histologically documented pulmonary sarcoidosis developed a severe hypokinetic rigid syndrome with a supranuclear gaze palsy following recurrent lymphocytic meningitis and occlusive hydrocephalus. Magnetic resonance imaging (MRI) showed multiple hyperintense foci in the CNS, not detectable by computed tomography (CT). Long‐term steroid therapy led to clinical complete remission.
A case of rabies in North America
Authors: Bhatt Mohit , Wolters Erik , Tsui Joseph , Snow Barry J. , Calne Donald B. 
Published: January 2005

AbstractA 25‐year‐old man developed classical hydrophobic rabies 3 months after being bitten on the face. We present his involuntary movements on videotape and discuss the clinical and epidemiological features of rabies. Though rabies is seen in tropical countries commonly and in North America occasionally, recording of the clinical features is rare.
Uniform frequency of parkinsonian resting tremor in the lips, jaw, tongue, and index finger
Authors: Hunker Chauncey J. , Abbs James H. 
Published: January 2005

AbstractParkinsonian resting tremor was examined in the lips, jaw, tongue, and index finger using electromyography (EMG) and displacement transduction. Long‐term spectral averaging of tremor movement and EMG signals revealed that resting tremor frequencies were remarkably uniform across these orofacial and hand structures within the same parkinsonian subjects. However, marked variability in tremor spectral amplitude across structures and subtle moment‐to‐moment variations in tremor period, amplitude, and waveform shape were also observed. Although these observations argue for a central source of tremor, it appears that interactive central and/or peripheral neural circuitry may yield variations in tremor characteristics.
Pharmacokinetic comparison of sinemet and atamet (Generic carbidopa/levodopa): A single‐dose study
Authors: Pahwa R. , Marjama J. , McGuire D. , Lyons K. , Zwiebel F. , Silverstein P. , Ward R. , Koller W. C. 
Published: January 2005

AbstractWe compared the pharmacokinetic and motor responses of Sinemet and Atamet (generic carbidopa/levodopa) in patients with Parkinson's disease. Thirty Parkinson's disease patients (10 previously untreated, 10 with early disease, and 10 with motor fluctuations/dyskinesia) participated in a single‐dose double‐blind study. Following administration of an equivalent oral dose of Sinemet and Atamet on two separate days, we compared the peak plasma concentration, time to peak plasma concentration, area under the curve, total motor score, tapping score, and walking time. The mean time to peak plasma concentration was 49 min with Sinemet and 47 min with Atamet, the mean peak plasma concentration was 1,273 ng/ml with Sinemet and 1,153 ng/ml with Atamet, and the mean area under the curve was 2,295 m̈g/ml/h with Sinemet and 2,330 m̈g/ml/h with Atamet. Similarly, there was no significant difference between total motor score, tapping score, of walking time. In this single‐dose study, there were no significant differences in pharmacokinetic and motor responses between Sinmet and Atamet.
Infiltrating lipoma: A cause of monomelic hypertrophy
Authors: Fetell Michael R. , Duffy Philip E. , Rowland Lewis P. 
Published: January 2005

AbstractMuscle biopsy and computerized tomography indicated that unilateral enlargement of the gastrocnemius muscle in a 12‐year‐old girl was the result of an infiltrating lipoma. This rare condition may cause diagnostic confusion before EMG and muscle biopsy studies are performed, but it can be readily distinguished from other causes of unilateral muscle enlargement using these methods.
Choline acetyltransferase and acetylcholinesterase in muscular dystrophic mice
Authors: Sung S. C. 
Published: January 2005

AbstractThe activities of choline acetyltransferase (CAT) and acetylcholinesterase (AChE) were assayed in various tissues of dystrophic (dy/dy) and normal mice of Bar Harbor strain 129. The brain weights of these dystrophic mice were not significantly different from those of normal mice, but the average body weight of these dystrophic mice was only 66.8% of that of the controls. The activity of CAT (expressed as unit activity per mg of protein) was very similar in the brains of both groups of animals, but the CAT activity (per mg of protein) in the hindlimb muscles of the dystrophic mice was significantly higher than that of the controls. The patterns of AChE activity, as separated by sucrose density gradient centrifugation, were distinctly different in extracts of dystrophic and normal muscle. Compared with controls, decreased activity of the 15‐S and 10‐S forms of AChE, with increased activity of a 4.3‐S forrn of AChE, was observed in dystrophic muscle.
Myosin polymorphism in human skeletal muscles
Authors: Libera Dchem L. Dalla , Margreth A. , Mussini I. , Cerri C. , Scarlato G. 
Published: January 2005

AbstractMyosins isolated from individual human muscles (primarily normal muscles) were investigated with respect to their structural and catalytic properties. The results indicate unexpected elements of uniformity shared by the several myosins, such as a three‐banded, electrophoretic pattern of light chains in sodium dodecylsulfate (SDS) gels and a low degree of alkaline lability. The pH activity profile and the effect of KCl on myosin ATPase activities were also found to be the same for the myosins from predominantly fast (e.g., vastus lateralis and rectus abdominis) and slow (e.g., soleus and pectoralis minor) muscles. Coelectrophoretic experiments lend further credence to the interrelationship between human myosin light chains and the light chains of rabbit fast‐muscle myosin. However, several kinds of circumstantial evidence, such as that derived from the study of myosin in nemaline myopathy, suggest that one should exercise caution in interpreting these results. On the other hand, human muscle myosins, like those of other mammalian species, can be divided into two main categories according to the peptide composition of tryptic heavy meromyosin (HMM) and the banding pattern of light meromyosin (LMM) paracrystals. These results, which are indicative of differences in the primary structure of the heavy chains, allow us to identify these heavy chains as the main site of heterogeneity among myosins in human muscles.
Early electrophysiologic changes in conduction block
Authors: Trojaborg W. 
Published: January 2005

AbstractFibrillation potentials and positive sharp waves were recorded in the paralyzed muscles of 31 patients with a conduction block of more than 14 days' duration. In 75% of the muscles, these effects were attributed to axonal damage in addition to the local demyelinating block, this conclusion being based on changes in the properties of the motor unit found electromyographically when recovery had taken place. In the remaining 25% of the muscles, the denervation‐like activity was thought to be an effect of the local demyelinating block alone. The delay of the sensory action potential recorded above the presumed site of nerve compression most likely arises from a block of the largest myelinated nerve fibers. This assumption is based on the fact that the amplitudes of the fastest‐ and the slower‐conducted components in the patients differed little from corresponding components in the normal nerve.
Experimental autoimmune myasthenia gravis in the rat: A preliminary report
Authors: Krarup Christian , Elfman Lena , Kamieniecka Zofia , Schmalbruch Henning 
Published: January 2005

AbstractIn chronic experimental myasthenia gravis (EAMG) in rat, the decrement of electrical and mechanical responses evoked by maximal repetitive (3–167/sec) stimuli to the nerve was greater in the slow‐twitch soleus (SOL) than in the fast‐twitch extensor digitorum longus (EDL) muscle. Excitation‐contraction coupling was impaired in moderate to severe EAMG, as evidenced by a diminished staircase phenomenon and by diminished posttetanic potentiation (PTP) of twitch tension in two EDLs. The only morphologic abnormality observed was an increase in length of the endplates in the EDLs of those animals that had had an acute phase of EAMG. The latter had more than a 90% reduction in the amplitude of the action potential and in the twitch tension of the EDL when stimuli were applied to the nerve. Stimuli applied directly to the muscle evoked a tetanic tension that was one‐third of normal. The staircase and PTP were normal. Necrosis occurred in the endplate and in the adjacent segment of muscle fiber; outside the endplate region, the muscle fiber was normal.
Elements in muscle measured in vivo and in vitro with X‐ray spectrometry
Authors: Yarom Rena , Havivi Yael , Notowitz Luta , Friedman Margalit , Gorodetsky Raphael , Zeimer Ran 
Published: January 2005

AbstractCalcium, iron, copper, and zinc concentrations in muscle were measured in vivo by x‐ray spectrometry (XRS). The undersurface of the tongue was examined in cardiomyopathic and in normal hamsters. Values were compared to in‐vitro analyses of various muscles from the same groups of animals using XRS and chemical analysis. Blood tests as well as histologic and histometric examinations were also performed. The correlated results show that XRS is a relatively sensitive and practical method for measuring chemical elements in muscle, and that it could be developed into a useful clinical test in certain neuromuscular and myocardial diseases. An interesting incidental finding was that, though all the examined muscles of myopathic animals had pathologic changes, only the tongue and myocardium, which are composed exclusively of red fibers, showed excessive calcium accumulation. This suggests either that the basic genetic aberration is not directly related to the oxidative metabolism, or that white muscles have a more efficient system for dealing with calcium overload.
Neuroanastomosis of orthotopically transplanted palmaris longus muscles
Authors: Maxwell L. C. , Faulkner J. A. , Markley J. M. , Winborn D. R. 
Published: January 2005

AbstractPalmaris longus (PML) muscles of rhesus monkeys were transplanted, with or without anastomosis of the median nerve, to the nerve stump of the autograft. Because PML autografts revascularize spontaneously, vascular anastomoses were not performed. Muscle fibers regenerated in all autografts with neuroanastomosis, but in only three of eight autografts without neuroanastomosis. Five autografts without neuroanastomosis were replaced by noncontractile connective tissue. Growth and differentiation of muscle fibers into three fiber types and development of capillarity were analyzed histochemically, and succinate oxidase activity of whole‐muscle homogenates was determined. None of these measures reached values for control PML muscles within 100 days of transplantation. In comparison to control muscles, autografts had slower times to peak tension and less absolute tension, but similar tension per square centimeter of muscle fiber cross‐sectional area. Monkey PML autografts with neuroanastomosis were similar in structure and function to cat extensor digitorum longus autografts that had not had neuroanastomosis.
F chronodispersion: A new electrophysiologic method
Authors: Panayiotopoulos Chrysostomos P. 
Published: January 2005

AbstractWe have introduced the term F chronodispersion to denote the scatter or dispersion of the relative latencies of statistically significant numbers of consecutively recorded F waves. Five patients with chronic renal failure and five control subjects were studied. The two groups were matched for age, sex, proximal and distal motor conduction velocity, and latency of fast F waves plotted against distance traveled. We found that the extent of F chronodispersion of the deep peroneal nerve did not exceed 7.5 msec in control subjects, while it was longer than this value in all uremic patients studied. Measurements of this type may prove to be a useful diagnostic tool for cases in which traditional electrophysiologic measurements are normal.
The autosomal recessive (Becker) form of myotonia congenita
Authors: Erich Kuhn S. , Fiehn Walter , Seiler Dieter , SchröDer Joachim‐Michael 
Published: January 2005

AbstractIn the last two decades, two genetically distinct forms of myotonia congenita have been identified—an autosomal dominant and an autosomal recessive form. The purpose of this review is to describe the features that enable us to distinguish between these two forms in the absence of sufficient genetic data. Thus far, it can be concluded that the only probable difference between the two forms is in the fatty‐acid pattern of muscle phospholipids. Clinical, histologic, ultrastructural, and electromyographic investigation may prove helpful, but they alone cannot provide a reliable means of identifying the genotype in an individual patient.
Type III glycogenosis with multicore structures
Authors: Pellissier S. J. F. , de Barsy Th. , Faugere M. C. , Rebuffel P. 
Published: January 2005

AbstractA case of an infantile type III glycogenosis (Forbes disease), confirmed by morphologic and biochemical studies, had light‐microscopic, histochemical, and electron‐microscopic evidence of multicore structures and type 1 fiber predominance with hypotrophy. This association is discussed with relation to the unusual clinical findings. The authors conclude that two distinct disease entities—Forbes disease and multicore myopathy—may coexist.
Myoadenylate deaminase deficiency
Authors: Shumate Jack B. , Katnik Richard , Ruiz Martha , Kaiser Kenneth , Frieden Carl , Brooke Michael H. , Carroll James E. 
Published: January 2005

AbstractMyoadenylate deaminase (adenosine monophosphate deaminase—AMPDA) was recently shown to be deficient in a group of patients by use of a histochemical and biochemical method based on the elaboration of ammonia by this enzyme as it deaminates 5′ AMP. We have confirmed the utility of this histochemical method and the existence of persons deficient in AMPDA through the use of an unrelated assay technique. The lack of enzyme activity is not associated with any inhibitory activity in the muscles of patients with this disorder. The clinical diversity of these patients suggests that this lack may represent a normal variant or a subclinical state rather than an actual disease. The occurrence of AMPDA deficiency in both sexes points to possible autosomal inheritance.
Muscle breakdown and repair in polymyositis: A case study
Authors: Edwards H. T. , Wiles C. M. , Round J. M. , Jackson M. J. , Young A. 
Published: January 2005

AbstractMuscle breakdown and repair were measured by metabolic balance techniques in a patient with polymyositis who was being treated with prednisolone and azathioprine. Changes in body muscle mass that had been estimated from nitrogen and phosphorus balances correlated with anthropometric assessments of thigh muscle mass and quadriceps strength. Decline in muscle strength was associated with a net rate of muscle breakdown of 148 g/day. Recovery was associated with a net rate of muscle repair of up to 100 g/day. Early reduction in corticosteroid treatment appeared to enhance the rate of repair. Changes in the isometric contraction force of the quadriceps muscle (but not in clinical symptoms, plasma creatine kinase [CK] or erythrocyte sedimentation rate [ESR]) were found to reliably indicate whether the muscle was in a state of breakdown or repair. Treatment of the individual patient may be quantitatively monitored by metabolic balance studies or, more simply, by measurement of muscle strength.
Contractile responses of rat fast‐twitch and slow‐twitch muscles to glucocorticoid treatment
Authors: Gardiner Phillip F. , Edgerton V. Reggie 
Published: January 2005

AbstractIn an attempt to determine whether chronic glucocorticoid treatment affects speed‐ and strength‐related properties of fast‐twitch muscle and slow‐twitch muscle, in‐situ contractile properties of soleus and gastrocnemius muscles were measured in rats after 14 days on a regimen of triamcinolone acetonide injected at a dosage of 2 mg/kg/day. No effects on contractile speed or strength were noted for either muscle. The time to peak tension in soleus muscle was significantly longer in the treated group (43.0 ± 1.0 msec) than in the control group (38.7 ± 0.7 msec); however, this was attributed to a prolongation of “active state” duration, as suggested by the fact that treated muscles also showed significantly elevated twitch‐tetanus ratios. The treatment‐induced enhancement of the specific twitch tension was more pronounced in the gastrocnemius muscles (198% ± 19% of control) than in the soleus muscles (153% ± 9% of control). The difference in response to steroid treatment may reflect structural and functional difference in fast‐ and slow‐muscle membrane systems.
Morphologic changes in extraocular muscles of the dystrophic hamster
Authors: Percy D. H. , Thakar J. H. , Strickland K. P. 
Published: January 2005

AbstractCardiomyopathic hamsters (UM‐X7.1 strain) have demonstrable myopathy involving both skeletal and cardiac muscle. In addition, they have multiple ocular abnormalities. In this study, extraocular muscles were examined by light and electron microscopy. Changes observed within affected muscle fibers were variable and included coagulation necrosis, lysis of myofibrils, mitochondrial changes, and infiltration by phagocytic cells. Regenerative changes included duplication of myoblast nuclei, proliferation of sarcoplasmic reticulum, and myofibrillogenesis. The lesions are presumably myogenic in origin. The cardiomyopathic hamster may be useful as an animal model for certain types of ocular myopathy in man.
Increased endoneurial fluid pressure in galactose neuropathy
Authors: Myers Robert R. , Costello Michael L. , Powell Henry C. 
Published: January 2005

AbstractEdema and increased endoneurial fluid pressure developed in peripheral nerves of rats that received a diet containing 40% galactose during a study of the role of sugar alcohols in producing neuropathy. Fluid pressure was elevated starting in the third month and progressed to a fivefold increase over control values by the fifth month. Microscopic examination confirmed the presence of edema as predicted by the sorbitol theory, which is often invoked to explain the pathogenesis of diabetic neuropathy.
Defective oxidative metabolism of myodystrophic skeletal muscle mitochondria
Authors: Lee C. P. , Martens M. E. , Jankulovska L. , Neymark M. A. 
Published: January 2005

AbstractA small‐scale procedure for preparing tightly coupled intact skeletal muscle mitochondria from myodystrophic (myd/myd) mice is described. Mitochondrial preparations derived from heart, liver, and skeletal muscle of myd/myd and their littermate (+/?) controls are characterized with respect to their cytochrome content and their oxidative and phosphorylative capacities. Our data indicate that there is an impairment in the NADH CoQ region of the respiratory chain of myodystrophic skeletal muscle mitochondria. Both heart and liver mitochondria of myd/myd exhibited normal activities of respiratory chain‐linked oxidative phosphorylation.
Experimental myotonia induced in denervated muscles by 2,4‐D
Authors: Eberstein Arthur , Goodgold Joseph 
Published: January 2005

AbstractRats were denervated in one hind limb and injected with 2,4‐dichlorophenoxyacetic acid (2,4‐D). Isotonic tetanic contractions of the muscles treated with 2,4‐D after more than 10 days of denervation revealed prolonged relaxation times similar to those of the intact side and characteristic of clinical myotonia. No myotonic discharges were observed in the muscles denervated for more than 10 days and treated with 2,4‐D. The increase in threshold for action potential generation secondary to denervation is suggested as the factor limiting the initiation of the repetitive discharges.