bluuebunny/crossref_metadata_2025_split huggingface dataset:
Antigenotoxicity of extracts from Pleurotus citrinopileatus
Authors: Wang Jinn‐Chyi , Hu Shu‐Hui , Liang Zeng‐Chin , Lee Min‐Yen 
Published: January 2005

AbstractWhile Pleurotus citrinopileatus is a widely used edible mushroom, little is known about its physiological effects. Extracts, including aqueous extract, water‐soluble polysaccharide (WSP), crude protein solution (CPS) and residue from chloroform–ethyl acetate–methanol elution (CEM), were obtained first from fruiting bodies, through a solid‐state culture, and then from the mycelium, through a submerged culture. This study explored the antigenotoxicity effects of these extracts from Pleurotus citrinopileatus via the Ames test and a spore rec‐Assay. The results showed that, regardless of where the extract came from, the fruiting body or the mycelium, the antigenotoxicity effect was highest for CEM, followed by CPS, aqueous extract and WSP. The results of the Ames test indicated that, among several mutagens, CEM had the highest inhibition rate against AFBl in TA98 and TA100 and the lowest inhibition rate against NQNO. The concentrations of the various extracts were as follows: water extracts were 1 mg ml−1 and 5 mg ml−1 WSP, while CPS and CEM were 0.4 mg ml−1 and 2 mg ml−1, respectively; the higher the concentration of the extract, the higher the antimutagenicity effect. The results of the rec‐Assay indicated that CEM had the highest anti‐DNA‐damaging activity with or without the S9 mixture; the higher the concentration, the more significant the effect (p < 0.05). The anti‐DNA‐damaging activities were lower in the water extract concentrations, at 30 µg disc−1 dry weight−1, while the WSP, CPS and CEM at 12, 150 and 60 µg disc−1, respectively, were high. Copyright © 2004 Society of Chemical Industry
Levodopa dose‐related fluctuations in presumed olivopontocerebellar atrophy
Authors: Lang A. E. , Birnbaum A. , Blair R. D. G. , Kierans C. 
Published: January 2005

AbstractThe parkinsonism that occurs in some patients with olivopontocerebellar atrophy (OPCA) can cause diagnostic confusion with idiopathic Parkinson's disease (IPD). The response to levodopa is usually a distinguishing feature, the OPCAs either failing to benefit or losing efficacy relatively quickly. A fluctuating response to levodopa in those OPCA patients who do benefit has not been emphasized in the literature previously. Reported here are three patients with presumed OPCA, dominated by parkinsonian features, who eventually developed typical fluctuations with morning akinesia, wearing off, and periodic lack of response related to meals. These fluctuations were a major source of disability and an important reason for diagnostic confusion with IPD. The possible mechanisms of these fluctuations are discussed.
Administration of MPTP to the common marmoset does not alter cortical cholinergic function
Authors: Garvey J. , Petersen M. , Waters C. M. , Rose S. P. , Hunt S. , Briggs R. , Jenner P. , Marsden C. D. 
Published: January 2005

AbstractThe administration of 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP) to common marmosets induced persistent motor deficits and decreased concentrations of dopamine, homovanillic acid, and 3,4‐dihydroxy‐phenylacetic acid (DOPAC) and [3H]dopamine uptake in the caudate‐putamen. There was an 80% reduction in tyrosine hydroxylase immunoreactive cells in substantia nigra. At 10 days following the start of MPTP administration, the activity of choline acetyltransferase in the thalamus and frontal cortex was unchanged compared with control animals. Similarly, specific [3H]QNB binding was unaltered. At 4–6 weeks following the start of MPTP treatment, choline acetyltransferase activity and [3H]QNB binding in the frontal cortex and thalamus remained unaffected. There was no evidence for cell loss in the nucleus basalis of Meynert or alteration in the intensity of staining for acetyl‐cholinesterase. MPTP treatment of the common marmoset produces a nigrostriatal lesion. In contrast, MPTP did not alter cortical cholinergic function and was not neurotoxic to the cholinergic cells in the nucleus basalis of Meynert.
Autosomal dominant episodic ataxia: A heterogeneous syndrome
Authors: Gancher Stephen T. , Nutt John G. 
Published: January 2005

AbstractWe describe six kindreds with autosomal dominant episodic ataxia, apparently representing three distinct syndromes. Four kindreds were characterized by episodic ataxia and response to acetazolamide, and in three, interictal nystagmus. One kindred was characterized by paroxysmal ataxia and in one member, paroxysmal choreoathetosis. The last kindred had brief attacks of ataxia and interictal neuromyotonia. The age of onset and severity of the disorder varied within each kindred. These kindreds illustrate the heterogenity of episodic ataxia as well as the variable expressivity within each kindred.
Focal asterixis caused by a small putaminal hemorrhage
Authors: Trejo José M. , Giménez‐Roldán Santiago , Esteban Angel 
Published: January 2005

AbstractUnilateral asterixis and mild pure motor hemiparesis contralateral to a small putaminal hemorrhage were observed in a 74‐year‐old hypertensive man. The motor deficit proved to be rapidly reversible, but the focal asterixis remained for some time as the only sign of the intracranial hemorrhage. This case suggests that altered striopallidal inhibitory input to the thalamus probably disturbed the circuit subserving the maintenance of postural tone.
Long‐term follow‐up after stereotaxic basal ganglia surgery in Parkinsonism. A neuroanatomical study of a case showing unusual postural disorders
Authors: Viallet F. , Gambarelli D. , Alonzo B. , Sedan R. , Khalil R. 
Published: January 2005

AbstractA 60‐year‐old man who had Parkinson's disease for 25 year became unable to maintain the upright position of the trunk while standing or walking. Because of a loss of postural fixation, his trunk sank forward until his back was horizontal. Fifteen years before, a bifocal implantation of radioactive yttrium‐90 had been performed by a stereotaxic procedure at the level of the globus pallidus (GP) and the ventrolateral thalamus, respectively on the right side. A second operation, 2 years later, had been made by electrocoagulation in the left ventrolateral thalamus. Postmortem examination showed the following features (a) neuronal cell loss in the pigmented nuclei of the brainstem with intracellular Lewy bodies and gliosis. These data corresponded with the usual pathology of idiopathic Parkinson's disease; (b) surgical lesions resulting from the previous stereotaxic operations in the right GP and the ventrolateral thalamus on both sides and (c) neuronal cell loss without gliosis in the right GP that was evident in regions spared by the surgical lesion. Moreover, these degenerative changes were particularly seen in the left GP, which was surgically unlesioned. The ansa lenticularis was demyelinated on both sides. These data might be consistent with a retrograde degenerative process affecting the pallidothalamic projections. Beside the role of the neuronal cell loss affecting the pars compacta of the substania nigra (SN) in akinesia, the role of the lesions affecting the G.P. in the genesis of postural disorders is suggested.
An investigation of the phenomenon of “set” in Parkinson's disease
Authors: Brown R. G. , Marsden C. D. 
Published: January 2005

AbstractIt has been suggested that patients with Parkinson's disease have a basic deficit in shifting “set.” Previous authors have used this hypothesis as an explanation for deficits on a range of cognitive tasks. An experiment was conducted to investigate this phenomenon. A reaction time paradigm was used in which the subject had to make left‐right decisions under two stimulus conditions. Both patients and controls showed a facilitation in performance with repetition within each blcok of trials, followed by a deterioration in performance when the stimulus type switched. Furthermore, the shapes of the reaction‐time functions were equivalent in both groups, suggesting that the patients had no deficit in either switching or establishing set in this task. In contrast, they were impaired on the Wisconsin Card Sorting Test. This dissociation suggests that patients with Parkinson's disease do not have a generalized deficit in switching set. It is suggested that patients with Parkinson's disease are impaired on tasks where they have to rely upon internal control for the regulation of behaviour, but are relatively unimpaired on tasks where external cues are available.
Crural and axial myoclonic dystonia following meralgia paraesthetica
Authors: Ter Bruggen J. P. , Tijssen C. C. 
Published: January 2005

AbstractA 49‐year‐old man developed a syndrome of crural‐axial dystonia combined with segmental myoclonus 3 months after the onset of meralgia paraesthetica of the left leg. The association of this remarkable movement disorder with the pain syndrome is discussed.
Oculo‐facio‐skeletal myorhythmia as a cerebral complication of systemic Whipple's disease
Authors: Hausser‐Hauw Chantal , Roullet Etienne , Robert René , Marteau René 
Published: January 2005

AbstractA fifth case of oculomasticatory myorthythmia associated with cerebral Whipple's disease is reported. This peculiar abnormal movement has never been described in association with cerebral dysfunction other than Whipple's disease. The present case exhibited rhythmic convergence of the eyes and synchronous (1–2 Hz) contractions of the masticatory muscles and of the proximal and distal skeletal muscles. These abnormal movements occurred 13 years after the beginning of the disease. They were persistent and unchanged until the death of the patient 3 months later. No treatment was effective to suppress the involuntary movements (clonazepam, baclofen, antibiotics). Associated neurological signs included global supranuclear ophthalmoplegia, facial weakness, bilateral ptosis, absent gag reflex, and intellectual deterioration.
Pathological report of four patients presenting with cranial dystonias
Authors: Gibb W. R. G. , Lees A. J. , Marsden C. D. 
Published: January 2005

AbstractSecondary dystonias and experimental models of dystonia suggest that mechanisms responsible for primary dystonias may lie in the basal gaglia or brainstem. A histological study ahs been done in three patients with cranial dystonia (blepharospasm with oromandibular dystonia in two, blepharospasm alone in one), and one patient with craniocervical dystonia (oromandibular dystonia with retrocollis). In the patient with blepharospasm alone, an angioma, 0.5 mm in diameter, was found in the dorsal pons at the site of the central tegmental tract, confirming that some patients presenting with primary dystonias may have longstanding lesions in the brainstem. In the three other cases, the striatum, pallidum, thalamus, and brainstem were examined and cell populations in the putamen, substantia nigra, and inferior olives were compared with age‐matched controls, but no significant abnormality was found.
Genetic prediction in Huntington's disease: What are the limitations imposed by pedigree structure
Authors: Misra V. Peter , Baraitser Michael , Harding A. E. 
Published: January 2005

AbstractThe major factor limiting use of the polymorphic DNA sequence D4S10, genetically linked to the Huntington's desease (HD) locus, in clinical practice is the fragmented nature of HD families. A population survey in South Wales suggested that genetic prediction would only be possible in 15% of adults at risk as a result of this. We have analysed pedigrees from 151 families, containing 482 adults between 18 and 45 years of age who were at high risk of developing HD, 157 of whom had attended genetic counselling clinics. Thirtyseven percent of adults at high rist in these kindreds had the appropriate pedigree structure needed for presymptomatic testing. It should be possible to perform fetal exclusion tests in about 80% of pregnancies at risk.
Concurrent hypnogenic and reflex paroxysmal dystonia
Authors: Lehkuniec Estela , Micheli Federico , De Arbeliaz Roberto , Torres Marta , Paradiso Guillermo 
Published: January 2005

AbstractA 29‐year‐old patient with nocturnal episodes of paroxysmal dystonia is described. In addition, attacks could be provoked by stimulation of his right foot. Treatment with phenytoin resulted in a marked reduction in the frequency of the episodes.
Neurochemical findings in neuroacanthocytosis
Authors: de Yebenes J. G. , Brin M. F. , Mena M. A. , De Felipe C. , del Rio R. M. , Bazan E. , Martinez A. , Fahn S. , Del Rio J. , Vazquez A. , de Seijas E. Varela 
Published: January 2005

AbstractWe performed a neurochemical study of the brain of two unrelated patients, living in different continents, with neuroacanthocytosis. The levels of monoamines and their metabolites, γ‐aminobutyric acid and substance P, were measured in several brain areas and the monoamine metabolites in cerebrospinal fluid. The binding of 3H‐spiperone to striatal membranes and to lymphoctytes was also measured. Both patients had a progressive neurological disorder with onset in the third decade of life and characterized by a complex movement disorder, epilepsy, muscular wasting, and changes in behavior. The movement disorder, epilepsy, muscular wasting, and changes in behavior. The movement disorder initially manifested with oromandibular dystonai and limb chorea, but at the time of death was characterized by a severe dystonic syndrome. The chemical changes were similar in the two patients. The most important neurochemical findings were a depletion of dopamine and its metabolites in most brain areas, most notably in the striatum, and elevation of norepiephrine levels in the putamen and globus pallidus. Substance P was markedly reduced in the striatum and substantia nigra. Our findings may provide clues to the neurochemical mechanisms underlying dystonia.
Off‐period belching due to a reversible disturbance of oesophageal motility in Parkinso's disease and its treatment with apomorphine
Authors: Kempster P. A. , Lees A. J. , Crichton P. , Frankel J. P. , Shorvon P. 
Published: January 2005

AbstractTwo L‐dopa‐treated patients with Parkinson's disease who developed distressing belching during “off” periods are reported. In each case, contrast cine radiography revealed disturbed oesophageal motility which disappeared after injection of the dopamine receptor agonist apomorphine. It is suggested that central dopaminergic abnormalities may be important in the aetiology of “off period belching”.
The use of glutethimide for treatment of essential tremor
Authors: McDowell Fletcher H. 
Published: January 2005

AbstractTwo patients with long‐standing intention tremor that was interfering with their ability to maintain employment were treated with glutethimide. In both patients, this agent successfully supressed tremor at doses of 1,000 mg and 4,000 mg per day. Glutethimide has been taken for as long as 14 years in one patient without evidence of side effects.
Choreoathetosis induced by cyproheptadine
Authors: Samie Mohammad Reza , Ashton Adam Keller 
Published: January 2005

AbstractCyproheptadine is an antihistamine with antiserotoninergic and anticholinergic properties. It is a relatively safe drug with many varied indications for usage, which rarely produces serious adverse neurologic sequelae. Reports of involuntary movement disorder secondary to cyproheptadine are rare. We present the case of a 17‐year‐old overdose victim which may be the first documented instance of cyproheptadine intoxication resulting in choreoathetosis. This hyperkinesia was most likely resultant from the anticholinergic properties of cyproheptadine. Although the choreoathetoid movement resolved spontaneously, the delirious state disappeared after administration of physostigmine.
Homolateral disappearance of essential tremor after cerebellar stroke
Authors: Dupuis M. J. M. , Delwaide P. J. , Boucquey D. , Gonsette R. E. 
Published: January 2005

AbstractThe case of a patient with essential tremor in whom tremor disappeared on the right side after a homolateral cerebellar infarct is reported. The pathophysiology of essential tremor remains unknown. Vascular and surgical lesions of dentorubrothalamic pathway have been reported to improve various kinds of tremor including essential tremor. In this patient, a lesion of this pathway related to the cerebellar infarct probably explains the homolateral cessation of essential tremor.
The sz mutant hamster: A genetic model of epilepsy or of paroxysmal dystonia?
Authors: Löscher Wolfgang , Fisher J. Edward , Schmidt Dieter , Fredow Gabriele , Hönack Dagmar , Iturrian W. B. 
Published: January 2005

AbstractAttacks of sustained dystonic postures of limbs and trunk can be initiated by mild environmental stimuli in an inbred line of Syrian hamsters. The trait is determined by an autosomal simple recessive genetic mutation, originally designated by the gene symbol sz, because the abnormal movements were thought to represent epileptic seizures. The attacks, which can be reproducibly initiated by placing the sz mutant hamsters in a new environment, begin with rapid twitches of the vibrissae, flattened ears, and flattened posture of the trunk while walking, followed by facial contortions, rearing, and sustained posturing of trunk and limbs, often resulting in falling over to the side or backwards. In the final stage, the hamsters became immobile, which can last for hours. An increased tone of limbs and trunk muscles can be palpated during the attack. Electromyographical recordings in awake, unrestrained mutant hamsters showed that the onset of the attack coincided with continuous tonic muscle activity and phasic bursts, which were present even when the animals did not move. During the attack, the animals continue to react to external stimuli. Bilateral electroencephalographic (EEG) recordings before and during motor disturbances in sz mutant hamsters showed no abnormalities. The severity of the dystonic syndrome in hamsters is age dependent with a peak at about 30–40 days of age. A score system for grading type and severity of dystonic attack was developed for use in drug activity studies. The severity of the attack was reduced or attacks were completely prevented by diazepam (1–2.5 mg/kg i.p.) and valproic acid (100–400 mg/kg i.p.) in a dose‐dependent fashion. The latency to dystonic movements was significantly increased by diazepam but markedly reduced by subconvulsive doses of pentylenetetrazol (40 mg/kg s.c.). Diazepam antagonized the latency‐reducing action of pentylenetetrazol in the hamsters. The pathophysiology and pharmacological sensitivity of the dystonic attacks in these animals remain to be further clarified, but the data indicate that the sz mutant hamsters might represent an interesting genetic model for paroxysmal dystonia. In view of these data, we propose that the hamster mutation should be re‐named dystonic and that the new gene symbol should be designated dtsz.
Adductor laryngeal dystonia (spastic dysphonia): Treatment with local injections of botulinum toxin (Botox)
Authors: Brin Mitchell F. , Blitzer Andrew , Fahn Stanley , Lovelace Robert E. , Gould Wilbur 
Published: January 2005

AbstractAdductor spastic dysphonia (SD) is a laryngeal dystonia characterized by a choked, constrained voice pattern with breaks in vocal flow. Treatment with a variety of therapies including speech and pharmacotherapy have minimal benefit; only one‐third of patients undergoing recurrent laryngeal nerve section have benefitted at 3 years. We have used local injections of botulinum toxin (Botox) bilaterally into vocalis muscles in 42 patients with SD. Injections were through a teflon‐coated hollow electromyography (EMG) recording needle. Unilateral small doses (2.53.75 U) were of no clinical benefit. Bilateral small doses resulted in sustained improvement lasting 84.4 ± 9.3 days. The degree of improvement was 61.1 ± 4.6%. Common side‐effects included a brief period of breathy hypophonia (8.5 ± 2.5 days) and a mild sensation of choking/aspiration of fluids (1.7 ± 0.6 days); there were no serious adverse effect. Vocal cord paralysis was not necessary for benefit. Follow‐up vocalis muscle EMGs revealed denervation. All patients responded to retreatment (longest follow‐up 3.5 years). Patients with prior recurrent laryngeal nerve surgery and residual uncomplicated dysphonia had similar results. Our results indicate that local injection of low‐dose Botox is the treatment of choice for SD.
Comparison of Sinemet CR4 and standard Sinemet: Double blind and long‐term open trial in parkinsonian patients with fluctuations
Authors: Jankovic Joseph , Schwartz Kenneth , Linden Chris Vander 
Published: January 2005

Abstract“Wearing‐off” effect, the most common form of levodopa‐induced fluctuations, seems to be related to the short plasma half‐life of the drug. More sustained plasma levodopa levels may be achieved with a new controlledrelease formulation of carbidopa/levodopa, Sinemet CR4. We studied 20 patients, 12 men and 8 women, with Parkinson's disease complicated by “wearing‐off” phenomenon. Mean age was 61.1 ± 8.1 years, duration of symptoms 8.3 ± 2.4 years, and the Hoehn‐Yahr stage 3.0 ± 0.9. In a 12‐week double‐blind study, the average number of tablets administered per day decreased from 5.7 ± 1.2 to 3.8 ± 0.7 when Sinemet CR4 (50/200) was substituted for the standard Sinemet (25/100) (p < 0.001). However, this was at the expense of reducing the “on” time (without dyskinesia) from 9.3 ± 4.6 to 7.5 ± 4.3 (p < 0.05), although the total “on” time did not significantly change. In a long‐term follow‐up of 18 patients, the “on” time with dyskinesia and morning dystonia significantly increased (p < 0.05). There was no significant change in the total daily dosage of levodopa, but the daily number of doses and tablets significantly decreased (p < 0.001). Despite increased dyskinesia, most patients preferred taking fewer tablets and have elected to continue taking Sinemet CR4 instead of standard Sinemet. Sinemet CR4 seems to offer a new and effective strategy for the management of levodopa‐related fluctuations.
Life‐threatening cranial dystonia following trihexyphenidyl withdrawal
Authors: Giménez‐Roldán S. , Mateo D. , Martíin M. 
Published: January 2005

AbstractA 64‐year‐old woman with blepharospasm, sustained clenching of the jaw, antecollis, and a strained, high‐pitched phonation continued chronic trihexyphenidyl therapy despite the lack of any obvious benefit. Abrupt, accidental withdrawal of trihexyphenidyl triggered severe exacerbation of the cranial dystonia associated with inspiratory stridor and acute respiratory difficulties, prompting emergency admission. On indirect laryngoscopy, hyperadduction of the vocal folds was not the cause of the upper airway obstruction. A more likely cause of the inspiratory obstruction appeared to be forward bending of the neck combined with mouth‐clenching spasms. Reinstitution of intravenous anticholinergic medication provided relatively prompt relief. We caution against abrupt interruption of anticholinergics in patients with severe segmental cranial dystonia, even in those cases in which no benefit is apparent to observers.
Diaphragmatic flutter, the moving umbilicus syndrome, and “belly dancer's” dyskinesia
Authors: Iliceto G. , Thompson P. D. , Day B. L. , Rothwell J. C. , Lees A. J. , Marsden C. D. 
Published: January 2005

AbstractFive patients presenting with focal abnormal involuntary movements of the abdominal wall are described. One was shown to have diaphragmatic flutter, which may represent a variant of the palatal myoclonus syndrome. The other four had writhing movements and contractions of the abdominal wall at frequencies of approximately 30 per minute. In two of the latter cases, these abdominal movements were profoundly influenced by respiratory manoeuvres. They were quite unlike the movements of spinal myoclonus or axial torsion dystonia. None of the patients were taking drugs that might have provoked their abnormal movements, or had any other evidence of neurological deficit. No cause was established in any of the cases.