bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long‐term changes of striatal dopamine D2 Receptors in patients with Parkinson's disease: A study with positron emission tomography and [11C]Raclopride
Authors: Antonini Angelo , Schwarz Johannes , Oertel Wolfgang H. , Pogarell Oliver , Leenders Klaus L. 
Published: May 2005

AbstractWe used [11C]raclopride (RACLO) and positron emission tomography (PET) to study longitudinally striatal dopamine D2 receptor binding in nine patients with Parkinson's disease (PD) at an early drug‐naive stage and 3–5 years later, when motor fluctuations had appeared in seven of them. Patients were treated with a combination of levodopa and dopamine agonists. Data were compared with 10 healthy controls in the same age range. Initially, patients with PD showed a significant increase of RACLO uptake in the putamen compared with controls (p < 0.04). The caudate nucleus revealed values in the normal range. After 3–5 years, RACLO binding was significantly reduced in the putamen (p < 0.03) and caudate nucleus (p < 0.03) compared with baseline. Values were now in the control range in the putamen and reduced in the caudate nucleus (p < 0.05). The clinical score at “off” had significantly worsened (p < 0.0005) compared with the first PET scan. The nine PD patients reported here had already been investigated 3–4 months after therapy began and at that time did not show a reduction of the initially increased RACLO binding capacity (data published previously). These results indicate long‐term downregulation of striatal dopamine D2 receptor binding in PD. Receptor changes in the striatum of patients with PD may be induced by chronic dopaminergic therapy or occur independently of treatment, as a result of structural adaptation of the postsynaptic dopaminergic system to the progressive decline of nigrostriatal neurons.
The neurological syndrome of infantile cobalamin deficiency: Developmental regression and involuntary movements
Authors: Grattan‐Smith P. J. , Wilcken B. , Procopis P. G. , Wise G. A. 
Published: May 2005

AbstractDevelopmental regression is the presenting symptom of most infants with cobalamin (vitamin B12) deficiency. We present a report of three infants with cobalamin deficiency in which the infants also developed a movement disorder. In each case the mother was a vegetarian and the infant was exclusively breast‐fed. In two of the infants, a striking movement disorder consisting of a combination of tremor and myoclonus particularly involving face, tongue, and pharynx appeared 48 h after the initiation of treatment with intramuscular cobalamin. This was associated with marked changes in plasma amino acid levels. Paradoxically, the onset of the movement disorder coincided with overall neurological improvement. The third infant had a persistent focal tremor, which appeared before the commencement of treatment. The movements slowly abated during a 3–6‐week period. The presence of a movement disorder in cobalamin deficiency has received less attention than other features, but in a mild form is probably common. It may offer an early clue to the diagnosis before the onset of profound neurological deterioration. The cause of the severe movement disorder that can appear after treatment is not known.
Clinical, polysomnographic, and genetic characteristics of restless legs syndrome: A study of 133 patients diagnosed with new standard criteria
Authors: Montplaisir Jacques , Boucher Sylvie , Poirier Gaétan , Lavigne Gilles , Lapierre Odile , Lespérance Paul 
Published: May 2005

AbstractOne hundred thirty‐three cases of restless legs syndrome (RLS), diagnosed with criteria recently formulated by an international study group, were studied by questionnaire and with all‐night polysomnographic recordings. Results show that RLS starts at a mean age of 27.2 years and before age 20 in 38.3% of patients. Symptoms often appear in one leg only and also involve upper limbs in about half of all cases. Most patients (94%) report sleep‐onset insomnia or numerous nocturnal awakenings due to RLS symptoms. A strong relationship was found between these complaints and polysomnographic findings; increasing sleep latency and number of awakenings and decreasing sleep efficiency were associated with worsening symptoms. Periodic leg movements in sleep (index > 5 movements/h sleep) were found in 80.2% of patients. This study shows that this percentage is increased when 2 recording nights are considered (most severe score). Eighty patients of 127 (63%) reported the presence of RLS in at least one of their first‐degree relatives. In these families, 221 of 568 first‐degree relatives (39%) were reported by the patients to be affected with RLS.
The effect of the catecholamine precursor L‐Dopa on sleep bruxism: A controlled clinical trial
Authors: Lobbezoo F. , Lavigne G. J. , Tanguay R. , Montplaisir J. Y. 
Published: May 2005

AbstractThe putative role of the dopaminergic system in sleep bruxism (SB) was studied in a double‐blind clinical trial by using low doses of short‐term L‐dopa in combination with benserazide. We recorded 10 patients with SB in our sleep laboratory for 3 consecutive nights. The first night was for habituation to the laboratory environment. During the second and the third nights, the patients received two doses of either L‐dopa or a placebo in a crossover fashion: the first dose 1 h before bedtime and the second, 4 h after the first one. The order of administration was reversed in half the patients. The efficacy of L‐dopa was analyzed by using multilevel models. L‐Dopa resulted in a significant decrease in the average number of bruxism episodes per hour of sleep, as well as in a significant reduction in the average value of the root‐mean‐square (RMS) electromyography (EMG) level per bruxism burst. This indicates that L‐dopa exerts an attenuating effect on SB. In addition, L‐dopa caused a reduction in the variance in RMS values, which suggests that L‐dopa normalizes the EMG activity patterns associated with SB.
The EDB Test—A clinical test for the detection of antibodies to botulinum toxin type A
Authors: Kessler Kirn R. , Benecke Reiner 
Published: May 2005

AbstractApproximately 5 to 10% of patients with cervical, segmental or multifocal dystonia receiving repetitive local injections with botulinum toxin A (BTX A) are estimated to develop secondary loss of treatment benefit (nonresponding) because of the formation of circulating serum antibodies against the neurotoxin. Because other reasons may account for loss of benefit during the course of treatment, the group of secondary nonresponders because of antibody formation need to be separated from the antibody‐negative group by appropriate testing. We present an easy clinical antibody test based on a test injection of BTX A into an indicator muscle, the extensor digitorum brevis (EDB), combined with amplitude measurements of compound muscle action potentials (CMAPs) elicited by electrical nerve stimulation of the peroneal nerve before and after the injection. The results show that in a group of clinically defined secondary nonresponders, who were serologically proven to be antibody negative, a marked decrease in CMAP amplitude can consistently be detected in the injected EDB 4 weeks after BTX A injection. In contrast, antibody‐positive patients are characterized by a lack of such decrease in amplitude. In all cases, results of the EDB test were in keeping with the standard mouse bioassay test. We conclude that the EDB test is a useful tool for clinicians faced with the question of whether a secondary nonresponding patient has in fact developed antibodies to BTX A.
Short‐term levodopa test assessed by movement time accurately predicts dopaminergic responsiveness in Parkinson's disease
Authors: Zappia Mario , Montesanti Rita , Colao Rosanna , Branca Damiano , Nicoletti Giuseppe , Aguglia Umberto , Quattrone Aldo 
Published: May 2005

AbstractShort‐term challenges with dopaminergic agents are used in patients with idiopathic Parkinson's disease (IPD) to predict the therapeutic effect of sustained levodopa treatment, but false‐negative results often occur. We prospectively evaluated 74 patients with clinically diagnosed IPD and compared the predictive value of a short‐term levodopa test assessed by movement time (MT) with the predictive value obtained by the evaluation with the motor examination part of the Unified Parkinson's Disease Rating Scale (UPDRS‐ME). The response to long‐term levodopa was accurately predicted in 96% of patients by assessing the response to the short‐term test with MT and in 80% of cases with UPDRS‐ME. Similar predictive values were obtained by separately analyzing 21 de novo patients. The short‐term test also accurately predicted the magnitude of improvement with long‐term treatment. We conclude that the predictive value for long‐term dopaminergic responsiveness may be further enhanced by evaluating the short‐term pharmacologic challenges with MT analysis. This is particularly useful to select de novo patients for drug trials with dopaminergic agents.
Ophthalmoplegia and leg myorhythmia in Whipple's disease: Report of a case
Authors: Rajput A. H. , McHattie J. D. 
Published: May 2005

AbstractWhipple's disease (WD) is a rare disorder that is more common in males than females. Progressive supranuclear ophthalmoplegia (SNO) in conjunction with oculomasticatory myorhythmia (OMM) or oculofacioskeletal myorhythmia are characteristic movement abnormalities when WD involves the nervous system. Limb myorhythmia without facial or ocular myorhythmia has not been reported in WD. We report such a case who had SNO and leg myorhythmia but no facial or ocular myorhythmia. She had onset of WD at age 28 and 16 years later developed SNO and leg myorhythmia. The neurological manifestations did not respond to antimicrobial agents or to the drugs used for parkinsonism or essential tremor. Valproate produced a remarkable improvement in leg myorhythmia, but the efficacy declined after 3 months. Because WD may manifest as a neurological disorder without gastrointestinal symptoms, all SNO cases, with or without OMM, and those with skeletal myorhythmia should be suspected of WD. These patients should be treated vigorously and followed carefully since neurological involvement is the most disabling feature and it has a propensity to relapse.
Cerebellar presentation of multiple system atrophy
Authors: Wenning G. K. , Kraft E. , Beck R. , Fowler C. J. , Mathias C. J. , Quinn N. P. , Harding A. E. 
Published: May 2005

AbstractEarly diagnosis of multiple‐system atrophy (MSA) is important in patients presenting with late‐onset cerebellar ataxia because it has a less favourable prognosis than other degenerative ataxic disorders. We report cerebellar presentation of MSA in a series of 16 patients, 3 of whom later developed parkinsonism. Two‐thirds of them had early evidence of impaired postural reflexes with a history of recurrent falls. Some of these had a narrow‐based, unsteady gait, unlike the more classic broad‐based gait ataxia of cerebellar disease. On review of the patients' histories, genitourinary dysfunction (particularly impotence) was present at the onset of, or preceding, cerebellar ataxia in 60% of patients, but this had often been attributed to age, or to urological or gynaecological causes. External striated anal or urethral sphincter electromyography (EMG) demonstrated features of chronic denervation and reinnervation in 14 (93%) of 15 patients, consistent with degeneration in Onuf's nucleus as occurs in MSA. Autonomic function tests were abnormal in 9 (64%) of 14 patients. Our data suggest that close enquiry into genitourinary function and analysis of the gait disorder can be useful pointers to a diagnosis of MSA in patients with an unexplained adult‐onset progressive cerebellar syndrome, and that sphincter EMG is the most useful investigation in this context.
Multiple system atrophy: A review of 203 pathologically proven cases
Authors: Wenning G. K. , Tison F. , ben Shlomo Y. , Daniel S. E. , Quinn N. P. 
Published: May 2005

AbstractWe report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa‐induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the latter. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.
Mechanism of action of clozapine‐induced modification of motor behavior in an animal model of the “super‐off” phenomenon
Authors: Dziewczapolski G. , Menalled L. B. , Savino M. T. , Mora M. , Stefano F. J. E. , Gershanik O. 
Published: May 2005

AbstractWe tested the effects of clozapine, an “atypical” neuroleptic with high affinity for the D4 (dopaminergic), and the 5‐HT1c and 5‐HT2 (serotonergic) receptor subtypes on locomotor activity in an animal model of Parkinson's disease showing a bimodal response curve to increasing doses of a D2 agonist. Sulpiride (D2 antagonist) and ritanserin (5‐HT1c and 5‐HT2 antagonist) were used for comparison. The D1 agonist SKF 38393 at a dose of 8 mg/kg significantly reversed the akinesia induced by chronic reserpine treatment (1 mg/kg for 5 days) and alpha‐methyl‐p‐tyrosine pretreatment (300 mg/kg). In this model, the addition of a low dose of a D2 agonist, LY 171555 (quinpirole, 1 μg/kg), inhibited the effects of SKF 38393, whereas the same drug at higher doses (5–50 μg/kg) restored and potentiated the stimulatory response to D1 stimulation. Clozapine inhibited the inhibitory phase and potentiated the stimulatory phase of the curve. Sulpiride inhibited both phases of the dose‐response curve (inhibitory/stimulatory), whereas ritanserin had no effect. We believe these results may reflect a disinhibition phenomenon possibly mediated by the blockade by clozapine of a subpopulation of inhibitory dopa‐mine (DA) receptors belonging to the D2 “family”.
Step initiation in Parkinson's disease: Influence of levodopa and external sensory triggers
Authors: Burleigh‐Jacobs Anne , Horak Fay B. , Nutt John G. , Obeso Jose A. 
Published: May 2005

AbstractWe studied anticipatory postural adjustments contributing to gait initiation deficits in patients with Parkinson's disease (PD) to determine if these deficits could be improved by administration of levodopa or by external stimuli. Ground reaction forces and body kinematics were recorded for self‐generated and cutaneous cue‐triggered step initiation in normal subjects and in PD subjects when OFF and when ON. The effects of assisting anticipatory postural sway with a surface translation coupled with a cutaneous cue were also examined. Decreased force production, decreased velocity of movement, and slowed execution of the anticipatory postural adjustments for self‐generated step characterized step initiation in PD subjects when OFF. These impairments were significantly less evident when the PD subjects were ON. Both PD and normal subjects increased force and velocity of movement when a cutaneous cue was used as a go signal. When subjects voluntarily initiated a step in response to the surface translation, both PD and normal subjects executed the anticipatory postural adjustments for step more rapidly, but the PD subjects, both ON and OFF, failed to increase force to execute push‐off more rapidly. In conclusion, dopaminergic therapy and an external stimulus similarly improve the deficient force production for the anticipatory postural adjustments associated with step initiation in PD. The findings also suggest that force production during the postural adjustment phase of self‐generated, but not externally triggered, step initiation is influenced by dopaminergic pathways.
A dose‐ranging study of selegiline in patients with Parkinson's disease: Effect on platelet monoamine oxidase activity
Authors: Andreu Nathalie , Damase‐Michel Christine , Senard Jean‐Michel , Rascol Olivier , Montastruc Jean‐Louis 
Published: May 2005

AbstractA dose‐ranging study of selegiline was performed in patients with Parkinson's disease to determine the minimal dosage of the drug able to inhibit ≥95% of platelet monoamine oxidase (MAO) activity. Different doses of selegiline (5 or 10 mg daily, 10 or 20 mg weekly) were studied in four groups of six patients with Parkinson's disease. Platelet MAO activity was measured before and after 1 month's treatment with selegiline. The doses of 5 or 10 mg daily and 20 mg (i.e., 10 mg × 2) weekly induced a complete inhibition of platelet MAO‐B activity from day 7 to day 28 (96.0–99.5%). In contrast, platelet MAO‐B inhibition was only 75.9% of the basal value after a dosage of 10 mg weekly. These results demonstrate that 20 mg weekly is the minimal dosage of selegiline able to induce a maximal and long‐lasting inhibition of platelet MAO‐B activity in patients with parkinsonism. Further clinical trials are needed to investigate the clinical efficacy of this dose.
Unchanged basal ganglia N‐acetylaspartate and glutamate in idiopathic Parkinson's disease measured by proton magnetic resonance spectroscopy
Authors: Clarke C. E. , Lowry M. , Horsman A. 
Published: May 2005

AbstractThe lentiform nucleus of five patients with idiopathic Parkinson's disease (IPD) was studied by quantitative magnetic resonance spectroscopy (MRS), both before and after administration of apomorphine, and the spectra were compared with those from a group of age‐matched normal subjects. The concentrations of the three major metabolites, choline, creatine, and N‐acetylaspartate (NAA), were quantified using tissue water content as an internal concentration reference. Glutamate concentration was assessed as the (glutamate + glutamine; GLX)/creatine peak area ratio. In normal subjects, the mean ± SD concentrations of the three metabolites were 2.4 ± 0.4 μmol/g wet wt for choline 11.5 ± 0.8 μmol/g for creatine, and 14.7 ± 2.8 μmol/g for NAA. The Glx/creatine ratio was 1.26 ± 0.12. There was n significant difference in these parameters in the lentiform nucleus of patients with IPD either before or after apomorphine. The absence of detectable differences in IPD in this study implies that the changes in glutamate metabolism in the basal ganglia predicted by animal work are more subtle than those currently observable by MRS.
Sensory and motor evoked potentials in multiple system atrophy: A comparative study with Parkinson's disease
Authors: Abbruzzese Giovanni , Marchese Roberta , Trompetto Carlo 
Published: May 2005

AbstractSomatosensory evoked potentials (SEPs) to median nerve stimulation and motor evoked potentials to transcranial magnetic stimulation (TMS) of the motor cortex were studied in 15 patients with multiple system atrophy (MSA) and compared with matched groups of 20 patients with idiopathic Parkinson's disease (PD) and of 20 normal controls (NCs). No SEP latency or amplitude abnormalities were observed, and, in particular, the frontal N30 component was not significantly depressed. No differences in TMS threshold for evoking responses in relaxed or active thenar muscles were observed. The mean central motor conduction time was normal for the biceps brachii and opponens pollicis muscles and prolonged in the MSA group for the tibilais anterior muscle. Recording SEPs is not useful to differentiate MSA from PD, while the presence of central motor conduction abnormalities may bring into question the diagnosis of idiopathic PD.
Comparison of swallowing function in Parkinson's disease and progressive supranuclear palsy
Authors: Johnston Brain T. , Castell June A. , Stumacher Sharon , Colcher Amy , Gideon R. Matthew , Li Qun , Castell Donald O. 
Published: May 2005

AbstractDysphagia is common in both Parkinson's disease (PD) and progressive supranuclear palsy (PSP). Although it is believed to be more common in PSP, there are no controlled data and no comparison of swallowing function between these two disorders. Our aim was to assess dysphagia and swallow function in patients with PSP and PD. Seven patients with PSP were matched to seven patients with PD on the basis of disease duration. Self‐rated dysphagia, movement disorder disability, modified barium swallow results, and abnormalities noted on manometry of the lower esophageal sphincter, esophageal body, upper esophageal sphincter, and pharynx were compared between the two groups. Neither severity nor duration of dysphagia differed between the two groups. Patients with PSP had a significantly greater degree of disability [median (range) Hoehn & Yahr score, 4 (3–5) vs. 2 (1–2); p < 0.002]. Manometric abnormalities were similar for the two groups. Oral‐phase abnormalities on modified barium swallow were significantly more frequent in PSP (four patients with PSP vs. no patients with PD; p < 0.005). Pharyngeal abnormalities did not differ. Modified barium‐swallow scores correalated well with self‐reported dysphagia severity for patients with PSP (r = 0.93; p < 0.05) but not for those with PD (r = 0.42; p = NS). The frequency of abnormalities noted during the oral phase was significantly increased in PSP. It is hypothesized that the sensory information conveyed due to this may account for the better correlation between symptoms and swallowing abnormalities and the belief that swallowing problems are more common in PSP.