bluuebunny/crossref_metadata_2025_split huggingface dataset:
Abnormalities of tracking behavior in Parkinson's disease
Authors: Hufschmidt Andreas , Lücking Carl‐Herrmann 
Published: December 2004

AbstractTracking behavior in Parkinsonian patients and in a control group of elderly subjects was studied using a one‐dimensional pursuit tracking task. The most obvious abnormality in patients with Parkinson's disease is a tendency to restrict movements to a limited range, thus “clipping” the extreme deviations of the target, reflected by movement arrests, reduced amplitude gain, and decreased velocity gain. Most of the tracking parameters were selectively sensitive to parkinsonian as opposed to age‐related impairment of motor functions, the only exception being directional errors, which were more frequent in the old age group. Movement range and amplitude gain were related to general disability as reflected by the Hoehn and Yahr stage. Amplitude gain was also the single factor with the strongest influence on tracking error and was smaller for large target deviations in both groups. A reduction in target velocity caused a significant increase in amplitude gain, suggesting a compensatory strategy (amplitude/lag trade‐off) as one possible cause of low‐amplitude gain. Decreased peak velocity did not contribute significantly to tracking error. These findings suggest that reduced movement amplitude and slowness are independent phenomena, of which only the former has a significant influence on tracking performance in the task used. Patients with Parkinson's disease showed no impairment of motor learning on this task.
The anterior olfactory nucleus in Parkinson's disease
Authors: Pearce R. K. B. , Hawkes C. H. , Daniel S. E. 
Published: December 2004

AbstractImpaired olfaction occurs in patients with idiopathic Parkinson's disease (PD), and Lewy bodies have been found in the olfactory bulb and tract. We now confirm the latter finding and show that this presence of Lewy bodies is associated with significant neuronal loss. A quantitative study of the anterior olfactory nucleus (AON) was performed in tissue obtained postmortem from seven patients with PD and seven age‐matched controls. Neuronal loss was seen in the PD bulb and tracts (p < 0.01), and a strong correlation of neuronal loss with disease duration was detected (R = −0.87). The presence of Lewy bodies was confirmed with immunocytochemical staining for ubiquitin in all the PD cases.
“Pure” striatonigral degeneration and Parkinson's disease: A comparative clinical study
Authors: Gouider‐Khouja Neziha , Vidailhet Marie , Bonnet Anne‐Marie , Pichon Jacques , Agid Yves 
Published: December 2004

AbstractStriatonigral degeneration (SND) is difficult to diagnose in vivo. The purpose of this study was to detect the best indicators for an early and reliable diagnosis of the disease. Eighteen patients clinically diagnosed as having SND were selected with rigorous inclusion criteria and compared to 18 patients with Parkinson's disease (PD) matched for age and disease duration. Apart from dysautonomia, the principal discriminant clinical features that distinguished SND from PD were the early appearance of the following symptoms and signs: (a) severe and atypical progressive parkinsonism characterized by bilateral bradykinesia and rigidity, slowness of gait, postural instability, and falls, and poor or absent response to adequate levodopa treatment; (b) increased tendon reflexes associated or not with frank pyramidal signs, severe dysarthria, and less consistently, dysphagia, stridor, antecollis, and stimulussensitive myoclonus, which, when present, are highly suggestive of the disease.
Antiparkinsonian therapies and brain mitochondrial complex I activity
Authors: Przedborski Serge , Jackson‐Lewis Vernice , Fahn Stanley 
Published: December 2004

AbstractAlterations in complex I activity, one of the enzymatic units of the mitochondrial respiratory chain, have been demonstrated in different tissues from patients with Parkinson's disease (PD). Subsequently, we showed that the chronic administration of levodopa can cause alterations in mitochondrial respiratory chain activity in rats, which suggests that the observed deficit in complex I activity in PD might be, at least in part, related to chronic levodopa therapy. Our study assessed the in vitro effects of different antiparkinsonian agents on complex I activity in rat brain. As previously reported, both levodopa and dopamine inhibit complex I activity in a dose‐dependent manner. In contrast, the two major metabolites of dopamine, homovanillic acid and 3, 4‐dihydroxyphenylacetic acid as well as 3‐O‐methyl‐dopa, had little or no effect on complex I activities. Bromocriptine, pergolide, trihexyphenidyl, molindone, and clozapine were all without significant inhibitory effects on mitochondrial function. Although vitamin C and deprenyl did not alter complex I activity, they did prevent the inhibitory effect of both levodopa and dopamine on complex I activity. This work indicates that among the different and usual antiparkinsonian agents, only levodopa and dopamine induced reductions in complex I activity. It also indicates that vitamin C and deprenyl are both effective in preventing the levodopa‐induced complex I inhibition. This latter finding provides further support to the use of antioxidants and monoamine oxidase inhibitors as therapeutic strategies in attempts to slow the progression of PD.
Lid opening apraxia is associated with medial frontal hypometabolism
Authors: Smith D. , Ishikawa T. , Dhawan V. , Winterkorn J. S. , Eidelberg D. 
Published: December 2004

AbstractWe used [18F]fluorodeoxyglucose with positron emission tomography to explore the functional basis of lid opening apraxia (LOA). We studied four patients with LOA without associated pyramidal or extrapyramidal motor dysfunction (mean age 65.5 ± 4.7 years). We compared measures of glucose metabolism with those calculated for 10 age‐matched normal volunteers (mean age 60.6 ± 11.4). All four patients demonstrated significant metabolic reductions (>2 SD) in the medial frontal lobe: bilaterally in two, unilaterally in two. Significant abnormal unilateral metabolic reductions in the left basal ganglia were evident in two patients. These findings suggest that medial frontal lobe hypometabolism is associated with LOA. Abnormal striatofrontal interactions may be involved in the pathogenesis of this condition.
Longitudinal evolution of prefrontal leucotomy in Tourett's syndrome
Authors: Kulisevsky Jaime , Berthier Marcelo L. , Avila Asunción 
Published: December 2004

AbstractWe report the case of a 69‐year‐old man with severe Tourette's syndrome (TS) who underwent a bilateral prefrontal leucotomy at the age of 29 years to alleviate coprolalia, copropraxia, and obsessive‐compulsive disorder. No improvement was observed during the 5 years after the leucotomy. Tics and associated behaviorial abnormalities disappeared thereafter, to recur at age 67. Long‐term follow‐up of single cases can help to estimate the role of neurosurgery in the treatment of TS.
Hypokinesia in Parkinson's disease: Influence of age, disease severity, and disease duration
Authors: van Hilten J. J. , Braat E. A. M. , van der Velde E. A. , Middelkoop H. A. M. , van Dijk J. G. , Ligthart G. J. , Roos R. A. C. 
Published: December 2004

AbstractThe aim of this cross‐sectional study was to compare the role of aging in measures reflecting diurnal activity and immobility in 60 parkinsonian patients with predominant features of hypokinesia and rigidity and 100 healthy subjects aged 50 to 98 years. In the patients, we also studied the relation between disease duration and subjective and objective measures of disease severity. Motor acitivity was recorded during 5 successive days at home with a wrist‐worn activity monitor. For each subject, two mean measures reflecting the diurnal activity level and the relative proportion of activity and immobility were calculated. Diurnal measures of activity revealed in both groups a prominent absolute reduction of activity and an increase of the time spent without movement (“immobility”) with advancing age. Parkinsonian patients showed significantly lower values for both motor‐activity measures than did the healthy subjects. The rate of the age‐related decline of both diurnal activity measures in both groups, however, is comparable. Disease duration showed no relation with subjective and objective measures reflecting disease severity. This study shows that if care is taken to control for disease severity, the rate of the age‐related decline of measures reflecting diurnal activity and immobility is similar in both groups. The lack of relation between disease duration and subjective and objective measures of disease severity suggests that the rate of progression of Parkinson's disease can be reliably studied only by means of longitudinal studies.
The blepharospasm disability scale: An instrument for the assessment of functional health in blepharospasm
Authors: Lindeboom R. , De Haan R. , Aramideh M. , Speelman J. D. 
Published: December 2004

AbstractAssessment of the functional status in patients with blepharospasm is of major importance for clinical practice and outcome studies. The Blepharospasm Disability Scale (BDS) is specifically directed to measure the disability in these patients. The metric properties of this instrument were evaluated. Reliability, validity, and responsiveness to within‐patient health changes over time of the BDS were assessed in 40 patients with essential blepharospasm treated with botulinum toxin injections. The reliability of the scale was sufficient for use on group level (Cronbach's α coefficient, 0.69). Evidence of discriminant validity was provided by the difference in median score on the BDS between 21 newly admitted patients and 19 patients already under treatment (p < 0.001). Convergent validity was supported by correlations between BDS and neurological impairment scores (range, Spearman correlation coefficients, 0.65‐0.79). Responsiveness to health changes was demonstrated by a significant difference between median BDS scores before treatment and 2 weeks after treatment with botulinum toxin (p < 0.01). The BDS is a useful disease‐specific instrument to assess disability. Completion of the questionnaire is easy and takes only a few minutes. The instrument is suitable for use in patient care, descriptive outcome studies, and should be considered in controlled clinical trials.
Click‐evoked vestibulocollic reflexes in torticollis
Authors: Colebatch J. G. , Lazzaro V. Di , Quartarone A. , Rothwell J. C. , Gresty M. 
Published: December 2004

AbstractA total of 26 patients with torticollis were studied using a recently developed technique for recording vestibulocollic reflexes form the sternocleidomastid muscles in addition to conventional caloric tests of vestibular function. Previous reports of abnormalities of vestibulo‐ocular reflexes in these patients were confirmed with just fewer than half having significant canal pareses or directional preponderances (nine of 20 tested). In addition, there was a high incidence of abnormal click‐evoked vestibulocollic reflexes (17 of 26 tested), which were not simply the result of prior treatment with botulinum toxin, nor due to unequal levels of muscle activation. In patients never previously treated with botulinum toxin (14 patients), the effect almost always consisted of suppressed responses in the sternocleidomastoid muscle ipsilateral to the direction of head turning. Because responses were not abnormal in all patients tested, and more commonly so in those with a history of torticollis of ≥5 years (eight of nine patients) than in de novo patients, we suggest that the changes are more likely to be compensatory than causal.
Hemiatrophy, juvenile‐onset exertional alternating leg paresis, hypotonia, and hemidystonia and adult‐onset hemiparkinsonism: The spectrum of hemiparkinsonism‐hemiatrophy syndrome
Authors: Lang Anthony E. 
Published: December 2004

AbstractA 45‐year‐old woman with a history of probable perinatal craniocerebral trauma resulting in mild asymptomatic right hemiatrophy developed right leg weakness and hypotonia alternating with dystonia only after prolonged exertion at age 12. At age 27, she developed right‐sided parkinsonism. Exertional paresis and dystonia and parkinsonism responded completely to levodopa; however, she developed a progressive reduction in the duration of action of levodopa over the first 4 years of treatment. Investigations including computed tomography, magnetic resonance imaging, [18F]fluorodopa, and [18F]fluorodeoxyglucose positron emission tomography scans suggested a static lesion involving the left substantia nigra. This unusual exertion‐induced weakness and hypotonia alternating with hypertonia and dystonia has not been reported previously. The role of dopamine deficiency in dystonia and the role of levodopa in the development of fluctuations in Parkinson's disease are discussed. Review of the literature, including this patient, emphasizes the heterogeneity of the syndrome of hemiparkinsonism‐hemiatrophy.
Hemimasticatory spasm in hemifacial atrophy: Diagnostic and therapeutic aspects in two patients
Authors: Ebersbach G. , Kabus C. , Schelosky L. , Terstegge L. , Poewe W. 
Published: December 2004

AbstractWe report two cases of hemimasticatory spasm in association with progressive hemifacial atrophy. On the basis of neurophysiological and magnetic resonance imaging assessments, a peripheral irritation of the trigeminal nerve–probably due to entrapment of the motor branches in the infratemporal fossa–is suggested as the cause of the involuntary movement. Local injections of botulinum toxin type A into the masticatory muscles proved to be a successful treatment in both patients.
Familial tremulous and myoclonic dystonia with white matter changes in brain magnetic resonance imaging
Authors: Bohlega S. , Stigsby B. , Al‐Kawi M. Z. , McLean D. R. , Ozand P. , Omer S. , Coates P. 
Published: December 2004

AbstractWe report two families with a disorder, probably autosomal recessive, characterized by tremor of juvenile onset, dystonia, and myoclonus with preserved cognitive, cerebellar, and peripheral nervous system functions. During 4 years' follow‐up, mild spasticity appeared. Magnetic resonance imaging (MRI) revealed mild diffuse changes in the white matter. Central conduction times for visual, motor, and sensory systems were all prolonged. Extensive metabolic work‐up failed to reveal lysosomal, peroxisomal, mitochondrial, or other metabolic abnormalities.
The epidemiology of Parkinson's disease in the county of Rogaland, Norway
Authors: Tandberg Elise , Larsen Jan P. , Nessler Ernst G. , Riise Trond , Aarli Johan A. 
Published: December 2004

AbstractThe objective of this investigation was to clarify the epidemiology of idiopathic Parkinson's disease (PD) in the county of Rogaland, Norway. Total case ascertainment and a high diagnostic accuracy were attempted through a detailed community study and the use of a new clinical diagnostic classification. The study population comprised 220, 858 inhabitants, and a total of nearly 400 patients was interviewed and examined by a neurologist. On prevalence day, January 1, 1993, 245 patients were included in the study. The diagnostic classification revealed 135 patients with clinically definite, 74 with probable, and 36 with possible PD. The crude prevalence rate was shown to be 110.9 per 100,000 inhabitants. The total age‐adjusted prevalence was calculated to be 102.4 per 100,000 and to 120.9 per 100,000 men and 89.8 per 100,000 women. Among the 245 patients, 28 patients had a tremor‐dominant disease, 50 patients an akinetic‐dominant disease, and 167 patients a mixed clinical pattern of PD. Age‐adjusted prevalence figures were slightly higher for rural compared to urban areas. About 50% of the PD patients were in need of public help, 15% had complaints about pain related to their parkinsonism, and after ∼6 years of levodopa treatment, 20% were suffering from clinical fluctuations. The study showed that 40% of the patients had some degree of thought disorder. The prevalence figures for PD in this study are slightly lower than those reported from most previous prevalence studies with a comparable study design for case finding. This may be due to a careful diagnostic evaluation with the use of specified diagnostic criteria, excluding patients with other parkinsonian syndromes.
No evidence for systemic oxidant stress in Parkinson's or Alzheimer's disease
Authors: Ahlskog J. Eric , Uitti Ryan J. , Low Phillip A. , Tyce Gertrude M. , Nickander Kim K. , Petersen Ronald C. , Kokmen Emre 
Published: December 2004

AbstractOxidant stress secondary to dopamine metabolism has been proposed as a pathogenic factor in the development of Parkinson's disease. Biochemical abnormalities extending beyond the central nervous system have been identified in patients with this condition. Previous investigators have found abnormally elevated concentrations of the lipid peroxidation product, malondialdehyde, in the plasma and serum of patients with Parkinson's disease. We attempted to replicate these findings but controlled for other factors that could influence malondialdehyde levels. We detected no significant elevations in mean serum malondialdehyde concentrations in either levodopatreated or untreated patients with Parkinson's disease, compared to normal controls; similarly, no elevation was found in a group of patients with dementia of Alzheimer's type. On the other hand, a group of subjects with diabetes mellitus but no neurodegenerative disease had significantly elevated mean serum malondialdehyde levels, consistent with previous studies of diabetic patients. Autoxidation is one of the two major routes by which dopamine and dopa metabolism may generate oxygen free radicals. We analyzed the autoxidation product of dopa, 5‐S‐cysteinyl‐dopa, in the plasma of these same groups of patients with neurodegenerative disease and normal controls; no significant differences were identified. Serum concentrations of two other antioxidant substances, α‐tocopherol and uric acid, were also statistically similar in these groups. In conclusion, analysis of several blood products relevant to oxidant stress, including malondialdehyde, 5‐S‐cysteinyl‐dopa, α‐tocophero, and uric acid, failed to distinguish patients with Parkinson's disease or dementia of Alzheimer's type from controls.
Long latency postural reflexes are under supraspinal dopaminergic control
Authors: Bloem Bastiaan R. , Beckley Dennis J. , van Vugt Jeroen P. P. , van Dijk J. Gert , Remler Michael P. , Langston J. William , Roos Raymund A. C. 
Published: December 2004

AbstractScaling of posturally stabilizing long latency (LL) reflexes in tibialis anterior muscles induced by “toe‐up” rotational perturbations is abnormal in standing patients with Parkinson's disease. To investigate the contribution of dopaminergic pathways to abnormal scaling, we studied LL reflexes in 22 patients with selective hypodopaminergic syndromes: 10 psychiatric patients taking chronic neuroleptic medication (7 with mild parkinsonism), 8 patients with young‐onset Parkinson's disease, and 4 patients with MPTP‐induced parkinsonism. Results were compared with those of 10 healthy controls. Stimuli consisted of (a) 10 serial (predictable) perturbations of 4° amplitude, (b) 10 serial (predictable) perturbations of 10° amplitude, and (c) 20 randomly mixed (unpredictable) perturbations of either 4 or 10° amplitude. In normal subjects, LL reflex amplitudes were adapted to match predictable variations in stimulus size, whereas under unpredictable conditions a “default” response emerged that anticipated the 10° perturbation. LL reflex scaling under predictable conditions was intact in patients with neuroleptic‐induced parkinsonism and young‐onset Parkinson's disease, but the large default LL response under unpredictable conditions was absent. In patients with MPTP‐induced parkinsonism, LL reflex scaling was absent during both predictable and unpredictable conditions. We conclude that abnormal scaling of posturally stabilizing LL reflexes is related to decreased supraspinal dopaminergic influence.
Neurological Wilson's disease studied with magnetic resonance imaging and with positron emission tomography using dopaminergic markers
Authors: Westermark K. , Tedroff J. , Thuomas K.‐Å. , Hartvig P. , Långström B. , Andersson Y. , Hörnfeldt K. , Aquilonius S.‐M. 
Published: December 2004

AbstractFour patients with neurological Wilson's disease were investigated using magnetic resonance imaging (MRI) and positron emission tomography (PET). All patients had dystonia as their major clinical manifestation but also had dysarthria and at the presentation of the disease had choreoathetoid movements in at least one limb. A multitracer approach with PET was used to visualize various aspects of dopaminergic function; [11C]‐(+)‐nomifensine (NMF), [11C]raclopride (RAC) and [11C]‐L‐DOPA (one patient). Correlation analysis of RAC and NMF binding as well as putamen/caudate uptake ratios showed corresponding reductions. The patient investigated with [11C]‐L‐DOPA had a normal striatal uptake. Generally, structural changes as shown by MRI corresponded to reductions both in NMF and RAC binding. There was no evident correspondence between PET findings and the severity of clinical symptoms seen in the individual patient. In two patients with discrete neurological impairment at the time of investigation, PET showed serious presynaptic dopaminergic lesions in the putamen. Our data suggest that the striatal degeneration seen in Wilson's disease comprises a complex pathology involving both afferent and efferent projections. The discrete neurological impairment seen in some patients with gross striatal pathology might be due to concomitant lesions in functionally counteracting basal ganglia circuits.
The movement‐related cortical potential is abnormal in patients with idiopathic torsion dystonia
Authors: Van Der Kamp W. , Rothwell J. C. , Thompson P. D. , Day B. L. , Marsden C. D. 
Published: December 2004

AbstractVoluntary movements, such as the self‐paced finger extension task used in the present experiments, are preceded by a slowly rising negative electroencephalographic potential [the movement‐related cortical potential (MRCP)]. The early NS1 component of the potential was no different in patients with primary dystonia affecting the arm (n = 6) compared with matched controls. In contrast, the peak amplitude of the MRCP was smaller in the patients, despite the fact that the movements made by the two groups were very similar; it was of equal size over both left and right hemispheres, rather than being larger on the side contralateral to the movement. These results are similar to those observed by others in patients with symptomatic dystonia secondary to lesions of the basal ganglia or their output pathways and may reflect abnormal basal ganglia input to motor areas of cortex before the onset of a self‐paced movement.