bluuebunny/crossref_metadata_2025_split huggingface dataset:
Motor response to levodopa and the evolution of motor fluctuations in the first decade of treatment of Parkinson's disease
Authors: McColl Craig D. , Reardon Katrina A. , Shiff Mark , Kempster Peter A. 
Published: December 2002

AbstractThirty‐four patients with Parkinson's disease were followed for a mean period of 8 years from the time of initiation of levodopa medication. Levodopa response was charted from the starting point of pharmacological treatment to give a longitudinal point of view of the changes that evolve as the disease progresses. Objective measurements of the motor response to levodopa test‐doses were made at approximately three yearly intervals. Motor fluctuations developed in 58% of the patient group after a mean treatment period of 35 months. Dyskinesia developed in parallel with fluctuations but appeared on average 7 months before symptomatic wearing‐off effects of levodopa doses. The patients with motor fluctuations had significantly better responses to levodopa. By contrast, nonfluctuators were more prone to develop increasing midline motor disability affecting speech, gait and balance. Comparison of test‐dose and pretreatment scores suggested that a substantial long‐duration response to levodopa remains after many years of treatment, and that lateralized motor deficits show a stronger long duration response than midline ones. Motor fluctuations are a consequence of disease progression but their early development is, on balance, associated with better long‐term functional ability because these patients have the greater capacity to respond to pharmacological treatment. © 2002 Movement Disorder Society
Switching of movement direction is central to parkinsonian bradykinesia in sit‐to‐stand
Authors: Mak Margaret K.Y. , Hui‐Chan Christina W.Y. 
Published: December 2002

AbstractPatients with Parkinson's disease (PD) are known to manifest slowness in movements. We sought to identify the particular kinematic and kinetic disorders that contribute to the slowness in performing sit‐to‐stand in these patients. Two inter‐related studies were carried out. In the first study, 20 patients with PD and 20 control subjects were instructed to perform sit‐to‐stand at a natural speed. In the second study, 15 control subjects were instructed to simulate the slower speed of sit‐to‐stand of the patients identified in the first study. Kinematic and kinetic data were recorded by a PEAK motion analysis system and two force platforms. The results showed that patients with PD generated smaller peak horizontal and vertical velocities during the task. They took a longer time to complete each individual phase as well as the whole movement of sit‐to‐stand. Patients also produced smaller peak hip flexion and ankle dorsiflexion joint torques and had prolonged time‐to‐peak torques from sit‐to‐stand onset. When control subjects simulated the patients' speed of sit‐to‐stand, there was no difference in all the kinematic and kinetic data between groups. The only exception was that they exhibited a shorter transition time between peak horizontal velocity (flexion phase) and seat‐off (extension phase) than the patients. This study demonstrated that the slowness of PD patients during sit‐to‐stand at a natural speed could be attributed to inadequate peak hip flexion and ankle dorsiflexion torques, a prolonged torque production, as well as a difficulty in switching from the flexion to extension direction during sit‐to‐stand. As the latter difficulty persisted when the control subjects performed the task at a speed similar to that of the patients, our findings suggest that a fundamental problem of patients with Parkinson's disease could be a switch between movement directions. © 2002 Movement Disorder Society
Selective loss of pyramidal neurons in the pre‐supplementary motor cortex in Parkinson's disease
Authors: MacDonald Virginia , Halliday Glenda M. 
Published: December 2002

AbstractThe nonprimary motor cortices have not previously been studied in Parkinson's disease, despite the selective pattern of dysfunction observed in these regions. In particular, the pre‐supplementary motor region is consistently underactive, with successful treatments correlating with increased excitatory drive to nonprimary motor regions. This finding could suggest a primary cortical abnormality in the pre‐supplementary motor area (pre‐SMA) in Parkinson's disease. We analysed and compared neuronal number in the pre‐SMA and dorsolateral premotor cortical regions in 5 cases of Parkinson's disease and 5 controls. For each cortical region, the total neuronal number as well as the estimated numbers of subpopulations of interneurons and pyramidal neurons was quantified using previously published unbiased techniques. The results showed a significant loss of cortico–cortical projecting pyramidal neurons in the pre‐SMA with no loss of other pyramidal neurons or interneurons either in this region or in the dorsolateral premotor region. These findings indicate a highly selective loss of pyramidal cells in the pre‐SMA in Parkinson's disease, consistent with previous imaging findings in this disease. Our results implicate the degeneration of the premotor projection from the pre‐SMA, along with dopaminergic basal ganglia dysfunction, in the pathogenesis of Parkinson's disease. © 2002 Movement Disorder Society
Progressive delayed‐onset dystonia after cerebral anoxic insult in adults
Authors: Kuoppamäki Mikko , Bhatia Kailash P. , Quinn Niall 
Published: December 2002

AbstractThe basal ganglia, especially the globi pallidi (GP), are highly vulnerable to generalized cerebral anoxia/hypoxia. We report on 2 new cases with delayed‐onset generalized dystonia due to cerebral anoxia. The onset of dystonia in both of our patients was delayed by about 2 months. In both cases, the unusual feature was the progressive worsening and the spread of dystonia over many years after delayed onset. Dystonia progressed for 16 years in Case 1 and for 4 years in Case 2. Furthermore, initial magnetic resonance imaging (MRI) scan of Case 1 showed mild changes of the internal capsule sparing the basal ganglia. Years later, in line with clinical progression, the follow‐up MRI scan showed isolated bilateral lesions involving the entire GP. MRI scans in Case 2 showed bilateral lesions of caudate and lentiform nuclei. There may be several mechanisms underlying delayed and progressive symptoms after time‐limited brain anoxia. We hypothesize that anoxia‐induced excitotoxicity resulting in mitochondrial dysfunction and subsequent apoptosis may explain, at least partly, the delayed‐onset and progressive extrapyramidal syndromes seen in these patients. © 2002 Movement Disorder Society
An imaging study of parkinsonism among African‐Caribbean and Indian London communities
Authors: Hu Michele T.M. , Chaudhuri K. Ray , Jarosz Jozef , Yaguez Lidia , Brooks David J. 
Published: December 2002

AbstractWe previously reported on 131 parkinsonian patients of African‐Caribbean and Indian origin attending movement disorders clinics in six London Hospitals, of whom approximately 20% manifested atypical parkinsonism with a late‐onset, akinetic‐rigid predominant syndrome, postural instability and minimal resting tremor refractory to levodopa therapy and dopamine agonists (see Hu et al., Neurology 2000;54[Suppl.3]: A188 and Hu et al., Mov Disord 2000;15[Suppl.3]:S212). To better elucidate the phenotype of these atypical patients 18FDG/18F‐dopa positron emission tomography (PET) were performed in a subgroup to look for cortical and striatal metabolic changes suggestive of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or dementia with Lewy bodies. Magnetic resonance imaging (MRI) rating of cerebral vascular lesion load, putaminal atrophy, and neuropsychological testing were also performed. Discriminant function analysis of 18F‐dopa/18FDG striatal metabolism in 43 patients failed to separate atypical ethnic minority from typical Caucasian Parkinson's disease (PD) patients. Additionally, atypical Indian and African‐Caribbean patients did not show cortical reductions in glucose metabolism suggestive of PSP, CBD, or DLB. Cerebral vascular lesion load rated in these patients did not differ between atypical and typical PD groups, and none of the atypical patients had MRI changes suggestive of MSA or PSP. Our results suggest the atypical parkinsonian phenotype seen in African‐Caribbean and Indian patients represents a levodopa‐refractory form of PD separate from MSA or PSP in most patients. © 2002 Movement Disorder Society
Beginning‐of‐dose and rebound worsening in MPTP‐treated common marmosets treated with levodopa
Authors: Kuoppamäki Mikko , Al‐Barghouthy Ghassan , Jackson Michael , Smith Lance , Zeng Bai‐Yun , Quinn Niall , Jenner Peter 
Published: December 2002

AbstractA wide range of motor fluctuations develop in Parkinson's disease (PD) patients after prolonged levodopa (L‐dopa) treatment, but few experimental models exist in which these can be investigated. We report on motor fluctuations occurring in MPTP‐treated common marmosets (Callithrix jacchus) treated repeatedly with L‐dopa. All animals showed an improvement in motor function in response to L‐dopa, and rapidly developed peak‐dose dyskinesia. During the period of L‐dopa action, brief periods of immobility were occasionally observed. After acute L‐dopa challenge, animals exhibited a worsening of motor function before improvement, and after the beneficial response to L‐dopa declined, motor performance showed rebound worsening to below‐baseline values. Before L‐dopa challenge and during wearing‐off and rebound worsening, leg dystonias were observed. Although these findings cannot necessarily be generalized to all MPTP‐treated nonhuman primates, they demonstrate that MPTP‐treated marmosets show a range of different motor fluctuations analogous to those seen in PD patients chronically treated with L‐dopa. Therefore, 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP)‐treated primates can provide a model in which the pathophysiology of treatment complications can be investigated. © 2002 Movement Disorder Society
Steele‐Richardson‐Olszewski syndrome in a patient with a single C212Y mutation in the parkin protein
Authors: Morales Blas , Martínez Armando , Gonzalo Isabel , Vidal Lidice , Ros Raquel , Gomez‐Tortosa Estrella , Rabano Alberto , Ampuero Israel , Sánchez Marina , Hoenicka Janet , García de Yébenes Justo 
Published: December 2002

AbstractSteele‐Richardson‐Olszewski syndrome (SROS) is a neurodegenerative disorder of unknown aetiology, most frequently sporadic. Familial cases of SROS have been described. An intronic polymorphism of the tau gene is associated with sporadic SROS and mutations of the tau gene are present in atypical cases of SROS. The role of tau has been excluded in other families with pathology proven SROS, suggesting that this syndrome may have multiple causes. An 82‐year‐old patient, father of 3 children with autosomal recessive juvenile parkinsonism due to combined heterozygous mutations of the parkin gene, developed clinical features of SROS 2 years before death. The diagnosis was confirmed by pathology. He carried the C212Y mutation of the parkin gene and was homozygous for the A0 polymorphism and for the H1 haplotype. The role of parkin in the processing of tau is discussed. © 2002 Movement Disorder Society
Phenomenology of “Lubag” or X‐linked dystonia–parkinsonism
Authors: Evidente Virgilio Gerald H. , Advincula Joel , Esteban Raymund , Pasco Paul , Alfon Jhoe Anthony , Natividad Filipinas F. , Cuanang Joven , Luis Amado San , Gwinn‐Hardy Katrina , Hardy John , Hernandez Dena , Singleton Andrew 
Published: December 2002

AbstractX‐linked dystonia–parkinsonism (XDP), or Lubag syndrome, is known to cause progressive dystonia, with or without parkinsonism, among Filipino male adults with maternal roots from the Philippine island of Panay. We present cinematographic material of 11 cases of Lubag carrying the XDP haplotypes who manifest with a wide spectrum of movement disorders, including dystonia, tremor, parkinsonism, myoclonus, chorea, and myorhythmia. Because of overlapping features, Lubag patients are commonly misdiagnosed as idiopathic dystonia, essential tremor, Parkinson's disease, or Parkinson's‐plus syndromes. Thus, it is imperative to elicit an exhaustive family history in any Filipino male adult who presents with a movement disorder. © 2002 Movement Disorder Society
Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease
Authors: Lefaucheur Jean‐Pascal , Bachoud‐Levi Anne‐Catherine , Bourdet Catherine , Grandmougin Thierry , Hantraye Philippe , Cesaro Pierre , Degos Jean‐Denis , Peschanski Marc , Lisovoski Fabrice 
Published: December 2002

AbstractAssessment programs recently designed to follow‐up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II). Electrophysiological studies comprised the recording of palmar sympathetic skin responses (SSRs), blink reflexes (BRs), thenar long latency reflexes (LLRs), cortical somatosensory evoked potentials (SEPs), and electromyographic silent periods evoked by transcranial magnetic stimulation (SPs). Results were analyzed with reference to disease duration and staging and to specific cognitive, psychiatric, and motor alteration. SEPs were the most and very sensitive markers, because they were abnormal in 94% of patients. Except for LLRs, alteration of electrophysiological results increased in parallel to the evolution of the disease. Except for LLRs and SSR latency, electrophysiological results correlated with those of specific clinical examinations. In particular, an increased BR latency or a reduced amplitude of the N20 component of SEPs correlated with the extent of bradykinesia, whereas a reduced amplitude of SSRs or of the N30 component of SEPs correlated with hyperkinesia. Overall, electrophysiological tests, in particular SEPs and BRs, appeared sensitive and interesting in the follow‐up of HD patients and correlated with various clinical parameters, suggesting that these easy to perform and noninvasive repeatable examinations could be added fruitfully to the assessment programs for HD. © 2002 Movement Disorder Society
Parkinson's disease and dementia with Lewy bodies: One disease or two?
Authors: Richard Irene Hegeman , Papka Michelle , Rubio Ana , Kurlan Roger 
Published: December 2002

AbstractParkinson's disease (PD) and dementia with Lewy bodies (DLB) have clinical features in common and are both characterized neuropathologically by the presence of Lewy bodies (LBs). We conducted a clinicopathological correlation pilot study to better understand whether PD and DLB represent two distinct nosological entities or rather exist along the spectrum of a single LB disease. A neuropathologist blinded to clinical diagnoses evaluated brains with largely pure LB pathology to determine LB distribution and frequency. Research clinicians blinded to LB distribution and frequency determined consensus clinical diagnoses. Clinical features separated cases into two groups, one having features most compatible with PD and the other with DLB. The groups were distinguishable mainly by the time course of clinical symptoms. Although the presence of neocortical LBs was more common in the group of patients with clinical features of DLB, neocortical LBs were also present in 1 member of the PD group and even in the clinically normal control subject. Thus, there appear to be two clinical syndromes, distinguished mainly by the time course of symptoms. The mechanisms responsible for the different clinical presentations are not known, and the issue of whether PD and DLB represent two distinct diseases remains unsettled. © 2002 Movement Disorder Society
Practice effects in three‐dimensional sequential rapid aiming in Parkinson's disease
Authors: Smiley‐Oyen Ann L. , Worringham Charles J. , Cross Carol L. 
Published: December 2002

AbstractOne hypothesized role of the basal ganglia, based largely on findings in Parkinson's disease (PD) patients, is the control of movement sequences. We examined changes in performance with practice of a movement sequence in PD patients and age‐matched controls (n = 8 per group). Subjects practiced a complex three‐dimensional sequential aiming task over 2 days, completing 180 trials, with the goal to minimize response time within specified accuracy limits. The results indicated that both groups became faster in planning the movement, and both groups moved more quickly through the sequence with practice. The PD group's decrease in movement time occurred primarily within the first 45 trials, whereas the control group continued to improve through the first 150 trials. Flight time (time between targets) to a small target decreased with practice in both groups, but flight time to a large target decreased only in the control group. This finding indicates that error corrective processes are more amenable to practice than ballistic processes in people with PD. There was little evidence that either group improved their performance by planning shorter (lower) trajectories, but rather increased mean velocity. Contact time (time on target) decreased with practice in both groups, and there were no group differences. These data indicate that practice can improve performance, both in planning and in specific execution processes. © 2002 Movement Disorder Society
Memory and executive function impairment predict dementia in Parkinson's disease
Authors: Levy Gilberto , Jacobs Diane M. , Tang Ming‐Xin , Côté Lucien J. , Louis Elan D. , Alfaro Brenda , Mejia Helen , Stern Yaakov , Marder Karen 
Published: December 2002

AbstractWe analyzed the association of neuropsychological test impairment at baseline with the development of dementia in idiopathic Parkinson's disease (PD) patients. A cohort of nondemented PD patients from northern Manhattan, NY was followed annually with neurological and neuropsychological evaluations. The neuropsychological battery included tests of verbal and nonverbal memory, orientation, visuospatial ability, language, and abstract reasoning. The association of baseline neuropsychological tests scores with incident dementia was analyzed using Cox proportional hazards models. The analysis controlled for age, gender, education, duration of PD, and the total Unified Parkinson's Disease Rating Scale motor score at baseline. Forty‐five out of 164 patients (27%) became demented during a mean follow‐up of 3.7 ± 2.3 years. Four neuropsychological test scores were significantly associated with incident dementia in the Cox model: total immediate recall (RR: 0.92, 95% CI: 0.87–0.97, P = 0.001) and delayed recall (RR: 0.73, 95% CI: 0.59–0.91, P = 0.005) of the Selective Reminding Test (SRT), letter fluency (RR: 0.87, 95% CI: 0.77–0.99, P = 0.03), and Identities and Oddities of the Mattis Dementia Rating Scale (RR: 0.85, 95% CI: 0.73–0.98, P = 0.03). When the analysis was performed excluding patients with a clinical dementia rating of 0.5 (questionable dementia) at baseline evaluation, total immediate recall and delayed recall were still predictive of dementia in PD. Our results indicate that impairment in verbal memory and executive function are associated with the development of dementia in patients with PD. © 2002 Movement Disorder Society
Clozapine withdrawal symptoms in a Parkinson's disease patient
Authors: Zesiewicz Theresa A. , Borra Sujatha , Hauser Robert A. 
Published: December 2002

AbstractAbrupt clozapine withdrawal can cause rebound psychosis and severe somatic symptoms in psychiatric patients. We report on the case of an advanced Parkinson's disease patient who developed myoclonus, tremor, rigidity, hyperreflexia, and stupor after abrupt clozapine withdrawal. The patient's symptoms resolved with treatment with cyproheptadine. This clinical picture suggests serotonergic rebound as an explanation for the patient's symptoms, although other pharmacological mechanisms are possible. Clozapine should be gradually withdrawn over a period of 1 to 2 weeks when possible, and abruptly discontinued only when necessary. © 2002 Movement Disorder Society
Application of the International Cooperative Ataxia Scale rating in multiple system atrophy
Authors: Tison François , Yekhlef Farid , Balestre Eric , Chrysostome Virginie , Quinn Niall , Wenning Gregor K. , Poewe Werner 
Published: December 2002

AbstractWe assessed the International Cooperative Ataxia Scale (ICARS) as a means of extracting and rating cerebellar signs in multiple system atrophy (MSA). Cross‐sectional analysis of internal consistency, factor structure, and correlation with parkinsonism severity (Unified Parkinson's Disease Rating Scale [UPDRS] III) of the ICARS, in 50 unselected MSA patients (mean age, 67.6 years; mean disease duration, 5.5 years), 50 age‐matched and disease duration‐matched Parkinson' disease (PD) patients, and 50 control subjects. Fifteen patients (30%) had MSA‐C (cerebellar subtype) and 35 (70%) MSA‐P (parkinsonism subtype), and 66% had at least one cerebellar sign. The total ICARS score was much higher (fivefold) in MSA compared to PD patients. The ICARS score was twofold higher in MSA‐C than in MSA‐P patients. MSA‐C patients had a higher score than MSA‐P mainly on posture and gait disturbances and kinetic functions subscores. All the ICARS items were significantly more severe in MSA than in PD patients, who in turn scored higher than the controls. In MSA, internal consistency was excellent (Cronbach = 0.93). Factor structure analysis revealed four clinically distinct subscores, in accordance with the scale structure, which accounted for 70% of the variance. The ICARS showed less consistency and accuracy in PD patients; however, the ICARS scores significantly correlated with the UPDRS‐III scores in both MSA and PD patients. The ICARS appears a useful tool to extract and rate the severity of cerebellar signs in MSA; however, it is clearly contaminated by parkinsonian features. © 2002 Movement Disorder Society
A method for measuring cerebral glucose metabolism in vivo by 13C‐NMR spectroscopy
Authors: Cohen David M. , Wei Jingna , O'Brian Smith E. , Gao Xiaolian , Quast Michael J. , Sokoloff Louis 
Published: December 2002

AbstractCurrent methods for estimating the rate of cerebral glucose utilization (CMRglc) typically measure metabolic activity for 40 min or longer subsequent to administration of [13C]glucose, 2‐[14C]deoxyglucose, or 2‐[18F]deoxyglucose. We report preliminary findings on estimating CMRglc for a period of 15 min by use of 2‐[6‐13C]deoxyglucose. After a 24‐hr fast, rats were anesthetized, infused with [1‐13C]glucose for 50 min, and injected with 2‐[6‐13C]deoxyglucose (500 mg/kg). During the subsequent 12.95 min the estimated value of CMRglc was 0.6 ± 0.4 micromol/min/g (mean ± SD, N = 7), in agreement with values reported for anesthetized rats studied with the 2‐[14C]deoxyglucose method and other 13C‐NMR methods that measure CMRglc. In rats injected with bicuculline methiodide (a known stimulant of CMRglc), CMRglc increased by more than 75% during 12.95 min following injection of bicuculline (Wilcoxon signed rank test, P = 0.042, N = 8). Magn Reson Med 48:1063–1067, 2002. © 2002 Wiley‐Liss, Inc.