bluuebunny/crossref_metadata_2025_split huggingface dataset:
Quantitative gait analysis in patients with vascular parkinsonism
Authors: Zijlmans J. C. M. , Poels P. J. E. , Duysens J. , van der Straaten J. , Thien T. , van't Hof M. A. , Thijssen H. O. M. , Horstink M. W. I. M. 
Published: December 2004

AbstractUntil now the clinical criteria for the diagnosis of vascular parkinsonism (VP) have been disputed. The purpose of the present study is to investigate whether quantitative gait analysis can differentiate between the gait pattern of patients with VP and the gait pattern of patients with idiopathic Parkinson's disease (PD). Twelve patients with VP, 12 patients with PD, and 10 neurologically nondiseased controls were examined by quantitative gait analysis. Patients with VP, having a similar gait velocity and stride length, showed relatively preserved arm swing with markedly more anteflexion in the shoulder on the forward sway of the arm swing than patients with PD. Patients with VP also showed less flexion dystonic posture of the elbow, hip, knee, and trunk than did patients with PD. There was no significant difference in the excursions and coordination of arm swing in the patients with VP compared with the control group. Both patient groups showed reduced leg movements, reduced hip extension, and reduced knee flexion and extension as compared with the controls.
Characteristics of individuals with Huntington disease in long‐term care
Authors: Nance M. A. , Sanders G. 
Published: December 2004

AbstractThis is a retrospective review of 97 Huntington disease (HD) patients living in long‐term care facilities in the Twin Cities. The purpose of the study was to describe the demographic features, patterns of behavior, weight change, nursing issues medication use, and hospitalization in this population. On admission to the nursing home, the “average” HD patient was of either sex, 45 years old, previously employed, a high school graduate, and not married. One third had severe behavior problems. Half gained and half lost weight; weight loss was not a predictor of death. Almost all used central nervous system‐active drugs, most commonly neuroleptics. Eighty‐four percent were ambulatory on admission, but 88% of those who died were nonambulatory at the time of death. We concluded that (a) HD patients are demographically different from other residents of long‐term care facilities, (b) weight gain can occur in some late‐stage patients, (c) negative behavior is a significant problem but is restricted to a subset of patients, and (d) adapting creatively to increasing multifaceted disability is the greatest challenge to the staff in facilities caring for HD patients.
A controlled trial of idebenone in Huntington's disease
Authors: Ranen Neal G. , Peyser Carol E. , Coyle Joseph T. , Bylsma Frederick W. , Sherr Meeia , Day Leslie , Folstein Marshal F. , Brandt Jason , Ross Christopher A. , Folstein Susan E. 
Published: December 2004

AbstractOne hundred patients with clinically diagnosed Huntington's disease (HD) were randomized to either idebenone, an antioxidant and enhancer of oxidative metabolism, or placebo, in a 1‐year, double‐blind, parallel‐group study aimed at slowing the rate of progression of the disease. Ninety‐one patients completed the study. There were no significant differences between groups on the primary outcome measures of the Huntington's Disease Activities of Daily Living Scale (ADL—an index of functional status) and the Quantified Neurologic Examination (QNE). Sample size calculations based on progression of the ADL and QNE in this study group revealed that a larger study group is necessary to detect any differences less than an almost complete halting of the disease. This argues for multicenter efforts for future therapeutic trials in HD.
Bilateral myoclonus of the trapezius muscles after distal lesion of an accessory nerve
Authors: Glocker Franz X. , Deuschl Günther , Volk Benedikt , Hasse Joachim , Lücking Carl H. 
Published: December 2004

AbstractObservations of rhythmic or semirhythmic myoclonus due to a peripheral nerve lesion are exceptional. We report on a patient with thorax trauma with multiple bilateral hematomas of the paravertebral musculature. Eight years later he developed rhythmic myoclonus of both trapezius muscles and thoracic pain. Infiltration of a paramedially located scar at the level of D5–6 with a local anesthetic agent led to an intermittent relief of the myoclonus as did anesthetic blockade of the left accessory nerve. Surgical excision of the scar, which contained multiple dystrophic axons on histological examination, cured the patient's symptoms as illustrated in a videotape. This indicates that peripheral afferents contributed to the myoclonus. Ephaptic transmission, ectopic excitation, or misdirected neuronal sprouting secondary to the trauma are possible peripheral mechanisms responsible for the movement disorder. Successful blockade of the left accessory nerve with bilateral relief of the symptoms suggests a secondary, more centrally located mechanism, e.g., in the brain stem, probably driven by an altered afferent input. It is concluded that rhythmic or semirhythmic and focal myoclonus need a careful workup to look for a peripheral cause because such a condition would be accessible for surgical treatment.
A new sublingual formulation of apomorphine in the treatment of patients with Parkinson's disease
Authors: van Laar T. , Neef C. , Danhof M. , Roon K. I. , Roos R. A. C. 
Published: December 2004

AbstractA new formulation of a sublingual tablet with 10 mg apomorphine was examined in 13 patients with Parkinson's disease. Vitamin C (250 mg) was added sublingually to lower the salivary pH. Four patients received sublingual apomorphine and nine received sublingual apomorphine as well as vitamin C. Subcutaneous apomorphine was given to all patients. The study was designed as a randomized three‐way cross‐over study. Tmax, Cmax, and bioavailability (F) were determined. Clinical efficacy was assessed by hand‐tapping during 30 s, walking time over 25 m, and a 4‐point tremor score. The mean Tmax after subcutaneous apomorphine was 14.5 ± 1.9 min with a mean Cmax of 19.2 ± 3.8 ng/ml. The mean clearance of all paients was 3.8 ± 0.6 L/min. The mean Tmax after sublingual apomorphine was 61.1 ± 6.9 min vs. 61.7 ± 8.2 min with vitmin C. The mean Cmax was 7.4 ± 1.0 ng/ml (– vitamin C) vs. 4.3 ± 1.3 ng/ml (+ vitamin C). These data resulted consequently in a not significantly different mean biovailability, varying from 17.6% (– vitamin C) to 6.1% ( + vitamin C). The latency of onset of clinical efficacy varied between 25.0 ± 8.5 min ( – vitamin C) and 26.0 ± 5.3 min ( + vitamin C). The duration of effect was lower (not significantly) when vitamin C was added: 88.0 ± 12.5 min ( – vitamin C) vs. 61.0 ± 11.9 min ( + vitamin C). These data show that 10 mg apomorphine sublingually was effective in 56% of the patients. The combination with vitamin C did not significantly change the latency of noset or duration of clinical efficacy. Sublingual apomorphine should be considered as an alternative in the treatment of “off” ‐periods in Parkinson's disease, in particular when patients have the capacity to anticipate their off‐periods.
Classical eyeblink conditioning in Parkinson's disease
Authors: Daum Irene , Schugens Markus M. , Breitenstein Caterina , Topka Helge , Spieker Sybille 
Published: December 2004

AbstractPatients with Parkinson's disease (PD) show impairments of a range of motor learning tasks, including tracking or serial reaction time task learning. Our study investigated whether such deficits would also be seen on a simple type of motor learning, classic conditioning of the eyeblink response. Medicated and unmediated patients with PD showed intact unconditioned eyeblink responses and significant learning across acquisition; the learning rates did not differ from those of healthy control subjects. The overall frequency of conditioned responses was significantly higher in the medicated patients with PD relative to control subjects, and there was also some evidence of facilitation in the unmedicated patients with PD. Conditioning of electrodermal and electrocortical responses was comparable in all groups. The findings are discussed in terms of enhanced excitability of brainstem pathways in PD and of the involvement of different neuronal circuits in different types of motor learning.
Amyloid β‐peptide and the dementia of Parkinson's diease
Authors: Jendroska Klaus , Lees Andrew J. , Poewe Werner , Daniel Susan E. 
Published: December 2004

AbstractIt is not known whether an increased incidence of dementia in patients with Parkinson's disease (PD) is due to a higher incidence of Alzheimer's disease (AD) or to “early” Alzheimer‐type pathology. To determine whether amyloid β‐peptide (Aβ) of AD occurs more frequently in brains of patients with PD, we examined 50 cases and 79 controls by using histoblots for Aβ. Twentythree cases with PD had dementia, including all nine with Aβ distributed throughout the entire cerebral cortex; three of these cases had AD. In contrast, five of 17 controls with comparable Aβ accumulation were not demented. Neither AD nor Aβ deposition was increased in PD; furthermore, there was no statistical correlation between the amount of Aβ and the number of Lewy bodies in cerebral cortex. In 14 patients with PD in whom dementia was unrelated to Aβ, there was cerebral vascular disease (four), numerous cortical Lewy bodies (three), or hydrocephalus (two); in five further cases, dementia was not well explained by histopathologic changes. Our data found no increase of either AD or “early” Alzheimer‐type pathology in cases of PD; however, a synergistic effect between the two pathologies was suggested as contributing to dementia.
Focal task‐specific tremors
Authors: Soland Valerie L. , Bhatia Kailash P. , Volonte Maria A. , Marsden C. David 
Published: December 2004

AbstractFocal task‐specific tremors besides primary writing tremor have rarely been reported in the literature. We describe inne cases of focal tremors induced by different specific tasks, involving a repetitive and frequently performed movement. Only one of these patients had a family history of any similar disorders. There was no overt dystonia in any of the cases, but these tremors may be forms of focal dystonia, rather than a manifestation of essential tremor.
Evidence of cortical metabolic dysfunction in early Huntington's disease by single‐photon‐emission computed tomography
Authors: Sax Daniel S. , Powsner Rachel , Kim Anthony , Tilak Shripad , Bhatia Rita , Cupples L. Adrienne , Myers Richard H. 
Published: December 2004

AbstractWe compared perfusion of prefrontal, motor, and sensory cortices and basal ganglia in 29 Huntington's disease (HD) patients and nine controls. We found a significant reduction in perfusion in patients with HD of short (<6 years, n=10), medium (6–10 years, n=8), and long duration (>10 years, n=11) compared with controls. Among short‐duration patients, we observed decreases in cortical perfusion before evidence of atrophy on magnetic resonance imaging, suggesting that decreases in neuronal activity, as reflected by perfusion levels, precede gross structural changes. As expected, decreased perfusion was marked in basal ganglia. The extent of cortical perfusion correlated with clinical assessments of functional capabilities as well as with the duration of disease. Perfrontal perfusion correlated with cognitive measures, and motor cortical perfusion correlated with physical disability and activities of daily living scores. We found no significant clinical correlations with sensory cortical perfusion. Single‐photon‐emission computed tomography may be a sensitive method for assessing disease progression in clinical trials and pharmacologic intervention.
Saccadic eye movements in idiopathic blepharospasm
Authors: Bollen E. , Van Exel E. , van der Velde E. A. , Buytels P. , Bastiaane J. , van Dijk J. G. 
Published: December 2004

AbstractVisually guided saccades, memory‐contingent saccades, and antisaccades were studied with an infra‐red reflection technique in 12 patients with idiopathic blepharospasm and in controls. Latencies of the three kinds of saccades were prolonged. Peak velocities and gains did not differ. Our results indicate an initiation defect in the saccadic system, which may be localized in the caudate nucleus.
Abnormal sensorimotor integration in writer's cramp: Study of contingent negative variation
Authors: Ikeda Akio , Shibasaki Hiroshi , Kaji Ryuji , Terada Kiyohito , Nagamine Takashi , Honda Manabu , Hamano Toshiaki , Kimura Jun 
Published: December 2004

AbstractTo investigate the pathophysiology of idiopathic focal dystonia, we recorded contingent negative variation (CNV) as a physiological index of sensorimotor intergration in 14 right‐handed patients with writer's cramp and compared it with normative data. CNV was recorded in the S2 choice reaction time paradigm: Two kinds of auditory S2 (Go or No‐Go) were given 2 s after the auditory S1, and the subject reacted only to the Go signal by extending the wrist. In normal subjects, the late CNV amplitude had no difference at Cz between left‐and right‐hand tasks and was symmetric without significant amplitude laterality irrespective of the side of hand movement. In patients with writer's cramp, the late CNV amplitude at Cz was relatively larger for the left‐hand task than the right‐hand task (p<0.03), and a significant amplitude laterality of the late CNV (larger on the right) was present especially at the central area for the right‐hand task (p<0.03). Since the late CNV is known to be generated, at least inpart, from primary and supplementary motor cortices, the significant laterality of the late CNV seen with the right‐hand task in patients with writer's cramp might represent functional abnormality of motor cortices possibly as the result of basal ganglia function.
Blink reflex excitability is abnormal in patients with periodic leg movements in sleep
Authors: Briellmann Regula S. , Rösler Kai M. , Hess Christian W. 
Published: December 2004

AbstractThe excitability of the late component of the blink reflex was measured in 19 patients with periodic leg movements in sleep (PLMS) and in 18 control patients with other sleep complaints. PLMS patients were not different from controls regarding age, body mass index, and apneas and hypopneas per hour of sleep. The latencies of the R1 and R2 components of the blink reflex were not different between the groups. However, in PLMS, the excitability of the R2 response was markedly enhanced: The second R2 response to a pair of stimuli given at an interval of 500 ms was in PLMS 59.5% (range 21–87%) of the first R2 response and in controls 18.8% (range 2–79%) (p < 0.0001, Mann‐Whitney U test). These results provide further evidence of an altered motor control in PLMS, which is likely to be located subcortically and possibly related to the dopaminergic striaitopallidal system.
Brainstem encephalopathy with stimulus‐sensitive myoclonus leading to respiratory arrest, but with recovery: A description of two cases and review of the literature
Authors: Kullmann D. M. , Howard R. S. , Miller D. H. , Hirsh N. P. , Brown P. , Marsden C. D. 
Published: December 2004

AbstractTwo unrelated patients developed bulbar symptoms, followed within several weeks by spontaneous myoclonus and painful, generalized, stimulus‐sensitive jerks triggered by unexpected noises and cutaneous stimuli. They progressed to respiratory arrest and required mechanical ventilation, but both patients subsequently made an almost full recovery. These cases stress the importance of persevering with supportive treatment despite rapid progression of this severe generalized movement disorder. The relationship of brainstem reflex myoclonus to hyperekplexia, progressive encephalomyelities with rigidity, and the stiff‐man syndrome is discussed.
Effects of central dopaminergic stimulation by apomorphine on swallowing disorders in Parkinson's disease
Authors: Tison F. , Wiart L. , Guatterie M. , Fouillet N. , Lozano V. , Henry P. , Barat M. 
Published: December 2004

AbstractSwallowing disorders are common in Parkinson's disease but are of obscure and complex nature and pathophysiology. The effect of central dopaminergic stimulation on disordered swallowing is not well known. We studied the effects of apomorphine (in combination with domperidone) on buccolinguofacial motoricity and on various swallowing stages by using videofluoroscopy in eight patients with dysphagia. Swallowing abnormalities more frequently encountered were vallecular stasis (n = 7), fragmentation of the bolus (n = 7), and buccal stagnation of the bolus. Apomorphine improved vallecular stasis and fragmentation in about half the cases and improved buccal stagnation in all cases. Direct laryngeal penetration was found in three cases and improved in two of them. The total swallowing duration was improved by apomorphine in a subset of patients (n = 5). This improvement correlated with an improvement of the buccolinguofacial motoricity and was combined with an improvement of pharyngeal transit time. Thus central dopaminergic stimulation by apomorphine improved swallowing in a subgroup of patients, mainly in its early stages.
Intrafamilial variability in Machado‐Joseph disease
Authors: Subramony S. H. , Currier R. D. 
Published: December 2004

AbstractDominantly inherited ataxias resulting from different gene mutations are difficult to distinguish based on clinical phenotypes. We believe the phenotypic variability within families can be a clue to clinical diagnosis. We illustrate the range of phenotypes extending from levodopa‐responsive extrapyramidal disease to more purely ataxic syndromes seen in two families with molecularly proven Machado‐Joseph disease.
N‐0923, A novel soluble dopamine D2 agonist in the treatment of parkinsonism
Authors: Calabrese Vincent P. , Alvin Lloyd K. , Brancazio Pasquale , Cefali Eugene , Martin Pauline , Wall James , Sica Dominic 
Published: December 2004

AbstractN‐0923, a novel aminotetralin dopamine D2 agonist, was shown to effectively reverse parkinsonian symptoms in nine dopa/agonist‐responsive Parkinson's disease patients. The drug was given up to 4.5 hours by continuous intravenous (IV) infusion using an IV pump. The onset of anti‐parkinsonian effect was seen within minutes of the initiation of the infusion and was absent within 90 minutes of cessation of the infusion. The short elimination half‐life of N‐0923 (90 min) would allow for the rapid initiation of drug effect when necessary and at the same time permit the effect to be terminated quickly if necessary. The drug would be useful in situations where oral medication is not feasible or is associated with erratic absorption. The patients tolerated the drug well. Dose escalation load was limited by nausea and vomiting. It should be noted that the doses were increased until these symptoms occurred, but therapeutic effects were noted well before the side effects occurred. Using a modified Columbia scale, maximum improvement consisted of a 27–95% drop in score. Maximum response was obtained at infusion rates varying from 2–16 μg/kg per hour and at blood levels of 0.11–1.49 μg/mL.
Pallidotomy improves motor responses and widens the levodopa therapeutic window in Parkinson's disease
Authors: Skalabrin Elaine J. , Laws Edward R. , Bennett James P. 
Published: December 2004

AbstractStereotactic posteroventral pallidotomy (PVP) as a treatment for Parkinson's disease (PD) symptoms has been increasingly used in moderate‐advanced disease. We examined the pharmacodynamic responses of PD patients to single oral levodopa doses and intravenous levodopa infusions before and after PVP surgery. Nine subjects with advanced PD received a single oral dose and ramped intravenous levodopa infusions before and 3–5 weeks after unilateral PVP. Timed motor tasks, Unified Parkinson's Disease Rating Scale (UPDRS) evaluations, and ordinal dyskinesia rating were performed after oral levodopa and during IV levodopa infusions. Serum prolactin and dopa levels were measured during the levodopa infusions. Overall timed motor but not motor UPDRS scores were improved after PVP in both the worst (“off”) and best (“on”) states. Contralateral but not ipsilateral limb dyskinesias were substantially reduced at all serum (dopa) levels after PVP. Ipsilateral and contralateral timed motor performance at low serum (dopa) levels was improved by PVP. Walking speeds at all serum (dopa) levels were not changed by PVP. Serum prolactin was reduced equally by increasing (dopa) preoperatively and postoperatively. PVP significantly and favorably altered oral and intravenous levodopa pharmacodynamics by improving bilateral limb motor function and contralateral dyskinesia but did not alter walking speed. PVP appears to widen significantly the therapeutic window for levodopa in PD.
Dopamine D3 receptor is decreased and D2 receptor is elevated in the striatum of Parkinson's disease
Authors: Ryoo Han L. , Pierrotti Dylan , Joyce Jeffrey N. 
Published: December 2004

AbstractThe mesolimbic dopamine (DA) system preferentially innervates the D3 receptor, whereas the D2 receptor is, in addition, a target of the nigrostriatal DA system. In human brain D3 receptors and D3 mRNA‐expressing neurons are largely segregated to brain regions that are the targets of the mesolimbic DA system and the efferents of the “limbic striatum.” Thus, D3 receptors may regulate effects of DA on the “limbic” cortico‐striatal‐pallidal‐thalamic‐cortical loop. The nigrostriatal DA system is considerably more damaged in Parkinson's disease (PD) than the mesolimbic DA system. We report here, using radioligands selective for the D2 and D3 receptor, that these receptors are independently changed in PD. Tissue collected at autopsy from nine subjects with a diagnosis of PD and eight age‐matched subjects with no evidence of a neurologic disorder was processed for [125I]epidepride binding to D2 receptors, [125I] trans‐7‐OH‐PIPAT binding to D3 receptors, [125I]RTI‐55 for the DA transporter (DAT), and immunoautoradiography for tyrosine hydroxylase (TH) using autoradiographic methods. Dopaminergic innervation to the caudal putamen was profoundly reduced and to a lesser extent in the rostral putamen in PD. DAT sites but not TH protein levels were reduced in the nucleus accumbens (NAS) in PD compared with age‐matched control subjects. This is consistent with a loss of dopaminergic innervation from the mesolimbic DA system but elevation in TH production. D3 receptors were significantly reduced in PD by 40–45% particularly in the NAS and putamen. D2 receptors were elevated in PD in the dorsal putamen by 15%. The reduction in D3 receptor number was not observed in PD cases with a diagnosis of less than 10 years. The changes in DA D3 receptor number is interesting in light of the development of antiparkinsonian agents that are D3‐preferring agonists.
Amantadine reduces levodopa‐induced dyskinesias in parkinsonian monkeys
Authors: Blanchet Pierre J. , Konitsiotis Spyridon , Chase Thomas N. 
Published: December 2004

AbstractThe antidyskinetic potential of the glutamate NMDA receptor channel blocker amantadine was evaluated in four levodopa‐primed parkinsonian monkeys using two different regimens (1.25 or 2.5 mg/kg administered subcutaneously twice daily for 3–6 days). When administered with a relatively low dose of levodopa, amantadine produced a near‐total suppression of choreiform dyskinesias and a substantial reduction in dystonic dyskinesias at the expense of a significant reduction in antiparkinsonian response. With a high dose of levodopa, amantadine had a smaller but still significant effect on dyskinesias without altering the antiparkinsonian response. These results lend support to the view that glutamate receptormediated mechanisms contribute to levodopa‐induced dyskinesias. They also suggest that amantadine could alleviate such complications in parkinsonian patients, especially with careful dose optimization.