bluuebunny/crossref_metadata_2025_split huggingface dataset:
A “combined” levodopa test as a useful method for evaluating the efficacy of dopamine agonists: Application to pergolide and bromocriptine
Authors: Bonnet A. M. , Serre I. , Marconi R. , Agid Y. , Dubois B. 
Published: December 2004

AbstractThe efficacy of pergolide as adjunct to levodopa therapy was compared to that of bromocriptine in 12 parkinsonian patients with fluctuating motor disability and levodopa‐induced dyskinesias (mean age of onset, 50.6 ± 8 years; Hoehn and Yahr stage between II and IV; mean basal UPDRS motor score, 30.6 ± 8.6), in a double‐blind crossover study. After an 8‐day habituation to each agonist, an acute challenge of a supraliminal dose of levodopa (“levodopa test”) was performed in association with either 1 mg pergolide or 10 mg bromocriptine. The delay to onset and the duration of therapeutic benefit, the percentage improvement in motor disability, and the severity of onset and peak‐dose dyskinesias were evaluated. Both agonists significantly increased the duration of therapeutic benefit, but pergolide more so than bromocriptine (p = 0.02). Pergolide also tended to reduce the severity of dyskinesias and wes globally perceived by the patients to be more efficacious than bromocriptine on parkinsonian symptoms and fluctuations. This study illustrated the usefulness of the “levodopa test” in evaluating, objectively, the effects of dopamine agonists.
Bilateral subthalamic nucleus stimulation for severe Parkinson's disease
Authors: Limousin Patricia , Pollak Pierre , Benazzouz Abdelhamid , Hoffmann Dominique , Broussolle Emmanuel , Perret Jean Edmond , Benabid Alim‐Louis 
Published: December 2004

AbstractSubthalamic nucleus (STN) lesions or high‐frequency stimulations could improve parkinsonian symptoms in monkeys treated by MPTP. We have applied the procedure of chronic stimulation to the STN in severely disabled parkinsonian patients. This article presents the case of the first patient operated on bilaterally. Bilateral STN stimulation has greatly improved akinesia and rigidity. The benefit was maintained ≤15 months after surgery. Unilateral stimulation induced motor effects mainly in contralateral limbs. Further studies are needed to evaluate the value of this procedure in the treatment of Parkinson's disease.
Intrathecal baclofen for motor disorders
Authors: Penn Richard D. , Gianino Janet M. , York Michelle M. 
Published: December 2004

AbstractTo test the efficacy of intrathecal baclofen in various movement disorders, 18 patients with dystonia, head trauma, cerebral palsy, rigidity, or painful spasms underwent a trial of intrathecal baclofen. Ten went on to permanent implantation with an infusion pump to provide long‐term treatment. Patients with a component of spasticity, painful spasms, or focal dystonias did best, and no response was seen in patients with rigidity due to anoxic encephalopathy. A videotape of responses is provided.
Involvement of respiratory muscles in adult‐onset dystonia: A clinical and electrophysiological study
Authors: Lagueny A. , Burbaud P. , Le Masson G. , Bergouignan F. X. , Ferrer X. , Julien J. 
Published: December 2004

AbstractInvolvement of respiratory muscles is unusual in dystonia, but its occurrence may be underestimated either because it is not conspicuous or because it is improperly imputed to another cause. Three patients who had adult‐onset dystonia and who were exhibiting respiratory problems were examined clinically and electrophysiologically. In the three patients the onset was focal‐cervical in two and blepharospasm in one. The respiratory problems appeared later. The first patient had involuntary deep and loud inspirations combined with spasms of axial dystonia, the second complained of breathing arrests, and the third had deep inspirations mainly on speaking or reading aloud, thus causing broken speech. Electromyographic findings, including of the diaphragm, were quite consistent with a respiratory involvement in these three cases of dystonia. Assuming that respiratory troubles could be the first sign of a focal dystonia, electrophysiological studies of respiratory muscles could be used to confirm this.
Chronic acquired hepatocerebral degeneration: Case reports and new insights
Authors: Jog Mandar S. , Lang Anthony E. 
Published: December 2004

AbstractChronic acquired hepatocerebral degeneration (CAHD) is a heterogeneous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Little has been added to the understanding of this disorder since the detailed, early clinical and pathological descriptions. The spectrum of clinical presentations can be neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, parkinsonism, myoclonus, dystonia), or both. Cortical laminar necrosis and polymicrocavitation in the cortex and basal ganglia are combined with cerebral and cerebellar atrophy. Microscopically, Alzheimer type II astrocytes and cytoplasmic glycogen granules are characteristic. Recent neuroradiological observations in patiensts with liver failure have shown a specific magnetic resonance (MR) imaging appearance with a hyperintense T1 signal in the pallidum, putamen, and, rarely, mesencephalon. Using clues from a similar MR appearance in patients receiving total parenteral nutrition as well as animals given parenteral manganese, and the knowledge that manganese is cleared by the hepatobiliary system, deposition of manganese in the brain is postulated in patients with CAHD. In this review we describe three cases of CAHD with detailed clinical and radiological documentation and discuss the aforementioned pathogenetic mechanisms.
The audiogenic startle response in Tourette's syndrome
Authors: Stell R. , Thickbroom G. W. , Mastaglia F. L. 
Published: December 2004

AbstractWe report the results of a study in which we examined the auditory startle responses (ASR) of 8 patients with Tourette's syndrome and 15 normal controls, none of whom reported or manifested exaggerated startle responses clinically. The ASR in two patients failed to habituate with repetition, a finding not present in any of the controls. There was no correlation between the presence or absence of exaggerated ASRs and the severity of the patients' tics. This study demonstrates that some patients with Tourette's syndrome have exaggerated audiogenic startle responses that may be clinically asymptomatic.
Modulation of electromyographic activity of wrist flexor and extensor muscles in patients with writer's cramp
Authors: Valls‐Solé Josep , Hallett Mark 
Published: December 2004

AbstractPatients with writer's cramp have two well‐recognized neurophysiological abnormalities: reduced reciprocal inhibition of the wrist flexor motoneurons at rest, and increased cocontraction of antagonist muscles of the forearm during voluntary activity. In this article we present evidence for an impaired integration of sensory inputs into the voluntary motor activity during performance of a force‐related task in patients with writer's cramp. Normal (control) subjects and patients activated wrist flexor and extensor muscles to maintain a predetermined level of force. Electrical stimuli were applied to median and radial nerve afferents and the modulatory effects induced in the electromyographic (EMG) activity were measured. For each muscle studied and nerve stimulated, we defined a characteristic sequence of excitatory (E) and inhibitory (I) phases of modulation of the averaged rectified EMG activity in control subjects. E and I phases were thought to represent excitation and inhibition, respectively, of the corresponding motoneuronal pool to homonymous or reciprocal afferent inputs. There were no differences between control subjects and patients regarding the level of background EMG activity in the agonist muscles during wrist flexion or wrist extension. In the agonist wrist flexors, patients had reduced reciprocal excitation and reciprocal inhibition. These results are compatible with an abnormal CNS processing of the inputs generated by external stimuli during voluntary contraction of wrist flexor and extensor muscles. Defective integration of inputs from peripheral nerve afferents into the command for voluntary movement may partly underlie the pathophysiology of the motor dysfunction in patients with writer's cramp.
Associated movement as a sequel to thoracotomy: Aberrant regeneration to the latissimus dorsi muscle
Authors: Kuwabara Satoshi , Fukutake Toshio , Kasahata Nobuyoshi , Shimoe Yutaka , Yamanaka Izumi , Hirayama Keizo 
Published: December 2004

AbstractWe describe two patients with unusual associated movements as a sequel to thoracotomy. The two patients developed involuntary twitchings of the latissimus dorsi, which were induced by contractions of the adjacent muscles, the serratus anterior muscle in patient 1 and the external intercostal muscle in patient 2, after lobectomy for lung cancer. Electrophysiological and radiological studies showed that aberrant regeneration from the adjacent nerves to the denervated latissimus dorsi could be responsible for the associated movements.
Environmental and genetic risk factors in Parkinson's disease: A case–control study in southern italy
Authors: De Michele G. , Filla A. , Volpe G. , De Marco V. , Gogliettino A. , Ambrosio G. , Marconi R. , Castellano A. E. , Campanella G. 
Published: December 2004

AbstractTo clarify the role of heredity and of some environmental risk factors in the etiology of idopathic Parkinson's disease, we performed a case‐control study in two regions of southern Italy, Campania and Molise. We selected two controls for each parkinsonian patient, the patient's spouse and a sex‐ and age‐matched neurological control. One hundred sixteen consecutive outpatients with Parkinson's disease (77 men, 39 women; mean age ± SD = 62.5 ± 9.9) and the same number of spouses and neurological controls were interviewed about five environmental risk factors (cigarette smoking, well‐water drinking, head trauma with loss of consciousness, strict diets, general anesthesia) and two genetic risk factors (family history of Parkinson's disease or of essential tremor). Well‐water drinking and family history of Parkinson's disease or essential tremor showed a positive association with Parkinson's disease; smoking showed a negative association. The most relevant risk factor was history of familial Parkinson's disease (odds ratio = 14.6; 95% confidence interval = 7.2 − 29.6); 33% of our patients had at least one affected relative. We also showed a unilateral distribution of ancestral secondary cases on the paternal or on the maternal side, which suggests a dominant inheritance. Clinical and epidemiologic features of cases with familial Parkinson's disease showed no peculiarity. The study suggests a strong role of the genetic factors in the etiology of Parkinson's disease.
Levodopa stability in solution: Time course, environmental effects, and practical recommendations for clinical use
Authors: Pappert Eric J. , Buhrfiend Colleen , Lipton Jack W. , Carvey Paul M. , Stebbins Glenn T. , Goetz Christopher G. 
Published: December 2004

AbstractWe defined the temporal stability characteristics of levodopa/carbidopa (LD/CD) solution, and determined the effects of temperature, ascorbate, and light on LD stability over 7 days. At room temperature and without ascorbate, LD levels significantly declined by 48 h. Ascorbate prolonged stability to 72 h. Refrigeration and freezing prevented a significant decline in LD levels for the full 7 days. Light or darkness had no effect on stability. LD/CD solution, if made daily, requires no special handling and longer stability is maintained with ascorbate, refrigeration, or freezing.
Unilateral injection of botulinum toxin in blepharospasm: Single fiber electromyography and blink reflex study
Authors: Girlanda P. , Quartarone A. , Sinicropi S. , Nicolosi C. , Messina C. 
Published: December 2004

AbstractWe studied six patients affected with blepharospasm (BSP). We injected botulinum toxin (BTX) around only one eye and saline solution around the other. Clinical rating of BSP was performed. Single fiber electromyography (SFEMG), compound motor action potential (cMAP) at the orbicularis oculi muscle by stimulation of the facial nerve, blink reflex, and blink reflex recovery curve were recorded. All clinical and electrophysiological investigations were carried out before, and 1, 2, and 4 weeks after treatment. Evidence of bilateral clinical benefit was provided. Following therapy, facial cMAP decreased bilaterally and SFEMG revealed statistically significant changes on both sides while the excitability curve of blink reflex remained unmodified. The results confirm that BTX affects merely the neuromuscular junctions. The clinical and neurophysiological effects are present on both sides also for unilateral injection probably because of toxin spreading.
Cricopharyngeal dysfunction in Parkinson's disease: Role in dysphagia and response to myotomy
Authors: Born Lawrence J. , Harned Roger H. , Rikkers Layton F. , Pfeiffer Ronald F. , Quigley Eamonn M. M. 
Published: December 2004

AbstractWe report five patients with Parkinson's disease and dysphagia who were found, by radiological and manometric evaluation, to have evidence of cricopharyngeal dysfunction, which included the presence of a Zenker's diverticulum in two. Cricopharyngeal myotomy was performed in four patients with excellent and sustained improvement in swallowing. We conclude that cricopharyngeal function should be carefully evaluated in patients with Parkinson's disease and dysphagia and that surgical treatment should be considered in appropriate cases.
Slow increase of homovanillic acid in cerebrospinal fluid after levodopa administration
Authors: Raftopoulos C. , Dethy S. , Laute M. A. , Goldman S. , Naini A. B. , Przedborski S. , Hildebrand J. 
Published: December 2004

AbstractConcentrations of major catabolites of dopamine were followed in the ventricular cerebrospinal fluid (CSF) in five patients undergoing intracranial pressure monitoring for chronic hydrocephalus. Determinations were made every 2 h following the administration of carbidopa/levodopa 25/250 mg (one Sinemet capsule) given 8h apart. The rise of homovanillic acid (HVA) concentrations was slow and progressive, reaching the level of statistical significance (p ≤ 0.01) only 8 h after the second administration of Sinemet. The rise in 3,4‐dihydroxy‐phenylacetic acid (DOPAC) was faster than the rise in HVA, with the peak value detected 4 h after the first administration of Sinemet. These data are interpreted as a confirmation, in humans, of a slow pool of exogenous levodopa, previously demonstrated in animal studies.
Prevalence of a history of shaking in persons 65 years of age and older: Diagnostic and functional correlates
Authors: Louis E. D. , Marder K. , Cote L. , Wilder D. , Tang M‐X. , Lantigua R. , Gurland B. , Mayeux R. 
Published: December 2004

AbstractThe purpose of this study was to determine the prevalence and functional and diagnostic correlates of reported shaking in the community‐dwelling elderly. We conducted a standardized neurological evaluation of 1,056 nondemented Medicare recipients in Washington Heights‐Inwood, Northern Manhattan (New York). Of 1,056 patients, 108 reported shaking (10.2%). The prevalence of reported shaking did not increase with age. It did differ between ethnic groups, but when adjusted for depression and score on the Schwab and England Activities of Daily Living (ADL) scale, this difference was insignificant. The age‐adjusted prevalence was similar for women and men. Neurological examination of the 108 who reported shaking showed that 8.3% had tremor at rest, 17.6% had tremor with action, 5.6% had dyskinesia or chorea, and an additional 28.7% had various problems in coordination or movement. The remaining 39.8% had neither tremor nor problems in coordination or movement. Only 2.9% of individuals with reported shaking had Parkinson's disease (PD), 8.7% had essential tremor, and 2.1% had oral‐buccal‐lingual dyskinesia. Of the remaining 86.3%, 29 (31%) had no identifiable medical condition. Those who reported shaking were less independent with ADLs, regardless of presence of tremor on examination. Shaking is commonly reported by the community‐dwelling elderly. It does not necessarily identify individuals with essential tremor and PD, and is related to decreased independence in ADLs.
Pure stimulus‐sensitive truncal myoclonus of propriospinal origin
Authors: Schulze‐Bonhage A. , Knott H. , Ferbert A. 
Published: December 2004

AbstractThe clinical and electrophysiological features of stimulus‐sensitive truncal myoclonus are described in a 49‐year‐old woman. Touching the skin of the back and abdomen would evoke jerks in both ipsilateral and contralateral axial muscles; there was no spontaneous jerking. Multichannel EMG recordings showed bilateral short‐latency muscle bursts at truncal recording sites both rostral and caudal to stimulus sites. The short latencies of muscle bursts in adjacent segments give evidence of a spinal origin of myoclonus; the pattern of recruitment and the velocity of spread suggest the involvement of propriospinal pathways. The presence of intrathecal IgG synthesis and of oligoclonal bands in the CSF point towards an inflammatory process which may underly the unusual type of myoclonus in this patient.
An appraisal of the antiparkinsonian activity of piribedil in 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine‐treated common marmosets
Authors: Smith Lance , De Salvia Maria , Jenner Peter , Marsden C. David 
Published: December 2004

AbstractThe D2 dopamine agonist piribedil is not widely used in the treatment of Parkinson's disease because it was thought to be effective mainly on parkinsonian tremor and to produce a high incidence of peripheral side effects, particular nausea. In this study, we used 1–methyl–4–phenyl–1,2,3,6–tetrahydropyridine (MPTP)–treated primates to reevaluate the antiparkinsonian ability of piribedil after its oral administration in the presence or absence of domperidone pretreatment. Adult common marmosets (Callithrix jacchus) were treated with the nigral toxin MPTP to induce a parkinsonian syndrome characterised primarily by brady kinesia and other motor deficits. Oral administration of a solution of piribedil [1–(3,4–methylenedioxybenzyl)–4–(2–pyrimidinyl) piperazine] produced a dose–related reversal of all MPTP locomotor and behavioural deficits. However, this effect was short lived and associated with unwanted effects, particular nausea and retching, which clearly hindered locomotion. In contrast, after pretreatment with the peripheral dopamine antagonist domperidone, administration of piribedil did not induce nausea or retching in MPTP‐treated marmosets. In these animals, piribedil caused a more marked and and longer lasting enhancement of locomotor activity and a further reduction in behavioural deficits than that observed after administration of piribedil alone. In addition, piribedil induced increased vigilance and awareness. These data show that piribedil can reverse akinesia and rigidity in MPTP‐treated primates. In addition, they show the drug to be effective without peripheral side effects when used in conjuction with domperidone. These data indicate that piribedil should be an effective monotherapy for Parkinson's disease.
Haplotype analysis at the DYT1 Locus in ashkenazi jewish patients with occupational hand dystonia
Authors: Gasser T. , Bove C. M. , Ozelius L. J. , Hallett M. , Charness M. E. , Hochberg F. H. , Breakefield X. O. 
Published: December 2004

AbstractGenetic haplotypes at five marker loci that are closely linked to the DYT1 gene on chromosome 9q were determined in 10 Ashkenazi Jewish patients with focal hand dystonia (eight with musician's cramp, two with writer's cramp). The founder haplotype associated with >90% of cases of generalized dystonia in the Ashkenazi Jewish population could not be constructed from any of the twenty chromosomes. Potential haplotypes were determined, and no common haplotype was discerned in these patients. These findings argue against a role for the founder mutation in the DYT1 gene in the etiology of occupational hand dystonia in this ethnic group. Further, if the DYT1 gene is involved in these later onset dystonias, there is no evidence for a common mutation in the Ashkenazic Jewish population. It appears that excessive, repetitive use, possibly in combination with ulnar neuropathy, may serve as the inciting cause of some focal dystonias.
Dominantly inherited cerebello‐olivary atrophy is not due to a mutation at the spinocerebellar ataxia‐I, machado‐joseph disease, or dentato‐rubro‐pallido‐luysian atrophy locus
Authors: Subramony S. H. , Fratkin J. D. , Manyam B. V. , Currier R. D. 
Published: December 2004

AbstractThe dominantly inherited ataxias are characterized both by phenotypic variablity (phenotypic heterogeneity) within the same genotype and overlapping phenotypes from different genotypes (genotypic heterogeneity). Therefore it is important to characterize specific clinical‐neuropathologic phenotypes as precisely as possible at the genetic level. We describe a family with dominantly inherited ataxia of late adult onset with relatively “pure” cerebellar signs. Neuropathologic examination in two individuals from this family revealed findings consistent with cerebello‐olivary atrophy, suggesting that this neuropathologic phenotype may run true within families. Mutations at the spinocerebellar ataxia‐I, Machado‐Joseph disease, and dentatorubropallidoluysian atrophy loci were excluded by direct DNA analysis on the leukocytes of one living affected member. Thus we provide evidence that these mutations are not responsible for this particular phenotype of dominantly inherited ataxia.
Abnormal involuntary movements induced by subthalamic nucleus stimulation in parkinsonian patients
Authors: Limousin Patricia , Pollak Pierre , Hoffmann Dominique , Benazzouz Abdelhamid , Perret Jean Edmond , Benabid Alim‐Louis 
Published: December 2004

AbstractChronic electrical subthalamic nucleus (STN) stimulation is under investigation for alleviating parkinsonian symptoms. STN alterations may carry the risk of provoking abnormal involuntary movements (AIMs). We took advantage of the reversibility of the stimulation technique to assess the possibility of inducing AIMs, using different electrical variables with or without concomitant levodopa intake. Above a given threshold voltage, stimulation could induce contralateral distal mobile AIMs or hemiballism in the off‐drug condition in two patients. AIMs occurred after a latency that varied from a few minutes up to several hours after switching on the stinulator. Hemiballism immediately disappeared upon switching off the stimulator. In these patients, levodopa had never provoked that type of AIMs before surgery. Levodopa‐induced AIMs were not modified by electrical stimulation, but off‐phase dystonia disappeared in one patient. Stimulation of the STN induced AIMs that resembled both those observed following spontaneous lesions of the STN and levodopa‐induced diphasic AIMs in parkinsonian patients. As electrical stimulation provoked AIMs and antiparkinsonian benefit occurred with different electical variables and different timing, the mechanisms responsible for these two effects could be distinct.