bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neuropathological studies in a mutant hamster model of paroxysmal dystonia
Authors: Wahnschaffe Ulrich , Fredow Gabriele , Heintz Peter , Löscher Wolfgang 
Published: December 2004

AbstractDystonic movements in a mutation of the Syrian golden hamster, named dtsz, have several features in common with clinically observed paroxysmal dystonic choreoathetosis. In this study the CNS of the mutant hamsters and age‐matched nondystonic controls was examined for morphological alterations at the age of 30 days, i.e., when the severity of the dystonic syndrome is fully developed. Particular interest was directed to those brain regions (caudate nucleus, putamen, globus pallidus, ventrolateral thalamus) that are presumably involved in symptomatic dystonia of humans, as well as to regions (e.g., spinal cord, dorsal root ganglia, nucleus ruber) for which neuropathologically detectable lesions have been found previously in the dystonia musculorum mouse. The neuropathological investigation was carried out on routine paraffin histology on step sections of the whole brain and spinal cord. In addition, a silver impregnation method was used for detection of pre‐ and/or postsynaptic degeneration. Light microscopic examination, including morphometry, of the nervous tissue failed to reveal any morphological or morphometric differences between control and dystonic hamsters. The only abnormality that was found in several control and dystonic hamsters was hydrocephalus. Breeding studies using magnetic resonance imaging for detection of hydrocephalus showed that hydrocephalus was hereditary but not related to dystonia. Virus infections as a cause of hydrocephalus or dystonia could be excluded by serological analysis with determinations of various virus antibodies in hamster sera. The lack of neuropathological alterations related to dystonic movements in the present study in dtsz hamsters is comparable to most cases of human hereditary or idiopathic dystonia, which show dystonic movements in the absence of morphological alterations.
Sustained‐release (+)‐PHNO [MK‐458 (HPMC)] in the treatment of Parkinson's disease: Evidence for tolerance to a selective D2‐receptor agonist administered as a long‐acting formulation
Authors: Cedarbaum Jesse M. , Clark Mary , Toy Linda H. , Green‐Parsons Alison 
Published: December 2004

Abstract4‐‐Propyl‐9‐hydroxynaphthoxazine, or MK‐458 (HPMC), a selective, nonergot D2 agonist administered orally twice a day in sustained‐release form, was studied as adjunctive therapy with carbidopa‐levodopa (Sinemet) in 12 Parkinson's disease patients with motor response fluctuations. The dosage of agonist was gradually increased over 12 weeks to a maximum tolerated level of up to 60 mg/day, and that of Sinemet was reduced concurrently. After 8 weeks, reduction of Sinemet averaged 45.1%, but over the next 4 weeks, despite a continued increase in dosage of the agonist, patients were unable to decrease their Sinemet further, and by 12 weeks mean reduction in Sinemet was only 32%. Only five patients completed the planned 24‐week study, mostly due to progressive loss of efficacy. The MK‐458 is capable of partially substituting for Sinemet in dosages employed in this study. Reduced sensitivity to the drug can appear over a relatively short time, perhaps as a result of downregulation of postsynaptic dopamine receptors.
Limb positioning and magnitude of essential tremor and other pathological tremors
Authors: Sanes Jerome N. , Hallett Mark 
Published: December 2004

AbstractWe examined the influence of maintaining different postural configurations of the upper extremity on the magnitude of tremor in patients with essential tremor and with postural tremor from a variety of other neurological disorders. Patients maintained postures requiring different angles of forward flexion in the sagittal plane, of horizontal flexion, and of elbow extension. The tremor of patients diagnosed with essential tremor was either unaffected or affected only little by changes in limb position. In contrast, patients with pathological tremors, of the cerebellar postural, parkinsonian, and other types, exhibited positional dependence of their tremor. When there was positional dependence of tremor, it was always largest when the hands were near the nose or chin. These observations suggest a practical method for assistance with the clinical discrimination of essential tremor from other postural tremors.
Blepharoclonus and parkinsonism associated with aqueductal stenosis
Authors: Gatto Mabel , Micheli Federico , Pardal Manuel Fernandez 
Published: December 2004

AbstractA 28‐year‐old man with a history of congenital hydrocephalus due to aqueductal stenosis shunted at 45 days of age is presented. At age 4 years the valve had to be removed because of septicemia. Twenty‐three years later he developed parkinsonian signs and abnormal, involuntary rhythmic contractions of the eyelids. The latter were elicited on gentle eye closure. Parkinsonism promptly improved after ventriculoperitoneal shunting, but blepharoclonus persisted unchanged.
Geographic distribution of deaths due to Parkinson's disease in canada: 1979–1986
Authors: Svenson Lawrence W. 
Published: December 2004

AbstractMortality figures for Parkinson's disease in Canada from 1979 to 1986 were examined by province and by sex. As seen in other studies, Parkinson's disease showed a statistically significantly higher prevalence in males than in females (p < 0.01). An uneven distribution across provinces was found when mortality rates were compared by males and when males and females were grouped together. Females did not show a significant variation across provinces. Five provinces showed a higher incidence of Parkinson's disease among males than in females (p < 0.05), whereas the other provinces showed no difference. British Columbia and Manitoba showed the highest rates for males as well as the highest rates when males and females were grouped together. The uneven geographic distribution of Parkinson's disease offers support for the possible involvement of environmental factors in the etiology of some forms of idiopathic Parkinson's disease. Suggestions for further research are outlined.
Phenytoin potentiation of neuroleptic‐induced dyskinesias
Authors: Sethi K. D. , Hitri Ana , Diamond Bruce I. 
Published: December 2004

AbstractClinically phenytoin‐induced movement disorders may resemble neuroleptic‐induced tardive dyskinesia (TD). Neuroleptic‐induced TD is thought to be related to an increase in the number and affinity of dopamine D2 receptors. We investigated the interaction of phenytoin with dopaminergic systems by measuring dopamine behavior and receptor changes in animals treated with phenytoin alone or in combination with chlorpromazine. Phenytoin alone or in combination produced significant behavioral supersensitivity without alteration of dopamine D2 receptor density or affinity. This animal study suggests that phenytoin may aggravate neuroleptic‐induced tardive dyskinesia through mechanisms other than the dopamine D2 receptors.
Stiff‐person syndrome: An autoimmune disease
Authors: Blum Philip , Jankovic Joseph 
Published: December 2004

AbstractStiff‐person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. Electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.
The hemodynamic effects of triglycyl‐lysine‐vasopressin (glypressin) in patients with parkinsonism and orthostatic hypotension
Authors: Rittig Søren , Arentsen Jens , Sørensen Keld , Matthiesen Thorsten , Dupont Erik 
Published: December 2004

AbstractThe hemodynamic effects of triglycyl‐lysine‐vasopressin (TGLVP) were investigated in a single‐blind study in seven patients with chronic orthostatic hypotension and parkinsonism. Blood pressure, heart rate, and stroke volume were measured in the supine position before and after bolus injection of either placebo or TGLVP (5.0, 7.5, or 10.0 μg/kg of body weight). After 40 min in the supine position, the patients were head‐up tilted to 45° for 20 min. All patients underwent four tilt studies with different medication. The TGLVP increased supine blood pressure by approximately 25% and total peripheral resistance by approximately 46%, and reduced heart rate by approximately 13%. No changes in supine stroke volume or cardiac output were seen. The TGLVP slightly reduced the relative fall in blood pressure and increased heart rate during the tilt. After TGLVP, blood pressure levels during tilt were similar to supine levels prior to medication. The TGLVP did not change the effects of tilt on stroke volume or cardiac output. Only few and mild side effects were experienced and no cardiotoxic effects were observed. In conclusion, TGLVP showed marked blood pressure effects of very small doses in this category of patients. The clinical effects of TGLVP and other vasopressor‐specific analogs of vasopressin should be tested in these patients.
Orthostatic tremor: An association with essential tremor
Authors: FitzGerald Patricia M. , Jankovic Joseph 
Published: December 2004

AbstractOrthostatic tremor is characterized by tremor of the trunk and legs while standing. Rapid frequency has been emphasized as an important criterion for the diagnosis of this tremor. We observed five patients who had the typical findings of orthostatic tremor but had a wide range of frequencies. All five also had postural hand tremor and a family history of essential tremor, suggesting a relationship between orthostatic tremor and essential tremor. This report also emphasizes the association of orthostatic tremor with painful cramps and a relatively consistent improvement with clonazepam.
Quantitative comparison of barbiturates in essential hand and head tremor
Authors: Sasso Enrico , Perucca Emilio , Fava Roberto , Calzetti Stefano 
Published: December 2004

AbstractThe tremorolytic effects of primidone and phenobarbital in essential tremor of hands and head were compared in a double‐blind, placebo‐controlled trial. Quantitative measurements of tremor were obtained in 15 patients by means of an accelerometric method. Only primidone proved to be superior to placebo in reducing hand tremor, suggesting that its tremorolytic effectiveness is largely dependent on the parent drug rather than its metabolite phenobarbital. Head tremor tended to improve only in three out of six patients with both primidone and phenobarbital, but, likely due to the small number of affected patients, the effect failed to reach statistical significance.
A therapeutic trial of milacemide in myoclonus and the stiff‐person syndrome
Authors: Brown P. , Thompson P. D. , Rothwell J. C. , Day B. L. , Marsden C. D. 
Published: December 2004

AbstractWe investigated the therapeutic effects of milacemide in seven patients with myoclonus and three patients with the stiff‐person syndrome in an open‐label trial. Milacemide was initiated at 800 mg/day and was gradually increased to a maximum dosage of 2,400 mg/day. No significant improvement occurred in the 10 patients.
Carpal tunnel syndrome: A complication of idiopathic torsion dystonia
Authors: Drory V. E. , Neufeld M. Y. , Korczyn A. D. 
Published: December 2004

AbstractCarpal tunnel syndrome (CTS) is usually an idiopathic disorder. Certain occupations that require frequent flexion movements of the hand at the wrist are recognized as precipitating the development of CTS. Dystonia can cause similar excessive movements at the wrists. We report the clinical and electromyographic findings of two patients with idiopathic torsion dystonia (ITD), with frequent flexion postures of the wrists, in both of whom typical CTS developed in the more involved hand. It is concluded that nerve entrapment such as CTS has to be considered a possible complication in patients with ITD and other motor control disorders.
Review and videotape recognition of idiopathic restless legs syndrome
Authors: Walters Arthur S. , Hening Wayne A. , Chokroverty Sudhansu 
Published: December 2004

AbstractThe motor phenomena associated with idiopathic restless legs syndrome (RLS) are infrequently seen in the physician's office because they are present only after prolonged sitting or lying and usually at night. These motor phenomena are captured on videotape in four unrelated patients with idiopathic RLS. The clinical features of idiopathic RLS are reviewed in detail, and therapeutic advances in its treatment are summarized.
Visuospatial cognition in Huntington's disease
Authors: Mohr Erich , Brouwers Pim , Claus Jules J. , Mann Ulrike M. , Fedio Paul , Chase Thomas N. 
Published: December 2004

AbstractThe notion of specificity of visuospatial dysfunction in Huntington's disease (HD) was evaluated in a sample of afficted patients as a function of symptom duration, age at onset, and overall dementia severity. Factor analytic procedures indicated that overall visuospatial processing capacity (factor 1) as well as the ability for spatial manipulation (factor 3) was markedly affected in HD patients. In contrast, consistency of spatial judgment (factor 2) appeared to remain relatively intact in these patients. Age at onset seemed to have no relationship with any of these variables, whereas dementia severity demonstrated a significant relationship with overall visuospatial processing capacity. Most importantly, duration of symptoms was significantly associated with the declining ability to mentally perform spatial manipulations. The observation of circumscribed visuospatial impairment in HD patients may have important consequences for the further understanding of the neurobehavioral consequences of this disorder.
Gastrointestinal symptoms in Parkinson's disease
Authors: Edwards L. L. , Pfeiffer R. F. , Quigley E. M. M. , Hofman Ruth , Balluff Mary 
Published: December 2004

AbstractWe have investigated the prevalence of gastrointestinal (GI) symptoms in 98 individuals with Parkinson's disease (PD) and in a control group of 50. Seventy‐nine of those with PD were being treated with dopaminergic medications and 19 were untreated. Those symptoms occurring more frequently in PD patients than in controls included abnormal salivation, dysphagia, nausea, constipation, and defecatory dysfunction. Except for defecatory dysfunction, symptoms did not correlate with treatment but instead correlated with disease severity. This suggests that the GI symptoms of PD reflect direct involvement in the GI tract by the primary disease process.
Two sisters with generalized dystonia associated with homocystinuria
Authors: Berardelli A. , Thompson P. D. , Zaccagnini M. , Giardini O. , D'Eufemia P. , Massoud R. , Manfredi M. 
Published: December 2004

AbstractTwo sisters with progressive dystonic syndromes and homocystinuria are presented. The biochemical defect was not accompanied by the typical clinical features of homocystinuria. Magnetic resonance imaging (MRI) revealed bilateral lesions of the basal ganglia. Homocystinuria should be considered among the causes of symptomatic or secondary dystonias associated with basal ganglia lesions.
Dystonia and akinesia due to pallidoputaminal lesions after disulfiram intoxication
Authors: Krauss Joachim K. , Mohadjer Mohsen , Wakhloo Ajay K. , Mundinger Fritz 
Published: December 2004

AbstractA case with segmental cranial plus crural dystonia of delayed onset and akinesia after acute intoxication with disulfiram is presented. Computed tomography showed bilateral pallidal lesions, whereas on magnetic resonance imaging aditional small lesions of the putamen could be detected. Long‐term observation with progression and a change of symptoms over a period of 10 years after the intoxication is demonstrated on videotape. Although other central side effects after intoxication with disulfiram are well known, movement disorders are uncommon. Carbon disulfide, a disulfiram metabolite, may be important in the etiopathogenesis.
A case‐control study of idiopathic torsion dystonia
Authors: Fletcher N. A. , Harding A. E. , Marsden C. D. 
Published: December 2004

AbstractA study of 71 patients with idiopathic torsion dystonia (ITD) and 71 matched controls was performed to investigate the range of possible clinical expression of ITD and the role of environmental factors in the development of the disease. A family history of tremor and stuttering were the only factors significantly associated with ITD. No associated environmental factor was identified.
Inhibition of forearm EMG by palatal myoclonus
Authors: Elble Rodger J. 
Published: December 2004

AbstractThe forearm electromyogram (EMG), pharyngeal EMG, and wrist tremor were recorded simultaneously from a 74‐year‐old woman with the syndrome of palatal myoclonus and progressive ataxia. Her wrist tremor had the characteristics of enhanced physiologic tremor. The enhancement of her tremor was attributable to 50‐ to 80‐ms silent periods in the forearm EMG that followed the 1.9‐Hz bursts of palatal myoclonus by 50 to 60 ms. This observation and those of previous authors support the notion that rhythmic olivocerebellar discharges can cause tremorogenic excitation and inhibition of postural EMG activity in the upper extremities.
Profile of patients enrolled in a new movement disorder clinic
Authors: Siemers Eric , Reddy V. 
Published: December 2004

AbstractTo assess the need for a regional expertise in movement disorders, the numbers of patients, clinic visits, and medication changes for a new movement disorder clinic were recorded. During 3½ years, 355 patients were seen, with 1,329 clinic visits. Idiopathic Parkinson's disease was the most common diagnosis, comprising 36% of the population, followed by dystonia (17%), tremor (12%), parkinsonism (i. e., Parkinson's plus syndromes, drug‐induced parkinsonism, etc.) (10%), chorea (10%), Tourette's syndrome (6.5%), and tardive dyskinesia (3.4%). Distribution of follow‐up visits was similar, with Parkinson's disease (52%) being most frequent and Tourette's syndrome (3.1%) least frequent. The relative utilization of medical care by each patient group was assessed by determining the number of medication changes and the number of clinic visits per follow‐up year. No differences in these measures were found using a one‐way analysis of variance. Of the Parkinson's disease patients, 67% had Hoehn and Yahr stages III‐IV and 77% of the clinic visits were made by this subgroup. When considered in light of the prevalence of each of the diseases, these data show a need for an expertise in movement disorders for a population base of the size we have served.
Steroid‐responsive chorea in moyamoya disease
Authors: Pavlakis Steven G. , Schneider Steven , Black Karen , Gould Robert J. 
Published: December 2004

AbstractWe report the case of a 13‐year‐old boy with disabling chorea due to moyamoya disease. His chorea seemed to improve with steroid therapy. We conclude that steroid therapy may ameliorate moyamoya‐associated chorea, and perioperative steroids can confound neurosurgical outcome. We are unable to assess the effect of cerebrovascular bypass procedures on the outcome of chorea in this patient.
Complex stereotypies after right putaminal infarction: A case report
Authors: Maraganore D. M. , Lees A. J. , Marsden C. D. 
Published: December 2004

AbstractA 17‐year‐old boy is described who experienced circling behavior interrupting his gait and complex stereotyped movements of the hands after a complicated repair of an aortic coarctation. An MRI scan showed a right putaminal infarct. The boy's behavior and stereotypies bear a close resemblance to those found in autism.
The coordination of posture and voluntary movement in patients with cerebellar dysfunction
Authors: Diener H.‐C. , Dichgans J. , Guschlbauer B. , Bacher M. , Rapp H. , Klockgether T. 
Published: December 2004

AbstractPostural adjustments associated with the task of rising on tiptoes were investigated in a reaction time paradigm in 10 normal subjects and 18 patients with cerebellar disorders. Cerebellar dysfunction was due to either degenerative cerebellar disease, tumor, or ischemia. Displacements of the center of foot pressure (CFP) were recorded. The task, accomplished by the triceps surae muscle (executional activity, mean latency of 411 ms), is mechanically effective only if the center of gravity has been shifted forward in advance. To this effect, a phasic burst of preparatory EMG activity in the tibialis anterior normally occurs at a mean latency of 163 ms, shifting the center of gravity forward. Shortly thereafter, activity of the quadriceps femoris (175 ms) extends the knee and aids the forward shift of the center of gravity. Different aspects of this motor sequence were disturbed in individual patients: Latencies of preparatory and executional activity were uncorrelated in 15 of the 18 patients. Executional (n = 16) or preparatory (n = 13) EMG activity was tonic instead of phasic. Latencies of either preparatory or executional EMG activities or both were prolonged (n = 10). The time interval between motor preparation and execution was increased (n = 9). The trial‐to‐trial variability of biomechanical parameters and EMG latency was increased. Preparatory EMG activity in the quadriceps was entirely missing (n = 9), resulting in knee bending at the unsuccessful attempt to rise on tiptoes. Patients who were most severely affected had no preparatory activity at all (n = 2), and therefore were unable to perform the task. In conclusion, the cerebellum contributes to the scaling of size and duration of preparatory and executional motor activity and controls their temporal relationships.
Is nocturnal paroxysmal dystonia a form of frontal lobe epilepsy?
Authors: Meierkord H. , Fish D. R. , Smith S. J. M. , Scott C. A. , Shorvon S. D. , Marsden C. D. 
Published: December 2004

AbstractThe sex ratio, age at onset, and ictal features of nine patients with nocturnal paroxysmal dystonia were compared with those of eight patients with daytime frontal lobe seizures and eight patients with nocturnal motor attacks of known epileptic origin. All patients underwent video electroencephalography telemetry. There were no clinical features that would allow distinction between the groups and no single phenomenon was uniquely seen in nocturnal paroxysmal dystonia. This study provides no evidence that nocturnal paroxysmal dystonia is a separate diagnostic entity.