bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical course of patients with idiopathic Parkinson's disease
Authors: Roos R. A. C. , Jongen J. C. F. , Van Der Velde E. A. 
Published: December 2004

AbstractWe determined the variables associated with the progression and duration of illness of patients with Parkinson's disease (PD) and investigated the cause of death. In 474 patients with parkinsonism, who visited the Academic Hospital between January 1, 1960 and August 31, 1993, we did a survival analysis with the following covariates: age at onset, intial symptom (tremor or rigidity/hypokinesia), age at reaching Hoehn and Yahr stage III, dementia‐free period, and levodopa treatment. A total of 345 patients with parkinsonism fulfilled the criteria of idiopathic PD; 258 of them were still alive on the closing date of this study. There were significantly more men than women (1.43:1). Medical advice was sought in an earlier stage by men and by patients with tremor as presenting symptom. For patients with rigidity/hypokinesia as first symptom, the duration of illness until reaching Hoehn and Yahr stage III was shorter than for patients starting with tremor alone. If the initial symptom is tremor, patients develop dementia less frequently and later after onset than when tremor is not involved. Reaching Hoehn and Yahr stage III and developing dementia both, limit the patient's survival time. The mortality risk for a patient was found to be increased form the moment levodopa treatment was started as compared to those patients who had not yet started with the treatment. The effect of levodopa on survival could not be disentangled from effects of other factors related to the start of levodopa treatment.
A randomized, double‐blind, placebo‐controlled study to evaluate botulinum toxin type A in essential hand tremor
Authors: Jankovic J. , Schwartz K. , Clemence W. , Aswad A. , Mordaunt J. 
Published: December 2004

AbstractTwenty‐five patients with hand tremor of 2 + (moderate) to 4 + (severe) on the tremor severity rating scale were randomized to receive either 50 U of botulinum toxin (BTX) type A (Allergan, Irvine, CA) or placebo injections into the wrist flexors and extensors of the dominant limb. If patients failed to respond to the initial injection, they were eligible to receive another injection of 100 U 4 weeks later. Rest, postural, and kinetic tremors were evaluated at 2‐ to 4‐week intervals over a 16‐week study period using tremor severity rating scales, accelerometry, and assessments of improvement and disability. A signigicant improvment (p < 0.05) was observed on the tremor severity rating scale 4 weeks after injection in patients treated with BTX as compared with placebo, and this effect was maintained for the duration of the study. Four weeks after injection, 75% of BTX‐treated patients vs. 27% of placebo‐treated patients (p < 0.05). reported mild to moderate improvement (peak effect rating ≥2). There were no significant improvements in functional rating scales, although trends were observed for some items. Postural accelerometry measurements showed a ≥30% reduction in amplitude in nine of 12 BTX‐treated subjects and in one of nine placebo‐treated subjects (p < 0. 05). Although all patients treated with BTX reported some degree of finger weakness, no severe, irreversible, or unexpected adverse events occurred. Chemodenervation with BTX may significantly ameliorate essential hand tremor in patients who fail to improve with conventional pharmacologic therapy.
Effect of stimulation in the ventral intermediate nucleus of the thalamus on limb control in parkinson7apos;s disease: A case study
Authors: Pfann K. D. , Penn R. D. , Shannon K. M. , Shapiro M. B. , Corcos D. M. 
Published: December 2004

AbstractThis study focuses on upper extremity strength and movement control in a patient with Parkinson's disease who had stimulating electrodes surgically implanted in the ventral intermediate nucleus (VIM) of the left thalamus. We examined torque generation and control of movements distance in single degree‐of‐freedom elbow movements under three different stimulation conditions: (a)no stimulation, (b)high stimulation, in which tremor was minimized but there was also tingling and perceived weakness, and (c) moderate stimulation, in which tremor was partially reduced, but there was also a subjective sense of increased strength compared with the high‐stimulation condition. The patient's ability to generate both steady torque and rapid movements was poorest with no stimulation. The patient generated the largest torque with moderate stimulation and performed the fastest movements with high stimulation. However, even with tremor minimized, the patient's electromyogram (EMG) burst patterns were not typical of those of neurologically healthy subjects, although the movements were clearly improved.
Prospects for gene therapy in Parkinson's disease
Authors: Freese Andrew , Stern Matthew , Kaplitt Michael G. , O'Connor William M. , Abbey Maureen V. , O'Connor Michael J. , During Matthew J. 
Published: December 2004

AbstractNumerous advances in in vivo and ex vivo gene‐therapy approaches to Parkinson's disease offer promise for direct clinical trials in patients in the next several years. These systems are predicated on introducing genes that encode enzymes responsible for dopamine biosynthesis or neurotrophic factors that may delay nigrostriatal degeneration or facilitate regeneration. We review the current status of experimental approaches to gene therapy for Parkinson's disease. Comparative advantages and disadvantages of each system are enumerated, and preclinical trials of some of the systems are evaluated. Although the specific in vivo or ex vivo methods used for gene transfer into the brain are likely to be supplanted by newer technology over the next decade, the principles and approaches developed in current studies likely will remain the same.
Cabergoline in Parkinson's disease complicated by motor fluctuations
Authors: Geminiani Guiliano , Fetoni Vincenza , Genitrini Silvia , Giovannini Paolo , Tamma Filippo , Caraceni Tommaso 
Published: December 2004

AbstractCabergoline is a long‐acting D2 dopamine (DA) agonist. We conducted an open study to investigate the effectiveness and tolerability of cabergoline, administered once a day orally, in 50 consecutive patients with Parkinson's disease complicated by motor fluctuations and dyskinesias. In 15 patients cabergoline replaced another direct DA agonist. Evaluation after 6 months of treatment (also including patients who dropped out during this period), showed an improvement in off or on hours, or both, in excess of 50% in 27 patients, comprising 20 of the 35 patients (57%) previously untreated with DA agonists and seven of the 15 patients (47%) already on DA agonists when the study began. Of the 22 patients who received the treatment for 1 year, the improvement was maintained up to final evaluation in the patients not on DA agonists at admission (n = 16), whereas a slight deterioration in clinical condition was observed in the patients already on DA agonists at admission (n = 6). Only six patients showed a detectable increase in dyskinesias. The most common side effects were gastric upset (n = 12), orthostatic hypotension (n = 3), and ankle edema (n = 3), all mild; also observed were two cases of pleural effusion/pulmonary fibrosis. Twenty patients (40%) failed to complete the treatment; of these, five (10% of total) dropped out because of adverse effects. It is concluded that once‐daily administrations of cabergoline are useful for treating patients with Parkinson's disease with motor fluctuations and may advantageously substitute other DA agonists. The side effects of the drug are generally mild, although two cases involving pleuropulmonary complications did emerge.
Reversal of behavioural abnormalities by fetal allografts in a novel rat model of striatonigral degeneration
Authors: Wenning G. K. , Granata R. , Laboyrie P. M. , Quinn N. P. , Jenner P. , Marsden C. D. 
Published: December 2004

AbstractWe have developed a rodent model of striatonigral degeration, one of the core pathologies underlying the disease multiple system atrophy (MSA). 6‐Hydroxydopamine (6‐OHDA) was administered into the left medial forebrain bundle of male Wistar rats, followed 3–4 weeks later by intrastriatal injection of quinolinic acid into the ipsilateral stratum. The 6‐OHDA lesion resulted in ipsilateral rotation to (+)‐amphetamine and contralateral rotation to apomorphine. Following the subsequent striatal lesion, amphetamine‐induced ipsilateral rotation persisted, but apomorphine‐induced contralateral rotation was reduced or abolished. Subsequently, the lesioned striatum was implanted with fetal CNS allografts consisting of cell suspensions derived from striatal primordium alone or combined with cografts of ventral mesencephalon. Cografted rats showed a reduction or reversal of amphetamine‐induced rotation. This was not observed in animals receiving striatal grafts alone. Apomorphine‐induced contralateral rotation was restored after striatal grafts alone, but only partially in animals receiving sham or cografts. Tyrosine hydroxylase (TH) and dopamine‐ and cyclic adenosine 3′:5′‐monophosphate‐regulated phosphoprotein (DARPP 32) immunocytochemistry showed mesencephalic and striatal graft survival in most animals. However, dopaminergic outgrowth was restricted to the graft deposit. The latter was surrounded by a markedly gliotic glial fibrillary acidic protein‐positive capsule continuous with corpus callosum. Dopaminergic reinnervation of denervated and lesioned adult striatum itself was absent, suggesting that rotational recovery was due to diffuse dopamine release. The study shows that combined unilateral lesioning of rodent medial forebrain bundle and striatum results in a characterstic drug‐induced rotational response that can be partly restored by mesencephalic/striatal cografts.
Dentatorubral‐pallidoluysian atrophy. Clinical features of a five‐generation danish family
Authors: Nielsen J. E. , Sørensen S. A. , Hasholt L. , Nørremølle A. 
Published: December 2004

AbstractWe describe the first Danish family with dentatorubral‐pallidoluysian atrophy (DRPLA), containing 16 clinically affected individuals in five generations. Inheritance is autosomal dominant. The disorder was diagnosed as Huntington's (HD), but analysis of the IT15 gene for HD revealed normal alleles. The diagnosis of DRPLA was based on the finding of elongated CAG repeats in the B37 gene on chromosome 12 in affected individuals. The age at onset ranged from 13 to 60 years, with most severe clinical picture being associated with onset in childhood. Clinical features included varying combinations of dementia, euphoria, visuomotor disturbances, speech problems, ataxia, tremor, epilepsy, and involuntary movements presenting as chorea, athetosis, and dystonia. We discuss characteristics of DRPLA that may enable the differentiation from HD on a clinical basis. In conclusion, DRPLA should be considered and DNA analysis is recommended in patients manifesting varying combinations of extrapyramidal and cerebellar symptoms, especially when clinical features show pronounced intrafamilial variability, and dyscoordination, tremor, myoclonus, epilepsy, and euphoria are part of the syndrome.
Severe motor tics causing cervical myelopathy in Tourette's syndrome
Authors: Krauss Joachim K. , Jankovic Joseph 
Published: December 2004

AbstractWe describe two patients with Tourette's syndrome in whom severe motor tics involving the neck were complicated by cervical myelopathy. The first patients, a 21‐year‐old man, had complex tics consisting of violent twisting and extending movements of the neck preceded by an irresistible urge to produce the abnormal postures. Two years after onset of these tics, paraesthesias, sensory deficits up to the level of C4, and a gait disturbance developed. Neuroimaging studies confirmed compressive myelopathy, and the symptoms gradually improved with botulinum toxin injections in the posterior cervical muscles. The second patient, a 42‐year‐old man, had violent clonic tics consisting of thrusting head jerks. The repetitive flexion‐extension neck movements had been present since the age 10 years. At age 23, he developed progressive weakness off all four extremities and bladder and sexual dysfunction. Myelography demonstrated cervical spinal canal stenosis with retrospondylosis from C3 through C5 and a Klippel‐Feil malformation. After cervical decompression by, C3‐C5 laminectomies, his spinal cord symptoms improved temporarily. The tics, however, continued, and the neurologic deficits of cervical myelopathy progressed again after age 34. He did not benefit from a second operation. This report draws attention to the possibility that some tics can produce disabling compressive myelopathy. Early diagnosis and treatment of the movement disorder is essential in such cases.
Persisting “Writer's Cramp” as a result of compensation of a temporary palsy due to a hereditary neuropathy with liability to pressure palsies
Authors: Straube Andreas , Mai Norbert , Walther Ernst , Mayer Michael 
Published: December 2004

AbstractA 49‐year‐old man reported several periods of acute paresis of different nerves after exposure to pressure. All palsies showed a good recovery over a period of days to months. The suspected diagnosis of a hereditary neuropathy with liability to pressure palsies was confirmed by the histology of a suralis nerve biopsy, which showed a distinct tomaculous swelling of the myelin. Nine years ago he noticed an acute, distally pronounced palsy of the right arm. A brachial plexus lesion was diagnosed. His profession required written work, thereby forcing him to compensate this weakness by increasign the coactivation of the elbow and upper arm. This coactivation persisted even after complete recovery from the plexus palsy. Thus, the patient showed the typical feature of classcal writer's cramp, with broad coactivation of the arm muscles, which has continued to the present. At the same time, however, the patient was able to perform other motoric tasks well, such as painting. After several practice sessions with a special program that makes use of preserved motor abilities to reestablish writing, the patient was again able to write with a normal kinematic profile as documented by the recorded handwriting. This suggests that the “writer's cramp” of our patient is one form of task‐related dystonia that may be related more to the perserveration of a misleading motor strategy than to a general inability of the motor system to control the movement properly.
Alleviation of experimental hemiparkinsonism by high‐frequency stimulation of the subthalamic nucleus in primates: A comparison with L‐dopa treatment
Authors: Benazzouz Abdelhamid , Boraud Thomas , Féger Jean , Burbaud Pierre , Bioulac Bernard , Gross Christian 
Published: December 2004

AbstractExperimental studies in 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP)‐treated monkeys have shown that akinesia and rigidity are linked to a hyperactivity of glutamatergic subthalamic nucleus neurons and that the lesion of this nucleus can ameliorate parkinsonian motor signs. In our study, high‐frequency stimulation applied at the subthalamic level was performed on two Macaca mulatta monkeys rendered hemiparkinsonian by unilateral infusion of MPTP. Its effects on rigidity and bradykinesia have been quantified. The results exhibit an important alleviation of both symptoms during the application of subthalamic stimulation comparable to that obtained during L‐Dopa treatment, but without the appearance of abnormal movements such hemiballism or dyskinesia. Our data show that subthalamic stimulation has a beneficial effect on experimental parkinsonian rigidity and bradykinesia and suggests a new therapy approach for the treatment of Parkinson's disease by using subthalamic high‐frequency stimulation instead of L‐Dopa treatment.
Clinical trial of piracetam in patients with myoclonus: Nationwide multiinstitution study in Japan
Authors: Ikeda Akio , Shibasaki Hiroshi , Tasgiro Kunio , Mizuno Yoshikuni , Kimura Jun 
Published: December 2004

AbstractSixty patients with disabling myoclonus excluding mainly spinal myoclonus were treated by piracetam as an open‐labeled study, and myoclonus score, neurological symptoms, functional disbility, and intensity of myoclonus were scored before and after treatment, including a blinded video inspection. Electrophysiological correlation also was investigated before and after treatment. Piracetam was effective in myoclonus, especially that of cortical origin, in both had positive benefits on gait ataxia and convulsions but not on dysarthria, and feeding and hand writing improved much significantly. Psychologically significant improvement was seen in decreased motivation, sleep distrubance, attention deficit, and depression, all of which might be possibly secondary benefits associated with improvement of myoclonus. There was no positive correlation between clinical and electrophysiological improvement. Tolerance was good, and side effects were transient. However, hematological abnormalities observed in at least two patients in the present study should be kept in mind when relatively large doses of piracetam are administered, especially in combination with other antimyoclonic drugs.
Subacute encephalomyelitis presenting as stiff‐person syndrome: Clinical, polygraphic, and pathologic correlations
Authors: Armon Carmel , Swanson Jerry W. , McLean John M. , Westbrook Philip R. , Okazaki Haruo , Kurtin Paul J. , Kalyan‐Raman Uma P. , Rodriguez Moses 
Published: December 2004

AbstractA 60‐year‐old woman presented with stiff‐person syndrome (SPS). Treatment with diazepam controlled her painful spasms initially. Two and one‐half years after the onset of SPS, new spells of paroxysmal legjerking and apnea developed. A spell was recorded with simultaneous video and polygraphic techniques that revealed simultaneous firing of motor unit potentials in several muscles (paraspinal, internal hamstring, and abdominal muscles). Apnea was associated with arterial oxygen desaturation. An increase in the dose of diazepam decreased the number and severity of these episodes. Seventeen months later, the patient began to taper the diazepam dose. Shortly thereafter, she had a cardiorespiratory arrest and subsequently died. Autopsy showed small chronic inflammatory foci in the pancreas (some associated with islets) and findings of diffuse encephalomyelitis characterized by perivascular cuffing in the spinal cord, brainstem, thalamus, hippocampus, and amygdala and a dense mononuclear infiltrate in the anterior horns of the lumbar and cervical cord, with relative preservation of axons and myelin. Cell typing showed this infiltrate was polyclonal and reactive. There have been rare cases of SPS associated with encephalomyelitis reported previously. Although the prolonged course in our patient suggested that SPS may have preceded encephalomyelitis, the more likely explanation is that the patient had an unusually long course of encephalomyelitis alone.
Foot dystonia and lumbar canal stenosis
Authors: Blunt Stavia B. , Richards Peter G. , Khalil Nofal 
Published: December 2004

AbstractWe describe a patient who developed involuntary, painless, dystonic contraction of the toes of the right foot on standing or walking. The development of this abnormal movement had been preceded by sensory disturbance on the soles of both feet, triggered by dorsiflexion of the feet. Examination showed that weight bearing on the right foot and walking brought on clawing of the toes of the right foot, which was relieved within seconds of taking pressure off the right foot. There was sensory and reflex evidence of bilateral S1 root disturbance confirmed by electrophysiology. Magnetic resonance imaging of the lumbar spine showed marked stenosis of the lumbar canal with compression of the L5 and S1 nerve roots bilaterally. The patient underwent a lumbar laminectomy with nerve root exit foramina decompresion, which abolished the foot dystonia and has considerably improved the sensory disturbance. This case demonstrates that lumbar canal stenosis and/or nerve root compression, may be responsible for foot dystonia. Amelioration of the abnormal movement by surgical decompression argues strongly in favour of this hypothesis.
Imaging of dopamine receptors with [123I]iodobenzamide single‐photon emission–computed tomography in neuroleptic malignant syndrome
Authors: Jauss M. , Krack P. , Franz M. , Klett R. , Bauer R. , Gallhofer B. , Dorndorf W. 
Published: December 2004

AbstractWith the tracer [123I] iodobenzamide;([123I]‐IBZM), it is possible to image dopamine receptor occupancy with singlephoton emission‐computed tomography (SPECT). We report follow‐up examinations with IBZM‐SPECT in neuroleptic malignant syndrome (NMS) to display D2‐receptor availability in the acute phase and during the course of remission. A 27‐year‐old man was admitted with severe akinesia, rigor, tachycardia, fever, and elevated creatine phosphokinase level (CK) after neuroleptic medication. NMS was diagnosed, was started with dantrolene, amantadine, and dopamine agonists. IBZM‐SPECT examination was performed on days 6, 34, 90, 107, 131, and 201. In the acute state of NMS, there was no binding of IBZM to D2‐receptors. SPECT reached almost normal values on day 131, but clinical examination still showed a mild parkinsonian syndrome. With SPECT, the D2‐receptor occupancy in NMS could be successfully shown in correlation with extrapyramidal signs. IBZM‐SPECT may therefore serve to monitor D2‐receptor occupancy in patients at risk for NMS.
Paroxysmal kinesigenic dystonia associated with a medullary lesion
Authors: Riley David E. 
Published: December 2004

AbstractA 67‐year‐old man experienced the abrupt onset of intermittent of intermittent spasms of tightening of his throat muscles and elevation of his tongue to the roof of his mouth. These were precipitated by initiating movements, either of his mouth (eating, drinking, speaking, yawning) or of his whole body (arising from bed or a chair, lifting heavy weights). Episodes occurred six to 20 times per day, lasted 10–30 s, then resolved spontaneously. Two years later, results of his general neurological examination including speech, were normal. Several spasms were provoked by arising from a seated or supine position or by drinking. Objectively, there was a strained dysphonia accompainied by palpable hardening of the supralaryngeal muscles. Each episode resolved within 15s. Magnetic resonance imaging (MRI) showed evidence of a remote hemorrhage in the medulla. No abnormal blood vessels were seen. Phenytoin 300 mg/day abolished the spasms within days. Decreasing the dose to 200 mg/day months later led to a partial return of symptoms. Relief has persisted for 3 years. This patient has paroxysmal kinesigenic dystonia (PKD) of structures (pharynx, larynx tongue,) innervated by lower cranial motor nerves and a medullar lesion on MRI. PKD has been associated with focal lesions at all levels of the central nervous system (CNS), although never before in the medulla. PKD seems to be a nonspecific phenomenon of the CNS in reaction to injury.