bluuebunny/crossref_metadata_2025_split huggingface dataset:
Movement disorders in adult hydrocephalus
Authors: Krauss Joachim K. , Regel Jens P. , Droste Dirk W. , Orszagh Miro , Borremans Jan J. , Vach Werner 
Published: May 2005

AbstractIn a prospective series of symptomatic adult hydrocephalus characterized by gait disturbance, cognitive impairment, and/or urinary incontinence, 88 of 118 patients (75%) had additional akinetic, tremulous, hypertonic, or hyperkinetic movement disorders. Their prevalence was highest in patients with idiopathic normal pressure hydrocephalus (NPH) of the elderly (56/65 patients, 86%), and they were less frequent in patients with secondary NPH (10/15, 66%), with nonhydrodynamic atrophic/other hydrocephalus (20/33, 61%), and with obstructive hydrocephalus/aqueductal stenosis (2/5, 40%). Akinetic symptoms were found in 73 of 118 patients (62%), and the most frequent movement disorder was upper extremity bradykinesia (55%). Akinetic, tremulous, hypertonic, and hyperkinetic movement disorders were exclusively secondary to causes not related to hydrocephalus in 24 of 118 patients (20%). The proportion of patients with movement disorders not attributable to only such causes was highest in the idiopathic NPH group (44/65, 68%). Thirteen of 118 patients (11%) presented with a parkinsonian syndrome. There was evidence for coexistent Parkinson's disease in four of these patients. Parkinsonism was found to be secondary to NPH in five patients and was found improved after shunting. Akinetic symptoms in patients with NPH generally responded favorably to CSF diversion, which was evident in 80% of a subset of this group. Various other movement disorders did not show definite improvement. The high prevalence of bradykinesia and other akinetic symptoms in NPH and the beneficial effect of shunting on such symptoms suggest that NPH may cause a more generalized disorder of motor function.
Comparison of transcranial sonography, magnetic resonance imaging, and single photon emission computed tomography findings in idiopathic spasmodic torticollis
Authors: Becker Georg , Naumann Markus , Scheubeck Maximilian , Hofmann Erich , Deimling Michael , Lindner Alfred , Gahn Georg , Reiners Christoph , Toyka Klaus V. , Reiners Karlheinz 
Published: May 2005

AbstractVarious lines of evidence suggest that the basal ganglia and thalamus are involved in the pathogenesis of idiopathic dystonia, but unfortunately neuroradiological and pathological data are sparse and controversial. In this study, we have examined 10 patients with spasmodic torticollis by neuroimaging techniques, including transcranial sonography (TS; n = 10), conventional (n = 10) and diffusion‐weighted (n = 5) magnetic resonance imaging (MRI), and single photon emission computed tomography (SPECT; n = 10), employing [123I]iodobenzamide (IBZM) as a ligand with a high affinity to the D2 receptor. In seven patients, TS showed small hyperechogenic lesions in the medial segments of the lentiform nucleus contralateral to the side of head deviation. In accordance with the site of TS abnormalities, diffusion‐weighted MRI displayed a hyperintense lesion in only one patient, while standard MRI of this area was normal in all patients. SPECT revealed a slight but statistically nonsignificant reduction of IBZM tracer uptake in an area corresponding to the dorsal portions of the striatum in 9 of the 10 patients. TS findings support the hypothesis that structural alterations of the pallidothalamic circuit contralateral to the side of head deviation are involved in the pathogenesis of idiopathic spasmodic torticollis. TS may be more sensitive in detecting basal ganglia alterations than MRI.
The variability in the clinical effect induced by botulinum toxin type A: The role of muscle activity in humans
Authors: Eleopra R. , Tugnoli V. , de Grandis D. 
Published: May 2005

AbstractWhen botulinum toxin (BT) is administered for the first time at fixed doses, variable clinical responses can be observed in patients with the same form of dystonic disorder. Many factors may contribute to this phenomenon, including the variability in the rate of absorption of the drug. Animal experimental models (rat diaphragm preparation) have demonstrated an increased absorption of BT in the terminal nerve endings of the muscle under repetitive electrical stimulation, suggesting that “muscle activity” also may play an important role. The aim of our study was to evaluate in humans the role of the muscle activity on the variability of the effect induced by BT type A. Eleven patients with blepharospasm and idiopathic facial hemispasm were studied by using neurophysiologic techniques. In nine patients, both extensor digitorum brevis (EDB) muscles were injected with low (3 IU), fixed doses of type A BT. For the first 24 h after administration of the drug, periodic electrical stimulation of only one EDB was used. The subsequent percentage changes in compound muscle action potential (CMAP) amplitude was calculated at different intervals over a 30‐day period. The percentage change in the CMAP for the stimulated EDB was compared with that of the contralateral nonstimulated side. We found that the effect of the induced neuromuscular blockade was significantly greater for the stimulated side. In the other two patients, we injected BT in one EDB and the same volume of normal saline solution in the contralateral muscle to assess the stability of the CMAP in untreated muscle over time. We observed that the CMAP was unchanged in the untreated EDB; therefore we concluded that muscle activity plays an important role in the variability of clinical response often seen.
The effects of chronic levodopa treatment on pre‐ and postsynaptic markers of dopaminergic function in striatum of parkinsonian monkeys
Authors: Rioux Lise , Frohna Paul A. , Joyce Jeffrey N. , Schneider Jay S. 
Published: May 2005

AbstractTherapeutic treatment of parkinsonian monkeys by chronic administration of levodopa (1‐DOPA) leads to the development of dyskinesias and other motor fluctuations. It is unclear whether there are alterations in the dopamine system that are related to the induction of dyskinesias by 1‐DOPA, but recent attention has focused on the D1 receptor system. The present study assessed the consequences of chronic 1‐DOPA treatment in monkeys made parkinsonian with 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP) on indices of the pre‐ and post‐synaptic dopamine (DA) system. Treatment with therapeutic doses of 1‐DOPA led to the induction of dyskinesias in the MPTP‐treated monkeys. High‐pressure liquid chromatography was used for measurement of tissue levels of DA and its metabolites, and quantitative autoradiography was used to examine the regional integrity of the presynaptic DA system (by measuring [3H]mazindol binding to DA uptake sites). Quantitative autoradiography was used to measure the number of postsynaptic D1 receptors (using [3H] SCH 23390) in the striatum and pallidum of normal, MPTP alone, and MPTP monkeys treated chronically with 1‐DOPA. In both MPTP‐treated monkeys, levels of DA and metabolites as well as [3H]mazindol binding were greatly reduced in the caudate and putamen, slightly more in dorsal than in ventral areas. However, the lack of increase in striatal DA levels along with higher [3H]mazindol binding in MPTP‐plus‐1‐DOPA‐treated monkeys suggested differences in the way DA was used after 1‐DOPA treatment. In MPTP‐treated monkeys, a significant increase (141–170% of normals) of D1 receptor numbers was observed in putamen and dorsal caudate. With 1‐DOPA treatment, the number of D1 receptor numbers was further elevated in caudal putamen (119–123%), dorsal caudate (110–130%), and in the internal segment of the globus pallidus (GPi; 164% of normals) of MPTP‐treated monkeys as compared with MPTP treatment alone. This suggested that in MPTP‐treated monkeys made dyskinetic by chronic pulsatile delivery of 1‐DOPA, there was enhanced production of D1 receptors in the direct striatal output to the GPi.
The clinical features and prognosis of chronic posthypoxic myoclonus
Authors: Werhahn K. J. , Brown P. , Thompson P. D. , Marsden C. D. 
Published: May 2005

AbstractThe clinical and neurophysiologic features of 14 patients with chronic posthypoxic myoclonus are presented. Patients were first seen a mean of 2.5 years (range, 2 to 105 months) after the hypoxic event and followed up for 3.7 years (range, 7 to 84 months) thereafter. All patients had had a cardiorespiratory arrest, most caused by an acute asthmatic attack (11 cases). All patients had multifocal action myoclonus. Eleven patients had additional stimulus‐sensitive myoclonus. There was late improvement in the myoclonic syndrome and the level of disability in all but one patient. Three patients were eventually able to discontinue antimyoclonic medication, and five patients were able to walk unaided. Cognitive deficits were found in seven patients and were usually mild. Other neurologic deficits were rare. Electrophysiologic investigation confirmed cortical action myoclonus in every case, although this could be combined with cortical reflex myoclonus, an exaggerated startle response, or brainstem reticular reflex myoclonus. We conclude that posthypoxic myoclonus typically consists of multifocal cortical action myoclonus that improves with time. It is only rarely associated with severe additional neurologic deficit.
Facial myokymia in multiple system atrophy
Authors: Blunt S. B. , Khalil N. M. , Perkin G. D. 
Published: May 2005

AbstractTwo patients are described with clinical and neuroimaging features consistent with a diagnosis of multiple system atrophy (MSA). The patients are unusual in that facial myokymia became apparent clinically at some stage in their illness. In each patient, the nature and severity of the involuntary facial movements evolved over the course of the illness. Electrophysiologically the movement pattern was consistent with myokymia, and studies of blink‐reflex responses suggested that the myokymic discharges were of brainstem origin. Involuntary facial movements described as facial action myoclonus with electrical characteristics consistent with myoclonus have been described previously in hereditary olivopontocerebellar atrophy (OPCA). Our report describes electrical and clinical features of facial myokymia in MSA with electrical features suggesting hyperexcitability of the facial motorneurons in the brainstem. Such myokymic movements may occur more frequently in MSA than previously recognised but may be missed clinically because of their evolving nature.
Clinical and pharmacological differences in early‐ versus late‐onset Parkinson's disease
Authors: Arevalo G. Gomez , Jorge R. , Garcia S. , Scipioni O. , Gershanik O. 
Published: May 2005

AbstractWe evaluated whether patients with early‐onset Parkinson's disease (EOPD) have a different clinical profile and pharmacological response than those with late‐onset disease (LOPD). We performed a retrospective analysis and an acute pharmacological challenge with L‐Dopa in 34 EOPD (age at onset between 21 and 40 years) and 34 LOPD (onset after age 60) patients. All patients complete a structured questionnaire cross‐checked against mediacal record charts and underwent an acute levodopa test. Most significant differences were in the mode of onset, time to diagnosis, and degree of initial improvement. We did not observe differences with regard to motor fluctuations. The acute levodopa test showed no differences in latency to response onset between groups. However, the magnitude of the response was greater and the duration shorter in EOPD patients. Younger patients had greater reductions in bradykinesia scores, whereas posture/gait symptomatology was less responsive in older patients. The type and severity of dyskinesias also differed significantly between groups. Our findings suggest that central pharmacokinetics, pharmacodynamics, and, possibly, nondopaminergic systems play a role in the age‐related differences observed in Parkinson's disease.
Mitochondrial respiratory chain function in multiple system atrophy
Authors: Gu M. , Gash M. T. , Cooper J. M. , Wenning G. K. , Daniel S. E. , Quinn N. P. , Marsden C. D. , Schapira A. H. V. 
Published: May 2005

AbstractMultiple system atrophy (MSA) is a clinicopathological entity distinct from idiopathic Parkinson's disease (PD) that is responsible for 5–10% of cases of parkinsonism. Degeneration of nigral neurones is a feature of both diseases. A specific deficiency of mitochondrial complex I activity has been found in PD substantia nigra. We have analysed mitochondrial function in substantia nigra and platelets from MSA patients to identify any respiratory chain defect in this disorder and to determine its tissue specificity. As our MSA patients had been on L‐DOPA, we also sought to establish whether this treatment could cause the complex I defect as seen in PD. We found no significant difference in respiratory chain activity corrected for mitochondrial mass between control and MSA patients in either of the tissues studied. These results provide a biochemical dimension to the differences between MSA and idiopathic PD. In addition, the fact that L‐DOPA failed to induce a complex I defect in MSA substantia nigra suggests that this treatment is unlikely to cause the complex I deficiency in PD, without additional factors that may operate in PD.
Cervical dystonia associated with tumors of the posterior fossa
Authors: Krauss Joachim K. , Seeger Wolfgang , Jankovic Joseph 
Published: May 2005

AbstractCervical dystonia was associated with posterior fossa tumors in three patients. The onset of dystonia paralleled the appearance of other focal neurologic signs. All patients had extra‐axial tumors located in the cerebellopontine angle that were removed via suboccipital approaches. The tumors were identified as schwannomas arising from the glossopharyngeal nerve and from the vagus/accessory nerves; and a meningioma. Postoperatively, the cervical dystonia improved markedly during a period of 8 years in one patient, and it remitted completely within 1 year in another patient. In the third patient, cervical dystonia persisted. The combination of the clinical findings and the temporal relationship of their appearance suggest a causal association between the posterior fossa tumors and cervical dystonia in these three cases. Possible pathogenic mechanisms are reviewed.
[123I]β‐CIT single‐photon emission tomography in DOPA‐responsive dystonia
Authors: Naumann Markus , Pirker Walter , Reiners Karlheinz , Lange Klaus , Becker Georg , Brücke Thomas 
Published: May 2005

AbstractThe radiotracer [123I]β‐CIT is a sensitive marker of dopamine uptake sites that can be used to visualize dopaminergic nerve endings in vivo in the human brain. We report on [123I]β‐CIT single‐photon emission computed tomography (SPECT) findings in a patient with DOPA‐responsive dystonia (DRD). [123I]β‐CIT SPECT showed a striatal radiotracer uptake in the upper range of normal, indicating intact dopamine transporters and structural integrity of nigrostriatal neurons. This differentiates DRD from clinically similar cases with juvenile‐onset parkinsonism with dystonia that have a considerably poorer prognosis. [123I]β‐CIT SPECT may provide a method equally as useful as fluorodopa positron emission tomography in DRD.
Sleep benefit in parkinson's disease
Authors: Merello Marcelo , Hughes Andrew , Colosimo Carlo , Hoffman Marion , Starkstein Sergio , Leiguarda Ramon 
Published: May 2005

AbstractSleep benefit (SB) In Parkinson's disease (PD) is not well characterized. To determine SB frequency, as well as to characterize and correlate it with other disease variables, we evaluated prospectively a consecutive series of 312 PD patients by means of a structured questionnaire: 55% reported having SB and 35% reported that awakening was their best time of the day. Because of SB, 21% of the entire population were able to skip or delay medication. The mean duration of the phenomenon was 85.4 ± 67 min. Patients with SB were significantly older (p < 0.0002), had disease longer (p < 0.05), and were often men (X2 = 3.5, df 1, p = 0.05). Patients with SB took sleep medication with similar frequency as those without SB. There were no differences in hours of sleep or sleep latency. Sleep problems such as nightmares or somnambulism, but not the number of sleep awakenings, were similar in both groups. In conclusion, SB is a frequent phenomenon, especially in men, elderly patients, and patients with longer disease duration. SB enables the morning L‐dopa dose to be postponed in ∼50% of patients.
Severe tardive dystonia: Treatment with continuous intrathecal baclofen administration
Authors: Dressler Dirk , Oeljeschläger Remt‐Otto , Rüther Eckart 
Published: May 2005

AbstractTreatment of tardive dystonia with oral baclofen produces ambivalent and overall disappointing results. However, because only a small proportion of the baclofen penetrates into the central nervous system when administered orally, we tested whether it is possible to increase the efficacy of treatment by continuous intrathecal infusion of baclofen (CITB) in a patient with severe tardive axial dystonia unresponsive to conventional therapy. A dose of 100 m̈g/day improved muscle tone, head control, posture, and walking distance; electromyography showed a marked decrease of dystonic muscle activity with fully preserved voluntary muscle activity, and the patient reported substantial reduction of pain. Apart from some minor discomfort at the site of operation, no side effects were noted. Further studies should be encouraged to evaluate the usefulness of CITB for other patients with severe and otherwise untreatable tardive dystonia.
A tourette‐like syndrome following cardiopulmonary bypass and hypothermia: MRI volumetric measurements
Authors: Singer Harvey S. , DelaCruz Pablito S. , Abrams Michael T. , Bean S. Charles , Reiss Allan L. 
Published: May 2005

AbstractWe present the case of an adolescent boy who developed a variety of simple and complex motor and vocal tics (Tourette‐like syndrome), along with inattentiveness and obsessive‐compulsive behaviors after cardiac surgery with cardiopulmonary bypass and profound hypothermia. A single photon emission computed tomography study 2 months after surgery showed reduced uptake in the left hemisphere and 2 years later a perfusion defect in the basal ganglia. Serial magnetic resonance imaging (MRI) studies were normal. Volumetric MRI studies were obtained 4 years after surgery and compared with published values for normal individuals and children with Tourette syndrome (TS), including subsets matched for age, sex, and handedness. Measurement of basal ganglia structures showed a right‐dominant asymmetry of the caudate and putamen, in part similar to findings previously reported in patients with TS. Other volumetric abnormalities included a >2‐SD reduction of cortical gray matter, a small decrease of total cerebral volume, and increase in cerebral white matter. Although a variety of neurological problems may occur after cardiopulmonary bypass, to our knowledge this case represents the first report of a chronic tic disorder following cardiac surgery with cardiopulmonary bypass and hypothermia.
Myoclonus in corticobasal degeneration
Authors: Carella Francesco , Ciano Claudia , Panzica Ferruccio , Scaioli Vidmer 
Published: May 2005

AbstractFive patients with unilateral myoclonus and a clinical diagnosis of corticobasal degeneration (CBD) were studied. All patients showed enhanced long‐loop responses in their myoclonic arms without enlarged somatosensory potentials. The cortical relay time of the long‐loop responses was studied in three patients, in two of whom it was <2 ms, even in the nonmyoclonic arm. Myoclonus in CBD is probably related to an enhanced long‐loop reflex whose pathway is unlikely to be the same as that in classic cortical reflex myoclonus.
Cerebellar hypermetria: Reduction in the early component of the antagonist electromyogram
Authors: Wild Barbara , Corcos Daniel M. 
Published: May 2005

AbstractSeveral articles have described the electromyographic (EMG) patterns underlying cerebellar hypermetria. However, the description of the EMG associated with hypermetria is not consistent between studies. It is hypothesized that the reason for this concerns the criteria used to define and describe the antagonist latency. Several studies on neurologically normal individuals have shown that there are two components to the antagonist EMG: an early component and a late component. However, many studies have identified only one component and have not defined whether it was the early or late component. A reanalysis of one published data set that had previously identified only one antagonist component was performed. The reanalysis suggests that hypermetria can be caused by an absence of an early antagonist EMG component, a delayed late component, or both. In addition, a new measure is suggested that allows this hypothesis to be further evaluated.
Globus pallidus internus pallidotomy for generalized dystonia
Authors: Lozano Andres M. , Kumar R. , Gross R. E. , Giladi N. , Hutchison W. D. , Dostrovsky J. O. , Lang Anthony E. 
Published: May 2005

AbstractThe authors present a young boy with severe generalized dystonia treated with bilateral simultaneous pallidotomy. Microelectrode recordings with the patient under propofol anesthesia showed that the mean discharge rate of globus pallidus internus (GPi) neurons was between 21 and 31 Hz. This contracts sharply with the mean GPi neuronal firing rates of approximately 80 Hz that are characteristic of Parkinson's disease. The patient had no immediate benefit from surgery, but a progressive improvement in both axial and limb dystonia began within 3 days. The Burke‐Fahn‐Marsden scores were 75 (maximum possible = 120) at baseline, 52 at 5 days, and 16 at 3 months after surgery. The mechanism of action of pallidotoy for dystonia and the reasons for the delayed and progressive improvement are unknown. Nevertheless, the magnitude of the improvement and the safety of the procedure in this one patient warrant a careful evaluation of pallidotomy for dystonia.
Symptoms and duration of the prodromal phase in parkinson's disease
Authors: Gonera Ed G. , Hof Martin Van't , Berger Hans J. C. , van Weel Chris , Horstink Martin W. I. M. 
Published: May 2005

AbstractTo investigate the duration of a prodromal phase before the onset of the classic symptoms of idiopathic Parkinson's disease the authors conducted a retrospective case control study of 60 patients with Parkinson's disease and 58 age‐ and sex‐matched control subjects, covering the decade preceding the onset of classic Parkinson's disease. The symptoms were derived from files of the patients general practitioners. Compared with control subjects, patients pre‐Parkinson's disease had more central nervous system, psychologic, musculoskeletal, and cardiovascular (i.e., autonomic) symptoms. Patients pre‐Parkinson's disease also made more visits to general practitioners and medical specialists. The results indicate that the onset of classic parkinsonism is frequently precede by a prodromal phase lasting from 4–6 years.