bluuebunny/crossref_metadata_2025_split huggingface dataset:
Marked reduction of striatal dopamine D2 receptors as detected by 123IBZM‐SPECT in a Wilson's disease patient with generalized dystonia
Authors: Schwarz J. , Tatsch K. , Vogl T. , Kirsch C. M. , Trenkwalder C. , Arnold G. , Gasser T. , Oertel W. H. 
Published: December 2004

Abstract[123I]iodobenzamide‐single photon emission computed tomography (IBZMSPECT) was employed to study the distribution of dopamine D2 receptors in a patient with biochemically proven Wilson's disease presenting with generalized dystonia. IBZM is a dopamine D2 receptor antagonist with high affinity and specific binding to basal ganglia detectable by SPECT. IBZM‐SPECT in this patient (age, 20 years) displayed a striatum to frontal cortex ratio of 1.2 compared to 1.55 ± 0.05 (mean ± SD) in normal controls (n = 7; mean age, 53.3 years). In parallel with this finding, MRI with heavily T2‐weighted sequences showed atrophy and low signal intensity changes of the basal ganglia. There was no improvement of dystonia after a subcutaneous injection of apomorphine. In contrast, IBZM‐SPECT of a neurologically asymptomatic Wilson's disease patient (age, 21 years) displayed a striatum to frontal cortex ratio of 1.6. The MRI scan of this patient was normal. It is suggested that the observed apomorphine‐unresponsive generalized dystonia in this Wilson's disease patient is related to striatal lesions proven by IBZM‐SPECT and MRI.
A case of bilateral alternating hemifacial spasms
Authors: van de Biezenbos J. B. M. , Horstink M. W. I. M. , van de Vlasakker C. J. W. , van Engelen B. G. M. , van Eikema Hommes O. R. , Barkhof F. 
Published: December 2004

AbstractWe report a 24‐year old woman who developed bilateral hemifacial spasm alternating from one side to the other. The spasms followed a left peripheral facial palsy 2 years previously. This unusual type of bilateral hemifacial spasm was possibly due to lesions of multiple sclerosis in the brainstem.
Hemiballism and chorea in a patient with parkinsonism due to a multisystem degeneration
Authors: Steiger M. J. , Pires M. , Scaravilli F. , Quinn N. P. , Marsden C. D. 
Published: December 2004

AbstractDyskinesias associated with dopaminergic treatment in idiopathic Parkinson's disease (PD) can be indistinguishable from those arising spontaneously in other conditions involving degeneration of, or damage to, the basal ganglia. However, those due to levodopa treatment of PD disappear on cessation of therapy. We report a patient with a clinical diagnosis of PD who, on levodopa treatment, developed hemiballism and chorea that were originally thought to be drug induced. However, the dyskinesias persisted despite stopping levodopa. Postmortem analysis showed a multisystem degeneration.
Posttraumatic segmental axial dystonia
Authors: Jabbari Bahman , Paul John , Scherokman Barbara , van Dam Bruce 
Published: December 2004

AbstractIsolated dystonia of trunk and neck muscles without involvement elsewhere has been termed segmental axial dystonia—a rare disorder. We report a 31‐year‐old man who developed marked dystonia of paraspinal muscles and progressive scoliosis 6 months after a closed head injury. Computed tomography (CT) disclosed three small areas of encephalomalacia, one involving the head of the caudate nucleus. Treatment with trihexyphenidyl resulted in significant improvement of the dystonia and scoliosis.
Pathophysiology of cerebellar ataxia
Authors: Diener H.‐C. , Dichgans J. 
Published: December 2004

AbstractHuman and animal experiments performed recently have resulted in a more detailed understanding of limb movement and body posture disorders associated with cerebellar dysfunction. The delay in movement initiation can be explained by a delay in onset of phasic motor cortex neural discharge owing to decreased input from the cerebellar hemispheres. Disorders of movement termination (dysmetria), which can occur for movements at proximal and distal joints, result from disturbances of the timing and intensity of antagonist electromyographic (EMG) activity necessary to break the movement. Disorders in velocity and acceleration of limb movements result from muscular activity that is smaller in amplitude and more prolonged. The cerebellum is important for control of constant force but not for generation of maximal force. Dysdiadochokinesia is explained by a combination of the abovementioned mechanisms. During complex movements in three‐dimensional space, the cerebellum contributes to timing between single components of a movement, scales the size of muscular action, and coordinates the sequence of agonists and antagonists. The basic structure of motor programs is not generated in the cerebellum. Hypotonia can be observed only in acute cerebellar lesions. Cerebellar tremor appears to result from a central mechanism, but is modulated or provoked through increased long‐loop EMG responses. The common assumption that cerebellar ataxia of stance does not improve with visual feedback is true only of vestibulocerebellar lesions, not for ataxia resulting from atrophy of the anterior lobe of the cerebellum.
Levodopa‐induced dyskinesias in Parkinson's disease: Clinical and pharmacological classification
Authors: Luquin M. R. , Scipioni O. , Vaamonde J. , Gershanik O. , Obeso José A. 
Published: December 2004

AbstractLevodopa‐induced dyskinesias (LID) in Parkinson's disease (PD) may be classified into three main categories: “On” dyskinesias, diphasic dyskinesias (DD), and “off” periods. The study of 168 parkinsonian patients showed that about half (n = 84) showed one pattern of LID only. A combination of two was present in 68, and 16 had the three presentation patterns. A fairly good correlation between type of dyskinesia and presentation pattern was established. Chorea, myoclonus, and dystonic movements occurred during the “on” period. Dystonic postures, particularly affecting the feet, were mainly present in the “off” period, but a few patients had a diphasic presentation. Repetitive stereotyped movements of the lower limbs always corresponded to DD. Acute pharmacological tests using dopamine agonists 9subcutaneous apomorphine 3–8 mg; intravenous lisuride 0. (1–0. 15 mg) and dopamine antagonists (intravenous sulpiride 200–400 mg and intravenous chlorpromazine 25 mg) were performed in 40 patients. Dopamine agonists enhanced “on” dyskinesias and markedly reduced or abolished “off” period dystonia and DD. Dopamine antagonists reduced all types of LID but usually aggravated parkinsonism. These clinical and pharmacological results indicate that LID in PD are a heterogeneous phenomenon difficult to explain on the basis of a single pathophysiological mechanism.
Effect of ethosuximide on rest tremor in the MPTP monkey model
Authors: Gomez‐Mancilla Baltazar , Latulippe Jean‐François , Boucher René , Bédard Paul J. 
Published: December 2004

AbstractBased on the hypothesis that low‐threshold calcium conductance in the thalamus might be involved in the pathophysiology of parkinsonian tremor, ethosuximide was given chronically to a monkey previously treated with MPTP and displaying exceptionally a typical rest tremor. After 5 days of daily treatment, the tremor was reduced by 60%. Diltiazem and verapamil which act on different calcium channels had no such effect. Ethosuximide also potentiated the antitremor effect of the dopamine D2 agonist LY‐171555.
Movement disorders as sequelae of central pontine myelinolysis: Report of three cases
Authors: Maraganore Demetrius M. , Folger W. Neath , Swanson Jerry W. , Ahlskog J. Eric 
Published: December 2004

AbstractIn three survivors of central pontine myelinolysis, dystonia (in two patients) and rest tremor (in one) were sequelae. The onset of these movements occurred 3 weeks to 5 months after the initial presentation with central pontine myelinolysis. Magnetic resonance imaging revealed basal ganglia lesions suggestive of extrapontine myelinolysis in all three patients. We propose that the movement disorders seen in our cases are clinical correlates of extrapontine myelinolysis.
Cerebrospinal fluid antibodies to coronavirus in patients with Parkinson's disease
Authors: Fazzini Enrico , Fleming John , Fahn Stanley 
Published: December 2004

AbstractThe etiology of Parkinson's disease remains unknown, and a search for environmental agents continues. In 1985, Fishman (10) induced infection of the basal ganglia by a coronavirus in mice. Although coronavirus is recognized primarily as a respiratory pathogen in humans, its affinity for the basal ganglia led us to investigate its possible role in human Parkinson's disease. The cerebrospinal fluid of normal controls (CTL) (n = 18), and patients with Parkinson's disease (PD(n = 20)) and other neurological disease (OND) (n = 29) was analyzed in a blinded manner by enzyme‐linked immunosorbent assay [measurements in optical density (OD) units] for antibody response to four coronavirus antigens: mouse hepatitis virus JHM (J) and A59 (A), and human coronavirus 229E (E) and OC43 (O). When compared with CTL, PD patients had an elevated (p < 0.05) mean OD response to J (0.0856 vs. 0.0207) and A (0.1722 vs. 0.0636). Response (p > 0.05) to O (0.0839 vs. 0.0071) was greater than that to E (0.1261 vs. 0.0743). When compared to OND, PD patients had an elevated mean OD response to J (0.0856 vs. 0.0267, p < 0.05). Responses (p > 0.05) to A (0.1722 vs. 0.0929) and O (0.0839 vs. 0.0446) were greater than that to E (0.1261 vs. 0.0946). These results suggest that there may be an association between coronavirus and PD.
Severe but temporary injury to rabbit orbicularis oculi muscle using dihematoporphyrin ether and laser photochemomyectomy
Authors: Wirtschafter Jonathan D. , Slovut David Paul , Stordal Leif , Valentino Joseph , McLoon Linda Kirschen 
Published: December 2004

AbstractThe use of local dihematoporphyrin ether (DHE) injections, followed by laser light activation, was investigated as a potential permanent myectomy treatment for muscle spasms, in particular blepharospasm and hemifacial spasm. DHE was injected into the eyelids of rabbits, followed by laser activation, as used in photochemotherapy. Four days after treatment, histological examination indicated that doses of ≥0.5 mg of DHE and laser treatment with an energy density of at least 100 J/cm2 resulted in an almost total destruction of the orbicularis oculi muscle in the treated eyelid. The amount of muscle injury was dependent on both dose of DHE and energy density levels. Histologically, the tarsal glands and conjunctiva were damaged. Glandular tissue was markedly reduced, and the conjuctival epithelium showed hyperplasia and a loss of mucous cells. Six months after DHE and laser treatment, the majority of the muscle tissue had regenerated, although there was evidence of previous injury. While DHE injections combined with laser light activation were lethal to muscle at the site of treatment, this treatment was not permanent. The orbicularis oculi muscle retained its ability to regenerate. However, photochemomyectomy may be studied further as an adjuvant treatment to temporarily injure and debulk large muscles when botulinum toxin is contraindicated due to the large doses involved or as permanent treatment when used togeter with an antimitotic agent such as doxorubicin.
Cellular interactions in the striatum involving neuronal systems using “classical” neurotransmitters: Possible functional implications
Authors: Nieoullon André , Goff Lydia Kerkerian‐Le 
Published: December 2004

AbstractThe neostriatum contains a wide variety of neuroactive substances associated with several well‐defined functional neuronal systems. This structure, which is the seat of numerous neurological pathological disorders, is commonly used as a model for studying the basic mechanisms of neurotransmitter interactions in the brain and their putative involvement in striatal functions. Increasing interest has been focusing lately on the cellular interactions that may occur between the corticostriatal putatively glutamatergic system and the nigrostriatal depaminergic input. Current evidence suggests that the activatory corticostriatal glutamatergic input may play a more crucial role in regulating striatal functions than was formely assumed in comparison with the dopaminergic input. The key role of cholinergic interneurons in the striatum may therefore be attributable to the fact that they modulate the glutamatergic transmission to GABA striatal efferent neurons. Likewise, dopamine may actually act indirectly in the striatum by “tuning down” the cortical excitation of striatal neurons. Consequently, an impairment of the dopaminergic transmission such as that occurring in parkinsonism may lead to an increase in the corticostriatal glutamatergic transmission, which may further contribute towards reinforcing the “imbalance” between subsets of striatal neuronal systems controlling the output of the basal ganglia.
Golfers' cramp: Clinical characteristics and evidence against it being an anxiety disorder
Authors: Sachdev Perminder 
Published: December 2004

AbstractThe clinical characteristics of 20 golfers suffering from golfers' cramp or the “yips” are described. The typical description is that of a middleaged golfer who has played competitive golf since his teens and develops the problem during a tournament in the form of a jerk, spasm, or freezing of movement while putting or chipping, with the rest of the game being relatively unaffected. The problem generally takes a chronic fluctuating course, and a number of trick strategies are partially or fully successful. In this study, the subjects were compared with a matched group of 20 unaffected golfers on a number of indices of psychopathology; no significant differences emerged. The more severely affected golfers also did not differ significantly from the mildly affected ones, except on the subjective report of anxiety. These data support the argument that golfers' cramp is not an anxiety disorder or a neurosis. The important role of anxiety and arousal in its manifestation is, nevertheless, recognized and its pathophysiology speculated upon.
Noise‐induced psychogenic tremor associated with post‐traumatic stress disorder
Authors: Walters Arthur S. , Hening Wayne A. 
Published: December 2004

AbstractTremors in post‐traumatic stress disorders have not been previously well characterized. A 67‐year‐old man has a 46‐year history of a noiseinduced exaggerated startle reflex followed by a large amplitude rest, postural and kinetic tremor that may persist for up to 3 days. This tremor is superimposed on a continuous mild organic postural/kinetic tremor whose electrophysiological characteristics are different from those of the overlying tremor. We attribute the exaggerated startle reflex and the noise‐induced tremor to Post‐Traumatic Stress Disorder (PTSD) and postulate a psychogenic origin for the noise‐induced tremor. The patient alos believes the noise‐induced tremor to be psychologically based and to be produced by the fear and anxiety he experiences when he hears loud, unexpected noises. The sudden onset of the noise‐induced tremor, its intermittent character, its temporary disappearance on distraction despite the patient's inability to suppress it, inconsistencies in handwriting and figure drawing, and the fact that the noise‐induced tremor is stimulus specific and persists long after the offending stimulus (noise) is no longer present all suggest a tremor of psychogenic origin.
Premorbid smoking, alcohol consumption, and coffee drinking habits in Parkinson's disease: A case‐control study
Authors: Jiméanez‐Jiméanez Félix Javier , Mateo Dolores , Giméanez‐Roldan Santigo 
Published: December 2004

AbstractA number of studies have reported lower cigarette consumption in patients with Parkinson's disease (PD) previous to onset of the disease. In an attempt to determine whether there existed a “premorbid attitude” by patients against the use of socially accepted “drugs,” the premorbid tobacco, alcohol, and coffee consumption habits were compared in 128 PD patients and 256 controls. Patients and controls were selected by case control method and were recruited from the same health area and socioeconomic stratum. In males, the habits of smoking more than 10 cigarettes/day (p < 0.001) and drinking more than 50 g/day of alcohol (p < 0.001) were significantly less frequent in the PD patients than in the controls, but the differences in coffee consumption were nonsignificant. In females behavior did not differ significantly between the PD group and the controls for any of the three habits. There was no correlation between the amount of smoking and alcohol drinking and age at onset of PD or current Hoehn and Yahr's staging. Our results suggest the existence of a premorbid personality in males with PD, possibly conditioning a restrictive attitude toward the consumption of such toxic substances as tobacco and alcohol, yet a more tolerant attitude toward habits more widely accepted socially, like coffee consumption.
Corticobasal degeneration: Decreased and asymmetrical glucose consumption as studied with PET
Authors: Blin J. , Vidailhet M.‐J. , Pillon B. , Dubois Bruno , Feve J.‐R. , Agid Y. 
Published: December 2004

AbstractCerebral energy metabolism was studied by positron emission tomography and [18F]fluorodeoxyglucose in five patients with clinical diagnoses of probable corticobasal degeneration. A reduction in glucose consumption was observed in most cortical and subcortical structures compared to agematched controls. The reduction was greatest on the side of the brain contralateral to the most affected limbs, as shown by the significantly lower ratios of contralateral to homolateral metabolic rates, in the temporal and sensorimotor cortex of patients compared to controls. A distinct asymmetry between the two hemispheres could be observed in a patient who was examined twice in the course of his illness. Detection of this asymmetrical decrease in brain cortical and subcortical glucose metabolism may prove useful as additional evidence supporting clinical diagnoses of the disease.
Isolated continuous rhythmic lingual myoclonus
Authors: Gobernado José M. , Galarreta Mario , de Blas Genma , Jimenez‐Escrig Adriano , Hernandez Amalia 
Published: December 2004

AbstractIsolated continuous lingual myoclonus is an exceptional entity, poorly documented and understood. A patient with a nonepileptic continuous rhythmical myoclonus, affecting the anterior portion of the tongue, as an independent involuntary disorder, is reported. Electromyography showed low frequency (2‐4 Hz) bursts of genioglossus muscles activity. The EEG, visual, auditory and somatosensory evoked responses were normal. Imaging techniques like CT and MRI failed to reveal any brainstem or cerebellar lesion. Lingual myoclonus showed a very good response to sodium valproate.
Reaction time responses in parkinsonian and hemiparkinsonian patients
Authors: Mazzucchi A. , Sinforiani E. , Ludovico L. , Turla M. , Pacchetti C. , Brianti R. , Parma M. , Nappi G. 
Published: December 2004

AbstractTwenty‐ one normal subjects, 32 bilateral parkinsonian patients (BPs) and 29 hemiparkinsonian patients (HP) were submitted to separate or sequentially associated motor tasks that included simple reaction times (RT), choice RTs, directional RTs, and movement RTs. The results showed that simple RTs, directional RTs, and movement times (MT) were slower in BPs as compared to normal subjects; for choice RTs there was no difference. Response patterns were similar in normal controls and BPs. In both groups RTs became more prolonged as sequentially programmed operations increased. If movement occurred at the end of the sequence, they prolonged the RTs of the preceding operations, but MTs per se did not vary. In HPs the same results were observed on the “bad” hand side versus normal controls and versus the healthy side, but a significant statistical level was reached mainly when the “bad” hand was the right one.
The cholinergic system‐dependent myoclonus of the baboon papio is a reticular reflex myoclonus
Authors: Rektor I. , Švejdová M. , Silva‐Barrat C. , Menini C. 
Published: December 2004

AbstractNeurophysiological studies were performed on four Papio papio baboons presenting with nonepileptic myoclonus (a startle response resembling stimulus‐sensitive jerk). Investigations of the EEG, back‐averaged EEG, and somatosensory evoked potentials revealed the absence of cortical correlates preceeding the jerks, and exclusion of cerebral cortex involvement. No long‐latency reflexes could be recorded in these animals. The jerks were symmetric when evoked by unilateral stimulation in normal baboons as well as in a split‐brain animal. Polymyographic records showed that the first muscle involved during the jerk was the trapezius; other muscles were involved with latencies increasing in both cranial and caudal directions. From these data, nonepileptic myoclonus of baboons can be classified as a reticular reflex myoclonus. The involvement of cranial nerves did not follow the layout of the nuclei in the brainstem, indicating that the jerk is most likely generated as a complete movement. The generating structure is probably under cholinergic control. Finally, the Papio papio baboon, which was already known as a model for cortical myoclonus elicited by intermittent photic stimulation in predisposed animals, can also be considered a model for the study of the reticular reflex myoclonus.
Time course of distant effects of local injections of botulinum toxin
Authors: Garner C. G. , Straube A. , Witt T. N. , Gasser T. , Oertel W. H. 
Published: December 2004

AbstractBotulinum toxin A (btx) is used to treat focal dystonias. From accidental intoxications it is known that btx can cause generalized pathologic single‐fiber electromyography (SFEMG) findings. We monitored the onset and course of these disturbances in eight patients who received a small dose of btx (2–22 ng) for therapy of focal dystonias in the head/neck region for the first time via repeated SFEMG investigations at days 0, 3, 6, 9, 12, 28, and 56. Recordings were performed in the extensor digitoum brevis muscle, and in two patients additionally in the tibialis anterior muscle. In six of these patients we found an increase of jitter and blocking. The onset of these changes was in the range of 3–13 days after injection. Fiber density showed a tendency to increase. There was no correlation between SFEMG findings and the dose of injected btx. Possible mechanisms for these observations may be either a very efficient local uptake and retrograde axonal transport via the spinal motor neurons or a systemic distribution via the blood circulation.
Inefficacy of propranolol in attenuation of drug‐induced parkinsonian tremor
Authors: Metzer W. Steven , Paige Stephen R. , Newton Joseph E. O. 
Published: December 2004

AbstractWe investigated the tremolytic effect of long‐acting propranolol (propranolol‐LA) in six subjects with drug‐induced parkinsonism (DIP), using a double‐blind, placebo‐controlled, crossover experimental design. Subjects received propranolol‐LA for 2 weeks and placebo for 2 weeks, with no change in neuroleptic treatment. Tremor frequency and amplitude were objectively quantified at the end of each 2‐week period by computerized tremorgram recording. There were no significant differences in attenuation of DIP tremor by propranolol‐ LA and placebo. Previous investigations reported in the literature have found propranolol to attenuate the tremor of idiopathic parkinsonism (IPD). It is expected that DIP and IPD tremor would respond similarly to propranolol if a solely peripheral or spinal cord tremolytic action were operative. A possible differential attenuation of IPD tremor and DIP tremor provides support for the concept of a higher central tremolytic mechanism of β‐adrenergic receptor‐blocking drugs.