bluuebunny/crossref_metadata_2025_split huggingface dataset:
Idiopathic cervical dystonia: Clinical characteristics
Authors: Chan Jane , Brin Mitchell F. , Fahn Stanley 
Published: December 2004

AbstractWe reviewed detailed clinical features of 266 patients with idiopathic cervical dystonia, commonly called spasmodic torticollis. Mean age at onset (41 years), female‐to‐male ratio (1.9:1), clustering of onset between ages 30 and 59 (70%), familial history of dystonia (12%), and remissions (9.8%) were similar to those found in previous studies. In contrast to the single prior large clinical study of this disorder, no predominance of right‐handers or significant thyroid disease was found. Pain, which occurred in 75% of patients and contributed to disability score (p <0.01), distinguishes this syndrome from all other focal dystonias. Pain was also strongly associated with constant (vs. intermittent) head turning, severity of head turning, and presence of spasm. Eighty‐three percent of patients had deviation of the head of >75% of the time when sitting with the head unsupported (constant head deviation at rest). Of the 97% who had head turning, 81% also had head tilting in various combinations. The 23% with hand tremor had an older age at onset (mean, 46 vs. 41 years; p <0.05). An earlier age at onset (p <0.05) was seen in patients with a family history of dystonia (mean, 36 years), with trauma shortly preceding symptoms (mean, 36 years), with a change in the direction of head turning (mean, 30 years), and with remissions (mean, 33 years). Jerky movements or forced transient spasms of the head occurred in 62% of the patients, and these patients would be the ones for whom the designation “spasmodic torticollis” could logically apply. Therefore, because the cervical dystonia in 38% of patients is not spasmodic, we propose that the term “spasmodic torticollis” is not a completely appropriate designation for this condition. We suggest labeling the condition either idiopathic cervical dystonia or idiopathic dystonic torticollis, preferably the former for reasons explained in the text.
Isolated lingual/palatal dystonia
Authors: Robertson‐Hoffman Doreen E. , Mark Margery H. , Sage Jacob I. 
Published: December 2004

AbstractWe report a 45‐year‐old woman with a 3‐year history of continuous dystonic movements of tongue and palate with intermittent episodes of noticeable worsening lasting 6 to 8 h. The movements began immediately after a viral illness. The only contributory history is that the patient received high doses of prochlorperazine 22 years earlier for hyperemesis gravidarum. The patient appears to have an unusual focal lower cranial dystonia. Proposed etiologies may be idiopathic or related to prior use of a phenothiazine with a viral trigger.
Hereditary paroxysmal ataxia with neuromyotonia
Authors: Vaamonde J. , Artieda J. , Obeso J. A. 
Published: December 2004

AbstractThe clinical manifestations of a patient with hereditary paroxysmal ataxia and neuromyotonia are described. Generalized tremor, triggered by sudden movemnts, and spasms of hand and foot muscles were the main clinical findings. Electromyogram (EMG) and nerve blocking studies led to the diagnosis of neuromyotonia. Treatment with acetozolamide was of no therapeutic value, confirming previous observations about the difference in response of paroxysmal ataxia with and without neuromyotonia.
Lesion of the subthalamic nucleus for the alleviation of 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP)‐induced parkinsonism in the primate
Authors: Aziz T. Z. , Peggs D. , Sambrook M. A. , Crossman A. R. 
Published: December 2004

AbstractCentral to the neural mechanism of MPTP‐induced parkinsonism is overactivity of the subthalamic nucleus. Lesion of the subthalamic nucleus has therefore been performed in two primates with this condition and found to produce a remarkable amelioration of the disorder.
L‐dopa does not affect electron transfer chain enzymes and respiration of rat muscle mitochondria
Authors: Dagani Fiorenzo , Ferrari Rosaria , Anderson Jeffrey J. , Chase Thomas N. 
Published: December 2004

AbstractAlterations in mitochondrial respiratory chain enzymes have been found in skeletal muscle of parkinsonian patients. Most of these patients had received treatment with L‐dopa in combination with an inhibitor of peripheral decarboxylase for several years. In order to determine whether these effects are only dependent on the disease or are partially due to its therapy, the effects of L‐dopa methyl ester and benserazide, a peripheral dopa decarboxylase inhibitor, were studied on various parameters related to energy metabolism in rat skeletal muscle mitochondria. The maximum activities related to complexes of the respiratory chain: rotenone‐sensitive NADH‐cytochrome c reductase, succinate‐cytochrome c reductase, cytochrome c oxidase, state 3, state 4, uncoupled state, and respiratory control ratio were measured after 17‐19 days of treatment. The results indicate that L‐dopa treatment does not interfere with any of the parameters investigated and suggest that changes in muscle mitochondrial function found in parkinsonian patients are the result of the disease process and not its treatment.
Idiopathic Parkinson's disease combined with multiple system atrophy. A clinicopathological report
Authors: Hughes Andrew J. , Daniel Susan E. , Lees Andrew J. 
Published: December 2004

AbstractThe clinical and postmortem findings of a patient with Lewy body pathology combined with multiple‐system atrophy are described. When alive the patient was diagnosed as having Parkinson's disease. Pathological examination found evidence of striatonigral and olivopontocerebellar degeneration, together with Lewy bodies at a number of sites characteristic for idiopathic Parkinson's disease. Taken together, the clinical history and histological findings support the coexistence of two disease processes.
Segmental ballistic movement overflow myoclonus: A clinical, electromyographic, and pharmacologic evaluation
Authors: Stübgen J.‐P. , Lotz B. P. 
Published: December 2004

AbstractA 27‐year‐old man presented with a sporadic, nonprogressive, right‐sided, segmental movement disorder that had started at the age of 2 years. Ballistic movement overflow myoclonus was diagnosed clinically and by surface electromyography. Special investigations did not detect anything except atrophy of the left temporal lobe on CT scan. The movement intensity was attenuated by alcohol but was minimally affected by high doses of oral propranolol (320 mg daily), clonazepam (36 mg daily), or trihexyphenidyl (32 mg daily). High‐dose intravenous biperidine (10 mg) resulted in dramatic improvement, but with unacceptable side effects. The possible role of a cholinergic disturbance in generating myoclonus is discussed.
Neurotoxicity of levodopa on catecholamine‐rich neurons
Authors: Mena María Angeles , Pardo Beatriz , Casarejos María José , Fahn Stanley , de Yébenes Justo García 
Published: December 2004

AbstractThe human neuroblastoma cells NB69 are a catecholamine‐rich cell line with pharmacological properties similar to dopamine neurons. This cell line was used to study the neurotoxicity of levodopa on catecholamine neurons. Levodopa, at 50 × 10−6 M or higher concentrations, produced a doseand time‐dependent reduction in the number of live cells, [3H]thymidine uptake, levels of protein and DNA, and an enhancement of the quinone formation. This is a specific effect of levodopa since it did not happen in NB69 cells incubated with equimolar concentrations of leucine and tryptophan. Treatment with deprenyl, an inhibitor of monoamine oxidase type B, partially prevented levodopa neurotoxicity, suggesting that the mechanism of toxicity was, at least in part, related to an increase in the metabolism of dopamine catalyzed by monoamine oxidase.
Persistent dystonia possibly induced by flecainide
Authors: Miller Lucinda G. , Jankovic Joseph 
Published: December 2004

AbstractA 55‐year‐old man developed clicking of his teeth, involuntary biting, chewing and grimacing within three days of initiation of flecainide. We suggest flecainide should be added to the list of benzamide derivatives associated with drug‐induced movement disorders.
Progressive dystonia symptomatic of juvenile GM2 gangliosidosis
Authors: Nardocci Nardo , Bertagnolio Barbara , Rumi Viviana , Angelini Lucia 
Published: December 2004

AbstractA 9‐year‐old boy showed a progressive generalized dystonia, with onset at the age of 4 years, combined with mental deterioration and behavioral disturbances. The values of β‐hexosaminidase activities studied in plasma, leukocytes, and fibroblasts obtained using two different substrates (MUG‐NAc and MUG‐NAc‐6‐S) were significantly reduced but higher than in Tay–Sachs disease and similar to those found in the juvenile chronic form of GM2 gangliosidosis. With anticholinergic therapy, for 1.5 years, the dystonic symptoms did not progress and the boy can still care for himself and attend school. The description of another case of the disease. clinically expressed as dystonia, corroborates the existence of dystonic phenotype of GM2 gangliosidosis.
Permanent bruxism as a manifestation of the oculo‐facial syndrome related to systemic whipple's disease
Authors: Tison François , Louvet‐Giendaj Catherine , Henry Patrick , Lagueny Alain , Gaujard Eric 
Published: December 2004

AbstractWe report here a new case of oculomasticatory syndrome related to systemic Whipple's disease. The patient presented typical ophthalmoparesis associated with ocular myorhythmia consisting of 1 Hz convergent oscillations of both eyes. The masticatory involvement was remarkable and consisted of a permanent bruxism leading to severe tooth abrasions. Possible pathophysiology of such a disorder is discussed.
The undulating toe flexion sign in brain death
Authors: McNair N. L. , Meador K. J. 
Published: December 2004

AbstractBrain‐dead patients may exhibit gross spontaneous and reflex movements (e.g., Babinski sign, sterotypic flexion of one or more limbs, and Lazarus sign). We report three brain‐dead patients who had unusual complex sequential movements of the toes. Undulating toe flexion was elicited by noxious stimuli to the lower extremities, and consisted of initial plantar flexion of the great toe, followed by sequential brief plantar flexion of the second, third, fourth, and fifth toes. The undulating toe flexion sign differs from previously described responses characterized by plantar flexion of the toes (e.g., Rosselimo's sign and the Mendel‐Bechterew sign) in that it consists of complex patterned sequential movements of the digits rather than brief simultaneous flexion and/or fanning of the toes. Neurologists should be aware of this unusual finding, which should not preclude the diagnosis of brain death.
Diffuse lewy body disease presenting with supranuclear gaze palsy, parkinsonism, and dementia: A case report
Authors: de Bruin V. M. S. , Lees A. J. , Daniel S. E. 
Published: December 2004

AbstractA 67‐year‐old man with a family history of parkinsonism had visual complaints due to difficulty in convergence, which was followed 2 years later by development of bradykinesia and rigidity. The diagnosis of Steele Richardson‐Olszewski syndrome was made on the basis of a supranuclear gaze palsy, bradykinesia, rigidity, and poor response to levodopa. However, subsequent neuropathological examination revealed diffuse Lewy body disease with no evidence of neurofibrillary tangles involving either subcortical or brain stem structures.
Recumbent tic
Authors: Davies L. , King P. J. L. , Leicester J. , Morris J. G. L. 
Published: December 2004

AbstractA 59‐year‐old man with a 30‐year history of an unusual movement disorder characterised by involuntary axial spasms that occur only in recumbency is described. Clinical and electrophysiological evidence suggest that this disorder is best characterised as a simple tic of unusual form. We have coined the term “recumbent tic” to describe this disorder.
Stimulus‐sensitive paroxysmal dyskinesias associated with a thalamic infarct
Authors: Nijssen P. C. G. , Tijssen C. C. 
Published: December 2004

AbstractWe describe a patient with the Dejerine‐Roussy syndrome (hemiparesis, hemiataxia, hemidysesthesia, and pain), due to posterolateral thalamic infarction, who also exhibits paroxysmal dyskinesias triggered by action and by sensory stimulation.
Phenytoin and dyskinesias: A report of two cases and review of the literature
Authors: Harrison M. B. , Lyons G. R. , Landow E. R. 
Published: December 2004

AbstractDyskinesia is a recognized but uncommon side‐ effect of treatment with phenytoin. Two additional cases of dyskinesia during treatment with phenytoin are described; both had radiographically documented thalamic infarctions. The reported experience to date with movement disorders induced by phenytoin is reviewed and the clinical features summarized. The available experimental evidence addressing the mechanism underlying this side effect is discussed.
Quantitative measurement of cervical range of motion in patients with torticollis treated with botulinum A toxin
Authors: Dykstra Dennis , Ellingham Corine , Belfie Albert , Baxter Tanya , Lee Myoung , Voelker Ann 
Published: December 2004

AbstractImprovement in cervical range of motion in patients with spasmodic torticollis by botulinum A toxin injection is difficult to objectively measure. Recently, a three‐dimensional cervical range of motion system (EMROM) that measures primary as well as secondary cervical angles has been developed. This system uses an electromagnetic tracking system for data collection and a personal computer for analysis and graphic display of the data. We have tested the EMROM system and, from our results, believe that it can be used clinically to objectively and accurately measure cervical range of motion in patients who have spasmodic torticollis and who receive botulinum toxin injections.
Single‐dose L‐dopa response in early parkinson's disease: Measurements with optoelectronic recording technique
Authors: Johnels Bo , Ingvarsson Páll E. , Holmberg Björn , Matousek Michael , Steg Göran 
Published: December 2004

AbstractTwenty‐five patients with suspected Parkinson's disease were submitted to optoelectronic movement analysis with the Posturo‐ Locomotor‐Manual (PLM) test before and 60 min after a single dose of L‐Dopa. They were then examined clinically for diagnosis. Two patients were excluded due to L‐Dopa intolerance. Seventeen of the remaining patients were classified as having Parkinson's disease. The movement time (MT) in the PLM test was increased for all these patients, and they improved their performance after L‐Dopa. The degree of improvement was roughly proportional to the pretreatment augmentation of MT in comparison to healthy subjects of the same age. The PLM phase analysis showed a specific disability profile for each individual. Six patients were given diagnoses other than PD. Some improvement was found in one patient with suspected olivopontocerebellar atrophy and one patient with multiple brain injury. Two patients with progressive supranclear palsy, one with suspected striatonigral degeneration, and one with functional disturbance deteriorated after L‐Dopa. In conclusion, truly objective and fully reproducible evaluation of the motor performance before and after a single L‐Dopa dose is easily accomplished with computer‐assisted modern optoelectronic recording equipment. The technique is a valuable tool for the quantitative measurement of treatment effects and contributes to the differential diagnosis.
Comparison of somatosensory evoked potentials with striatal glucose consumption measured by positron emission tomography in the early diagnosis of huntington's disease
Authors: Kuwert T. , Noth J. , Scholz D. , Schwarz M. , Lange H. W. , Töpper R. , Herzog H. , Aulich A. , Feinendegen L. E. 
Published: December 2004

AbstractBoth somatosensory evoked potentials (SEP) and striatal glucose consumption (rCMRGlc) measured by positron emission tomography (PET) have been reported to be abnormal early in the course of Huntington's disease (HD). To compare their diagnostic value, SEP and rCMRGlc were measured in a group of 18 first degree off‐ spring of HD families: 6 had manifest HD with chorea and the remaining 12 individuals were chorea‐ free subjects at risk for HD. In five patients with manifest disease, both SEP and striatal rCMRGlc were significantly abnormal, defined in SEP as having either a bilaterally absent frontal N30 amplitude or a reduction of the parietal N20/P25 amplitude below 1 μV on at least one side; in PET as exhibiting a reduction of the cerebellar ratio (CR) of both caudate and lentiform rCMRGlc below the 99%confidence limits of these variables determined in 20 normal volunteers. The remaining patient with manifest HD had questionably abnormal SEP and significantly reduced indices of striatal rCMRGlc. The five persons at risk for HD who had normal SEP also had normal striatal rCMRGlc; those three at‐risk patients with abnormal SEP also had a reduction of the CR of both caudate and lentiform rCMRGlc. Of the remaining four individuals at risk for HD who had questionably abnormal SEP, three had CR values of striatal rCMRGlc in the normal range and one a reduction of the CR of lentiform rCMRGlc. In at‐risk patients, the SEP diagnosis correlated significantly with caudate (r =−0.8; p < 0.002) and lentiform (r = −0.76; p < 0.005) rCMRGlc. These data indicate a parallel deterioration of SEP and striatal rCMRGlc early in the course of HD even before the development of chorea.
Does tolerance develop to levodopa? Comparison of 2‐and 21‐h levodopa infusions
Authors: Nutt John G. , Carter Julie H. , Woodward William , Hammerstad John P. , Gancher Stephen T. 
Published: December 2004

AbstractTo determine if acute tolerance to levodopa develops, we compared the response to 2‐ and 21‐h levodopa infusions with the rate adjusted during the long infusion to yield the same plasma concentration as at the end of the 2‐h infusion. The duration of response after discontinuing the long infusions was briefer than after discontinuing the short infusion, suggesting the development of tolerance. Furthermore, dyskinesia severity was greater during long infusions. We conclude that continuous dopaminergic stimulation with levodopa may not offer optimal control of parkinsonism in patients with response fluctuations.
Tremor due to stroke
Authors: Ferbert A. , Gerwig M. 
Published: December 2004

AbstractWe report on four patients with unilateral tremor stemming from cerebrovascular accidents. In two patients with proven lesions of the thalamus, the tremor was irregular and, in addition, there was dystonic posturing of the affected arm. Tremor and dystonic posturing had appeared after the stroke. In the other two patients tremor had occurred immediately at the onset of the stroke and lasted only a few days. The tremors were of small amplitude and high frequency, and lesions could not be found on CT or MRI in these two patients. None of our patients showed signs of the so‐called rubral tremor.
Postneuroleptic laryngeal dyskinesias: A cause of upper airway obstructive syndrome improved by local injections of botulinum toxin
Authors: Fève A. , Angelard B. , Fé;nelon G. , Logak M. , Guillard A. , Saint‐Guily J. Lacau 
Published: December 2004

AbstractWe report a case of laryngeal dyskinesia resulting in severe rest and exercise dyspnea. A 51‐year‐old man treated for 2 years with flupentixol, an incisive neuroleptic, developed severe dyspnea due to intermittent, rhythmic, and dystonic movements of the vocal cords and upper airway. Local injections of botulinum toxin resulted in spectacular regression of laryngeal spasms and major improvement in breathing. This case emphasizes the risk of upper respiratory dyskinesias associated with neuroleptic treatment and shows the feasibility of a new local treatment in this life‐threatening disorder.
Familial idiopathic strio‐pallido‐dentate calcifications with late onset extrapyramidal syndrome
Authors: Martinelli P. , Giuliani S. , Ippoliti M. , Martinelli A. , Sforza A. , Ferrari St. 
Published: December 2004

AbstractA family with autosomal dominant inheritance of idiopathic strio‐pallidodentate calcifications and late onset of extrapyramidal symptoms is reported. Clinical features consisted of parkinsonism in one member and postural tremor in two. Depression and dysarthria were present in all cases. All symptomatic members showed a peculiar biochemical abnormality consisting of reduced 25‐OH vitamin D3 with normal levels of 1,25(OH)2 vitamin D3, suggesting an inborn error of Vitamin D metabolism. The biochemical, clinical, and genetic pattern of this family distinguishes this syndrome from the larger group of secondary familial basal ganglia calcifications.
Ataxia without telangiectasia
Authors: Friedman Joseph H. , Weitberg Alan 
Published: December 2004

AbstractAtaxia telangiectasia (AT) is an autosomal recessive hereditary disorder characterized by onset in infancy or childhood of a cerebellar and later extrapyramidal disorder associated with telangiectasias and an immune deficit. Only a handful of cases have been described in which the features were not stereotypic. This report describes a case that is classic except for the absence of telangiectasias through age 17. This and other cases suggest that a new, more inclusive term be used to describe the syndrome of ataxia with immune deficit until the genetic abnormalities in these disorders become identifiable.
Dopamine receptors in the basal ganglia: Relevance to parkinson's disease
Authors: Strange Philip G. 
Published: December 2004

AbstractAt least five receptors for dopamine (D1–D5) have been recognised from molecular biological studies, and their pharmacological properties and brain localisations have been determined. The D1 and D2 subtypes are the principal subtypes in brain, and their cellular localisations in the caudate nucleus and putamen have been determined. With recent advances in the understanding of basal ganglia neuronal function, these localisation data enable insights into the mode of action of drugs used at present and in the futue to treat Parkinson's disease.
The detection of preclinical Parkinson's disease: What is the role of positron emission tomography?
Authors: Sawle Guy V. 
Published: December 2004

AbstractOn clinical criteria alone, the diagnosis of early Parkinson's disease can be difficult and, by definition, the prospective recognition of preclinical Parkinson's disease is impossible. Positron emission tomography (PET) using [18F]dopa as tracer has been proposed as a means of identifying patients with preclinical disease. The number of subjects detected to date has been few; most have been identified by serendipity or during the course of family studies. This review examines the significance of a single abnormal scan in an apparently healthty subject in terms of the relationship between normal and abnormal values and the time course of the disease.
Ascorbic acid protects against levodopa‐induced neurotoxicity on a catecholamine‐rich human neuroblastoma cell line
Authors: Pardo Beatriz , Mena María Angeles , Fahn Stanley , de Yébenes Justo García 
Published: December 2004

AbstractLevodopa, at concentrations of 0.25 × 10−4 M or larger, is toxic for the human neuroblastoma cell NB69. Toxicity is associated with high levels of quinones, increased activity of complex II‐III, and lack of changes of complex I of the mitochondrial respiratory chain. Deprenyl, which does not alter the production of quinones, has a partial protective effect. Tocopherol, 23 or 115 × 10−6 M, lacks significant preventive effect on levodopa toxicity, but ascorbic acid, 10−3 M, prevents levodopa toxicity and quinone formation. Deprenyl, 10−4 M, provides additional protection in cultures treated with levodopa and ascorbic acid. Our results indicate that ascorbic acid and deprenyl prevent levodopa neurotoxicity by unrelated mechanisms. Both compounds should be considered as complementary drugs to test for slowing the progression of Parkinson's disease.