bluuebunny/crossref_metadata_2025_split huggingface dataset:
Limb dystonia following electrical injury
Authors: Tarsy Daniel , Sudarsky Lewis , Charness Michael E. 
Published: December 2004

AbstractElectrical injuries of the extremities may cause paralysis, muscle atrophy, sensory deficit, causalgia, and reflex sympathetic dystrophy (RSD). Limb dystonia has rarely been reported following electrical injury to an extremity, although it may result from cerebral hemisphere electrical trauma. Following electrical injury to the upper extremity, three patients developed limb dystonia accompanied by severe pain and sensory symptoms in two patients and features of RSD in one patient. Two patients received botulinum toxin injections without functional benefit. The mechanism of dystonia following peripheral trauma is unknown but may relate to reorganization of central synaptic connections, possibly in the spinal cord.
Autosomal‐dominant dentatorubropallidoluysian atrophy in the United Kingdom
Authors: Warner T. T. , Lennox G. G. , Janota I. , Harding A. E. 
Published: December 2004

AbstractDentatorubropallidoluysian atrophy (DRPLA) has been described chiefly in Japan and appears to be rare in Europe. It is of autosomal dominant inheritance. We report the first British family with DRPLA, which contains four affected individuals in two generations. The diagnosis was made at autopsy in one case. The age of onset of symptoms ranged from 15 to 38 years, and clinical features included ataxia, dementia, chorea, and dystonia; three patients had generalized seizures. The three living patients resemble those with early Huntington's disease clinically. Three main phenotypes of DRPLA have been proposed: an ataxo‐choreoathetoid type, a pseudo‐Huntington type, and a myoclonic epilepsy type. The variation in clinical presentation in our family demonstrates the difficulty in applying such classifications to this and other dominantly inherited disorders with phenotypic variation. DRPLA is likely to be confused with Huntington's disease in European families.
Adductor laryngeal breathing dystonia in a patient with lubag (X‐linked dystonia‐parkinsonism syndrome)
Authors: Lew M. F. , Shindo M. , Moskowitz C. B. , Wilhelmsen K. C. , Fahn S. , Waters C. H. 
Published: December 2004

AbstractWe report a patient with Lubag (X‐linked dystonia‐parkinsonism) who presented with severe respiratory stridor form adductor laryngeal breathing dystonia. Emergency tracheostomy was necessary, and subsequent laryngeal injection with botulinum toxin led to worsening aspiration. Botulinum toxin injection for severe lingual dystonia was successful.
Is cranial computerized tomography useful in the diagnosis of multiple system atrophy?
Authors: Wenning G. K. , Jäger R. , Kendall B. , Kingsley D. , Daniel S. E. , Quinn N. P. 
Published: December 2004

AbstractCranial computer tomographic (CT) images of 33 patients with multiple system atrophy (MSA) and of 40 age‐matched controls were blindly analyzed by two neuroradiologists. All patients had autonomic dysfunction, all but one had parkinsonism, and 13 had cerebellar signs. The scans were judged entirely normal in 21%. Moderate or severe infratentorial atrophy was found in 42%. Cerebellar atrophy was present in 39%, and pontine atrophy was present in 18%. Of the 13 patients with cerebellar signs, only eight had cerebellar atrophy. Of the 20 patients without cerebellar signs, five had cerebellar atrophy. Supratentorial involvement was much less common and less severe. Thus, CT demonstrated system involvement that was not evident clinically in five of 33 cases (15%). However, in all five the clinical diagnosis was already evident from the presence of both autonomic and pyramidal signs in addition to parkinsonism. We conclude that CT imaging is of limited diagnostic use in individual patients with MSA.
Reliability of symptom onset assessment in Parkinson's disease
Authors: Richards M. , Marder K. , Cote L. , Mayeux R. 
Published: December 2004

AbstractTest‐retest reliability of onset date and type of initial symptoms of Parkinson's disease (PD) was assessed in 45 patients with PD who were drawn from a community‐dwelling cohort in New York City. Patients were the sole information providers on both rating occasions. Reliability of symptom‐onset data was found to be high, even when the interval between test and retest was up to 3 years and when the information was elicited by different neurologists. However, these patients were less consistent about the type of early symptoms. When interpreting their own disease history, patients with PD may attach more importance to when their disease began than to how it began.
Reversible intermittent rhythmic myoclonus with fluoxetine in presumed pick's disease
Authors: Lauterbach Edward C. 
Published: December 2004

AbstractA 61‐year‐old man with presumed Pick's disease was successfully treated with fluoxetine for pathological affect. Severe intermittent, rhythmically repetitive trains of myoclonus developed suddenly the following year. A dystonic‐like component involving the shoulder region and a decrescendo frequency pattern were observed. Myoclonus involved the face, palate, shoulder, neck, upper chest and back, diaphragm, hips and upper extremities, especially on the right side. Movements were not influenced by postural adjustments, startle, or other stimuli. Movements proved insensitive to benztropine but abated with discontinuation of fluoxetine. Rechallenge with fluoxetine or trazodone evoked the movements, whereas clonazepam and chloral hydrate abolished the movements. The pattern of myoclonus is unlike previous reported cases and may relate to activation of serotonin 5HT1A receptors (possibly supersensitive in Pick's disease) or to hypodopaminergia. Although fluoxetine may be useful as adjunct therapy in dementing disorders, caution may be warranted in its use in Pick's disease.
The prevalence of parkinsonism in Italy: An epidemiological survey of the disease in general practice
Authors: Beghi E. , Monticelli M. L. , Sessa A. , Simone P. ,   The Italian General Practitioner Study Group (IGPSG)
Published: December 2004

AbstractThe prevalence and characteristics of parkinsonism have been assessed in two separate geographic areas in Italy (Arcisate and San Giovanni Rotondo). A total of 28, 377 patients of 23 general practitioners (GPs) were the reference population. Fifty‐eight patients were traced by the GP as having typical parkinsonian features or being treated with antiparkinson drugs. Among 53 subjects, 21 of them untreated, parkinsonism was subsequently confirmed neurologically. The overall crude prevalence rate was 1.87 cases per 1,000 (Arcisate 1.81; San Giovanni Rotondo 2.01). The age‐ and sex‐adjusted prevalence rates were 1.84 in Arcisate and 2.04 in San Giovanni Rotondo. After exclusion of drug‐related parkinsonism, the rates were 1.48 and 1.90, respectively. Even with some inconsistencies within the two study areas, the prevalence tended to be higher in San Giovanni Rotondo, to prevail in women, and to increase significantly with age. Presumed etiological factors for parkinsonism were recorded in 34% of the cases (mostly drugs). In two thirds of the cases the disease ran a mild course. The present study shows that the GP proves a valuable reference for surveys of parkinsonism in Italy.
Perturbation of a prehension movement in Parkinson's disease
Authors: Scarpa Marina , Castiello Umberto 
Published: December 2004

AbstractMovement kinematics of the transport and manipulation components of a double‐step prehension task were studied in eight Parkinson patients and eight control subjects. The aims were to (a) assess the effects of a spatial perturbation upon the movement for the two groups and (b) add data to the controversy about the damage/preservation of predictive behaviour in Parkinson patients. The results showed: (a) Both groups are able to preprogram a movement. (b) In both groups, the perturbation results in an anticipation of all kinematic parameters, both of the transport and manipulation components. (c) Parkinson patients, when adopting a predictive behavior, show a delay between the beginning of the two components, and thus activate them in sequence rather than simultaneously. This delay is significantly reduced by the perturbation, indicating that Parkinson patients, when using a responsive behavior, can recouple the two motor components.
Abnormalities of somatosensory evoked potentials in spasmodic torticollis
Authors: Mazzini L. , Zaccala M. , Balzarini C. 
Published: December 2004

AbstractThis study examined the N20 and N30 waves of somatosensory evoked potentials (SEPs) from median nerve stimulation at three different stimulation rates (1, 3, and 6 Hz) in patients with idiopathic spasmodic torticollis (ST). The data were compared with those collected from a group of patients affected by Parkinson's disease (PD) and normal age‐matched subjects. N30 amplitude was significantly decreased in both groups of patients with respect to the controls. The decrease was larger in patients with ST. The N20 wave remained stable. The latencies of both waves were unchanged. When the stimulus rate was increased, the N30 amplitude decreased significantly, with a similar trend observed in both patients and controls. The isolated abnormalities of the N30 wave in both ST and PD support the hypothesis of a common physiopathogenetic mechanism that involves the basal ganglia or their connections with the supplementary motor area.
Vascular chorea: Case report with pathology
Authors: Bhatia Kailash P. , Lera Gabriel , Luthert Philip J. , Marsden C. David 
Published: December 2004

AbstractWe report a patient with long‐standing systemic hypertension who developed progressive generalized chorea and dementia beginning at 70 years of age with no family history or other features to suggest Huntington's disease. At postmortem examination, congophilic angiopathy and atherosclerosis causing neostriatal neuronal loss and gliosis were found, in addition to plaques and neurofibrillary tangles in the cortex. This case is a rare demonstration of a vascular pathology causing late onset generalized chorea in association with dementia due to Alzheimer's‐type cortical changes.
Parkinsonian syndromes associated with hydrocephalus: Case reports, a review of the literature, and pathophysiological hypotheses
Authors: Curran Terry , Lang Anthony E. 
Published: December 2004

AbstractWe present nine cases of obstructive hydrocephalus (OH) associated with marked parkinsonism. Four patients had noncommunicating OH (NCOH) [three nontumoral aqueductal stenosis (AS), one tumoral AS]. The presentation was that of acute or subacute parkinsonism, usually at the time of acute recurrent ventricular obstruction. Three had a marked response to levodopa and required short‐term treatment after shunting. However, one has remained levodopa dependent after 21/2 years. Three of the five patients with communicating OH (COH) presented with shunt‐responsive normal pressure hydrocephalus (NPH), only later to develop progressive parkinsonism. One of these was found to have progressive supranuclear palsy (PSP) at autopsy and PSP was clinically susepected in one other patient. A third had an atypical course suggestive of PSP; however, autopsy demonstrated the combination of Lewy body parkinsonism and the sequelae of hydrocephalus. The remaining two COH patients presented with levodopa‐responsive parkinsonism. Subsequent clinical features and imaging studies suggested the presence of NPH. The pathophysiology of hydrocephalic parkinsonism probably involves variable sites of dysfunction in the nigrostriatal pathway and/or the cortico‐striato‐pallido‐thalamo‐cortical circuit. At certain locations these pathways lie in close proximity to the ventricular system and may be subjected to mass effects and ischemic changes secondary to ventriculomegaly. The additional importance of possible associations between subcortical cerebral ischemia, NPH, and “degenerative” disorders such as PSP and Parkinson's disease is discussed.
Characteristics of handwriting of patients with huntington's disease
Authors: Phillips J. G. , Bradshaw J. L. , Chiu E. , Bradshaw J. A. 
Published: December 2004

AbstractPatients with Huntington's disease exhibit poorer‐quality handwriting, sometimes clinically exhibiting macrographia, an increase in the size of handwriting. To characterize deficits in handwriting of patients with Huntington's disease, we compared the writing of 12 young, 12 age‐matched controls, and 12 patients with Huntington's disease. Subjects were asked to write the letter “I” four times, at a constant length, on a graphics table that sampled pen position at 200 Hz. Huntington's disease causes chorea (involuntary movement), akinesia (difficulty in maintatining voluntary movement). To distinguish changes in handwriting quality due to involuntary movement from impairments of voluntary movement, handwriting samples with obvious choreic movements were analyzed separately from other handwriting samples. Several measures of quality of handwriting were considered, based on: the regularity and consistency of handwriting, the efficiency of movement trajectories, and the proportions of movement occurring at specific frequencies. Results suggested that Huntington's disease increases variability of movement parameters, and causes problems in producing smooth movements. Choreic movement was best characterized by the number of zero crossings in the velocity function relative to the prescribed number of writing strokes. We hypothesize that macrographia in Huntington's disease occurs when chorea predominates over bradykinesia. Comparisons were made between the handwriting of patients with Huntington's and Parkinson's diseases.
Visual thresholds to low‐contrast pattern displacement, color contrast, and luminance contrast stimuli in Parkinson's disease
Authors: Haug Bernhard A. , Trenkwalder Claudia , Arden Geoffrey B. , Oertel Wolfgang H. , Paulus Walter 
Published: December 2004

AbstractWe used the computerized Moorfield Vision System to demonstrate specific increases in various perceptual visual thresholds in idiopathic Parkinson's syndrome. Fifteen patients were compared to 13 age‐matched normals. Motion detection was impaired maximally (2p < 0.01 and better in two‐tailed t test) at luminance contrasts of 3–7%. Stimulus was an achromatic vertical 4 cycles/°sine wave grating subtending 3° × 2°, centered 5° in the nasal field and oscillating at 5 Hz. In addition, stationary color and luminance contrast thresholds were tested with flashed display of 5° × 6° random letters, which were presented for 200 ms (color) and 50 ms (achromatic). Color discrimination was impaired in the tritan axis only (2p < 0.05 in two‐tailed t test). All achromatic stimuli–luminance increments, decrements, and phase reversing stimuli–were equally well seen by patients and controls. We conclude that the dopaminergic deficit of retinal amacrine cells in Parkinson patients can be monitored by combined low‐contrast and motion (displacement) stimuli. Future studies will determine if moving colored targets are more effective in discriminating patients from controls than are the achromatic gratings used in this work.
Motor effects of the partial dopamine agonist (−)‐3‐(3‐hydroxyphenyl)‐N‐n‐propylpiperidine (preclamol) in Parkinson's disease
Authors: Metman L. Verhagen , Sethy V. H. , Roberts J. R. , Bravi D. , Hoff J. I. , Mouradian M. M. , Chase T. N. 
Published: December 2004

AbstractThe motor effects of the partial dopamine agonist (–)‐3‐(3‐hydroxyphenyl)‐N‐n‐propylpiperidine [(–)‐3‐PPP, preclamol] were evaluated in nine patients with Parkinson's disease using a double‐blind, placebocontrolled design. (–)‐3‐PPP monotherapy had an antiparkinsonian effect in five of nine patients at a mean dose of 37 ± 10 mg intramuscularly. The co‐administration of (–)‐3‐PPP and a mildly dyskinetic dose of levodopa, infused intravenously at steady‐state, resulted in complete suppression of dyskinesias and reemergence of parkinsonian signs in two of seven patients. These dopamine antagonist effects of (–)‐3‐PPP occurred at relatively low (2.5 and 5 mg) doses. Our results suggest that partial dopamine agonists can exert agonist or antagonist activity in parkinsonian patients depending on concurrent dopaminergic tone. Although this dual action of (–)‐3‐PPP and other partial agonists could be therapeutically important on theoretical grounds, the small number of patients manifesting a clinically significant response and the frequently inconsistent effects could indicate that this class of agents may have relatively limited clinical utility.
“Apraxia of lid opening,” a focal eyelid dystonia: Clinical study of 32 patients
Authors: Krack P. , Marion M. H. 
Published: December 2004

AbstractWe have seen 32 patients with “apraxia of lid opening” (ALO) in the following clinical settings: as an isolated condition (3 patients), idiopathic blepharospasm (BSP, 20 patients, including 4 familial cases), progressive supranuclear palsy (PSP, 7 patients), and dystonic parkinsonian syndrome (2 patients). Twenty‐nine patients treated with botulinum toxin into the orbicularis oculi muscle were rated before and after treatment and 83% of the patients improved on a clinical scale. Best results were obtained with injections directed toward the junction of the preseptal and pretarsal parts of the palpebral orbicularis oculi. Several patients also improved on anticholinergic drugs. Besides medical treatment, lid crutches, in conjunction with botulinum toxin injections, were useful in some patients. ALO is not a true apraxia; it constitutes an eyelid dystonia as shown by its clincal and electrophysiological features as well as pharmacological reactions and is encountered in a clinical spectrum ranging from an isolated from to predominant BSP. It was an important cause of treatment failures in botulinum toxin‐treated BSP but by modifying our injection strategy and by adding anticholinergic drugs and also lid crutches, we obtained a good functional benefit.
Reliability among neurologists in the severity assessment of blepharospasm and oromandibular dystonia: A multicenter study
Authors: Defazio G. , Lepore V. , Abbruzzese G. , Barontini E. , Berardelli A. , Caraceni T. , Carella F. , Girlanda P. , Manfredi M. , Messina C. , Maurri S. , Ferrari E. , Livrea P. 
Published: December 2004

AbstractThe reliability of a scale of 0 to 4 (where 0 is normal) in rating the severity of blepharospasm (BS) and oromandibular dystonia (OMD) was evaluated by the concordance among six neurologists from different neurological institutions. As expressed by k index, interobserver agreement was moderate either for BS or for OMD according to the Landis classification. Neurologists showed different rating attitude towards BS and OMD. In fact, the category analysis showed that raters were inclined to overestimate BS and to underestimate OMD. The familiarity with dystonia influenced reliability more than the length of professional experience in neurology. In fact, when examiners were subdivided into subgroups (each of three raters) according to the former criteria, the level of interobserver agreement increased significantly. Almost perfect agreement was obtained in intrarater comparisons. These results may be of value with regard to the conduct of multicenter epidemiologic and clinical studies on focal dystonias.
Coprolalia in younger patients with Gilles de la tourette syndrome
Authors: Goldenberg James N. , Brown Stuart B. , Weiner William J. 
Published: December 2004

AbstractThe objective of this study was to determine the frequency of coprolalia in younger patients with Gilles de la Tourette syndrome (GTS). Coprolalia, which is the least understood and perhaps most unusual symptom of GTS, is reported to occur in 4–60% of all patients with GTS. Most reports indicate a prevalence >30%. The widely varying prevalence figures for coprolalia may reflect cultural differences, severity of disease, and the age of the population surveyed. This study is a chart review of 112 patients seen in a pediatric neurology clinic in South Florida who met the Diagnostic and Statistical Manual of Mental Disorder (3rd ed., revised) criteria for GTS. Only 8% of the patients in the study exhibited coprolalia. This unusually low frequency of coprolalia suggests that coprolalia may be rarer than previously believed in younger patients. It may also better reflect the frequency of coprolalia in a community‐based pediatric neurology practice.
Cortical myoclonus in huntington's disease
Authors: Thompson P. D. , Bhatia K. P. , Brown P. , Davis M. B. , Pires M. , Quinn N. P. , Luthert P. , Honovar M. , O'Brien M. D. , Marsden C. D. , Harding A. E. 
Published: December 2004

AbstractWe describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.
Symptomatic and essential palatal tremor. 2. Differences of palatal movements
Authors: Deuschl G. , Toro C. , Hallett M. 
Published: December 2004

AbstractPalatal tremor, a rhythmic movement disorder of the soft palate, may be described as two separate entities: symptomatic palatal tremor (SPT) and essential palatal tremor (EPT). The symptomatic form is associated with brain stem or cerebellar disease, whereas the essential form has no known etiology. A cardinal symptom of EPT is the presence of ear clicks, which do not occur in SPT. Visual observation of the movements in the two disorders suggests that the difference in symptoms is due to the activation of different palatal muscles, the levator veli palatini in SPT and the tensor veli palatini in EPT. Electromyographic recording from the levator veli palatini muscle showed abnormal bursting activity time locked to the palatal movements in patients with SPT, but not in those with EPT. Because the two palatal muscles are innervated by different cranial nerves, SPT and EPT are likely to have separate origins.
Does levodopa therapy delay death in Parkinson's disease? A review of the evidence
Authors: Clarke C. E. 
Published: December 2004

AbstractStudies examining the mortality from Parkinson's disease have been reviewed to assess the impact of levodopa therapy. These include national mortality data taken from death certificate returns, cohort studies compring the deaths observed in a group of patients with those expected from population statistics, and case‐control studies comparing deaths in patients with those in a matched control group. It is concluded that the decrease in crude mortality rate in the early 1970s in several western countries was mirrored by a decrease in observed to expected mortality rates in cohort studies over the same period. The rise in crude mortality in the late 1970s and early 1980s paralleled an increase in observed to expected mortality in cohort studies. These trends are compatible with the hypothesis that levodopa delayed the death of a cohort of frail elderly parkinsonian patients who succumbed ∼5 years later, leading to an apparent “catch‐up” increment in national mortality data. Whilst crude mortality in England and Wales does not appear to be increasing as fast as before the introduction of levodopa, recent cohort and case‐control studies report mortality ratios comparable to those seen in the prelevodopa era. Further case‐control studies are required to determine the position with current treatment regimens.