bluuebunny/crossref_metadata_2025_split huggingface dataset:
Iron in parkinsonian and control substantia nigra—A mössbauer spectroscopy study
Authors: Gała̧zka‐Friedman J. , Bauminger E. R. , Friedman A. , Barcikowska M. , Hechel D. , Nowik I. 
Published: December 2004

AbstractWe used Mössbauer spectroscopy to study the iron content, the redox state, and the binding site of iron in substantia nigra (SN) from parkinsonian (PD) and control brains. Measurements performed on fresh‐frozen, formalin‐fixed, and lyophilized samples demonstrated the presence of ferric (Fe3+) iron only, both in PD and control SN. Ferrous iron, if present at all, may represent at most 5% of the total iron. We found no difference in the total amount of iron in SN between PD and control brains. The Mössbauer spectra observed at 4.1 K in fresh (frozen or lyophilized) samples were different from those obtained in formalin‐fixed (frozen or lyophilized) samples. In the fresh samples, only ferritin‐like iron was observed, whereas in the samples frozen or lyophilized from formalin, non‐ferritin iron was detected.
Quantification of essential tremor in writing and drawing
Authors: Elble Rodger J. , Brilliant Mikhail , Leffler Keith , Higgins Constance 
Published: December 2004

AbstractEighty‐seven patients, aged 15–84 years (mean, 61.8 ± 16.2 SD), with essential tremor wrote two series of cursive e's and cursive l's on a standard sheet of ruled paper that was mounted on a commercially available digitizing tablet. Forty patients also drew an Archimedes spiral. Postural wrist tremor was measured with a triaxial accelerometer on the dorsum of the horizontally extended hand. The digitizing tablet was sufficiently sensitive to measure sustained visible tremor. Very severe tremor could not be recorded when it prevented a patient from keeping the ballpoint pen on the tablet. The intertrial variability of the handwriting data was such that a 36.0% change in mean acceleration amplitude (cm/s2) and an 8.3% change in mean tremor frequency (Hz) could be detected in a hypothetical population of 30 patients (paired‐sample t test, p = 0.01, power = 90%). The intertrial changes detectable with accelerometry were 35.9% (amplitude) and 7.8% (frequency). The correlations between wrist tremor and writing tremor were <0.60 for amplitude and <0.25 for frequency. Significant correlations between patient age and tremor frequency and between tremor amplitude and frequency existed for postural tremor but not for writing or drawing tremor. Standard digitizing tablets for personal computers are useful in the quantitative assessment of writing tremor. The amplitude and frequency characteristics of tremor in posture, writing, and drawing may differ significantly.
Perioperative use of botulinum toxin for movement disorder–induced cervical spine disease
Authors: Adler Charles H. , Zimmerman Richard S. , Lyons Mark K. , Simeone Fred , Brin Mitchell F. 
Published: December 2004

AbstractPatients with cervical dystonia or tics of the nuchal muscles can develop serious cervical spine disease. We report a series of four patients who received botulinum toxin injections to control their movement disorders prior to their required surgery. One patient with cervical tic–induced radiculomyelopathy required botulinum toxin injection postoperatively to facilitate stabilization of the cervical fusion. Two patients with torticollis‐induced cervical radiculomyelopathy, and one patient with dystonia‐induced C5 fracture, had botulinum toxin injected pre‐operatively to facilitate postoperative recovery. Botulinum toxin appears to be a useful adjunct in the treatment of cervical movement disorders prior to or following surgery for associated cervical spine disease.
Essential myoclonus and myoclonus dystonia
Authors: Quinn Niall P. 
Published: December 2004

AbstractThis review explores the history and use of the terms essential myoclonus and myoclonie dystonia. In addition, the review proposes that herediatry essential myoclonus and dominantly inherited myoclonic dystonia, with lightning jerks and dramatic response to alcohol, are the same disease, although proof of this hypothesis must come from ongoing genetic studies.
Core assessment program for intracerebral transplantation in Huntington's disease (CAPIT‐HD)
Authors: Quinn Niall , Brown Richard , Craufurd David , Goldman Serge , Hodges John , Kieburtz Karl , Lindvall Olle , MacMillan John , Roos Raymund 
Published: December 2004

AbstractStudies in Parkinson's disease using the Core Assessment Program for Intracerebral Transplantation (CAPIT) protocol have demonstrated that grafts of embryonic mesencephalic cells into striatum can survive, grow, and exert useful clinical effects. Attention is now being directed toward neural grafting in other conditions, such as Huntington's disease. As a precondition for grafting of embryonic striatal cells into diseased striatum in this complex motor and psychiatric disorder, not only is further basic research needed, but also a thorough and wide‐ranging assessment protocol is essential. This article presents such a CAPIT‐HD assessment protocol.
Proton magnetic resonance spectroscopy in Huntington's disease: Evidence in favour of the glutamate excitotoxic theory?
Authors: Taylor‐Robinson S. D. , Weeks R. A. , Bryant D. J. , Sargentoni J. , Marcus C. D. , Harding A. E. , Brooks D. J. 
Published: December 2004

AbstractThe gene responsible for Huntington's disease (HD) has been located, but its action and the pathophysiology of HD remain unclear. Glutamate excitotoxicity may contribute to the striatal neurodegeneration seen in HD. We used localised proton magnetic resonance spectroscopy (MRS) of the brain to investigate five patients with early HD, one symptom‐free gene carrier, and 14 healthy volunteers. Peak area ratios of choline‐containing compounds (Cho), glutamine and glutamate (Glx), and N‐acetyl moieties including N‐acetylaspartate (NAx), relative to creatine (Cr), were calculated. Spectra were analysed from the striatum and the occipital and the temporal cortex. The HD patients all had an elevated Glx/Cr in spectra localised to the striatum, compared with healthy controls, and one patient also had an elevated thalamic Glx/Cr. The mean Glx/Cr was unaltered in the cortical spectra of HD patients. The asymptomatic gene carrier displayed no spectral abnormalities. Our findings suggest disordered striatal glutamate metabolism and may support the theory of glutamate excitotoxicity in HD.
Basal ganglia iron content in Parkinson's disease measured with magnetic resonance
Authors: Ye Frank Q. , Allen Peter S. , Martin W. R. Wayne 
Published: December 2004

AbstractThe possibility of using magnetic resonance (MR) to evaluate the severity of the pathological changes of Parkinson's disease (PD) is suggested by the known accumulation of iron in the basal ganglia in PD and the reduced signal evident from this area with conventional reduced signal evident from this area with conventional T2‐weighted MR imaging. To improve the specificity of MR for the measurement of tissue iron content, we have developed a method that quantifies the effects of paramagnetic centers sequestered inside cellular membranes, based on the echo time dependence of the decay of transverse magnetization caused by the local field inhomogeneities which are due to intracellular iron. This method enables an index of local tissue iron content to be calculated for structures of the basal ganglia. We report here the application of this method to a series of patients with PD (n = 12) and of normal, age‐matched controls (n = 13). Our objective was to determine whether this measurement of basal ganglia iron concentration correlates with the presence and severity of PD. We observed a significant increase in iron content in both the putamen and pallidum in PD as well as a correlation with the severity of clinical symptomatology. More severely affected patients had a higher iron content in both of these structures. Our results suggest that this MR measurement may provide a noninvasive method of measuring the severity of the pathological changes underlying PD.
Influence of dopaminergic medication on automatic postural responses and balance impairment in Parkinson's disease
Authors: Bloem Bastiaan R. , Beckley Dennis J. , van Dijk J. Gert , Zwinderman Aeilko H. , Remler Michael P. , Roos Raymund A. C. 
Published: December 2004

AbstractIt is still unclear why balance impairment in Parkinson's disease (PD) often responds insufficiently to dopaminergic medication. We have studied this issue in 23 patients with idiopathic PD and 24 healthy controls. Our specific purposes were (a) to investigate the contribution of abnormal automatic postural responses to balance impairment in PD and (b) to assess the influence of dopaminergic medication on abnormal automatic postural responses and balance impairment. Standing subjects received 4° “toe‐up” rotational perturbations of a supporting forceplate. We bilaterally recorded posturally destabilizing medium latency (ML) responses from the stretched gastrocnemius muscles and functionally corrective long latency (LL) responses from the shortened tibialis anterior (TA) muscles. We also assessed changes in the center of foot pressure (CFP) and the center of gravity (COG). All patients were tested “off” and “on” phase. All controls were tested and retested after 1 h. During the off phase, we found enlarged ML amplitudes and diminished LL amplitudes in patients, together with a markedly increased posterior displacement of the COG. The abnormal ML and LL responses were partially responsible for the increased body sway in patients because the initial forward (destabilizing) displacement of the CFP was increased, while the subsequent backward displacement of the CFP (a measure of the corrective braking action of LL responses) was delayed. Abnormal late automatic or possibly more voluntary postural corrections also contributed substantially to the increased body sway. During the on phase, ML amplitudes were reduced in patients but remained increased compared with controls. LL amplitudes no longer differed between both groups due to a modest, possibly dopamine‐related in crease in patients and a simultaneous decrease in controls. The abnormal CFP displacement was only partially improved by dopaminergic medication. The later postural corrections were not improved at all. Consequently, the increased posterior COG displacement was not ameliorated during the on phase. We conclude that (a) a combination of abnormal automatic and perhaps more voluntary postural corrections contributes to increased body sway in PD and (b) dopaminergic medication fails to improve balance impairment in PD because early automatic postural responses are only partially corrected, while later occurring postural corrections are not improved at all. These electrophysiological results support clinical observations and suggest that nondopaminergic lesions play a significant role in the pathophysiology of postural abnormalities in PD.
Hashimoto's myoclonic encephalopathy: An underdiagnosed treatable condition?
Authors: Ghika‐Schmid F. , Ghika J. , Regli F. , Dworak N. , Bogousslavsky J. , Städler C. , Portmann L. , Despland P. A. 
Published: December 2004

AbstractWe report two patients with subacute diffuse encephalopathy characterized by confusion, myoclonic encephalopathy, and mild akineto‐rigid extrapyramidal signs in one case and by apathy, memory deficit, and partial complex seizures in the other. Hashimoto's thyroiditis with high titers of antithyroglobulin antibodies was diagnosed in both patients, who were unresponsive to anticonvulsant medication, but showed rapid neurological improvement following steroid treatment. On neuropsychological examination, predominant frontotemporal dysfunction was noted. Electroencephalographic activity was remarkable for its rhythmical delta activity, unresponsive to, or even paradoxically increased by, anticonvulsant treatment. On magnetic resonance imaging, atrophy with temporal predominance was found. These observations support the idea that this potentially treatable dementia and movement disorder should be classified as a separate clinical entity.
Postencephalitic stereotyped involuntary movements responsive to L‐Dopa
Authors: Picard F. , de Saint‐Martin A. , Salmon E. , Hirsch E. , Marescaux C. 
Published: December 2004

AbstractIn 1954, at the age of 5 years, our patient had an encephalitic syndrome associated with a prolonged lethargic state. After this episode, he developed a severe parkinsonian syndrome that, after a few years, was associated with axial dystonia and stereotyped abnormal movements of the upper limbs. This complex and progressive extrapyramidal syndrome had many similarities to the encephalitis lethargica as described by von Economo. Results of cerebral computed tomography and magnetic resonance imaging were normal. Fluorodopa positron emission tomography showed a significant bilateral reduction of tracer accumulation in both putamen, similar to that observed in patients with idiopathic Parkinson's disease. However, in this patient, treatment with L‐Dopa suppressed all akinetic, dystonic and dyskinetic symptoms. The effectiveness of L‐Dopa was abolished by administration of a D2 antagonist and was fully reproduced by a D2 agonist. In conclusion, this patient presented a complex postencephalitic, extrapyramidal syndrome, with akinetic symptoms and involuntary movements. These symptoms appeared to be related to a limited lesion of the dopaminergic neurons of the zona compacta of the substantia nigra.
Striatal interneurons in Huntington's disease: Selective increase in the density of calretinin‐immunoreactive medium‐sized neurons
Authors: Cicchetti Francesca , Parent André 
Published: December 2004

AbstractThe marked atrophy of the striatum seen in Huntington's disease (HD) is largely due to a massive neuronal loss that affects the striatal projection neurons more severely than the local circuit neurons. We recently reported the existence of a new class of interneurons characterized by their immunoreactivity for the calciumbinding protein calretinin in the human striatum. In the present immunohistochemical study, we compared the distribution and relative density of the calretininexpressing interneurons in the striata of four normal individuals and four patients with HD (grade 1 to 3). The population of calretinin‐containing interneurons comprised (a) a small subset of large (17‐to 44‐μm), multipo‐ lar neurons with five to seven long, aspiny, and highly branched dendrites and (b) a large number of medium‐ sized (8‐to 18‐μm), round‐to‐oval neurons with two to three long, varicose, and poorly branched dendrites. Both types of chemospecific neurons occurred throughout the striatum in all specimens examined, but the density of the medium‐sized neurons was much higher in patients with HD than in controls. A quantitative analysis showed a significant (p < 0.01) twofold increase in the density of the striatal medium‐sized neurons and a similar decrease in the density of the large neurons in patients with HD compared with controls. This differential effect on the densities of the two types of interneurons suggests that calretinin may protect the medium‐sized but not the large neurons against neurodegeneration in HD.
Effects of diminished and conflicting sensory information on balance in patients with cerebellar deficits
Authors: Gatev Plamen , Thomas Sherry , Lou Jau‐Shin , Lim Meerana , Hallett Mark 
Published: December 2004

AbstractWe studied the effects of altered sensory information on standing balance in 25 patients with cortical cerebellar atrophy (CCA), nine patients with olivopontocerebellar atrophy (OPCA), and 10 normal subjects. The total sway path and its components, the antroposterior (AP) sway path and the lateral sway path, were measured under six conditions: (1) standing on a fixed platform with the eyes open and visual surroundings fixed, (2) standing on a fixed platform with the eyes closed, (3) standing on a fixed platform with the eyes open and visual surroundings AP sway referenced, (4) standing on an AP sway‐referenced platform with the eyes open and visual surroundings fixed, (5) standing on an AP sway‐referenced platform with the eyes closed, and (6) standing on an AP sway‐referenced platform with the eyes open and visual surroundings AP sway referenced. Patients swayed more than normal subjects during normal stance (condition 1), when the visual information was absent (condition 2) or distorted (condition 3), and when the proprioceptive information from the ankles was distorted (condition 4). Patients swayed much more than normal, and most fell, when two sensory modalities were affected under condition 5 (proprioceptive information distorted and visual information absent) and condition 6 (both proprioceptive information and visual information distorted). When the patients' sway was normalized to that of the first condition, however, only their lateral sway was greater than the sway in normal subjects. Unlike in normal subjects, the patients' lateral sway varied with the AP sway to approximately the same degree in each condition for conditions 1–5. Clinical ratings of gait and balance were highly correlated with the sway measures. Quantitative testing of standing balance with altered sensory information has better sensitivity than normal stance testing.
Sleep‐related periodic leg movements associated with spinal cord lesions
Authors: Lee M. S. , Choi Y. C. , Lee S. H. , Lee S. B. 
Published: December 2004

AbstractWe describe three patients who developed progressive paraparesis and sleep‐related periodic leg movements (SRPLM) associated with thoracic spinal cord lesions; one patient had a schwannoma and two had intramedullary lesions. The patients showed periodic repetitive involuntary movements involving one or both lower limbs. The involuntary movements consisted of a single rapid dorsiflexion of the great toe or ankle, two to four repetitive dorsiflexions of the toes and ankle, and a mixture of repetitive jerks and prolonged spasms causing flexion of the hip and knee and dorsiflexion of the ankle and toes. In the patient with a schwannoma, paraparesis and SRPLM improved completely after surgical removal of the mass lesion. In one patient the SRPLM associated with an intramedullary lesion improved markedly after levodopa treatment. We suspect that thoracic spinal lesions partially disinhibit the lumbosacral generator. Such disinhibition seems to be enhanced by the activation of the neuronal systems related to periodic somatic and vegetative phenomena during sleep.
Chorea caused by lithium intoxication: A case report and literature review
Authors: Podskalny Gerald D. , Factor Stewart A. 
Published: December 2004

AbstractWe report a patient who developed an acute, reversble, generalized choreiform disorder from lithium (Li) intoxication. This medication was prescribed for manic‐depressive disorder, and serum levels became elevated after the addition of a diuretic for the treatment of hypertension. There were no other apparent causes for the movement disorder, and it was associated with other known features of Li intoxication, including ataxia and encephalopathy. There was a delay between the intial symptoms of Li intoxication and the onset of chorea. The chorea improved as serum Li levels diminished, with some lag time. This represents the eleventh case report of Li‐induced chorea, but only the sixth in a patient without concomitant neuroleptic therapy, and the first presented with videotape confirmation. A review of these other cases is included, and possible mechanisms are discussed.
A double‐blind, placebo‐controlled study of intranasal apomorphine spray as a rescue agent for off‐states in Parkinson's disease
Authors: Dewey Richard B. , Maraganore Demetrius M. , Ahlskog J. Eric , Matsumoto Joseph Y. 
Published: December 2004

AbstractNine patients with advanced levodopa‐responsive Parkinson's disease were enrolled in a double‐blind, placebocontrolled crossover trial of intranasal apomorphine as rescue therapy for parkinsonian off‐states. Patients were assigned in random order to each of four possible combinations of apomorphine, trimethobenzamide antiemetic, and their matched placebos and received detailed in‐office motor scoring during each of the four study periods. Patients also completed diaries describing the effectiveness of the nasal spray for reversing off‐states. A statistically significant reduction in the Unified Parkinson's Disease Rating Scale (UPDRS) motor score was seen following active apomorphine during in‐office evaluation visits but not following placebo nasal spray. Patient diaries revealed that active apomorphine had a latency to onset of 11 minutes and a duration of 50 minutes. Significant nausea from apomorphine spray was seen in only one patient whereas nasal irritation was disabling in three and mild in two. We conclude that intranasal apomorphine is an effective rescue agent for parkinsonian off‐states although nasal irritation is a limiting factor.
Total structural assignment and absolute configuration of terreinol by 13C and 1H NMR
Authors: de Macedo Fernando César , Marsaioli Anita Jocelyne 
Published: December 2004

AbstractThe carbon–carbon connectivity of terreinol, a new metabolite isolated from Aspergillus terreus, and its previous 13C assignments were confirmed by a two‐dimensional INADEQUATE experiment using a few milligrams of the compound with natural 13C abundance. The carbon–carbon correlations were determined by computational analysis (with >99% probability) of this experiment. Additionally, the absolute configuration of terreinol was achieved indirectly via its corresponding secondary alcohol by the modified Mosher method allied to conformational analysis. The shielding effect of the phenyl group of methoxytrifluoromethylphenylacetic acid (MTPA) on the substituents of the carbonylic centre gave a fully regular ΔδSR sign distribution, allowing reliable assignment of the R configuration for terreinol. Copyright © 2004 John Wiley & Sons, Ltd.
Proton MR CSF analysis and a new software as predictors for the differentiation of meningitis in children
Authors: Subramanian Arunachalam , Gupta Abhishek , Saxena Swapnil , Gupta Ashish , Kumar Raj , Nigam Anjali , Kumar Rashmi , Mandal Sudhir K. , Roy Raja 
Published: December 2004

AbstractThis article describes proton MR spectroscopic analysis of cerebrospinal fluid of 167 children suffering from meningitis and 24 control cases. Quantification of 12 well‐separated and commonly observed cerebrospinal fluid metabolites viz., β‐hydroxybutyrate, lactate, alanine, acetate, acetone, acetoacetate, pyruvate, glutamine, citrate, creatine/creatinine, glucose (total) and urea was carried out using Bruker's NMRQUANT software with respect to a known concentration of sodium‐3‐(trimethylsilyl)‐2,2,3,3‐d4‐propionate (TSP), serving as an external reference. The assignment of urea in CSF is reported for the first time by NMR. The presence of cyclopropane, observed for the first time in tuberculous meningitis overall in 85.1% of cases, acts as a finger‐print marker for the differential diagnosis. Multivariate discriminant function analysis was carried out for the proton MR‐detected metabolite information and the clinical symptoms data of the meningitis and control cases to find the important descriptors for classification, followed by a re‐validation of the entire database. It was found that the control could be differentiated from the disease group with a success rate of 96.4%, followed by the differential diagnosis of tuberculous meningitis with a corresponding value of 77.2%. Excluding the presence of cyclopropane, bacterial meningitis could be classified 84.4% correct and viral meningitis with a rate of 83.3%. It is proposed that the NMR spectroscopic information, along with other routine clinical features, may serve as an additional diagnostic tool for the differential diagnosis of meningitis in children. Copyright © 2004 John Wiley & Sons, Ltd.