bluuebunny/crossref_metadata_2025_split huggingface dataset:
A high‐resolution three‐dimensional T1‐weighted gradient echo sequence improves the detection of disease activity in multiple sclerosis
Authors: Filippi M. , Rovaris M. , Comi G. , Yousry T. , Alkadhi H. , Horsfield M. A. , Campi A. , Voltz R. 
Published: January 2005

AbstractIn this study, we performed a post‐contrast magnetization‐prepared rapid acquisition gradient echo (MP RAGE) sequence with subsequent reconstruction of axial slices with 5,3‐, and 1‐mm thickness in 26 patients with multiple sclerosis (MS) to investigate the disease activity going on below the resolution of conventional scans. One hundred three enhancing lesions were detected using MP RAGE reconstructed with 1‐mm‐thick slices, 93 using MP RAGE reconstructed with 3‐mm‐thick slices and 82 using MP RAGE reconstructed with 5‐mm‐thick slices. The detection rate using 1‐mm MP RAGE was 25.6% higher compared with 5‐mm MP RAGE and 12% higher compared with 3‐mm MP RAGE. Using 1‐mm MP RAGE, we detected five enhancing lesions in 2 of the 12 patients (16.6%) who showed no enhancing lesions on the 5‐mm MP RAGE and one enhancing lesion in 1 of the 10 patients (10%) who showed no enhancing lesions on the 3‐mm MP RAGE. The increased sensitivity of 1‐mm MP RAGE over the other two reconstruction slice thicknesses was due to improved detection of small lesions, located mainly in cortical/subcortical areas. These data indicate that the size of enhancing lesions in MS is highly variable, and that a significant proportion of disease activity goes undetected when performing conventional scans.
Botulinum toxin treatment of muscle cramps: A clinical and neurophysiological study
Authors: Bertolasi Laura , Priori Alberto , Tomelleri Giuliano , Bongiovanni Luigi G. , Fincati Emiliana , Simonati Alessandro , De Grandis Domenico , Rizzuto Nicolo' 
Published: January 2005

AbstractBotulinum toxin is now widely used in the treatment of severla hyperkinetic movement disorders. To evaluate its efficacy in treating muscle cramping syndromes, we studied clinical and neurophysiological variables before and after botulinum toxin injections into calf muscles and small flexor muscles of the foot in patients with an inherited benign crampfasciculation syndrome. At each assessment the clinical severity of cramp was scored and the cramp threshold frequency was measured with repetitive electrical peripheral nerve stimulation. Botulinum toxin injection signifcantly lowered our patients' clinical cramp severity scores (mean ± SD: before, 3.80 ± 0.44; after, 1.40 ± 0.54), left muscle strength unchanged and significanlty increased their cramp threshold frequencies (before, 4.22 ± 2.26 Hz; after, 10.0 ± 3.74 Hz). The clinical beefit induced by botulinum toxin lasted about 3 months. Boutlinum toxin injections also significantly reduced fasciculation potentials in relaxed muscles (before, 0.86 ± 0.19 fasciculations/sec; after, 0.45 ± 0.11 fasciculations/sec). These findings show that local intramuscular injection of botulinum toxin provide effective, safe, and long‐lasting relief of cramps possibly by reducing presynaptic cholinergic stimulation of motor nerve terminals and by impairing the input/output function of intrafusal and extrafusal motor end plates.
Marked age‐dependent neuroprotection by brain‐derived neurotrophic factor against neonatal hypoxic—ischemic brain injury
Authors: Cheng Yu , Gidday Jeffrey M. , Yan Qiao , Shah Aarti R. , Holtzman David M. 
Published: January 2005

AbstractHypoxic‐ischemic brain injury in survivors of perinatal asphyxia is a frequently encountered clinical problem for which there is currently no effective therapy. Neurotrophins, such as brain‐derived neurotrophic factor (BDNF), can protect responsive neurons against cell death in some injury paradigms. While the role of BDNF in hypoxic‐ischemic brain injury is not clear, evidence suggests that BDNF may have different effects in the developing, as opposed to the adult, brain. We found that a single intracerebroventricular (ICV) injection of BDNF resulted in rapid and robust phosphorylation of trk receptors in multiple brain regions in the postnatal day (PD) 7 rat brain. BDNF also markedly protected against hypoxic‐ischemic brain injury at PD7. It protected against 90% of tissue loss due to hypoxic‐ischemia when given just prior to the insult and against 50% of tissue loss when give after the insult. In contrast, ICV injection of BDNF in PD21 and adult rats resulted in little trk phosphorylation and less dramatic protection against unilateral hypoxic‐ischemic injury at PD21. Because of its potent neuroprotective actions in the developing brain, BDNF may be a potential treatment for asphyxia and other forms of acute injury in the perinatal period.
The impact of inpatient rehabilitation on progressive multiple sclerosis
Authors: Freeman J. A. , Langdon D. W. , Hobart J. C. , Thompson A. J. 
Published: January 2005

AbstractOne of the primary aims of rehabilitation for patients with multiple sclerosis (MS) is to reduce their levels of disability and handicap, yet little systematic research into the outcomes of this intervention has been undertaken. This stratified, randomized, wait‐list controlled study evaluated the effectiveness of a short period of multidisciplinary inpatient rehabilitation in people with MS. Sixty‐six patients in the progressive phase of the disease were assessed at 0 and 6 weeks with validated measures of impairment (Expanded Disability Status Scale and Functional Systems), disability (Functional Independence Measure), and handicap (London Handicap Scale). Both groups were comparable in terms of age, sex, disease duration and severity, disability, and handicap. At the end of 6 weeks, although the level of impairment on both groups remained the same, those who participated in a short period of inpatient rehabilitation (average of 25 days) significantly improved their level of disability and handicap compared with those in the wait‐list control group. Despite unchanging impairment, inpatient rehabilitation resulted in reduced disability and handicap in patients with progressive MS.
Calcium channels in neurological disease
Authors: Greenberg David A. 
Published: January 2005

AbstractChannels involved in the influx and intracellular mobilization of calcium have been implicated as targets of diverse genetic and immune‐mediated neurological diseases. These include the L‐type voltage‐gated calcium channel of skeletal muscle (hypokalemic periodic paralysis), the neuronal P/Q‐type voltage‐gated calcium channel (familial hemiplegic migraine, episodic ataxia type 2, spinocerebellar ataxia 6, and Lambert–Eaton myasthenic syndrome), and the skeletal muscle ryanodine receptor (malignant hyperthermia and central core disease). The discovery of these and other calcium channelopathies should help to clarify how different mutations affect shannel function and how altered channel function produces disease, and may lead to new treatments for these conditions.
Long‐term effects of pancreatic transplantation on diabetic neuropathy
Authors: Navarro Xavier , Sutherland David E. R. , Kennedy William R. 
Published: January 2005

AbstractRestoration of a long‐lasting euglycemic state by a functioning pancreatic transplantation (PTx) is the most logical treatment for insulin‐dependent diabetes mellitus and for amelioration of secondary complications, including neuropathy. We evaluated neurological function by clinical examination, nerve conduction studies, and autonomic function tests in 115 patients with a functioning PTx and in 92 control patients treated with insulin, at baseline and 1, 2, 3.5, 5, 7, and 10 years later. In control patients, neuropathy progressively worsened during follow‐up. The clinical examination score and composite indices of abnormality of motor and sensory nerve conduction decreased significantly at all intervals tested. Autonomic function indices also decreased, but significantly only after 1 year. In patients who received a successful PTx the neuropathy improved. The motor and sensory nerve conduction indices increased significantly at all intervals after transplantation, whereas the clinical examination and autonomic tests improved only slightly. Patients who received either a PTx alone, a PTx after a kidney graft, or simultaneous pancreatic and kidney transplantations improved similarly over the follow‐up. These results indicate that a functioning PTx halts the progression and improves the signs of diabetic polyneuropathy by restoration of a normoglycemic state.
Vulnerable neuronal subsets in Alzheimer's and Pick's disease are distinguished by their τ isoform distribution and phosphorylation
Authors: Delacourte A. , Sergeant N. , Wattez A. , Gauvreau D. , Robitaille Y. 
Published: January 2005

AbstractAggregated τ proteins constitute the basic matrix of neuronal inclusions specific to numerous neurodegenerative disorders. Monodimensional and two‐dimensional Western blot analyses performed on cortical brain homogenates allowed discrimination between disease‐specific τ protein profiles. These observations raised the issue of the physiopathological significance of such specificities. Alzheimer's disease (AD) pathological τ proteins (PTPs) (τ 74, 69, 55) were compared with those of Pick's disease (PiD) (τ 64, 55) using a panel of antibodies against peptidic sequences of τ isoforms corresponding to exons 2, 3, and 10. AD and PiD could then be critically differentiated by the absence of translated τ isoforms with exon 10 in PiD PTPs, along with the absence of the phosporylation site on Ser262. Immunohistochemical studies corroborate these findings. Indeed, Pick bodies were strongly immunostained by an anti‐“exon 2” antibody but failed to reveal any anti‐exon 10 reactive epitope. Tangles in AD contained exon 2, 3, and 10 epitopes. Altogether, our results demonstrated that Pick bodies develop within specific neuronal subsets that express specific patterns of τ isoforms lacking exon 10 peptidic sequence. We conclude that neurodegenerative disorders imply attrition of selectively vulnerable neuronal subsets, a process revealed, and may be sustained by specific τ isoform patterns.
In vivo [11C] flumazenil‐PET correlates with ex vivo [3H] flumazenil autoradiography in hippocampal sclerosis
Authors: Koepp Matthias J. , Hand Kieran S. P. , Labbé Clarie , Richardson Mark P. , van Paesschen Wim , Baird Virginia H. , Cunningham Vincent J. , Bowery Norman G. , Brooks David J. , Duncan John S. 
Published: January 2005

AbstractBy using [11C]flumazenil‐positron emission tomography ([11C]FMZ‐PET), we have previously shown that reductions of central benzodiazepine receptors (cBZRs) are restricted to the hippocampus in mesial temporal lobe epilepsy (mTLE) caused by unilateral hippocampal sclerosis (HS). Receptor autoradiographic studies on resected hippocampal specimens from the same patients demonstrated loss of cBZRs that was over and above loss of neurons in the CA1 subregion. Here, we report the first direct comparison of in vivo cBZR binding with [11C]FMZ‐PET and ex vivo binding using [3H]FMZ autoradiography. We applied a magnetic resonance imaging‐based method for partial volume effect correction to the PET images of [11C]FMZ volume of distribution ([11C]FMZ Vd) obtained in 10 patients with refractory mTLE due to unilateral, hisologically verifed HS. Saturation autoradiography was performed on the hippocampal specimens obtained from the same patients, allowing calculation of receptor availability ([3H]FMZ Bmax). After correction for partial volume effect, [11C]FMZ Vd in the body of epileptogenic hippocampus was reduced by a mean of 42.1% compred with normal controls. [3H]FMZ bmax, determined autoradiographically from the same hippocampal tissue, was reduced by a mean of 42.7% compared with control hippocampi. Absolute in vivo and ex vivo measurements of cBZR binding for the body of the hippocampus were significantly correlated in each individual. Our study demonstrates that reduction of available cBZR on remaining neuron in HS can be reliably detected in vivo by using [11C]FMZ‐PET after correction for partial volume effect.
Temporal distribution of partial seizures: Comparison of an animal model with human partial epilepsy
Authors: Quigg Mark , Straume Martin , Menaker Michael , Bertam Edward H. 
Published: January 2005

AbstractSeizures do not often strike randomly but may occur in circadian patterns. We compared daily times of partial seizures determined by continuous electroencephalography among patients with mesial temporal lobe epilepsy (MTLE; n = 64), those with extratemporal lobe (XTLE; n = 26) or lesional temporal lobe epilepsy (LTLE; n = 8), and a rat model similar to MTLE in which rats become epileptic after electrically induced limbic status epilepticus (postlimbic status [PLS]; n = 20). Rats were maintained on a 12‐hour light/dark cycle with lights on at 0700 hours. The distributions of seizures were fitted by cosinor analysis to determine time of peak seizure incidence ± 95% confidence interval (95% CI). The mean fraction ± SD of seizures recorded during light was 63 ± 17% in PLS animals and 60 ± 21% in humans. Peak incidence of seizures for PLS rats (547 seizures) was 1645 (95% CI = 1448,1830) and for MTLE subjects (774 seizures) was 1500 (95% CI = 1324,1636). Seizures from XTLE (465 seizures) and LTLE (48 seizures) did not fit a cosinor model and occurred no more frequently during light than dark. In conclusion, limbic seizures in humans and PLS rats occur more often during light than dark and have similar cosinor daily distributions. The chronological similarity between human MTLE and PLS rat epilepsy suggests that limbic seizure occurrence has a relation to the circadian regulatory system.
Cerebral functional magnetic resonance imaging of vestibular, auditory, and nociceptive areas during galvanic stimulation
Authors: Bucher Stefan F. , Dieterich Marianne , Wiesmann Martin , Weiss Alexander , Zink Reto , Yousry Tarek A. , Brandt Thomas 
Published: January 2005

AbstractCerebral activation was investigated with functional magnetic resonance imaging (FMRI) during galvanic stimulation of the mastoid in 6 normal volunteers. Cutaneous stimulation at the neck C4–5 level served as a control. During mastoid stimulation, bilateral vestibular activation occurred in the posterior insula (parietoinsular vestibular cortex, PIVC), the transverse temporal (Heschl's) gyrus, and thalamic pulvinar. The cutaneous pain elicited by galvanic stimulation caused bilateral activity of the medial part of the insula and the anterior median thalamus. Thus, galvanic stimulation at the mastoid level activates cortical areas of three different sensory systems in the insulathalamic region, the vestibular, the auditory, and the nociceptive systems.
Mitochondrial DNA in focal dystonia: A cybrid analysis
Authors: Tabrizi S. J. , Cooper J. M. , Schapira A. H. V. 
Published: January 2005

AbstractThe cause and pathophysiology of dystonia remain unknown. The recent identification of mitochondrial complex I deficiency in platelets from patients with sporadic focal dystonia suggests that a defect of energy metabolism may be relevant in a proportion of patients. We have addressed the possible contribution of mitochondrial DNA (mtDNA) to the complex I deficiency in dystonia by the use of genome transfer technology. Platelets from patients dificient for complex I were fused with A549 p0 (mtDNA‐less) cells to form cybrids comprising the A549 nucleus and dystonia mtDNA. Mixed cybrid cell lines were analyzed for 9 controls and 9 dystonia patients, and clonal cybrid lines were generated for 2 control and 2 dystonia patients. Subsequent biochemical analysis showed that the dystonia complex I defect was complemented in both the mixed and the clonal cybrid lines. These results contrast with similar studies in mitochondrial myopathy and Parkinson's disease patients, in which the mitochondrial defect was maintained in at least a proportion of A549 cybrids, and suggest that the complex I defect in dystonia is not caused by an mtDNA mutation.
Failure to replicate a protective effect of allele 2 of NACP/α‐synuclein polymorphism in Alzheimer's disease: An association study
Authors: Hellman Nathan E. , Grant Elizabeth A. , Goate Alison M. 
Published: January 2005

AbstractRecently, a dinucleotide repeat polymorphism was identified in the promoter of the nonamyloid component of plaques (NACP) gene, and it was shown that the NACP allele 2 was significantly associated with healthy elderly control individuals with at least one apolipoprotein E ε4 allele, suggesting a protective role for this allele in Alzheimer's disease. We genotyped the same NACP polymorphism in a comparable number of individuals diagnosed with dementia of the Alzheimer's type and in healthy, elderly controls. In our analysis, however, no protective effect for NACP allele 2, or any of the other NACP alleles, was observed.
Measurement of regional N‐Acetylaspartate after transient global ischemia in gerbils with and without ischemic tolerance: An index of neuronal survival
Authors: Nakano Misa , Ueda Hirokazu , Li Ji‐Yao , Matsumoto Masayasu , Yanagihara Takehiko 
Published: January 2005

AbstractWe investigated the correlation between N‐acetylaspartate (NAA) level and neuronal density in the hippocampal CA1 region of the brain after occlusion of both common carotid arteries for 5 minutes and reperfusion for 3 hours to 4 weeks in gerbils with and without ischemic preconditioning (tolerance). Animals were divided into four groups—the sham operated group, the nonpreconditioning (non‐p) group, the single‐preconditioning (single‐p) group with 2‐minute ischemia once 2 days before 5‐minute ischemia, and the double‐preconditioning (double‐p) group with 2‐minute ischemia twice 2 days before 5‐minute ischemia (n = 6 for each group). The CA1 region was dissected out from freeze‐dried sections for high‐performance liquid chromatographic assay of NAA, and adjacent sections were stained with cresy1 violet for measurement of the neuronal density. Both NAA (pmol/μg dry weight) and the neuronal density (cells/mm) decreased in the non‐p group after 3 days (NAA = 24.0 ± 3.0; neuronal density = 65 ± 38 cells/mm) and 7 days (NAA = 17.9 ± 2.5; neuronal density = 20 ± 15 cells/mm) and in the single‐p group after 7 days (26.4 ± 3.0, 106 ± 30) compared with the control group (NAA = 32.9 ± 3.0; neuronal density = 203 ± 9 cells/mm). There was no decrease in the double‐p group. The NAA level and the neuronal density showed a good linear correlation. The regional NAA level may be used as an index of neuronal viability.
Cerebrospinal fluid F2‐isoprostane levels are increased in Alzheimer's disease
Authors: Montine T. J. , Markesbery W. R. , Morrow J. D. , Roberts L. J. 
Published: January 2005

AbstractPostmortem studies have associated Alzheimer's disease (AD) with regionally increased oxidative damage to brain. Lacking, however, is a specific marker of oxidative damage to brain that may be measured during life. We tested the hypothesis that cerebrospinal fluid (CSF) concentrations of F2‐isoprostanes (F2‐IsoPs), stable products of arachidonate peroxidation, are increased in CSF of AD patients. CSF from lateral ventricles (VF) was analyzed from 11 AD patients and 11 control subjects who participated in a rapid autopsy program. VF F2‐IsoP concentrations were significantly elevated in AD patients compared with control subjects (72 ± 7 vs 46 ± 4 pg/ml) and were significantly linearly correlated with brain weight (–0.3 pg/ml/g, r2 = 0.32). These results suggest that quantification of CSF F2‐IsoP concentrations may provide a useful biomarker of central nervous system oxidative damage in AD.
Slow vertical saccades in motor neuron disease: Correlation of structure and function
Authors: Averbuch‐Heller Lea , Helmchen Christoph , Horn Anja K. E. , Leigh R. John , Büttner‐Ennever Jean A. 
Published: January 2005

AbstractWe studied eye movements and brainstem pathology in 2 patients with slow vertical saccades and autopsy‐proven amyotrophic lateral sclerosis (ALS). In both patients, the main ocular motor finding was supranuclear vertical gaze impairment with slow vertical saccades. The second patient had difficulty opening his eyes on command, with preserved spontaneous eyelid opening. Postmortem examination in both patients demonstrated cell loss in the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) and substantia nigra, along with histopathological findings consistent with ALS. The extent of the pathological changes in the riMLF corelated well with the degree of functional impairment as reflected in the slow vertical saccades. We suggest that motor neuron disease with early involvement of vertical saccades represents a distinct clinicopathological entity.
Epilepsia partialis continua associated with a homoplasmic mitochondrial tRNASer(UCN) mutation
Authors: Schuelke Markus , Bakker Melan , Stoltenburg Gisela , Sperner Jürgen , von Moers Arpad 
Published: January 2005

AbstractEpilepsia partialis continua (EPC) is a rare epileptic syndrome characterized by continuous focal seizures. We report on a 16‐year‐old girl who died of prolonged pharmacoresistant EPC in whom we identified a 7472insC mutation within the mitochondrial transfer ribonucleic acid (tRNA)Ser(UCN). Additional symptoms included ataxia, lactic acidosis, myopathy, sensorineural hearing loss, severe headaches, and mental retardation. Quantification revealed 100% mutant mitochondrial DNA (mtDNA) in the patient, 4% in her mother, and none in her half‐sister. This highly skewed mtDNA distribution is most improbable (∼3 × 10−30) if only explained by random genetic drift. Clustering of dysfunctional mitochondria and replicatory advantage of mutant mtDNA may play a role in the rapid segregation towards homoplasmy within one generation.
Myelin mosaicism and brain plasticity in heterozygous females of a canine X‐linked trait
Authors: Cuddon Paul A. , Lipsitz David , Duncan Ian D. 
Published: January 2005

AbstractThe shaking (sh)pup, an animal model of Pelizaeus‐Merzbacher disease, is characterized by severe central nervous system dysmyelination in affected males, and myelin mosaicism in some female heterozygotes as a result of X‐linked inactivation. Heterozygous females develop a tremor of varying severity that usually disappears at 4 to 6 weeks, whereas male hemizygotes have severe, generalized tremor that persists throughout life. We have used these two myelin‐deficient models to study the potential for recovery with time as reflected by brainstem auditory evoked responses (BAERs). At set time points, the state of myelination in the trapezoid body was studied microscopically. Sequential BAERs demonstrated consistently prolonged interpeak latencies during the period of gross tremor in heterozygotes, with the trend continuing to a lesser extent after tremor cessation. The random nature of X‐linked inactivation resulted in variable myelin mosaicism that was reflected in variations in BAER changes within animals in the same litter. In most heterozygotes, the tremor resolved with time, the BAE returned to near normal, and myelin mosaicism was lost. In contrast, in the affected males, the severity of tremor and lack of recovery was demonstrated by consistent abnormalities in BAER waves at all times studied, and severe and persistent myelin deficiency in the trapezoid body. These findings show that despite the normal tightly programmed temporal development of myelin in the brain in the heterozygous mosaic state, sufficient plasticity persists during the neonatal period for late‐stage myelination to occur.
Histological analysis of the Nissl‐pattern and ‐substance of nerve cells
Authors: Einarson Lárus 
Published: January 2005

AbstractThe influence of the variety and pH of the fixative upon the Nissl pattern has been studied, together with the action of warm water, acids and Na2CO3 after ordinary fixation. The effect of NH4OH is characteristic. The stainability is considerably modified, but through alternating the use of the gallocyanin method and of a new stain, gallamin blue, desribed in the text, the Nissl substance is always stainable. After fixation in alcohol the substance does not stain with purified methylgreen.