bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intrinsic epileptogenicity of human dysplastic cortex as suggested by corticography and surgical results
Authors: Palmini André , Gambardella Antonio , Andermann Frederick , Dubeau Francois , da Costa Jaderson C. , Olivier André , Tampieri Donatella , Gloor Pierre , Quesney Felipe , Andermann Eva , Paglioli Eduardo , Paglioli‐Neto Eliseu , Andermann Ligia Coutinho , Leblanc Richard , Kim Hyoung‐Ihl 
Published: January 2005

AbstractCortical dysplastic lesions (CDyLs) are often associated with severe partial epilepsies. We describe the electrographic counterpart of this high degree of epileptogenicity, manifested by continuous or frequent rhythmic epileptogenic discharges recorded directly from CDyLs during intraoperative electrocorticography (ECoG). These ictal or continuous epileptogenic discharges (I/CEDs) assumed one of the following three patterns: (1) repetitive electrographic seizures, (2) repetitive bursting discharges, or (3) continuous or quasicontinuous rhythmic spiking. One or more of these patterns were present in 23 of 34 patients (67%) with intractable partial epilepsy associated with CDyLs, and in only 1 of 40 patients (2.5%) with intractable partial epilepsy associated with other types of structural lesions. I/CEDs were usually spatially restricted, thus contrasting with the more widespread interictal ECoG epileptic activity, and tended to colocalize with the magnetic resonance imaging–defined lesion. Completeness of excision of cortical tissue displaying I/CEDs correlated positively with surgical outcome in patients with medically intractable seizures; i.e., three‐fourths of the patients in whom it was entirely excised had favorble surgical outcome; in contrast, uniformly poor outcome was observed in those patients in whom areas containing I/CEDs remained in situ. We conclude that CDyLs are highly and intrinsically epileptogenic, and that intraoperative ECoG identification of this intrinsically epileptogenic dysplastic cortical tissue is crucial to decide the extent of excision for best seizure control.
Lyme neuroborreliosis
Authors: Garcia‐Monco Juan Carlos , Benach Jorge L. 
Published: January 2005

AbstractNeuroborreliosis, an infection of the nervous system caused by spirochetes of the genus Borrelia, has achieved worldwide attention in the last decade as part of the clinical spectrum of Lyme disease. This disorder, caused by Borrelia burgdorferi, has increased in incidence to become the most frequent arthropod‐borne infection in North America and Europe. As a result of this rapid rise in incidence and of its protean neurological manifestations, this disease has created an important challenge to modern neurology. The diverse manifestations of neuroborreliosis require that it be included in the differential diagnosis of many neurological disorders. This review takes a chronological approach to clinical neuroborreliosis to summarize its most important aspects. The limitations as well as the benefits of laboratory diagnosis are also considered, with the aim of providing assistance in this area. Recent advances in neuroimmunology regarding the pathogenesis of neuroborreliosis that may elucidate its protean clinical spectrum are summarized.
Low platelet mitochondrial complex I and complex II/III activity in early untreated parkinson's disease
Authors: Haas Richard H. , Nasirian Fatemeh , Nakano Kazutoshi , Ward David , Pay Mary , Hill Robert , Shults Clifford W. 
Published: January 2005

AbstractFollowing the discovery of inhibition of electron transport complex I by the neurotoxin 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP), which produces a parkinsonian syndrome in humans, monkeys, and mice, several laboratories have reported abnormalities of complex I and other electron transport complexes (ETCs) in various tissues from patients with Parkinson's disease (PD). Criticism of the significance of these findings in the etiology of PD has centered on whether drug treatments or the debilitation of the disease process itself produced the low ETC activities. We present results from a blinded study of platelet mitochondrial ETC activities in 18 early untreated PD patients and 18 age‐ and sex‐matched controls and in 13 spousal controls. Lower complex I activity in platelet mitochondria of PD patients was seen in early untreated disease and thus cannot be due to debilitation or drug therapy. Home environmental factors seem an unlikely explanation for the reduced complex I activity in PD patients but have not been excluded. Complex II/III activity was also reduced by 20% in PD compared with age‐/sex‐matched controls. The low complex I and II/III activities in platelet mitochondria appear to be related to the etiology of PD.
Expression of heme oxygenase‐1 in the senescent and alzheimer‐diseased brain
Authors: Schipper H. M. , Cissé S. , Stopa E. G. 
Published: January 2005

AbstractHeme oxygenase‐1 is a cellular stress protein expressed in brain and other tissue in response to oxidative challenge and other noxious stimuli. Using immunohistochemistry and immunofluorescent labeling in conjunction with laser scanning confocal microscopy, we observed intense immunoreactivity of heme oxygenase‐1 in neurons of the hippocampus and temporal cortex of Alzheimer‐diseased (AD) brain relative to age‐matched control specimens. Furthermore, we demonstrated consistent colocalization of heme oxygenase‐1 to glial fibrillary acidic protein–positive astrocytes, neurofibrillary tangles, and senile plaques in the AD specimens. In AD hippocampus, approximately 86% of glial fibrillary acidic protein–positive astrocytes expressed heme oxygenase‐1, whereas only 6.8% of hippocampal astrocytes in normal senescent control specimens were immunopositive for heme oxygenase‐1 (p < 0.0001). In regions other than the hippocampus and neocortex, such as the substantia nigra, the proportion of astrocytes expressing heme oxygenase‐1 in the experimental group (12.8%) was not significantly different from that in the controls (6.4%, p > 0.05). Robust 32‐kd bands corresponding to heme oxygenase‐1 were observed by Western blotting of protein extracts derived from AD temporal cortex and hippocampus after sodium dodecyl sulfate–polyacrylamide gel electrophoresis. Heme oxygenase‐1 bands were very faint or absent in protein extracts prepared from control specimens. These results indicate that heme oxygenase‐1 is significantly overexpressed in neurons and astrocytes of AD hippocampus and cerebral cortex relative to control brains. Upregulation of heme oxygenase‐1 in AD brain supports the contention that the affected tissues are experiencing chronic oxidative stress. In addition, the excessive generation of carbon monoxide, a metabolite of heme degradation, may participate in the pathogenesis of AD.
Tonic vibration reflex and muscle afferent block in writer's cramp
Authors: Kaji Ryuji , Rothwell John C. , Katayama Mari , Ikeda Tomoko , Kubori Tamotsu , Kohara Nobuo , Mezaki Takahiro , Shibasaki Hiroshi , Kimura June 
Published: January 2005

AbstractPatients with focal dystonia take advantage of certain cutaneous or proprioceptive sensory inputs to alleviate their symptoms (“sensory trick”). We examined the effects of increasing muscle spindle activity by the tonic vibration reflex maneuver and Decemberreasing it by intramuscular injection of lidocaine. The vibration was applied to the palm or the tendon of forearm muscles in 15 patients with writer's cramp and 15 age‐matched normal subjects. In 11 patients, the vibration induced dystonic postures or movements typical of those seen during writing. Normal subjects showed either no response to the vibration or a gradually developing tonic vibration reflex only in the wrist and finger flexors, which produced visible movements with a significantly longer latency (12.5 ± 6.7 seconds [mean ± standard deviation]) than what was observed in the patients (2.7 ± 2.5 seconds, p < 0.0001). Local injection of lidocaine (0.5%, 5–40 ml/muscle) attenuated the tendon reflex with relatively little effect on the M response. Injection into muscles with increased activity produced Marchked reduction of dystonic movements and significant clinical improvement in 13 patients, whereas injection into the other muscles had no effect. The clinical benefit lasted for 1 to 24 hours after injection. In 13 patients who had additional injections of 10% ethanol, which blocks sodium channels for a longer period than does lidocaine, the duration of action was prolonged to 5 to 21 days. These findings suggest that muscles causing dystonic movements have abnormal sensitivities to vibration at rest and that muscle afferents may play a pivotal role in producing dystonic movements. Local injection of lidocaine or ethanol reduces the effectiveness of muscle spindle afferent and may provide a means of treating patients with writer's cramp without causing unwanted weakness.
Frontal lobe phosphorus metabolism and neuropsychological function in aging and in alzheimer's disease
Authors: Smith Charles D. , Creed Pettigrew L. , Avison Malcolm J. , Kirsch John E. , Tinkhtman Alexander J. , Schmitt Frederick A. , Wermeling Daniel P. , Wekstein David R. , Marchkesberry William R. 
Published: January 2005

Abstract31P Magnetic resonance spectroscopy of the frontal lobe was performed in 17 patients with Alzheimer's disease (AD), 8 elderly controls (EC), and 17 young controls (YC). The phosphocreatine/inorganic phosphate (PCr/Pi) ratio in AD (2.32 ± 0.26 SD) was significantly lower than in EC (2.65 ± 0.41). In AD patients, a correlation was observed between the PCr/Pi ratio and the dementia rating scale (r = −0.50, p = 0.04). A significant positive correlation between PCr/Pi ratio and age was observed in both AD (r = 0.67, p 0.003) and YC (r = 0.63, p 0.006) groups, however, suggesting caution in interpretation of this ratio in AD. We did not find differences between AD, EC, or YC in any other spectroscopic measure. A significant sex difference in the phosphomonoester/phosphodiester ratio (PME/PDE) ratio was observed in AD brain. Females had a lower PME/PDE ratio than males.
Exons 16 and 17 of the amyloid precursor protein gene in familial inclusion body myopathy
Authors: Sivakumar K. , Cervenáková L. , Dalakas M. C. , Leon‐Monzon M. , Isaacson S. H. , Nagle J. W. , Vasconcelos O. , Goldfarb L. G. 
Published: January 2005

AbstractAccumulation of β‐amyloid protein (Aβ) occurs in some muscle fibers of patients with inclusion body myopathy and resembles the type of amyloid deposits seen in the affected tissues of patients with Alzheimer's disease and cerebrovascular amyloidosis. Because mutations in exons 16 and 17 of the β‐amyloid precursor protein (βAPP) gene on chromosome 21 have been identified in patients with early‐onset familial Alzheimer's disease and Dutch‐type cerebrovascular amyloidosis, we searched for mutations of the same region in patients with familial inclusion body myopathy. Sequencing of both alleles in 8 patients from four unrelated families did not reveal any mutations in these exons. The amyloid deposition in familial forms of inclusion body myopathy may be either due to errors in other gene loci, or it is secondary reflecting altered βAPP metabolism or myocyte degeneration and cell membrane degradation.
Proton nuclear magnetic resonance spectroscopic imaging of human temporal lobe epilepsy at 4.1 T
Authors: Hetherington Hoby , Kuzniecky Ruben , Pan Jullie , Mason Graeme , Morawetz Richard , Harris Cheryl , Faught Edward , Vaughan Thomas , Pohost Gerald 
Published: January 2005

AbstractWe performed proton magnetic resonance spectroscopic imaging (MRSI) at high magnetic field (4.1 T) to study N‐acetylaspartate, creatine, and choline levels in the brains of normal control subjects and patients with intractable temporal lobe epilepsy. We compared the results of MRSI to those of other presurgical techniques to determine the sensitivity of this method in the lateralization of the epileptic focus. The normal hippocampal creatine—N‐acetylaspartate ratio was 0.71 ± 0.14 with no differences between left and right. Using the mean control hippocampal creatine—N‐acetylaspartate ratio plus 2 standard deviations to identify statistically significant changes, we found lateralizing metabolic abnormalities corresponding to the operated temporal lobe in all patients. Four patients (40%) had contralateral abnormalities, and 2 of them had bilateral independent seizure onset confirmed by intracranial electroencephalographic studies. Statistically significant increases in the choline—N‐acetylaspartate ratio in comparison to healthy volunteers were observed in 8 of the 10 patients. With the creatine—N‐acetylaspartate ratio, MRSI demonstrated a 100% sensitivity compared to magnetic resonance imaging, which identified pathology in 70% of the patients. These findings suggest that proton MRSI yields a distinctive metabolic profile in patients with temporal lobe epilepsy and is sensitive in detecting bilateral metabolic abnormalities in some patients. These preliminary findings suggest that MRSI is more sensitive than magnetic resonance imaging in the lateralization of epileptic foci in temporal lobe epilepsy.
Recovery of hypermetria after a cerebellar stroke occurs as a multistage process
Authors: Manto M. , Jacquy J. , Hildebrand J. , Godaux E. 
Published: January 2005

AbstractIn a prospective study, we repeatedly recorded fast goal‐directed wrist movements of 8 patients who had experienced an acute cerebellar hypermetria due to a stroke and who had subsequently recovered clinically. Movements and the associated agonist and antagonist electromyographic (EMG) activities were recorded before and after addition of inertial loads. Four stages characterized the recovery process. At stage 1, hypermetria was present in the basal state and was not modified by the addition of inertial loads. At stage 2, hypermetria, which was present in the basal state, was enlarged by mass addition. At stage 3, hypermetria was absent in the basal state, but was revealed by an inertial load increase. At stage 4, as in healthy subjects, there was no hypermetria without or with addition of inertial loads. At stage 1, the patients presented several defects. (1) Facing an increased inertia, they could not increase their agonist EMG activity. (2) The onset latency of their antagonist EMG activity was delayed. (3) Facing an increased inertia, they could not increase their antagonist EMG activity. Among these three defects, the first disappeared at stage 2, the second at stage 3, and the third at stage 4.
Apolipoprotein E ϵ4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis
Authors: Mui Stina , William Rebeck G. , McKenna‐Yasek Diane , Hyman Bradley T. , Brown Robert H. 
Published: January 2005

AbstractApolipoprotein E allele 4 (apo E ϵ4) is known to be in genetic disequilibrium with Alzheimer's disease and is associated with an earlier age at onset of dementia. Whether apo E ϵ4 is a specific risk factor for Alzheimer's disease or is a more general susceptibility factor that shifts the age at onset of neurodegenerative diseases to earlier ages is unknown. To test these possibilities, we determined the apolipoprotein E genotypes of subjects with familial or sporadic amyotrophic lateral sclerosis (ALS). ApoE allele frequencies of the the apoE gene of the ALS subjects (n = 170, ϵ2 = 0.071, ϵ3 = 0.771, ϵ4 = 0.159) were found to be comparable to the allele frequencies of the general population. Furthermore, no significant association was observed between the age at onset or the duration of ALS and the inheritance of apoE ϵ4: subjects with at least one copy of ϵ4 (sporadic ALS: n = 15, onset at 57.7 ± 13.9 years; familial ALS: n = 23, onset at 53.6 ± 9.5 years, duration [n = 14] of 2.6 ± 1.6 years) had comparable ages at onset and durations to subjects without ϵ4 (sporadic ALS: n = 28, onset at 53.1 ± 17.0 years; familial ALS: n = 56, onset at 50.8 ± 12.1 years, duration [n = 30] of 1.9 ± 0.8 years). The lack of association of apoE ϵ4 with the age at onset and the duration of ALS suggests that apoE ϵ4 does not have a global effect on the pathogenesis of other neurodegenerative diseases.
Cerebral transport and metabolism of 1‐11C‐D‐glucose during stepped hypoglycemia
Authors: Powers William J. , Dagogo‐Jack Samuel , Markham Joanne , Larson Kenneth B. , Dence Carmen S. 
Published: January 2005

AbstractAttempts to measure blood‐to‐brain glucose transport and cerebral glucose metabolism with 11C‐glucose have been hampered by methods that require jugular venous sampling or do not adequately account for the efflux of labeled metabolites from the brain. We performed eight positron emission tomography studies with 1‐11C‐d‐glucose in macaques at arterial plasma glucose concentrations of 8.43 to 1.51 μmol ml−1 (152–27 mg dl−1) using a model that includes a fourth rate constant to account for regional egrees of all 11C‐metabolites. Values for blood‐to‐brain glucose influx, cerebral glucose metabolism, and brain free glucose concentration agreed closely with values obtained in mammals by other investigators. Values for net extraction fraction corresponded closely to simultaneously measured arteriovenous values. We demonstrated that utilization of a model that includes a fourth rate constant to account for regional egress of all 11C‐metabolites with positron emission tomography and 1‐11C‐d‐glucose provides accurate measurements of blood‐to‐brain glucose transport and cerebral glucose metabolism in vivo without need for jugular venous sampling, even under conditions of severe hypoglycemia.
Localization of subclinical ictal activity by functional magnetic resonance imaging: Correlation with invasive monitoring
Authors: Detre John A. , Sirven Joseph I. , Alsop David C. , O'Connor Michael J. , French Jaqueline A. 
Published: January 2005

AbstractFunctional magnetic resonance imaging (fMRI) with susceptibility‐based contrast was used to detect focal changes in cerebral blood flow and metabolism in a patient with focal epilepsy. The patient presented with frequent partial motor seizures involving his right lower face that spread to produce speech arrest and occasionally right arm jerking. Consciousness was never impaired during these events. A multislice echoplanar technique was used to acquire 16 contiguous axial slices every 4 seconds for 11 minutes. Although no overt seizures were observed or reported by the patient during the scanning, a time series analysis of the functional data revealed focal signal‐intensity changes in the posterior left frontal lobe, which correlated well both in duration and spatial localization with ictal activity subsequently recorded by invasive electrophysiological monitoring. The spatial localization of fMRI was more accurate than electroencephalography recorded from a subdural grid in predicting the site of successful surgical therapy. These results illustrate the potential of functional MRI for localizing seizure foci with high spatial and temporal resolution. Such studies can be readily combined with high‐resolution anatomical imaging, task‐activation studies, and other magnetic resonance techniques.
Movement‐related cortical potentials in writer's cramp
Authors: Deuschl G. , Toro C. , Matsumoto J. , Hallett M. 
Published: January 2005

AbstractMovement‐related cortical potentials in response to simple, self‐paced, brisk index finger abduction movements were recorded in patients with simple and complex writer's cramp and compared with those of age‐matched control subjects. Analysis of the movement‐related cortical potential waveforms showed that the Bereitschaftspotential, the peak of the negative slope, and the frontal peak of the motor potential did not differ in the two groups, except for the average amplitude of the early part of the negative‐slope peak, which was decreased in the patient group during the interval of 300 to 200 msec prior to electromyographic onset. This finding was restricted to the electrodes overlying the contralateral and midline central electrodes. Movement‐related cortical potentials from patients and control subjects could be equally accounted for by a four‐dipole source model with sources located in the contralateral and ipsilateral sensorimotor regions and the supplementary motor area. There was a trend for a reduction in the strength of the sensorimotor sources active during the premotor period in the patient group, but the difference did not reach a significant level for any individual source. No differences were found between the movement‐related cortical potentials elicited by movements of the affected and unaffected hand, or between those of patients with simple or complex hand cramps. This result suggests a deficiency of contralateral motor cortex activation just prior to the initiation of voluntary movements in patients with focal dystonia.
Familial subarachnoid hemorrhage: Distinctive features and patterns of inheritance
Authors: Bromberg Jacoline E. C. , Rinkel Gabriel J. E. , Algra Ale , van Duyn Cornelia M. , Greebe Paut , Ramos Lino M. P. , van Gijn Jan 
Published: January 2005

AbstractTo delineate the distinctive features of familial subarachnoid hemorrhage, we compared gender and age at the time of subarachnoid hemorrhage, as well as site and number of aneurysms, in patients with familial subarachnoid hemorrhage (at least 1 first‐degree relative with subarachnoid hemorrhage) and patients with sporadic subarachnoid hemorrhage (no subarachnoid hemorrhage in first‐ or second‐degree relatives), in a prospective, hospital‐based series of patients. In addition we studied the pattern of inheritance in 17 families with familial subarachnoid hemorrhage. Mean age at the time of hemorrhage in patients with the familial form was 6.8 years lower than that in those with the sporadic form, and middle cerebral artery aneurysms occurred more often in patients with familial disease. Sex distribution and number of aneurysms were similar in the two groups. Inheritance was compatible with autosomal dominant transmission in some families, and with autosomal recessive or multifactorial transmission in others. In our 5 families as well as in all 18 previously reported families with two affected generations, the age at the time of subarachnoid hemorrhage was invariably lower in later generations, which is suggestive of anticipation. We conclude that familial subarachnoid hemorrhage is a separate entity with occurrence at a young age, predilection for aneurysms of the middle cerebral artery, and variable modes of inheritance, including autosomal dominant inheritance with possible anticipation.