bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ganaxolone, a selective, high‐affinity steroid modulator of the γ‐aminobutyric acid‐A receptor, exacerbates seizures in animal models of absence
Authors: Snead O. Carter 
Published: January 2005

AbstractGanaxolone (3α‐hydroxy‐3β‐methyl‐5α‐pregnan‐20‐one) is a novel neurosteroid which has anticonvulsant properties in a number of seizure models as well as the ability to enhance function of the γ‐aminobutyric acid‐A (GABAA) receptor complex via a neurosteroid binding site. The object of these experiments was to ascertain the efficacy of ganaxolone against absence seizures. Ganaxolone was assessed in the low‐dose pentylenetetrazol (PTZ) and the γ‐hydroxybutyric acid (GHB) model of absence seizures in rats. Ganaxolone pretreatment resulted in a significant prolongation of absence seizure in both the PTZ and GHB models. Further, ganaxolone in doses above 20 mg/kg alone produced bilaterally synchronous spike wave discharges (SWDs) associated with behavioral arrest. These data suggest that augmentation of GABAA receptor complex function by neurosteroids has the potential to result in or exacerbate absence seizures.
The phenotypic spectrum of CADASIL: Clinical findings in 102 cases
Authors: Dichgans M. , Mayer M. , Uttner I. , Brüning R. , Müller‐Höcker J. , Rungger G. , Ebke M. , Klockgether T. , Gasser T. 
Published: January 2005

AbstractCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an increasingly recognized autosomal dominant disorder that leads to cerebrovascular manifestations in early adulthood. This study delineates the phenotypic spectrum and the natural history of the disease in 102 affected individuals from 29 families with biopsy‐proven CADASIL. Recurrent ischemic episodes (transient ischemic attack [TIA] or stroke) were the most frequent presentation found in 71% of the cases (mean age at onset, 46.1 years; range, 30–66 years; SD, 9.0 years). Forty‐eight percent of the cases had developed cognitive deficits. Dementia (28%) was frequently accompanied by gait disturvance (90%), urinary incontinence (86%), and pseudobulbar palsy (52%). Thirty‐nine patients (38%) had a history of migraine (mean age at oneset, 26.0 years; SD, 8.2 years), which was classified as migraine with aura in 87% of the cases. Psychiatric disturbances were present in 30% of the cases, with adjustment disorder (24%) being the most frequent diagnosis. Ten patients (10%) had a history of epileptic seizures. To delineate the functional consequences of ischemic deficits, we studied the extent of disability in different age groups. The full spectrum of disability was seen in all groups older than age 45. Fifty‐five percent of the patients older than age 60 were unable to walk without assistance. However, 14% in this age group exhibited no disability at all. Kaplan‐Meier analysis disclosed median survival times of 64 years (males) and 69 years (females). An investigation of the 18 multiplex families revealed marked intrafamilial variations.
Prospective magnetic resonance imaging identification of focal cortical dysplasia, including the non—balloon cell subtype
Authors: Chan Stephen , Chin Steven S. , Nordli Douglas R. , Goodman Robert R. , DeLaPaz Robert L. , Pedley Timothy A. 
Published: January 2005

AbstractThe purpose of this study was to determine the role of high‐resolution T2‐weighted fast multiplanar inversion‐recovery (FMPIR) magnetic resonance (MR) imaging in detecting and delineating microscopic focal cortical dysplasia (FCD). We performed MR scans with FMPIR on 42 patients with suspected neocortical epilepsy. Ten MR studies were read prospectively as showing FCD; these case histories, electroencephalographic studies, and neuroimaging data were reviewed. Eight of these patients subsequently underwent focal cortical resection guided by intraoperative electrocorticography. The MR findings were correlated with pathological findings in these 8 patients. For purposes of radiological‐pathological correlation, the FCD lesions were divided into two classes. Radiological classification was based on the absence (type A) or presence (type B) of T2 prolongation of the subcortical white matter. Pathological grading as type I or type II was based on a previously described pathological grading system. Specific MR findings associated with FCD included focal blurring of the gray‐white matter interface (n = 9), thickening of the cortical ribbon (n = 7), and T2 prolongation of the subcortical white matter (n = 4). In 3 patients, the only MR finding that suggested FCD was localized blurring of the gray‐white matter junction. In 2 of these 3 patients, the MR diagnosis of FCD could be made only by FMPIR. FCD was confirmed histologically in 7 of 8 patients, with insufficient tissue for complete histopathological evaluation in 1 case. Radiological classification of FCD agreed with pathological classification in 5 of 7 cases. Correlation of MR findings with intraoperative electrocorticography results indicated that the MR study localized the epileptogenic lesion correctly in 8 of 8 cases. Scalp ictal electroencephalographic studies localized the epileptogenic lesion in 5 of 8 cases; positron emission tomographic scans were focally abnormal in 3 of 3 cases. FMPIR MR imaging permitted accurate diagnosis and localization of FCD in all patients with pathologically proved FCD. MR identification of FCD aided presurgical planning and intraoperative management of these patients.
Enhanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis
Authors: Liu Rugao , Althaus John S. , Ellerbrock Brenda R. , Becker David A. , Gurney Mark E. 
Published: January 2005

AbstractMutations of the SOD1 gene encoding copper/zinc superoxide dismutase (CuZnSOD) cause an inherited form of amyotrophic lateral sclerosis. When expressed in transgenic mice, the same SOD1 mutations cause progressive loss of spinal motor neurons with consequent paralysis and death. In vitro biochemical studies indicate that SOD1 mutations enhance free radical generation by the mutant enzyme. We investigated those findings in vivo by using a novel, brain‐permeable spin trap, azulenyl nitrone. Reaction of azulenyl nitrone with a free radical forms a nitroxide adduct that then fragments to yield the corresponding azulenyl aldehyde. Transgenic mice expressing mutant SOD1‐G93A show enhanced free radical content in spinal cord but not brain. This correlates with tissue‐specific differences in the level of transgene expression. In spinal cord, the increase in free radical content is in direct proportion to the age‐dependent increase in mutant human CuZnSOD expression. This increase precedes motor neuron degeneration. The higher level of human CuZnSOD expression seen in spinal cord compared with brain, and consequent difference in free radical generation, provides a basis for understanding the selective vulnerability of the spinal cord in this disease model.
Electrophysiological classification of guillain‐barré syndrome: Clinical associations and outcome
Authors: Hadden R. D. M. , Cornblath D. R. , Hughes R. A. C. , Zielasek J. , Hartung H.‐P. , Toyka K. V. , Swan A. V. ,   The Plasma Exchange/Sandoglobulin Guillain‐Barré Syndrome Trial Group
Published: January 2005

AbstractWe performed electrophysiological and serological testing within 15 days of symptom onset on 369 patients with Guillain‐Barré Syndrome (GBS) enrolled in a trial comparing plasma exchange, intravenous immunoglobulin, and both treatments. Patients were classified into five groups by motor nerve conduction criteria; 69% were demyelinating, 3% axonal, 3% inexcitable, 2% normal, and 23% equivocal. Six of 10 (60%) patients with axonal neurophysiology had had a preceding diarrheal illness compared with 71 of 359 (20%) in other groups. Antiganglioside GM1 antibodies were present in a higher proportion of patients with axonal physiology or inexcitable nerves than other patients. The number dead or unable to walk unaided at 48 weeks was greater in the group with initially inexcitable nerves (6 of 12, 50%) compared with the rest (52 of 357, 15%), but was not significantly different between the axonal (1 of 10, 10%) and demyelinating (44 of 254, 17%) groups. Sensory action potentials and clinical sensory examination were both normal in 53 of 342 (16%) patients, and these “pure motor GBS” patients were more likely than other GBS patients to have IgG antiganglioside GM1 antibodies and to have had preceding diarrhea but had a similar outcome. The axonal group was more likely than other groups to have normal sensory action potentials. The outcomes in response to the three treatments did not differ in any subgroup (including patients with pure motor GBS or preceding diarrhea) or any neurophysiological category.
Somatic and limbic cortex activation in esophageal distention: A functional imaging study
Authors: Binkofski F. , Schnitzler A. , Enck P. , Frieling T. , Posse S. , Seitz R. J. , Freund H.‐J. 
Published: January 2005

AbstractLittle is known about the cerebral representations of visceral sensations in humans. Using functional magnetic resonance imaging (fMRI), we mapped the cortical areas of the human brain that were activated by mechanical stimulation of the esophagus in 5 healthy volunteers. Stimulation probes were placed into the distal part of the esophagus and inflated to produce a local distention. The cerebral activation pattern was related to the strength and quality of the stimulus. The weakest stimulus accompanied by a well‐localized albeit weak retrosternal sensation activated only the parietal opercular cortices, probably including the secondary somatosensory cortex (SII). Additional activation of the primary sensorimotor cortex (SI) at the level of the face and mouth representation as well as of the right premotor cortex was found during repetitive distention of the esophagus at 0.5 Hz. Repetitive stimulation at 1 Hz additionally activated the insulabilaterally. The strongest distention stimulus, which caused a painful retrosternal sensation, resulted in an activation of the anterior cingulate cortex. Our findings demonstrate that SII is the primary cortical target of visceral afferents originating in the esophagus. Limbic structures become engaged when the visceral senstion is unpleasant or painful.
Protein modification by the lipid peroxidation product 4‐hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients
Authors: Pedersen Ward A. , Fu Weiming , Keller Jeffrey N. , Markesbery William R. , Appel Stanley , Smith R. Glenn , Kasarskis Edward , Mattson Mark P. 
Published: January 2005

AbstractWe report increased modification of proteins by 4‐hydroxynoneal (HNE), a product of membrane lipid peroxidation, in the lumbar spinal cord of sporadic amyotrophic lateral sclerosis (ALS) patients versus that of neurologically normal controls. By immunohistochemistry, HNE‐protein modification was detected in ventral horn motor neurons, and immunoprecipitation analysis revealed that one of the proteins modified by HNE was the astrocytic glutamate transporter EAAT2. Given that the function of proteins modified by HNE can be severely compromised as previously demonstrated for glutamate transporters in cortical synaptosome preparatations, our findings suggest a scenario in which oxidative stress leads to the production of HNE, impairment of gluatmate transport, and excitotoxic motor neuron degeneration in ALS.
Imaging epileptogenic tubers in children with tuberous sclerosis complex usingα‐[11C]Methyl‐L‐tryptophan positron emission tomography
Authors: Chugani Diane C. , Chugani Harry T. , Muzik Otto , Shah Jagdish R. , Shah Aashit K. , Canady Alexa , Mangner Thomas J. , Chakraborty Pulak K. 
Published: January 2005

AbstractSeveral reports have indicated that cortical resection is effective in alleviating intractable epilepsy in children with tuberous sclerosis complex (TSC). Because of the multitude of cortical lesions, however, identifying the epileptogenic tuber(s) is difficult and often requires invaise intracranial electroencephalographic (EEG) monitoring. As increased concentrations of serotonin and serotonin‐immunoreactive processes have been reported in resected human epileptic cortex, we used α‐[11C]methyl‐L‐tryptophan ([11C]AMT) position emission tomography (PET) to test the hypothesis that serotonin synthesis is increased interictally in epileptogenic tubers in patients with TSC. Nine children with TSC and epilepsy, aged 1 to 9 years (mean, 4 years 1 month), were studied. All children underwent scalp video‐EEG monitoring, PET scans of glucose metabolism and serotonin synthesis, and EEG monitoring during both PET studies. [11C]AMT scans were coregistred with magnetic resonance imaging and with glucose metabolism scans. Whereas glucose metabolism PET showed multifocal cortical hypometabolism corresponding to the locations of tubers in all 9 children, [11C]AMT uptake was increased in one tuber (n = 3), two tubers (n = 3), three tubers (n = 1), and four tubers (n = 1) in 8 of the 9 children. All other tubers showed decreased [11C]AMT uptake. Ictal EEG data available in 8 children showed seizure onset corresponding to foci of increased [11C]AMT uptake in 4 children (including 2 with intracranial EEG recordings). In 2 children, ictal EEG was nonlocalizing, and in 1 child there was discordance between the region of increased [11C]AMT uptake and the region of ictal onset on EEG. The only child whose [11C]AMT scan showed to no regions of increased uptake had a left frontal seizure focus on EEG; however, at the time of his [11C]AMT PET scan, his seizures had come under control. [11C]AMT PET may be a powerful tool in differentiating between epileptogenic and nonepileptogenic tubers in patients with TSC.
β‐Trace protein in cerebrospinal fluid: A blood–CSF barrier–related evaluation in neurological diseases
Authors: Tumanin Hayrettin , Nau Roland , Felgenhauer Klaus 
Published: January 2005

Abstractβ‐Trace protein concentrations in cerebrospinal fluid (CSF) and serum from 113 patients with variuos neurological diseases and 65 controls were determined with a sensitive and specific immunonephelometric assay. In adult control patients, β‐trace concentrations were 14.6 ± 4.6 mg/L in CSF and 0.46 ± 0.13 mg/L in serum, that is 32‐fold higher in CSF. β‐trace levels in CSF correlated with age as well as with the albumin CSF/serum ratio (QA1b), which is considered a measure for blood‐CSF barrier function. The relationship between CSF β‐trace levels and elevated QA1b values was studied in various neurological diseases with CSF protein increase. In spinal canal stenosis, CSF β‐trace (mean = 29.5 ± 10.5 mg/L) correlated positively with increasing QA1b values. In bacterial meningitis, CSF β‐trace (mean = 8.7 ± 3.9 mg/L) remained invariant to changes of QA1b values. In Guillain‐Barre syndrome, CSF β‐trace (mean = 14.4 ± 6.8 mg/L) was below the QA1b‐dependent reference range. In multiple sclerosis and viral meningoencephalitis, β‐trace levels were within the reference range. β‐Trace concentration in CSF can be used in conjunction with QA1b to distinguish between different neurological pathologies associated with CSF protein increase.
Risk of unprovoked seizure after acute symptomatic seizure: Effect of status epilepticus
Authors: Hesdorffer Dale C. , Logroscino Giancarlo , Cascino Gregory , Annegers John F. , Hauser W. Allen 
Published: January 2005

AbstractWe asked whether acute symptomatic status epilepticus (SE) increases the risk for subsequent unprovoked seizure compared with less prolonged acute symptomatic seizure. We also explored whether the risk of unprovoked seizure differs by cause. We ascertained all first episodes of acute symptomatic seizure among residents of Rochester, Minnesota, through the Rochester Project's records‐linkage system. Information was collected on seizure duration, age, sex, cause, and subsequent unprovoked seizure. At 10 years of follow‐up, the risk of unprovoked seizure was 41% for those with acute symptomatic seizure with SE and 13% for those without SE. Controlling for age, sex, and cause, SE increased the risk for subsequent unprovoked seizure 3.3‐fold (95% confidence interval, 1.8–6.1) compared with brief acute symptomatic seizures. Among patients with SE, the risk of unprovoked seizure was increased 18.8‐fold for patients with anoxic encephalopathy, 7.1‐fold for patients with a structural cause, 3.6‐fold for patients with a metabolic cause. The increased risk for unprovoked seizure after SE compared with shorter seizures may be due to SE being a marker for severity of injury, damage caused by SE, or a biological substrate associated with the tendency to experience SE.
The mitochondrial DNA A8344G mutation in leigh syndrome revealed by analysis in paraffin‐embedded sections: Revisiting the past
Authors: Santorelli F. M. , Tanji K. , Shanske S. , Krishna S. , Schmidt R. E. , Greenwood R. S. , DiMauro S. , de Vivo D. C. 
Published: January 2005

AbstractIn 1975, we presented the results of a study on a family with a constellation of features that included a chronic spinocerebellar syndrome, neuropathologically proven Leigh syndrome, and sudden death in infancy or childhood affecting several members over three generations. Inheritance was thought to be autosomal dominant. Twenty years later, we reinterpreted the inheritance pattern as maternal. Mitochondrial DNA (mtDNA) extracted from paraffin‐embedded brain samples from the proband revealed the A8344G myoclonic epilepsy and ragged‐red fiber (MERRF) mutation as the molecular basis for this multifaceted neurological syndrome. This re‐evaluation of archival material is an instructive example of “medical archeopathology”.
Utility of anti‐Hu antibodies in the diagnosis of paraneoplastic sensory neuropathy
Authors: Molinuevo Jose Luis , Graus Francesc , Serrano Carmen , Reñe Ramón , Guerrero Antonio , Illa Isabel 
Published: January 2005

AbstractAnti‐Hu antibodies (Hu‐Abs) were positive in 40 patients with paraneoplastic sensory neuropathy (PSN) and in 1 patient with idiopathic sensory neuropathy in a series of 126 patients who presented with clinical features suggestive of PSN. The specificity of Hu‐Abs was 99% and the sensitivity was 82%. Nine (18%) PSN patients were Hu‐Ab–negative, and their sera did not harbor other specific anti‐neuronal of anti‐ganglioside antibodies. Small cell lung carcinoma (SCLC) was the leading neoplasm in the Hu‐Ab–positive (79%) and Hu‐Ab–negative (44%) groups. This study confirms the value of Hu‐Abs for the diagnosis of PSN and SCLC and also emphasizes that in patients with possible PSN, the absence of Hu‐Abs does not exclude cancer, particularly in those patients with risk factors for SCLC.
Orbital shaker technology for the cultivation of mammalian cells in suspension
Authors: Muller Natalie , Girard Philippe , Hacker David L. , Jordan Martin , Wurm Florian M. 
Published: January 2005

AbstractFor large‐scale applications in biotechnology, cultivation of mammalian cells in suspension is an essential prerequisite. Typically, suspension cultures are grown in glass spinner flasks filled to less than 50% of the nominal volume. We propose a superior system for suspension cultures of mammalian cells based on orbital shaker technology. We found that “square‐shaped” bottles (square bottles) provide an inexpensive but efficient means to grow HEK‐293 EBNA and CHO‐DG44 cells to high density. Cultures in agitated 1‐L square bottles exceeded the performance of cultures in spinner flasks, reaching densities up to 7 × 106 cells/mL for HEK‐293 EBNA cells and 5 × 106 cells/mL for CHO‐DG44 cells in comparison to (2.5–4) × 106 cells/mL for cultures of the same cells grown in spinner flasks. For 1‐L square bottles, optimal cell growth and viability were observed with a filling volume of 30–40% of the nominal volume and an agitation speed of 130 rpm at a rotational diameter of 2.5 cm. Transient reporter gene expression following gene delivery by calcium phosphate–DNA co‐precipitation was the same or slightly better for HEK‐293 EBNA cells grown in square bottles as compared to spinner flasks. Reductions in cost, simplified handling, and better performance in cell growth and viability make the agitated square bottle a new and very promising tool for the cultivation of mammalian cells in suspension. © 2004 Wiley Periodicals, Inc.
Experimental innervation of muscles by the central ends of afferent nerves (establishment of a one‐neurone connection between receptor and effector organ), with functional tests
Authors: Weiss Paul 
Published: January 2005

AbstractIn adult toads the central end of the ninth or tenth dorsal root was disnonnected from the spinal cord and inserted instead into a denervated muscle which has previously been tranplated to the back. In this way a direct connection between peripheral sense organs and muscle fibers has been achieved by a single sensory neurone (‘mono‐neuronal’ connecttion), eliminating the central nervous system as intermediary between receptor and effector organs.
The cortical area concerned with coordinated walking in the rat
Authors: Maier Norman R. F. 
Published: January 2005

AbstractCortical lesions in the rat do not produce paralysis, but they may greatly handicap its performance when running on narrow poles. To determine whether or not this handicap is due to the loss of some particular area, sixy‐six rats were subjected to various patterns of lesions and then tested to determine their skill on narrow poles. The performance of eacht rat was rated with 0 (no handicap), 1, 2, or 3, according to the degree of diability in walking on the poles.
Electroencephalogram of the chicken recorded from the skull under various conditions
Authors: Ookawa Takanori , Gotoh Jiro 
Published: January 2005

AbstractElectroencephalographic studies were carried out on chickens to determine the normal electroencephalogram (EEC) patterns from wakefulness to sleep and the effect of anesthesia. Sixteen electrodes were fixed to the frontal and parietal bones. EEC was recorded under unstressed condition.In the excited state, fast waves of 30 to 60 cps and of low amplitude were predominant. In the resting period, these waves shifted to slower ones of 14 to 24 cps, the amplitude remaining low as in the excited state. As the bird fell asleep, three main stages could be distinguished according to the correlation between the EEC patterns and behavioral states. Under ether anesthesia, four stages could be distinguished according to the EEC patterns and the responsiveness to afferent stimuli. By increasing the dose of nembutal, the correlation between the EEC pattern and the depth of anesthesia was studied.The “spindle burst”, or “barbiturate burst,” and the “fast wave” shown in mammals by the injection of barbiturates were not observed in the chicken.
An autoradiographic study on the origin and fate of the sub‐pial glioblast in the embryonic chick spinal cord
Authors: Fujita Setsuya 
Published: January 2005

AbstractGliogenesis in the spinal cord was studied in chick embryos ranging in age from 3 to 14 days of incubation using H3‐thymidine autoradiography. It has been reported by the author that the neuron production of the spinal cord begins on the third day and ends at the eighth day of incubation. At eight days of incubation glioblasts with small dark round nuclei first appear in the sub‐pial region. Using the selective labeling technique with H3‐thymidine the author demonstrated that no transition exists from pial cells to sub‐pial glioblasts. The glioblasts are produced from the matrix cells by their migration and transformation. These glioblasts mature into neuroglia (oligodendroglia and astrocytes), as development proceeds. The suggestion that the stage of neuroglia differentiation follows that of neuron production in development of the central nervous system has been reconfirmed.