bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and genetic evaluation of a family with a mixed dystonia phenotype from south tyrol
Authors: Klein Christine , Pramstaller Peter P. , Castellan Claudio C. , Breakefield Xandra O. , Kramer Patricia L. , Ozelius Laurie J. 
Published: January 2005

AbstractThe gene causing early‐onset torsion dystonia (DYT1) has recently been identified, and two new dystonia genes, one for adult‐onset focal dystonia (DYT7) and one for a mixed dystonia phenotype (DYT6), have been mapped. We evaluated clinically a family from South Tyrol (Northern Italy) with 6 definitely affected individuals who display an unusually large phenotypic range of dystonic symptoms. We excluded the GAG deletion in the DYT1 gene and linkage to any of the above‐mentioned dystonia loci, thus suggesting an as yet undefined dystonia gene in our family.
Gene therapy with HSP72 is neuroprotective in rat models of stroke and epilepsy
Authors: Yenari Midori A. , Fink Sheri L. , Sun Guo Hua , Chang Louis K. , Patel Maitrya K. , Kunis David M. , Onley David , Ho Dora Y. , Sapolsky Robert M. , Steinbrg Gary K. 
Published: January 2005

AbstractBrain areas damaged by stroke and seizures express high levels of the 27‐kd heat shock protein (HSP72). Whether HSP72 represents merely a marker of stress or plays a role in improving neuron survival in these cases has been debated. Some induced tolerance experiments have provided correlative evidence for a neuroprotective effect, and others have documented neuroprotection in the absence of HSP72 synthesis. We report that gene transfer therapy with defective herpes simplex virus vectors overexpressing hsp 72 improves neuron survival against focal cerebral ischemia and systemic kainic acid administration. HSP72 overexpression improved striatal neuron survival from 62.3 to 95.4% in rats subjected to 1 hour of middle cerebral artery occlusion, and improved survival of hippocampal dentate gyrus neurons after systemic kainic acid administration, from 21.9 to 64.4%. We conclude that HSP72 may participate in processes that enhance neuron survival during transient focal cerebral ischemia and excitotoxin‐induced seizures.
Posterior vermal split syndrome
Authors: Bastian Amy J. , Mink Jonathan W. , Kaufman Bruce A. , Thach W. Thomas 
Published: January 2005

AbstractWe have studied a battery of movements in 5 children (age, 6–15 years) after transection of the posterior inferior cerebellar vermis. In each case, the surgery destroyed the midline vermis only (ranging from lobules VI–X). Tandem gait was badly impaired in all subjects. No subjects had impairments of kicking, reaching, pinching, or speech. Regular gait, standing, and hopping on one leg were relatively unimpaired. Cutting the parallel fibers that cross the midline may be the critical variable causing incoordination in tandem gait.
Amelioration of flulike symptoms at the onset of interferon β‐ 1b therapy in multiple sclerosis by low‐dose oral steroids is related to a decrease in interleukin‐6 induction
Authors: MartíNez‐CáCeres E. M. , Río J. , Barrau M. , Durán I. , Borrás C. , Tintoré M. , Montalban X. 
Published: January 2005

AbstractLow‐dose oral steroid use at the onset of interferon β‐1b (IFNβ‐1b) therapy in relapsing‐remitting multiple sclerosis (RR‐MS) patients reduces flulike symptoms. To determine the mechanism by which steroid treatment minimizes these side effects, we analyzed the percentage of interleukin‐6 (IL‐6)–, interferon‐γ (IFN‐γ)‐, tumor necrosis factor α (TNF‐α)–, and IL‐10–producing cells before and after 3 months of IFNβ‐1b therapy onset. Our results support a relationship between IL‐6 induction and fever. Such side effects can be ameliorated by steroids.
Malignant glioma: Who benefits from adjuvant chemotherapy?
Authors: Deangelis Lisa M. , Burger Peter C. , Green Sylvan B. , Cairncross J. Gregory 
Published: January 2005

AbstractWe combined two randomized prospective Brain Tumor Study Group data sets to analyze the effects of prognostic factors on survival by treatment group. Adjuvant chemotherapy increased long‐term survival regardless of prognostic factors. Pathological review revealed that oligodendrogliomas were overrepresented among long‐term survivors independent of therapy. Prognostic factors do not predict benefit from adjuvant nitrosourea in malignant gliomas, and long‐term survival with chemotherapy is not explained by oligodendroglial tumors.
Central nervous system nitric oxide formation in cerebral systemic lupus erythematosus
Authors: Brundin L. , Svenungsson E. , Morcos E. , Andersson M. , Olsson T. , Lundberg I. , Wiklund N. P. 
Published: January 2005

AbstractSystemic lupus erythematosus (SLE) is an inflammatory disease in which up to two thirds of the patients present neurological symptoms. The diagnosis of the disease is based on clinical findings and the presence of autoantibodies, and the pathogenesis is unclear. The purpose of this study was to determine if the pathogenesis was partly mediated via nitric oxide (NO) formation. Cerebrospinal fluid (CSF) samples from 15 patients with cerebral SLE were analyzed for the NO metabolites nitrite and nitrate using capillary electrophoresis. The severity of neurological symptoms was scored by dividing the patients into two groups with either mild or moderate/severe CNS involvement. All patients with cerebral SLE showed increased levels of NO metabolites. In CSF, there was a relationship between signs of NO production and clinical results showing that increased levels of nitrite and nitrate were associated with more severe neurological symptoms. These findings may shed new light on the pathogenesis of cerebral SLE, and analysis of nitrate and nitrate may prove to be of value in monitoring the activity of the disease.
Familial partial epilepsy with variable foci: A new partial epilepsy syndrome with suggestion of linkage to chromosome 2
Authors: Scheffer Ingrid E. , Phillips Hilary A. , O'Brien Catherine E. , Saling Michael M. , Wrennall Jacqueline A. , Wallace Robyn H. , Mulley John C. , Berkovic Samuel F. 
Published: January 2005

AbstractFamilial partial epilepsy with variable foci (FPEVF) joins the recently recognized group of inherited partial epilepsies. We describe an Australian family with 10 individuals with partial seizures over four generations. Detailed electroclinical studies were performed on all affected and 17 clinically unaffected family members. The striking finding was that the clinical features of the seizures and interictal electroencephalographic foci differed among family members and included frontal, temporal, occipital, and centroparietal seizures. Mean age of seizure onset was 13 years (range, 0.75–43 years). Two individuals without seizures had epileptiform abnormalities on electroencephalographic studies. Penetrance of seizures was 62%. A genome‐wide search failed to demonstrate definitive linkage, but a suggestion of linkage was found on chromosome 2q with a LOD score of 2.74 at recombination fraction of zero with the marker D2S133. FPEVF differs from the other inherited partial epilepsies where partial seizures in different family members are clinically similar. The inherited nature of this new syndrome may be overlooked because of relatively low penetrance and because of the variability in age at onset and electroclinical features between affected family members.
Vigabatrin increases human brain homocarnosine and improves seizure control
Authors: Petroff Ognen A. C. , Mattson Richard H. , Behar Kevin L. , Hyder Fahmeed , Rothman Douglas L. 
Published: January 2005

AbstractHomocarnosine, a dipeptide of γ‐aminobutyric acid (GABA) and histidine, is thought to be an inhibitory neuromodulator synthesized in subclasses of GABAergic neurons. Homocarnosine is present in human brain in greater amounts (0.4–1.0 μmol/g) than in other animals. The antiepileptic drug vigabatrin increases human cerebrospinal fluid homocarnosine linearly with daily dose. By using 1H nuclear magnetic resonance spectroscopy, serial occipital lobe GABA and homocarnosine concentrations were measured in 11 patients started on vigabatrin. Daily low‐dose (2 g) vigabatrin increased both homocarnosine and GABA. Larger doses of vigabatrin (4 g) further increased homocarnosine but changed GABA levels minimally. Seizure control improved with increasing homocarnosine and GABA concentrations. Patients whose seizure control improved with the addition of vigabatrin had higher mean homocarnosine, but the same mean GABA concentrations, than those whose seizure control did not improve. Increased homocarnosine may contribute to improved seizure control.
Bilateral high‐frequency stimulation of the internal globus pallidus in advanced Parkinson's disease
Authors: Volkmann J. , Sturm V. , Weiss P. , Kappler J. , Voges J. , Koulousakis A. , Lehrke R. , Hefter H. , Freund H.‐J. 
Published: January 2005

AbstractWe report here the results of an open prospective study in 9 patients suffering from severe Parkinson's disease with on/off fluctuations and restricted off‐period mobility, who underwent bilateral implantation of stimulating electrodes in the internal pallidum. At 3‐month follow‐up, the total Unified Parkinson's Disease Rating Scale (UPDRS) motor score in the medication‐off state was reduced from 54.1 ± 14.8 to 23.9 ± 11.7 (44.2%) when stimulation was turned on. Comparison of UPDRS subscores revealed significant improvements for tremor, rigidity, bradykinesia, gait and posture, and dyskinesias. The results of the clinical scoring could be confirmed by significant changes in the quantitative assessment of hand function and walking. Bilateral pallidal stimulation reduced the amount and severity of on/off fluctuations. Additional follow‐up at 6 months (n = 6), 9 months (n = 6), and 12 months (n = 4) did not show a decline in effectiveness of stimulation. There was no permanent morbidity associated with the procedure. A subtle reduction of verbal fluency, which was not evident to the patients, was the only cognitive side effect of the procedure in neuropsychological testing. Chronic bilateral high‐frequency stimulation of the internal pallidum seems to be a neurologically safe and highly effective treatment for “off” symptoms, dyskinesias, and motor fluctuations in advanced stages of Parkinson's disease.
A dystrophin missense mutation showing persistence of dystrophin and dystrophin‐associated proteins yet a severe phenotype
Authors: Goldberg Laura R. , Hausmanowa‐Petrusewicz Irena , Fidzianska Anna , Duggan David J. , Steinberg Lisa S. , Hoffman Eric P. 
Published: January 2005

AbstractA muscle biopsy from an X‐linked muscular dystrophy pedigree showed normal dystrophin and dystrophinassociated proteins. Linkage to multiple markers within the dystrophin gene (LOD = 2.7, ⊖ = O) indicated a primary dystrophinopathy. Sequencing of the entire dystrophin RNA revealed a single missense mutation (D3335H) in the unique carboxyl‐terminal domain. This is the first report showing that a relatively severe dystrophinopathy can occur despite the correct localization of dystrophin and dystrophin‐associated proteins.
The development of the pillar cells, tunnel space, and Nuel's spaces in the organ of Corti
Authors: van der Stricht O. 
Published: January 2005

AbstractThe tunnel space is developed around the spiral nerve bundle, which runs between the nucleated portions of the inner and outer pillar cells. It is originally an intercellular cleft, the fluid contents of which are elaborated in the vacuolated cyto plasm of the pillar cells and discharged into the adjoining space. Parts of this secreting protoplasm undergo a process of cytolysis or liquefaction, so that the cleft enlarges and the fluid contents increase in quantity at their expense.
Studies of living nerves. III. Phenomena of nerve irritation and recovery, degeneration and repair
Authors: Speidel Carl Caskey 
Published: January 2005

AbstractThe reaction of single internodal segments of myelinated nerve fibers of living frog tadoples have been observed in minute detail, following many varieties of experimental irritation of both acute and chronic types. The changes that characterize nerve irritation bear a marked resemblance to the early changes that characterize tgrophic (wallerian) de generation after nerve section.
Localization of the terminals of the tongue afferents in the nucleus of the solitary tract
Authors: Blomquist A. J. , Antem A. 
Published: January 2005

AbstractThe nerves innervating the tongue were stimulated electrically and multineuron responses were recorded in the nucleus of the solitary tract. Stimulation of the chorda tympani and the IXth nerves evoked responses in the lateral and medial ipsilateral solitary nucleus in a rostral‐caudal order. Responses to lingual nerve stimulation were found only in the lateral, larger‐celled portion of the ipsilateral solitary nucleus overlapping the chorda tympani and the IXth nerve areas. Stimulation of the above three nerves also evoked somewhat longer latency responses in the ambiguus nucleus. Responses to lingual nerve stimulation were found in the XIIth nerve motor nucleus and the solitary nucleus at the level of the obex using high voltage levels of stimulation. The results were discussed in terms of the anatomical discreteness of the relays of the gustatory and the other tongue modalities in the solitary nucleus. These results were obtained in the white rats.
Post‐natal growth of neuroglia cells and blood vessels of the cervical spinal cord of the albino mouse
Authors: Sakla Francois B. 
Published: January 2005

AbstractThe post‐natal growth of three types of neuroglia cells viz. Astroglia, oligodendroglia and microglia cells and the growth of the blood vessels of the cervical spinal cord of the albino mouse were studied at the following ages: 1, 10; 20, 30 and 120 days in preparations stained with: Golgi‐Cox, Rio‐Hortega and Gallocyanin stains.Their total number was doubled in the first ten days as a result of mitoses in the ependymal cells of the central canal forming new astroglia and oligodendroglia cells in both the gray and white matter and mitoses in the cells of the pia‐mater which form new microglia cells. After the tenth day their total number became fixed and thus they were dispersed in the cord until the 120th day. A correlation was established between the increase in the number of oligodendroglia cells and myelination.The total number of cells forming the wall of the central canal was estimated.In mice successfully injected with China ink the blood vessels in the gray and white matter respectively were measured. Their total length and total volume were estimated. A correlation between the vascularization of the cord and the process of myelination, growth of the nerve cells and neuroglia cells was established.
The forebrain of the guinea pig in stereotaxic coordinates
Authors: Tindal J. S. 
Published: January 2005

AbstractA stereotaxic atlas has been prepared of the forebrain of the guinea pig (body weight 700–830 g) using a standard cat‐monkey stereotaxic instrument. The coordinates used were the vertical interaural plane (APO) and the horizontal orbital‐interaural plane (HO). The atlas consists of transverse sections of the brain taken at 0.5 mm intervals from A5 through A15.
Geniculo‐striate connections in the rabbit. II. Cytoarchitectonic structure of the striate region and of the dorsal lateral geniculate body; organization of the geniculo‐striate projections
Authors: Rose Jerzy E. , Malis Leonard I. 
Published: January 2005

AbstractRetrograde changes in the dorsal lateral geniculate body were studied following cortical radiation lesios.

The cytoarchitectonic structure of the striate region is described. The peristriate as well as the striate areas are shown to receive projections form the dorsal lateral geniculate body. Thus — as far as this set of connections is concerned — the peristriate are is a subdivision of the striate region. It is further shown that the striate region as defined cytoarchitectonically is —to the extent that it was validated — coextensive with the cortical projection area of the dorsal lateral geniculate body.
The dorsal lateral geniculate body is divided into an alpha and a beta sector. Evidence indicates that each vertical column of cells in the striate region receives projections from an appropriate geniculate column which extends throughout the thickness of the alpha sector. The alpha cell columns project upon the striate region in an orderly manner. Details of this organization are described.
The cells of the beta sector also project upon the striate region. Whether the pattern of this projection is similar to that of the alpha sector or is more generalized has not been established.
Some electrophysiological and experimental‐anatomical evidence relevant to the findings is discussed.
Cellular localization of monoamines in the upper brain stem of the pigeon
Authors: Fuxe Kjell , Ljunggren Lars 
Published: January 2005

AbstractWith the help of a highly sensitive and specific fluorescence method developed by Falck and Hillarp, in combination with a pharmacological approach, it has been possible to demonstrate the presence in birds of specific Neurons of two distinctly different types, which in all probability form and store a primary catcholamine and 5‐hydroxytryptamine respectively. The amines are in low concentrations in the cell bodies and axons but stored in very high concentrations in the terminals, especially in the abundant varicosities. At least three large ascending monoamine systems appear to be present within the upper brain stem. Many — if not most — of the ascending fibers arise in all probability from cell bodies within the mesencephalon and run rostrally mainly in the medial forebrain bundle. Two of the Neuron systems produce and store a primary catecholamine, one probably giving rise mainly to terminals within the hypothalamus and the preoptic area, the other within the corpus striatum. The remaining system produces and stores 5‐hydroxytryptamine.The modified ependyma seems to consist — at least partly — of two types of cells forming and storing either a primary catecholamine or 5‐hydroxytryptamine.
The pyramidal tract of tupaia, compared to that in other primates
Authors: Verhaart W. J. C. 
Published: January 2005

AbstractThe pyramidal tract of Prosimii proves to lack coarse fibers and to be much smaller than in Macaca, whereas in Saimiri it resembles the latter very much. Crossing and course within the cord in the Prosimii examined are identical to those in Macaca and Man. In Tupaia the pyramidal tract is very different, as it is very small, crosses to the dorsal funiculus and ends in the lower theoracic levels.The other large tracts descending into the cord seem to enlarge where the pyramidal tract decreases in size and length in Tupaia. The large difference between the pyramidal tract of Tupaia and that of other Prosimii is emphasized.
Contribution to the quantitative study of the nervous tissue. A new method for measurement of the volume and surface area of neurons
Authors: Mannen Hajime 
Published: January 2005

AbstractIn our histological analysis of the brain stem of the kitten with the Golgi‐Cox method, it was found that the application of reflecting illumination provides a clear outline of the soma surface including its relationship with the dendrites. Utilizing this method of illumination it was possible to develop a new approach for following the total course of dendrites in successive serial sections and a method for the photogrammetrical representation of neuron soma by means of microscopic stereophotograms. This has provided a means for estimating precisely the dimensions of the neuron.Five cells of the magnocellular nucleus of bulbar and pontine reticular formation have been measured as test cases by means of this new method. The following results were obtained: the mean area and volume of the neuron soma obtained by means of photogrammetry are 13,580μ2 and 109,820μ3 respectively; the area and volume of the entire dendritic portion of the neuron are 66,640 μ2 and 70,060 μ3 respectively. In the case of serial sections 250 μ thick, about 25% of the dendrites are lost in the neighboring sections with respect to length, and 16% to 14% with respect to area and volume. Thus the ratios of the dendritic area to the soma area and that of the dendritic volume to the soma volume average 5.1 and 0.6 respectively.
Lumbosacral dorsal root terminations in the nucleus gracilis of the cat. Some observations on terminal degeneration in other medullary sensory nuclei
Authors: Hand Peter J. 
Published: January 2005

AbstractFollowing lumbosacral dorsal rhizotomies (L1, L5, L6 and S1), fiber degeneration could be traced throughout three rostro‐caudal regions of nucleus gracilis by utilizing the Nauta method. In rostral region, fiber degeneration was less dense and localized with greater intersegmental overlap than in middle region, where roots terminated in fairly specific cell clusters. Little could be stated about the caudal region because of sparsity of terminal degeneration. Fiber degeneration in rostral region appeared to be distributed primarily in the dendritic fields while that in the middle region terminated on both cell somata and dendrites. Overlap of dermatomes in periphery and centrally in nucleus parallel each other. Throughout the nucleus, greatest overlap of root distribution was encountered in more ventral regions. A root supplying a more distal dermatome (e.g. L6) compared to a more proximal one (e.g. L1), terminates more densely and dorsally in the nucleus. An occasional degenerated fiber passed into the contralateral gracile nucleus at obex level. Root degeneration proceeded, ipsilaterally, into rostro‐dorsal regions of lateral cuneate, cuneate, and caudo‐lateral regions of 2 nuclei. Bilateral degeneration from all roots, excepting S1, was noted in rostral pole of subnucleus caudalis and the subnucleus interpolaris of spinal trigeminal complex and nucleus reticularis parvicellularis. The pathway to the spinal trigeminal and parvicellular nuclei was undetermined. The concept of the non‐homogenous organization of the gracile nucleus was supported.
The organization of the posterior ventral cochlear nucleus in the rat
Authors: Harrison J. M. , Irving R. 
Published: January 2005

AbstractThe ventral cochlear nucleus was examined in 31 rats' brains prepared according to the protargol method of Bodian. The following regions were delimited on the basis of synaptic, cellular and axonal criteria.Region II, which occupies the central part of the nucleus, consists principally of cells (type g)which receive modified bulbs of Held from the acoustic nerve and which send large axons to the trapezoid body. The cells were graded in size, the smallest (high frequency) being located dorsally and the larges (low frequency) being located ventrally. Associated with region II are the cells (type b) of the acoustic nerve nucleus. These cells receive boutons from the acoustic nerve and send very large axons to the trapezoid body.Region IV is located in the posterior part of the ventral cochlear nucleus and is composed of large multipolar cells (type k). The dendrites of these cells are specifically organized with respect to the fibers of the acoustic nerve. Synaptic endings, from the descending branch of the acoustic nerve, consist of boutons on cell body and dendrites. The axons of these cells compose the stria of Held.Region V is located in the posterior part of the ventral cochlear nucleus and appeared to have only intrinsic connections.All regions received synaptic endings in addition to those which arose from the acoustic nerve.