bluuebunny/crossref_metadata_2025_split huggingface dataset:
A subgroup of multiple sclerosis patients with anticardiolipin antibodies and unusual clinical manifestations: Do they represent a new nosological entity?
Authors: Karussis Dimitrios , Leker Ronen R. , Ashekenazi Avi , Abramsky Oded 
Published: January 2005

AbstractThe presence of antibodies to cardiolipin (ACL Abs) has been reported in some patients suffering from multiple sclerosis (MS), especially of the “neuromyelitic” type. In addition, bright T2‐imaging foci (unidentified bright objects) are occasionally detected on brain magnetic resonance imaging (MRI) scans, in patients with antiphospholipid syndrome. From a cohort of 100 patients with a probable or definite diagnosis of MS according to Poser's criteria, we isolated a subgroup of 20 patients (8 males and 12 females) consistently positive for ACL Abs. These patients were followed up neurologically for 1 to 3 years and brain MRI scanning and a complete autoimmune screening were performed. Nineteen (19 of 20) of our patients had the classic neuroimaging features of MS (multiple white‐matter T2 bright foci on the MRI scan). The most common neurological syndrome was chronic, slowly progressing myelopathy (presenting as myelopathy, neuromyelitis optica, or spinocerebellar syndrome; 15 of 20), and optic neuropathy (6 of 20). Headache was a dominant symptom in 8 of 20 patients. Less common symptoms included cognitive and psychiatric disorders and chronic fatigue. The mean levels of ACL Abs were 38.8 ± 28.2 GPL (normal values up to 7.5). Oligoclonal bands in the cerebrospinal fluid were detected in only 3 of 20 patients. Patients were treated with acetylsalycilic acid and occasionally with short courses of steroids. The progression of the chronic myelopathic/spinocerebellar syndrome was slower than expected in MS (only 2 patients deteriorated whereas 4 improved during a mean follow‐up period of 20.8 ± 7.1 months). We conclude that patients with probable or definite diagnosis of MS, and consistently elevated levels of ACL Abs show a slower progression and some atypical (for MS) features, such as persistent headaches and absence of oligoclonal bands in the cerebrospinal fluid. In these patients, other, presumably vascular, mechanisms may be involved in the pathogenesis of the neurological symptoms. Therefore, management should include antiplatelet or even anticoagulant agents.
Presence of 4‐hydroxynonenal in cerebrospinal fluid of patients with sporadic amyotrophic lateral sclerosis
Authors: Smith R. Glenn , Henry Y. Kay , Mattson Mark P. , Appel Stanley H. 
Published: January 2005

AbstractA marker of lipid peroxidation 4‐hydroxynonenal (HNE) was elevated in the cerebrospinal fluid (CSF) of a patient with sporadic amyotrophic lateral sclerosis (sALS) compared with that of most patients with other neurological diseases. Such elevations of HNE were sufficient to kill cyclic adenosine monophosphate (cAMP)‐differentiated motor neuron hybrid cells in vitro, and anti‐oxidants prevented this HNE‐dependent cell death. These data suggest that oxidative stress and lipid peroxidation are associated with and may promote motor neuron degeneration in sALS.
Clinical approach to inherited peroxisomal disorders: A series of 27 patients
Authors: Baumgartner M. R. , Poll‐The B. T. , Verhoeven N. M. , Jakobs C. , Espeel M. , Roels F. , Rabier D. , Levade T. , Rolland M. O. , Martinez M. , Wanders R. J. A. , Saudubray J. M. 
Published: January 2005

AbstractTo illustrate the clinical and biochemical heterogeneity of peroxisomal disorders, we report our experience with 27 patients seen personally between 1982 and 1997. Twenty patients presented with a phenotype corresponding either to Zellweger syndrome, neonatal adrenoleukodystrophy, or infantile Refsum disease, 3 of whom had a peroxisomal disorder due to a single enzyme defect. One patient had a mild form of rhizomelic chondrodysplasia punctata, 1 had classic Refsum disease. Finally, 5 patients presented with clinical manifestations that were either unusually mild or completely atypical, and initially did not arouse suspicion of a peroxisomal disorder. They showed multiple defects of peroxisomal functions with one or several functions remaining intact, suggesting a peroxisome biogenesis disorder. The defect in peroxisome biogenesis was further characterized by variable expression in different tissues and/or individual cells in 5 patients. Studies restricted to fibroblasts failed to identify abnormalities in this group. We demonstrate that clinical manifestations of peroxisomal disorders may be very mild or completely atypical, and therefore, peroxisomal disorders should be considered in a variety of clinical settings. Furthermore, we suggest performing extensive peroxisomal investigations in every patient suspected of suffering from a peroxisomal disorder, even when the clinical presentation is typical.
Genetic contribution of the tumor necrosis factor region in guillain‐barré syndrome
Authors: Ma J. J. , Nishimura M. , Mine H. , Kuroki S. , Nukina M. , Ohta M. , Saji H. , Obayashi H. , Kawakami H. , Saida T. , Uchiyama T. 
Published: January 2005

AbstractWe studied genetic polymorphisms in the tumor necrosis factor (TNF) region in 81 Japanese patients with Guillain‐Barré syndrome (GBS) and 85 controls. A significantly higher frequency of the 100–base pair (bp) (TNFa2) allele of the TNFa microsatellite marker, which is associated with high TNFα production, existed in Campylobacter jejuni‐positive (Cj+) GBS patients than in controls, suggesting the involvement of a genetic predisposition to high TNFα secretion in the development of c. jejuni‐related GBS.
Seasonal variation of interferon‐γ production in progressive multiple sclerosis
Authors: Balashov Konstantin E. , Olek Michael J. , Smith Derek R. , Khoury Samia J. , Weiner Howard L. 
Published: January 2005

AbstractMultiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system. There is increased interferon (IFN)‐γ secretion in MS patients in vitro, and IFN‐γ administration induces exacerbations of disease suggesting a link between IFN‐γ and disease activity. We observed significantly increased IFN‐γ production in the autumn and winter months compared with the spring and summer months in chronic progressive MS, and this increase was linked to endogenous interleukin (IL)‐12 production. Increased seasonal IFN‐γ was not observed in normal control subjects, and there were no seasonal changes in IL‐10 in progressive MS. These results suggest a potential environmental link between dysregulated IFN‐γ production and MS disease progression and pathogenesis.
The Glu318Gly mutation of the presenilin‐1 gene does not necessarily cause Alzheimer's disease
Authors: Mattila Kari M. , Forsell Charlotte , Pirttila Tuula , Rinne Juha O. , Lehtimaki Terho , Roytta Matias , Lilius Lena , Eerola Anne , George‐Hyslop Peter H. St , Frey Harry , Lannfelt Lars 
Published: January 2005

AbstractIn early‐onset familial Alzheimer's disease pathogenic mutations have been found in the amyloid precur sor protein (APP) gene and in the presenilin (PS)‐1 and PS‐2 genes. We screened for mutations in these genes in 20 patients with familial AD from the Finnish population. In addition, we sampled 41 sporadic AD patients and 59 controls to test for mutations identified in our familial AD cases. We detected an A‐to‐G transition in the PS‐1 gene, resulting in a glutamic acid (G1u)‐to‐ glycine (Gly) substitution at codon 318 in 2 familial and 2 sporadic AD patients. The Glu318Gly mutation has previously been reported to cause AD. We also found the Glu318Gly mutation in 4 healthy aged controls (range, 74–87 years). We thus conclude that the mutation is most likely a rare polymorphism not related to AD.
Muscle fiber immaturity and inactivity reduce myonecrosis in duchenne muscular dystrophy
Authors: Kimura Shigemi , Sugino Shigeto , Ohtani Yoshinobu , Matsukura Makoto , Nishino Ichizo , Ikezawa Makoto , Sakata Ayako , Kondo Yuichi , Yoshioka Kowashi , Huard Johnny , Nonaka Ikuya , Miike Teruhisa 
Published: January 2005

AbstractWe report on the first case of X‐linked recessive myotubular myopathy (MTM1) coinciding with Duchenne muscular dystrophy (DMD). The muscle biopsy specimens of the patient show only the characteristic findings of MTM1, without the findings of DMD. We theorize that this was caused by the muscle fiber immaturity and inactivity.
The endocranial blood vessels of Amblystoma tigrinum
Authors: Roofe Paul Gibbons 
Published: January 2005

AbstractThe arterial blood supply of the brain of Amblystoma is essentially similar to that of other urodeles so far as these have been described and is comparable in most respects with that of Anura. Individual variations in the courses of even the major arteries are surprisingly great, and the courses of the veins are even more erratic.
The mode of termination and temporal course of degeneration of cortical association pathways in the cerebellum of the cat
Authors: Eager Robert P. 
Published: January 2005

AbstractThe Nauta‐Laidlaw silver degeneration technique was used to study the mode of termination and temporal course of degeneration of cerebellar cortical association pathways in the cat. All associational pathways examined appeared to terminate identically in their respective folia of termination. Two specific cerebellar cortical areas were chosen for detailed discussion and illustration. In the vermis lobules (V and VI), fibers pass from one folium to the adjacent caudal two or three folia, and these pathways can be studied conveniently in the sagittal plane. Long connections extend from anterior lobe paravermal cortex (lobule V) to caudal hemispheral folia of the same side, and these terminations could be examined at a site some distance from the lesion.Projections of associational pathways displayed a rather strict localization, with terminal areas corresponding in size and location to the area of damaged cortex giving rise to the pathway.Terminal degeneration was found distributed at Purkinje cell levels and in basal portions of the molecular layer. Some of the degenerated fibers in the molecular layer showed relationships with Purkinje cell dendrites, suggesting that they might be climbing fibers.Early degenerative changes occurred at two days postoperatively. Maximal fine fiber degeneration occurred at eight days. Fragments of large degenerated fibers were stainable in terminal areas and in folial white matter for as long as 41 days postoperatively.
“Descending” neuronal units in the commissure of the crayfish central nervous system; and their integration of visual, tactile and proprioceptive stimuli
Authors: Wiersma C. A. G. , Mill P. J. 
Published: January 2005

AbstractThe integrative properties of “descending” neuronal units in respect to visual, tactile and proprioceptive stimuli were studied at the commissure level of the crayfish central nervous system and 41 entities were established, bringing the total number of established descending entities at this level to 69. All but three of these are interneurons, over half of which have an entirely homolateral input. Altogether 227 interneurons have so far been established in the crayfish central nervous system, 73 of which extend through more than one of the studied levels. Compared to the thoracic‐abdominal level, interneurons transmitting both anteriorly and posteriorly are very scarce. The properties of high order interneurons are discussed. In addition to those which respond to visual stimuli, or are multimodal, a number react only to mechanorecptive stimulation, and some of this latter type have peripheral sensory fields exclusively posterior to the recording level. A few interneurons, both visual and non‐visual, exibit inhibitory phenomena of various kinds, again mirroring the findings in the mammalian central nervous system. Visual interneurons were shown to respond to specific types of optic stimulation such as movement of objects or changes in light intensity in correspondence with units found in the optic nerves of other decapod crustaceans.
The function of the tectum for attention to auditory stimuli in the cat
Authors: Jane John A. , Masterton R. Bruce , Diamond Irving T. 
Published: January 2005

AbstractCats were trained to avoid a shock by moving from one to the other compartment of a double grill box at the onset of a compound signal which consisted of a soft low tone and a bright flashing light. After training on the compound signal unreinforced test trials were given on the tone alone and the light alone. By this procedure the relative potency of sound and light was established for normal cats and cats deprived of various structures in their auditory system. On the basis of this and other subsidiary experiments also reported here, several conclusions seem warranted:1. In normal cats sound is a prepotent stimulus over light. 2. The prepotency of sound does not depend on the intensities of the competing stimuli. 3. Even with approximately 90% of the second order auditory fibers destroyed, sound is still prepotent over light. 4. Bilateral removal of auditory cortex does not affect the prepotency of sound. 5. Deep bilateral section of the brachia of the inferior colliculus and small bilateral lesions in the apices of the inferior colliculi abolish the prepotency of sound. 6. The loss of the prepotency of sound after midbrain lesions is not the result of a change in absolute threshold for the sound.
The isodendritic core of the brain stem
Authors: Ramón‐Moliner E. , Nauta W. J. H. 
Published: January 2005

AbstractAccording to their degree of morphological specialization, the cell populations of the brain stem may be classified into three groups: isodendritic, allodendritic and idiodendritic. The isodendritic neurons, or generalized neurons, are the most frequently encountered. If one discards those isodendritic centers that by common definition are sensory or motor, an isodendritic core is left which displays very little histological variation throughout the whole extent of the brain stem. This core corresponds, with certain restrictions, to the regions that are usually regarded as reticular formation. It constitutes a continuum of overlapping dendritic fields that extends from the spinal cord to the diencephalon. In view of the similarities that exist between the histology of the isodendritic core and the relatively disorganized nervous system of the lower vertebrates, it is postulated that it represents a pool of pluripotential neurons which in the course of phylogeny have remained relatively undifferentiated and in charge of processing afferent signals of very heterogeneous origin. By contrast, the allodendritic and idiodendritic centers can be regarded as relatively specialized centers from the point of view of their dendritic morphology, connections and functions. Attention is paid to the fact that the diffuse characteristics of the isodendritic core do not necessarily entail ill‐defined physiological properties.
Growth alteration in the auditory cortex of visually deprived mice
Authors: Gyllensten Lars , Malmfors Torbjörn , Norrlin Marie‐Louise 
Published: January 2005

AbstractThe diameter of cell nuclei and mean volume of internuclear material per nucleus were determined in the auditory cortex in growing and adult mice. The influence of stay in complete darkness for 2–4 months was investigated.In growing mice, a decrease in nuclear size and relative volume of internuclear material was observed after two months in the dark from birth. Prolonged stay in the dark caused an increase in nuclear size and internuclear material, and the hypotrophy of the auditory cortex was succeeded by hypertrophy after four months in the dark from birth. In adult mice, reared in darkness for three months from four to seven months of age, similar hypertrophy of the auditory cortex was recorded.Compared to the findings in the striate cortex of visually deprived mice (in a previous investigation), the initial hypotrophy of the auditory cortex after visual deprivation was less pronounced than the hypotrophy of the auditory cortex after visual cortex. In the latter, no late hypertrophy occurred after prolonged stay in the dark, and hypotrophy was found in the adult, visually deprived mice, and not hypertrophy as in the auditory cortex. The possibility of compensatory auditory training and cortical hypertrophy is discussed.
Behavior of neuro‐epithelial cells during closure of the neural tube
Authors: Langman Jan , Guerrant Richard L. , Freeman Bruce G. 
Published: January 2005

AbstractChick embryos ranging in age from 18 to 32 hours were treated with thymidine‐H3 to examine the cell cycle and nuclear migration of the neuro‐epithelial cells during closure of the neural tube.By plotting the percentage of labeled metaphases against the tune of thymidine‐H3 application, the DNA‐synthetic period was found to last five hours, the postduplication (G2) stage and prophase two and one‐half hours and the preduplication (G1) stage to be brief or non‐existent. The total cell cycle time was calculated to be eight hours. During DNA synthesis the nuclei are located near the basement membrane; subsequently they migrate to the lumen to undergo mitosis, after which they return to the outer zone to start DNA synthesis for the next cell division.To determine the life span of the neuro‐epithelial cells by a different method, chick embryos were treated with vincristine, a drug known to arrest the mitosis in the metaphase. Fifteen, 26, 34 and 41% of the neuro‐epithelial cells were arrested in the metaphase, one, two, three and four hours after treatment, respectively. Since the “mitotic index” of the neuro‐epithelial cells was 5.2, the duration of the mitosis was calculated to last 25 minutes, and the life span of the neuro‐epithelial cell to be eight hours.Immediately after closure of the neural tube, a different cell type characterized by a round nucleus, pale nucleoplasm and a dark‐staining nucleolus appeared at the periphery of the tube near the basement membrane. These cells, which originate by division from the neuro‐epithelial cells at the lumen, did not incorporate thymidine‐H3 at any time during further development. They are considered to be primitive neuroblasts and to form the first representatives of the mantle layer.
A comparative study of the reticular formation of the guinea pig
Authors: Petrovicky Pavel 
Published: January 2005

AbstractThe author investigated the reticular formation in the guinea pig, using one sagittal and three frontal series of the brain stem stained according to the method of Nissl. The nuclei of the reticular formation in the guinea pig were identified and mapped.In the second part of the work the author compared the individual nuclei of the reticular formation in nine representatives of five orders of mammals: primates, carnivores, rodents, insectivores and chiroptera. A table illustrates the occurrence of reticular nuclei in the orders studied.
H3‐Thymidine autoradiographic studies on the cell proliferation and differentiation in the external and the internal granular layers of the mouse cerebellum
Authors: Fujita Setsuya , Shimada Morimi , Nakamura Tuneo 
Published: January 2005

AbstractCell proliferation and migration in the external granular layer of the mouse cerebellum were studied with autoradiography after cumulative labeling with H3‐thymidine. The germinative cells in the external granular layer were considered as externally dislocated matrix cells. Their generation time, presynthetic time, duration of DNA synthesis, postsynthetic time and mitotic time were determined in one‐, three‐, seven‐ and ten‐day‐old mice. The entire sequence of the ontogeny of the external granular cell‐system was separated into three consecutive stages; stage 1 or stage of pure external matrix cell proliferation, stage 2 or stage of neuroblast production, and stage 3 or stage of neuroglia differentiation. Production of neuroblasts in the external granular layer at seven and ten days of life and their migration into the internal granular layer were demonstrated by means of autoradiography. Transit times of the neuroblasts migrating across the external mantle layer and the molecular layer of ten‐day‐old mice were estimated at 21 and four hours, respectively. More than 50% of the inner granular cells migrated from the external granular layer later than ten days of life and almost 81 to 92% were produced later than seven days of postnatal life. In conclusion, on the basis of the matrix cell concept, the authors tried to unify observations of previous and present investigators and presented a scheme of pre‐ and postnatal histogenesis of the mouse cerebellum.
Comparative fine structure of vertebrate neuroglia: Teleosts and reptiles
Authors: Kruger Lawrence , Maxwell David S. 
Published: January 2005

AbstractElectron microscopic examination of aldehyde perfused fish and reptile brains reveals the presence of three distinct varieties of glial elements; ependyma, astrocytes and oligodendrocytes. Ependymal cells line the ventricular cavity, possess cilia and extend long fibrillar processes to the pial surface. Several cytoplasmic organelles and specialized plasma membrane contacts are unique to this glial variety. Astrocytes possess long processes which can be traced as pericapillary and sub‐pial end‐feet. Their cytoplasm contains fine filaments (less than 100 Å) and quantities of glycogen granules. Oligodendrocytes display only occasional long processes and usually have thin cytoplasmic rims, especially when related to myelinated axons. A wide range of nuclear and cytoplasmic matrix densities, numerous free ribosomes and an extensive microtubular system characterize these cells. On the basis of distinctive cytoplasmic organelles it appears possible to establish the homology of lower vertebrate and mammalian glial elements.
Cortical projections from the dorsal lateral geniculate nucleus of cats
Authors: Glickstein Mitchell , King Richard A. , Miller Josef , Berkley Mark 
Published: January 2005

AbstractLesions were placed in the dorsal lateral geniculate nucleus of cats (LGN) and cortical degeneration studied with a modified Nauta method. Besides local degeneration attributable to the electrode track, dense projections to Areas 17 and 18 ipsilateral to the lesion were found. Medium or dense degeneration was found on both banks of the suprasylvian fissure ipsilaterally and light degeneration widely distributed in Area 19 and on the suprasylvian and posterior ectosylvian gyri. In addition to commissural degeneration homotopic to the electrode track, degenerating fibers were found in the visual areas of the opposite hemisphere, suggesting the presence of a crossed geniculo‐cortical pathway.
The development of hippocampal and dorsolateral pallial region of the cerebral hemisphere in fetal rabbits. V. Sixty millimeter stage, glial cell morphology
Authors: Stensaas L. J. 
Published: January 2005

AbstractThe structure of the cerebral hemisphere of the 60 mm rabbit embryo was studied with aniline and Golgi stains. Two types of neuroglial cells are present for the first time at this stage and are described in detail: (1) Young neuroglial cells are elongate cells whose smooth somata are connected to an arbor of fine porcesses by a peduncle of varying length. They appear to develop from freely arborizing spongioblasts as somata lose their attachment to the ventricular surface and migrate toward the cortical plate. This suggests that astrocytes will be formed at a later state from freely arborizing spongioblasts rather than from typical spongioblasts whose connection to the pia has atrophied. (2) Small glial cells are present at all levels above the matrix lamina. They have small, smooth somata and fine, mossy processes arising from a short trunk at one pole of the cell. Although their origin is unknown, their small soze suggests they arise directly from germinal cells rather than from freely arborizing spongioblasts. Lacking conclusive evidence of their identity, they are tentatively regarded as a primitive type of oligodendrocyte.