bluuebunny/crossref_metadata_2025_split huggingface dataset:
Association of α1‐antichymotrypsin polymorphism with cerebral amyloid angiopathy
Authors: Yamada Masahito , Sodeyama Nobuyuki , Itoh Yoshinori , Suematsu Naomi , Otomo Eiichi , Matsushita Masaaki , Mizusawa Hidehiro 
Published: January 2005

AbstractIn search of genetic risk factors of sporadic cerebral amyloid angiopathy (CAA), we investigated the association of a polymorphism in the signal peptide sequence of α1‐antichymotrypsin (ACT) with the severity of CAA in 155 autopsy cases of the elderly, including 48 patients with Alzheimer's disease. In the total cases, there was no significant association of the ACT genotypes (AA, AT, and TT) with the severity of CAA. Within the Alzheimer's disease group, however, a significant correlation was found between the ACT A allele frequency and the severity of CAA.
Normal serum iron and ferritin concentrations in patients with Friedreich's ataxia
Authors: Wilson Robert B. , Lynch David R. , Fischbeck Kenneth H. 
Published: January 2005

AbstractFriedreich's ataxia (FRDA) is caused by point mutations or trinucleotide repeat expansions in both alleles of the gene encoding frataxin. Studies of frataxin homologues in lower eukaryotes suggest that mitochondrial iron accumulation may underlie the pathophysiology of FRDA. To evaluate the possible role of iron‐chelation therapy for FRDA, we measured serum iron and ferritin concentration in 10 FRDA patients. The measurements were within normal limits, suggesting that iron‐chelation therapy for FRDA may be problematic.
Parallel loss of presynaptic and postsynaptic dopamine markers in normal aging
Authors: Volkow N. D. , Wang G. J. , Fowler J. S. , Ding Y. S. , Gur R. C. , Gatley J. , Logan J. , Moberg P. J. , Hitzemann R. , Smith G. , Pappas N. 
Published: January 2005

AbstractAging of the human brain is associated with a decline in dopamine (DA) function, generally interpreted as reflecting DA cell loss. Postitron emission tomography studies revealed that in healthy individuals, the age‐related losses in DA transporters (presynaptic marker) were associated with losses in D2 receptors (postsynaptic marker) rather than with increases as is known to occur with DA cell loss. This association was specific for DA synaptic markers, because they were not correlated with striatal metabolism. Furthermore, the association was independent of age, suggesting that a common mechanism regulates the expression of receptors and transporters irrespective of age.
Hippocampal developmental changes in patients with partial epilepsy: Magnetic resonance imaging and clinical aspects
Authors: Baulac Michel , De Grissac Nathalie , Hasboun Dominique , Oppenheim Catherine , Adam Claude , Arzimanoglou Alexis , Semah Franck , Lehéricy Stephane , Clémenceau Stephane , Berger Brigitte 
Published: January 2005

AbstractDevelopmental disorders of the hippocampal formation (HF) have been described in epileptic syndromes associated with lissencephaly, but HF malformations can be found without widespread cortical changes. We report 19 patients with partial epilepsy and abonormal HF patterns on magnetic resonance imaging (MRI). The changes consisted of incomplete folding with abnormal medial location along the choroid fissure, globular shape and/or verticalization, and were observed in the following three contexts: (1) diffuse disorder of neuronal migration (n = 1); (2) temporal lobe malformation (n = 5), including heterotopia, abnormal gyration, and, in 2 cases, reduced HF volume; and (3) apparently isolated HF changes (n = 13, bilateral in 3 cases). The clinical features were heterogeneous in terms of severity of epilepsy and, when the focus could be determined, in localization (temporal or extratemporal). In 4 patients with apparently isolated HF changes, MRI was suggestive of both abnormal development and hippocampal sclerosis. In these patients, presurgical investigation and postoperative results suggested multiple epileptogenic foci, involving the frontal lobes in 2 cases. One HF specimen was large enough for the observation of developmental abnormalities corresponding to the canges seen on MRI. HF changes in shape and/or position should be included among the structural abnormalities associated with partial epilepsies. They may represent the visible part of a more extensive or more distant disorder of brain development.
Second malignancies in young children with primary brain tumors following treatment with prolonged postoperative chemotherapy and delayed irradiation: A pediatric oncology group study
Authors: Duffner Patricia K. , Krischer Jeffrey P. , Horowitz Marc E. , Cohen Michael E. , Burger Peter C. , Friedman Henry S. , Kun Larry E. ,   And The Pediatric Oncology Group
Published: January 2005

AbstractBetween 1986 and 1990, the Pediatric Oncology Group conducted a study in which 198 children younger than 3 years of age with malignant brain tumors were treated with prolonged postoperative chemotherapy in an effort to delay irradiation and reduce long‐term neurotoxicity. Children younger than 2 years of age received 24 months of chemotherapy followed by irradiation, and those between 2 and 3 years of age received 12 months of chemotherapy plus irradiation. Chemotherapy was given in 28‐day cycles (AAB, AAB), with cycle A = vincristine (0.065 mg/kg) intravenously on days 1 and 8 and cyclophosphamide (65 mg/kg) intravenously on day 1, and cycle B = cisplatinum (4 mg/kg) intravenously on day 1 and etoposide (6.5 mg/kg) intravenously on days 3 and 4. Five of the 198 children developed second malignancies, with a cumulative risk at 8 years of 11.3% (95% confidence interval [CI], 0–39%). Four of the five second malignancies occurred in children younger than 2 years of age at diagnosis, with a cumulative risk at 8 years of 18.9% (CI, 0–70%). Initial diagnoses were choroid plexus carcinoma (2 children), ependymoma (1 child), desmoplastic infantile ganglioglioma (2 children), and medulloblastoma (1 child). Duration from diagnosis of initial tumor to second malignancy was 33, 35, 57, 66, and 92 months. Three children younger than 2 years of age developed Iymphoproliferative disease, that is, myelodysplastic syndrome (2 children), both with monosomy 7 deletions, and acute myelogenous leukemia (1 child), after 24 to 26 cycles of chemotherapy, including 8 cycles of etoposide. Two of 3 received craniospinal irradiation (2,560/3,840 cGy) and (3,520/5,320 cGy). Time to second malignancy was 7 years 8 months, 4 years 9 months, and 2 years 9 months. Two children developed solid tumors, at 5 years 6 months and 2 years 11 months, respectively, after initiation of treatment. A sarcoma developed after 26 cycles of chemotherapy and no irradiation, and a meningioma developed after 12 cycles of chemotherapy and local craniospinal irradiation. Potential causative factors for this high rate of secondary malignancies include prolonged use of alkylating agents and etoposide with or without irradiation.
Overexpression of copper/zinc superoxide dismutase: A novel cause of murine muscular dystrophy
Authors: Rando Thomas A. , Crowley Rebecca S. , Carlson Elaine J. , Epstein Charles J. , Mohapatra Pramit K. 
Published: January 2005

AbstractOxidative injury underlies the cellular injury and cell death in a variety of disease states. In muscular dystrophies, evidence from in vivo and in vitro studies suggests that muscle degeneration may be secondary to an increased susceptibility to oxidativ stress. To address the role of free radical metabolism in the pathogenetic process of muscular dystrophies, we examined the muscle of transgenic mice that overexpress copper/zinc (Cu/Zn) superoxide dismutase. Overexpression of this enzyme can sensitize cells to oxidative injury, and Cu/Zn superoxide dismutase activity was elevated approximately fourfold above control levels in skeletal muscle of the transgenic strain. Examination of serum creatine phosphokinase levels in these mice revealed significant elevations after 2 months of age, indicative of active muscle breakdown. By 8 months of age, there was gross atrophy of the quadriceps muscle, and other hindlimb muscles were variably affected. Histologically, there was evidence of widespread muscle necrosis and regeneration, fiber splitting, and replacement of muscle with adipose and fibrous connective tissue, typical of a muscular dystrophy. Associated with the development of this degeneration was an increase in the levels of lipid peroxidation in the muscle of Cu/Zn superoxide dismutase transgenic mice, highlighting the central role of oxidative injury in this pathogenetic process. These results demonstrate that oxidative damage can be the primary pathogenetic process underlying a muscular dystrophy.
Glial cytoplasmic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble α‐synuclein
Authors: Tu Pang‐hsien , Galvin James E. , Baba Minami , Giasson Benoit , Tomita Taisuke , Leight Susan , Nakajo Shigeo , Iwatsubo Takeshi , Trojanowski John Q. , Lee Virginia M.‐Y. 
Published: January 2005

AbstractRecently, α‐synuclein was shown to be a structural component of the filaments in Lewy bodies (LBs) of Parkinson's disease (PD), dementia with LBs (DLB) as well as the LB variant of Alzheimer's disease, and this suggests that α‐synuclein could play a mechanistic role in the pathogenesis of these disorders. To determine whether α‐synuclein is a building block of inclusions in other neurodegenerative movement disorders, we examined brains from patients with multiple system atrophy (MSA) and detected α‐synuclein, but not β‐ or γ‐synuclein, in glial cytoplasmic inclusions (GCIs) throughout the MSA brain. In MSA white matter, α‐synuclein–positive GCIs were restricted to oligodendrocytes, and α‐synuclein accumulated selectively in MSA white matter with α‐synuclein–positive GCIs. Taken together with evidence that LBs contain insoluble α‐synuclein, our data suggest that a reduction in the solubility of α‐synuclein may induce this protein to form filaments that aggregate into cytoplasmic inclusions, which contribute to the dysfunction or death of glial cells as well as neurons in neurodegenerative disorders with different phenotypes.
Seizure outcome after epilepsy surgery in children and adolescents
Authors: Wyllie Elaine , Comair Youssef G. , Kotagal Prakash , Bulacio Juan , Bingaman William , Ruggieri Paul 
Published: January 2005

AbstractFew epilepsy surgery outcome data are available from series of pediatric patients. We studied seizure outcome in 136 pediatric patients who had surgery for intractable epilepsy at The Cleveland Clinic between January 1990 and June 1996, with a postoperative follow‐up of 1 to 7.5 years (mean, 3.6 years). Sixty‐two children (3 months to 12 years old at time of surgery) were compared with 74 adolescents (13–20 years old). Extratemporal or multilobar resections and hemispherectomies were similarly frequent among children (50%) and adolescents (44%), but these procedures strongly predominated in infancy (90% of patients 0–2 years of age). The remaining patients had temporal resection. Cortical dysplasia and low‐grade tumor were the most common causes and hippocampal sclerosis was rare. Seizure‐free outcome was achieved for 69% of adolescents, 68% of children, and 60% of the infant subgroup, overall; for 23 (74%) of 31 children and 33 (80%) of 41 adolescents after temporal resection; for 11 (58%) of 19 children and 15 (52%) of 29 adolescents after extratemporal or multilobar resection; and for 8 (67%) of 12 children and 3 (75%) of 4 adolescents after functional hemispherectomy. Seizure‐free outcome was more frequent after temporal resection (56 of 72, 78%) than after extratemporal or multilobar resection (26 of 48, 54%; 41 of 48 with a focal lesion on magnetic resonance imaging), and among patients with tumor (36 of 44, 82%) versus cortical dysplasia (16 of 31, 52%). The frequency of seizure‐free outcome after epilepsy surgery was similar for infants, children, and adolescents, and comparable with results from adult series. Most patients in each age, surgery type, and causal group were free from seizures after surgery. These results suggest that children should be considered for surgical evaluation at whatever age they manifest with severe, intractable, disabling localization‐related epilepsy.
Unilateral postherpetic neuralgia is associated with bilateral sensory neuron damage
Authors: Oaklander Anne Louise , Romans Katharine , Horasek Sylvia , Stocks Adelaine , Hauer Peter , Meyer Richard A. 
Published: January 2005

AbstractShingles can cause chronic neuropathic pain (postherpetic neuralgia) long after skin lesions heal. To investigate its causes, we quantitated immunolabeled sensory neurites in skin biopsies from 18 subjects with and 16 subjects without postherpetic neuralgia after unilateral shingles. Subjects rated the intensity of their pain. Punch skin biopsies were evaluated from the site of maximum pain or shingles involvement, the homologous contralateral location, and a site on the back, distant from shingles involvement. Sections were immunostained with anti‐PGP9.5 antibody, a pan‐axonal marker, and the density of epidermal and dermal neurites determined. The group with postherpetic neuralgia had a mean density of 339 ± 97 neurites/mm2 in shingles‐affected epidermis compared with a density of 1,661 ± 262 neurites/mm2 for subjects without pain. Neurite loss was more severe in epidermis than dermis. Unexpetedly, the lack of contralateral shingles eruptions or pain, correlated epidermis. Contralateral damage ocurred despite the lack of contralateral shingles eruptions or pain, correlated with the presence and severity of ongoing pain at the shingles site, and did not extend to the distant site. Thus, the pathophysiology of postherpetic neuralgia pain may involve a new bilateral mechansm.
Abnormal somatosensory homunculus in dystonia of the hand
Authors: Bara‐Jimenez William , Catalan Maria Jose , Hallett Mark , Gerloff Christian 
Published: January 2005

AbstractAbnormalities of the sensory system have been proposed as causative factors for dystonia. By mapping the human cortical hand somatosensory area of 6 patients with focal dystonia of the hand, we found an abnormality of the normal homuncular organization of the finger representations in the primary somatosensory cortex (S1). Although a remote antecedent event or even a developmental anomaly cannot entirely be ruled out, our findings may support the concept that abnormal plasticity is involved in the development of dystonia.
The nervus facialis of the albino mouse
Authors: Rhinehart D. A. 
Published: January 2005

AbstractThe facial nerve of the mouse corresponds very closely to that of other mammals and man, and more closely resembles the glossopharyngeal than any of the other cranial nerves. It consists of two parts: one part made up of those fibers which arise inthe motor nucleus and form the motor part of the nerve, the other part being formed by the'nervus intermedius and its peripheral continuation.
Application of the Marchi method to the study of the radix mesencephalica trigemini in the guinea‐pig
Authors: Allen William F. 
Published: January 2005

AbstractThe results of the previous experiments confirm May and Horsley's conclusions that the mesencephalic trigeminal root contains both ascending and descending fibers. The former take origin from sensory cells in the semilunar ganglion and the latter from globular, unipolar cells in the alar (sensory) plate of the mesencephalon, and from a caudal continuation of these cells, known as the locus. coeruleus, which extend downward into the motor area of the pons.
The effect of over‐activity on the morphological structure of the synapse
Authors: Marui Kiyoyasu 
Published: January 2005

AbstractCareful investigation of the cell body as well as the synapse of the fatigued Mauthner cell in Ameiurus revealed a number of interesting findings, which can be summarized as follows: The cell body was found either swollen or shrunken; the turgescence was regarded as the result of overactivity and the shrinkage as that, of exhaustion. The Nissl substance was in a more or less advanced stage of chromatolysis and thereby the cytoplasm was stained variously deeply. On the border of the nucleus a mass of stainable substance was observed as the restitution phenomenon of the Nissl bodies op the side of the nucleus. It was also accepted that a mutual interchange of substance occurs between nucleus and protoplasm in activity. The nucleus was also either swollen or shrunken; in the swollen nerve cell it was sometimes renioved to one side of the cell body. The nucleolus mas also found swollen sometimes and other times shrunken and it was of angular or otherwise irregular shape.
The distribution of sympathetic nerve fibers to the hind limb of the cat
Authors: Burns B. I. 
Published: January 2005

AbstractIn six cats the trunks of the third or fourth lumbar to the third sacral nerves were cut between the dorsal root ganglion and the gray ramus. Studies were made of serial sections of the nerve trunks with their intact gray rami, sections of the peripheral nerves, including most of the motor and sensory nerves of the posterior limb, and serial sections of the blood vessels. The latter were cut into segments and the serial sections made of each segment. IN addition, similar studies of a large number of normal vessels were made.
The human posterior commissure
Authors: Tomasch Joseph 
Published: January 2005

AbstractMidline cross‐sections of the posterior commissures of five adult males were used for a determination of their cross‐sectional area and fiber‐density. Due to non‐uniform fiber‐size distribution a stratified sample involving from 12–20 areas was collected from each case. A mean cross‐sectional area of 3,587 mm2 with a standard variation of ±0.820 mm2 was measured for the five cases. Fiber density was found to vary in the different strata between 86,400 to 432,000 ± 10% per mm2 on axon counts taken from sections stained by Häggqvist's method. Counting myelin sheaths on sections stained by Weigert's method fiber density varied from 40,000 to 158,400 fibers per mm2 ± 30%. Using these values for calculating a total fiber number estimate, based on the axon count, fiber number varies from 504,566 to 887,849 fibers. Comparing this total fiber estimate with a similar estimate taken from the anterior commissure, the figure appears low. This difference is due to the presence of a great number of large diameter fibers in the posterior commissures, not found in either the anterior commissure or the corpus callosum. Attaching a probability level of t0.05 to this sample of five cases, a hypothetical posterior commissure may be expected to have a cross‐sectional area of 3.587 ± 0.942 mm2 and from 497,404 to 1,090,270 fibers. In one case the habenular commissure was also studied and found to contain 112,706 fibers (cross‐sectional area 0.697 mm2).
A trigeminal midbrain‐cerebellar fiber connection in the rat
Authors: Cupédo R. N. J. 
Published: January 2005

AbstractCerebellar collaterals of the mesencephalic trigeminal root have been studied by the use of various histologic techniques in adult and fetal brains of rat and mouse. In addition, brains of rats with lesions in the mesencephalic trigeminal system were studied.Cells of the mesencephalic trigeminal system can be found in the brachium conjunctivum near the cerebellar hilus in the adult and fetal rat and in the fetal and full‐term mouse. Such cells located in the anterior medullary velum were found in fetal rat material and m the human brain stem. In the rat collaterals of the mesencephalic trigeminal root course through the brachium conjunctivum and anterior medullary velum, terminating in the nodulus cerebelli and possibly also in the neighborhood of the primary fissure. These fibers coursing through the anterior medullary velum decussate and intermingle with fibers of the ventral spino‐cerebellar tract and fibers of the trochlear nerve. They course ventrally from the tectocerebellar tract, which consists of smaller myelinized fibers. Termination in the cerebellum is by means of mossy fibers. The findings are discussed as they relate to previous studies.It is probable, that the cerebellum utilizes proprioceptive impulses conducted by mesencephalic fifth fibers in its coordinating influence on the activity of the muscles of mastication.
Sites and mode of termination of reticulo‐spinal fibers in the cat. An experimental study with silver impregnation methods
Authors: Nyberg‐hansen Rolf 
Published: January 2005

AbstractFollowing lesions in the brain stem reticular formation in 20 cats the ensuing degeneration within the spinal cord has been studied with silver impregnation methods with special reference to the laminar organization of the gray matter described by Rexed ('52, '54).Reticule‐spinal fibers do not arise in the mesencephalon. Pontine reticulo‐spinal fibers descend in the ventral funiculus, almost exclusively homolaterally. Some cross to the contralateral side in the anterior commissure. Medullary reticulo‐spinal fibers descend bilaterally in the ventral part of the lateral funiculus, mostly homolaterally. Both contingents of fibers descend the whole cord throughout. Pontine reticulo‐spinal fibers are more numerous than medullary ones.Pontine fibers terminate bilaterally in lamina VIII and adjacent parts of lamina VII. Contralateral terminations are established mainly by way of fibers crossing in the anterior commissure. Medullary fibers terminate chiefly in lamina VII. A few terminations are found in lamina IX, possibly on large α‐motoneurons and on small neurons.Reticulo‐spinal fibers of both contingents terminate on cells of all sizes, on somata as well as on dendrites.
Organization of the visual projection upon the optic tectum of a reptile (Alligator mississippiensis)
Authors: Heric Thomas M. , Kruger Lawrence 
Published: January 2005

AbstractElectrical responses were recorded from the optic tectum of the alligator with steel microelectrodes, and the receptive field for each of a series of systematically spaced recording sites was delineated on a transparent plastic hemisphere positioned in front of the contralateral eye. A precise retinotopic relationship was determined with the inferior portion of the retina projecting onto the dorsal surface of the tectum, and the superior portion of the retina represented on the lateral and ventral aspects of the tectum. The equatorial line of the hemisphere is situated laterally and runs obliquely across the tectum from the rostrolateral pole to the caudomedial corner. The center of the visual field occupies a disproportionate amount of tectal space providing for functional magnification. Receptive field size and spacing diminish for the representation of the central region implying a retinal area for increased visual acuity. It is suggested that this alinearity between the visual field and the tectum is expressed in its fiber architecture and is probably a consequence of specialization of the eye rather than an evolutionary modification within the reptilian central nervous system.
Organization of the visual projection upon the optic tectum of some freshwater fish
Authors: Schwassmann Horst O. , Kruger Lawrence 
Published: January 2005

AbstractIn four species of freshwater fish, bass, carp, bluegill and goldfish, the topographical relations of the visual field with the contralateral optic tectum were investigated, using a small light as stimulus in the visual field and recording the electrical response of the tectum with a metal microelectrode. The results show a precisely organized visual projection onto the contralateral tectum in which the anterior visual field lies anteriorly on the tectum, the temporal field posteriorly, the dorsal field medio‐dorsally, and the ventral field in the latero‐ventral part of the tectum. The projection was found to be essentially linear and uniform with no indication of a specialized area in the visual field in those species investigated. Some receptive fields extended into the anterior binocular visual field.