bluuebunny/crossref_metadata_2025_split huggingface dataset:
The biological behavior of primary cerebral neuroblastoma: A reappraisal of the clincal course in a series of 70 cases
Authors: Bennett James P. , Rubinstein Lucien J. 
Published: January 2005

AbstractThe outcome in 70 patients with primary cerebral neuroblastoma was evaluated in light of their age incidence, histological subtypes, and modes of treatment. In more than 80% of the patients, primary cerebral neuroblastoma occurred in the first decade, most often before the age 2 years (26%). Over half of the tumors were histologically of the classic variant, the remainder showing varying degrees of mesenchymal stromal response (desmoplastic or transitional variants). Patients with a tumor of the desmoplastic or transitional variant without ganglionic differentiation had a lower mean age at the time of diagnosis than those harboring a tumor of the classic variant. Age could not be correlated with the presence of ganglionic differentiation.The overall 3‐year survival rate was 60%. At least 30% of patients have a 5‐year postoperative survival. Elight patients survivied 7 years after diagnosis, and 3 for more than 10 years. The period of risk for tumor recurrence or death was a function of the initial age at diagnsis (Collin's law). Most deaths or fatal recurrences occurred within the first 3 years after operation. No corelation could be made between survival times and histological variant of the tumor or the presence of ganglionic differentiation.Our data do not indicate that, overall, postoperative irradiation with or without chemotherapy improved length of survival, but an occasional favorable clinical response to chemotherapy did occur. Because of the high incidence of local recurrence and the relative frequency of cerebrospinal metastasis, prophylactic whole brain and spinal irradiation is probably justified.
Activity of multiple sclerosis during pregnancy and puerperium
Authors: Korn‐Lubetzki Isabelle , Kahana Esther , Cooper Gerald , Abramsky Oded 
Published: January 2005

AbstractThe influence of pregnancy on multiple sclerosis was studied in 338 women by determining in each trimester of pregnancy and post partum the number of relapses and the corresponding relapse rate. Eighty‐five relapses occurred in association with 199 pregnancies, most (65) in the postpartum period, and a low number of relapses (2) were recorded in the last trimester of pregnancy. Comparing the average exacerbation rate of the study group with that of patients with multiple sclerosis in Israel (0.28 relapses per person per year), we found a statistically significant decrease in the third trimester (0.04) and a high increase in the first three months post partum (0.82). This pattern of remissions at the end of pregnancy and exacerbations post partum is similar to that observed in other putative autoimmune diseases.
Effect of vasodilating drugs on intracortical and extracortical vascular resistance following middle cerebral artery occlusion in cats
Authors: Date H. , Hossmann K.‐A. 
Published: January 2005

AbstractIn 24 lightly anesthetized cats acute cerebral infarction was produced by transorbital occlusion of the left middle cerebral artery. Pial artery pressure and blood flow were measured in the center of the middle cerebral artery territory, and changes of segmental resistance were determined upstream and downstream of the pial arterial recording site. After middle cerebral artery occlusion, pial arterial pressure fell from 59 to 13 mm Hg, and blood flow from 67.4 to 19.6 ml/100 gm/min. Downstream (intracortical) vascular resistance slightly decreased from 0.93 to 0.72 mm Hg · ml−1. 100 gm · minute, and upstream (extracortical) resistance rose form 0.89 to 6.18 mm Hg · ml−1. 100 gm · min. Before middle cerebral artery occlusion, intracarotid infusion of papaverine (625 μg/kg/min) and prostacyclin (500 ng/kg/min) caused a reduction of downstream resistance, and intracarotid infusion of nimodipine (670 ng/kg/min) reduced both downstream and upstream resistance. After vascular occlusion all three drugs reduced mainly upstream resistance but cerebral blood flow did not significantly improve because blood pressure also fell. Intracerebral steal phenomena were never observed.
Value of ultrasound for identification of acute hemorrhagic necrosis of thalamus and basal ganglia in an asphyxiated term infant
Authors: Kreusser Katherine L. , Schmidt Robert E. , Shackelford Gray D. , Volpe Joseph J. 
Published: January 2005

AbstractAn infant with profound intrapartum and postpartum asphyxia demonsstrated striking bilateral echodensities in the region of the thalamus and basal ganglia on ultrasound scan. Neuropathological examination confirmed the presence of symmetrical areas of hemorrhagic necrosis involving the thalamus, caudate, putamen, and globus pallidus. The findings demonstrate the value of ultrasonography in the identification of this variety of perinatal hypoxic‐ischemic injury.
Adrenoleukodystrophy: Survey of 303 cases: Biochemistry, diagnosis, and therapy
Authors: Moser Hugo W. , Moser Ann E. , Singh Inderjit , O'Neill P. 
Published: January 2005

AbstractAdrenoleukodystrophy (ALD) is a genetically determined disorder associated with progressive central demyelination and adrenal cortical insufficiency. All affected persons show increased levels of saturated unbranched very‐long‐chain fatty acids, particularly hexacosanoate (C26:0), because of impaired capacity to degrade these acids. This degradation normally takes place in a subcellular organelle called the peroxisome, and ALD, together with Zellweger's cerebrohepatorenal syndrome, is now considered to belong to the newly formed category of peroxisomal disorders. Biochemical assays permit prenatal diagnosis, as well as identification of most heterozygotes. We have identified 303 patients with ALD in 217 kindreds. These patients show a wide phenotypic variation. Sixty percent of patients had childhood ALD and 17% adrenomyeloneuropathy, both of which are X‐linked, with the gene mapped to Xq28. Neonatal ALD, a distinct entity with autosomal recessive inheritance and points of resemblance to Zellweger's syndrome, accounted for 7% of the cases. Although excess C26:0 in the brain of patients with ALD is partially of dietary origin, dietary C26:0 restriction did not produce clear benefit. Bone marrow transplant lowered the plasma C26:0 level but failed to arrest neurological progression.
The frequency of multiple sclerosis in Italy: A descriptive study in Ferrara
Authors: Granieri Enrico , Tola Rosaria , Paolino Ezio , Rosati Giulio , Carreras Mirko , Monetti V. Cinzia 
Published: January 2005

AbstractResult of intensive prevalence surveys on multiple sclerosis carried out in different small regions of Italy have suggested that this country falls into the high‐frequency zone for the disease. To verify this hypotiesis by studying a large population, we conducted an intensive incidence and prevalence survey in the province of Ferrara, northern Italy (mean population, 386, 000). Based on 128 patients, the mean incidence per year for the period 1965 through 1979 was 2.2 cases per 100, 000. On October 24, 1981, the prevalence rate was 46.1 cases per 100, 000. The results support the view that northern Italy is a high‐risk area for multiple sclerosis.
Evolution of computed tomographic abnormalities in leptomeningeal metastases
Authors: Jaeckle Kurt A. , Krol George , Posner Jerome B. 
Published: January 2005

AbstractWe obtained serial cranial computed tomography (CT) scans from 25 patients with documented leptomeningeal metastases in orders to correlate the course of clinical symptoms and cerebrospinal fluid abnormalities with the evolution of CT abnormalities. In almost one‐third of patients, relatively specific CT finding such as enhancement of the meninges, obliteration of cisterns or sulci, and hydrocephalus with transependymal edema preceded clinical symptoms. By the time patients became symptomatic, 10 of 25 (40%) had specific abnormalities, and 4 more became specifically abnormally with time. The first scan after cerebrospinal fluid tumor cells were discovered showed specific abnormalities in only 6 patient, but 7 more became specifically abnormal with time. Ventriculomegaly occurred in 18 of 25 patients (72%) and was associated with sulcal and/or cisternal obliteration in one‐half of the patients. In 4 patients sulcal and cisternal obliteration developed prior to ventricular enlargement. Multiple small, enhancing cortical nodules were detected prior to symptom onset and discovery of leptomeningeal tumor in 5 patients.
Dermatomal somatosensory evoked potentials unilateral lumbosacral radiculopathy
Authors: Aminoff Michael J. , Goodin Douglas S. , Barbaro Nicholas M. , Weinstein Philip R. , Rosenblum Mark L. 
Published: January 2005

AbstractWe examined scalp‐recorded somatosensory evoked potentials (SSEPs) to electrical stimulation of the peroneal nerves and to stimulation in the L5 and S1 dermatomes in 19 patients with unilateral radiculopathies involving these segments. For the dermatomal studies at least two trials of 512 responses were recorded from the vertex with reference to both the midfrontal and contralateral parietal electrodes, using an averaging technique. Findings on the symptomatic and asymptomatic sides were compared in each patient. We found that peroneal SSEPs were normal in all patients. Dermatomal SSEPs correctly identified the lesion in 5 patients. In 1 patient dermatomal SSEPs lateralized the lesion correctly but localized it to the adjacent root. In 10 cases dermatomol SSEPs gave misleading information, indicating an abnormality on the asymptomatic side in 1 patient and no abnormality in 9. In the remaining 3 patients, both SSEPs and radiological contrast studies failed to identify any lesion, although the radiculopathy was confirmed by electromyography. These findings raise doubt about the ultimate utility of these evoked potential techniques in the evaluation of patients with suspected radiculopathies.
Disturbance of smooth pursuit caused by infarction of occipitoparietal cortex
Authors: Leigh R. John , Tusa Ronald J. 
Published: January 2005

AbstractTwo distinct deficits of ocular smooth pursuit could be demonstrated using two types of visual stimulus motion in a patient who had suffered bilateral occipitoparietal lobe infarction. First, in response to constant‐velocity (zero accleration) target motion, smooth pursuit gain (eye velocity/target velocity) was mildly impaired for tracking to the right but near normal for tracking to the left. For example, for a target moving at 28.5 degrees/sec, gain was 0.65 to the right but 0.85 to the left. Second, in response to high‐acceleration sinusoidal target motion, there was a deficit in maximum eye acceleration for targets moving right and left. The patient was unable to accelerate his eyes faster than 48 degrees/sec2 to the right and 95 degrees/sec2 to the left (normal is more than 300 degrees/sec2). It is suggested that smooth pursuit should be routinely tested using stimuli with a wide range of accelerations.
Regional perfusion in normal and ischemic rat sciatic nerves
Authors: Sladky John T. , Greenberg Joel H. , Brown Mark J. 
Published: January 2005

AbstractWe measured regional blood flow in rat sciatic‐posterior tibial nerve using an iodo‐[14C] antipyrine autoradiographic unique. Average flow was 12.1 ± 3.3 ml/100 gm/min, considerably less than that of both gray and white matter structures in the cerebral hemispheres. Perfusion was homogenous along the proximal‐distal extent of this nerve and along its radial axis. Acute occlusion of the femoral artery reduced regional sciatic blood flow. Flow was especially depressed within the endoneurial core of the proximal posterior tibial branch, which lies in a watershed zone between adjacent segmental arterial fields. The topography of the arterial supply to nerves and their abundant collateral circulation may explain the centrifascicular distribution of nerve infarcts, and in part can account for the apparent resistance of peripheral nerves to ischemia.
Herpes simplex virus type 2 encephalitis in two homosexual men with persistent lymphadenopathy
Authors: Dix Richard D. , Waitzman David M. , Follansbee Stephen , Pearson Brion S. , Mendelson Terri , Smith Peggy , Davis Richard L. , Mills John 
Published: January 2005

AbstractWithin a 5‐month period, 2 homosexual men with persistent lymphadenopathy developed clinical findings consistent with herpes simplex virus (HSV) encephalitis. These signs included abrupt change in mental status, seizures, cerebrospinal fluid pleocytosis, and localized electroencephalographic abnormalities showing temporal lobe involvement. Intial computed tomographic scans were unremarkable. Treatment with adenine arabinoside was instituted and temporal lobe biopsies were performed. Although virus‐specific antigens were detectable in only 1 patient, cultures of biopsy tissue from both patients yielded HSV type 2 organisms. Spiking fevers persisted and the patients failed to improve, prompting administration of acyclovir. Both patients recovered gradually after their second course of antiviral therapy and survived with severe neurological deficits. These patients should raise concerns about an increased incidence of type 2 encephalitic illness among homosexual men with persistent lymphadenopathy or acquired immune deficiency syndrome. In addition, the importance of using HSV type 2 antibody in the immunofluorescence test of brain biopsy tissue for rapid diagnosis of the disease is emphasized.
Endogenous opioid immunoreactivity in rat spinal cord following traumatic injury
Authors: Faden Alan I. , Molioneaux Christopher J. , Rosenberger John G. , Jacobs Thomas P. , Cox Brain M. 
Published: January 2005

AbstractIt has been postulated that endogenous opioids play a pathophusiological role in spinal cord injury, based on the therapeutic effects of the opiate receptor antagonist naloxone in certain experimental models. The high doses of naloxone required to exert a therapeutic action suggest that naloxone's effects may be mediated by non‐μ opiate receptors, such as the κ receptor. This notion is supported by recent than naloxone in improving outcome after spinal cord injury. Moreover, dynorphin‐postulated to be the endogenous ligand for the κ receptor–is unique among opioids in producing hindlimn paralysis following intrathecal administration in the rat. In the present studies we have examined changes in endogenous opioid immunoreactivity following traumatic spinal cord injury in the rat. Dynorphin A was found to increase progressively with graded injury; changes were restricted to the injury segment and adjacent areas and were time dependent. Dynorphin A‐(1–8) showed no marked changes. Mehtionine and leucine enkephalin were either unaltered or reduced at the injury site; changes were not well localized and were not clearly related to the injury variables. These findings provide further support for a potential pathophysiological role of prodynorphin‐derived peptides in spinal cord injury.
An immunochemical study of the pyruvate dehydrogenase deficit in Alzheimer's disease brain
Authors: Rex Sheu Kwan‐Fu , Kim Young‐Tai , Blass John P. , Weksler Marc E 
Published: January 2005

AbstractThe activity of the pyruvate dehydrogenase complex (PDHC; EC 1.2.4.1, EC 2.3.12, and EC 1.6.4.3) was reduced to about 30% of control values in histologically unaffected occipital cortex of the brains of patients with Alzheimer's disease, as well as in histologically affected frontal cortex. In contrast, activity of another mitochondrial enzyme, glutamate dehydrogenase, was normal. Neither age nor time untill postmortem study correlated significanctly with PDHC activity in either Alzheimer or control samples, and PDHC was not inactivated significantly on incubation with homogenates of either Alzheimer or control brain. Antibodies against the highly purified bovine PDHC inhibited Alzheimer and control PDHC equally per unit of enzyme activity. Immunoblots also indecated that the PDHC antigens were not different in normal and Alzheimer brain. This antibody, however, inhabited Alzheimer PDHC more effectively than it did control PDHC, based on milligrams of protien, suggesting a reduced amount of normal PDHC protien. Other data suggest that the PDHC deficiency is related to mitochondrial damege and to impaired calcium homeoestasis in Alzheimer nerve cells, which may then mediate a variety of other cellular impairments.
Hereditary motor sensory neuropathy type II with neurofilament accumulaion: New finding or new disorder?
Authors: Vogel P. , Gabriel M. , Goebel H. H. , Dyck P. J. 
Published: January 2005

AbstractPeroneal muscular atrophy is now known to be heterogeneous and to be due to various underlying genetic mechanisms. Exploring this heterogeneity further, we report on a German kinship with the clinical, genetic, and nerve conduction features of hereditary motor and sensory neuropathy type II (HMSN type II) but whose sural nerves on biopsy were found to show infrequent axonal swellings with neurofilament accumulations not previously described. The dominant inheritance and absence of kinky hair set this disorder apart from giant axonal neuropathy. There was no history of toxic exposure to industrial chemicals. We conclude that the disorder either is a new type of HMSN or is HMSN type II with previously unencountered neurofilament accumulations. Neurofilament accumulation indicates that the axon could be a site for primary derangement and may implicate an abnormality of slow axonal flow. In addition, some of the patients exhibited features suggestive of a cardiomyopathy.
Experimental lead neuropathy: Inorganic lead inhibits proliferation but not diffrentiation of Schwann cells
Authors: Sobue Gen , Pleasure David 
Published: January 2005

AbstractSchwann cells were prepared from the sciatic nerves of newborn rats and cultured in a monolayer. Addition of lead acetate at concentrations between 0.4 and 10.0 μmUg/ml, levels comparable to those occurring in neural tissues and physiological fluids of lead‐intoxicated rats, diminished both the baseline rate of proliferation of the Schwann cells and their response to the mitogens, axolemmal fragment, glial growth factor, and the adenosine 3′:5′‐cyclic monophosphate (cAMP) analogues 8‐bromo‐cAMP and dibutyryl‐cAMP. This demonstrates a direct toxic effect of inorganic lead on Schwann cell. Lead acetate in this concentration range did not, however, inhibit the cAMP analogue‐induced appearance of the “myelin marker” lipid galactocerebroside on the surfaces of the cultured Schwann cells.
Low cancer rates among patients with Parkinson's disease
Authors: Jansson Birger , Jankovic Joseph 
Published: January 2005

AbstractAmong 406 Patients with Parkinson's disease, the cancer rate (all sites combined) was about one‐third that for the general population. The risk of cancer increased during the treatment period but remained significantly low. Malignant and benign thyroid neoplasms were significantly more frequent than expected among patients with Parkinson's disease. We suggest that high level of total body potassium in patients with Parkinson's disease is the projective factor against cancer.
Mitochondrial myopathies
Authors: DiMauro Salvatore , Bonilla Eduardo , Zeviani Massimo , Nakagawa Masanori , DeVivo Darryl C. 
Published: January 2005

AbstractMitochondrial myopathies are clinically heterogeneous disorders that can affect multiple systems besides skeletal muscle (mitochondrial encephalomyopathies or cytopathies) and are usually defined by morphological abnormalities of muscle mitochondria. There are a few distinctive syndromes, such as the Kearns‐Sayre syndrome; myoclonus epilepsy with ragged‐red fibers; and mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. Biochemically, mitochondrial myopathies can be divided into defects of substrate utilization, oxidation‐phosphorylation coupling, and the respiratory chain. Because mitochondria have their own DNA and their own translation and transcription apparatuses, mitochondrial myopathies can be due to defects of either a nuclear or mitochondrial genome and can be transmitted by mendelian or maternal inheritance.
Human telencephalic angiogenis
Authors: Kuban Karl C. , Gilles Floyd H. 
Published: January 2005

AbstractWe provide new observations regardingthe histogenesis and regional development of the human telencephalic microvasculature during the last half of gestation. Endothelium‐lined trunks extending from pia to the subventricular plexus, evident early in gestation, persisit during the last half of gestation. The courses of these trunks are modified and become complex as the bulk of the telencephalon, straitum, and thalamus increases and as guri grow. New cerebral tissue is supplied by increasing numbers of shorter penetrating vessels. All extrastriatal vessels have many lateral right‐and acute‐angle branches that join nearby trunks and shorter vessels. Striatal vessel branches predominanatly have acute angles. Most extratriatal channels remain devoid of apparent muscular is until the final weeks of gestation. In gestation. In contrast, striatal arteries begin to muscularize at about 24 weeks of gestation. Muscularization appears to occur in a centripetal direction and is apparent in the caudate at approximately 30 weeks' gestation. We did not identify transventricular, paraventricular, or recurrent arteries ending in deep white matter.
Nucleus raphe dorsalis in Alzheimer's disease: Neurofibrillary tangles and loss of large neurons
Authors: Yamamoto Toru , Hirano Asao 
Published: January 2005

AbstractDiffuse serotonergic fibers are presumed to project to the talencephalon from the nucleus raphe dorsalis (NRD) of the mid brain, in a manner similiar to the cholinergic projections from the nucleus basalis of Meynert (nbM) to the cerebral cortex. Neuropathological changes in both of these nuclei have been reported in Alzheimer's disease (AD). Although many morphometric studies of the nbM in AD have been documented, only one such study of the NRD has been documented, only one such study of the NRD has been conducted so far; it demonstrated a six fold increase in neurofibrillary tangles in AD but no statistically significant difference in the number of neurons in patients with AD and age‐matched controls. A study of the NRD utilizing different stains and wider anatomical boundaries is detailed in this report of 5 patients with AD and 7 age‐matched controls. In AD the NRD showed 39 times more neurofibrillary tangles and the number and cell density of large neurons were reduced to 23% and 28%, respectively, of those in the controls. A small number of senile plaques were found in the NRD in all patients with AD but none were found in the controls.
The clinical value of free phenytoin levels
Authors: Theodore William H. , Yu L. , Price Beth , Yonekawa Wayne , Porter R. J. , Kapetanovic I. , Moore Henry , Kupferberg Harvey 
Published: January 2005

AbstractThe relationship between total and free phenytoin levels and drug toxicity was studied in 80 patients. Twenty‐four were taking phenytoin alone. Drug toxicity was assessed by a “blind” rater using an eight‐point standardized scoring system. The mean free phenytoin fraction was 0.076 in patients taking phenytoin alone or phenytoin and carbamazepine and 0.11 in patients taking valproic acid (p < 0.001). The free fraction did not change with the total level over the range tested (6.7 to 39.9 μg/ml total phenytoin). There was a strong correlation between free and total levels (r = 0.84). Both free (r = 0.59) and total (r = 0.49) phenytoin levels were positively correlated with the toxicity score. Only total phenytoin levels showed a weak positive correlation with decreasing seizure frequency. Our results suggest that routine free phenytoin level monitoring is not necessary in most clinical situations.