bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute demyelinating disease in a chimpanzee three years after inoculation of brain cells from a patient with MS
Authors: Rorke Lucy Balian , Iwasaki Yuzo , Koprowski Hilary , Wroblewska Zofia , Gilden Donald H. , Warren Kenneth G. , Lief Florence S. , Hoffman Steven , Cummins Larry B. , Rodriguez Arthur R. , Kalter Seymour S. 
Published: January 2005

AbstractBrain cells from a patient with classic multiple sclerosis were inoculated intracerebrally into the frontal lobe of a newborn chimpanzee. The animal developed acute quadriplegia three years, two months later and was killed four days after the onset of symptoms. Central nervous system lesions were primarily localized in the spinal cord at root entry zones; these were characterized by demyelination and regeneration of myelin by Schwann cells.
Mechanisms underlying hemispatial neglect
Authors: Heilman Kenneth M. , Valenstein Edward 
Published: January 2005

AbstractIf patients with left‐sided hemispatial neglect bisect lines incorrectly because hemianopia or sensory hemiinattention prevents them from seeing how far the line extends to the left, a strategy that ensures their seeing the left side of the line in their normal field should improve performance. If patients have hemispatial hypokinesia, moving the line toward the normal half of body space should improve performance.Six patients with left‐sided neglect from right hemisphere infarctions were required to identify a letter at either the right or the left end of a line before bisecting that line. The task was given with the lines placed at either the right, the center, or the left of the body midline.Performance in trials when subjects were required to look to the left before bisecting a line did not differ from when they were required to look right. Performance was significantly better when the line was placed to the right side of the body than to the left. These observations support the hypothesis that patients with hemispatial neglect have hemispatial hypokinesia. An alternative hypothesis is that these subjects had a hemispatial memory defect. Although they saw the left side of the line in their normal field, they were incapable of forming a stable trace and performed as if they did not see the left side of the line.
Phosphorylation of the membrane‐bound acetylcholine receptor: Inhibition by diphenylhydantoin
Authors: Gordon Adrienne S. , Milfay Dale , Diamond Ivan 
Published: January 2005

AbstractPurified postsynaptic membranes can be used as a model system to study the regulation of synaptic membrane proteins. These membranes contain protein kinase activity that phosphorylates the acetylcholine receptor (AChR). We find that diphenylhydantoin (DPH) interacts with these membranes to inhibit phosphorylation of the membrane‐bound AChR. DPH appears to alter the availability of postsynaptic membrane proteins for phosphorylation by a synaptic membrane protein kinase. The concentration of DPH that produces half‐maximal inhibition of AChR phosphorylation is about 5 × 10−5 M. This suggests that one of the specific effects of DPH in the nervous system may be related to inhibition of phosphorylation of postsynaptic membrane proteins.
Osmotic opening of the blood‐brain barrier and local cerebral glucose utilization
Authors: Pappius H. M. , Savaki H. E. , Fieschi C. , Rapoport S. I. , Sokoloff L. 
Published: January 2005

AbstractThe effects of osmotic opening of the blood‐brain barrier (BBB) on local cerebral glucose utilization (LCGU) were studied in rats with the carbon 14–deoxyglucose method. The BBB was opened to Evans blue dye by unilateral carotid artery perfusion with hypertonic mannitol solution. (14C)‐Deoxyglucose was injected 15 minutes or two to three hours later. Osmotic opening of the BBB resulted in focal areas of intense LCGU in affected areas of the perfused hemisphere. In the contralateral hemisphere, glucose utilization was diminished, especially in cerebral cortical areas. The effects were reversible. LCGU was normal in both hemispheres when permeability to Evans blue returned to normal two to three hours after carotid perfusion. In 3 to 5 experiments the administration of high doses of intravenous diazepam immediately following carotid artery perfusion with mannitol prevented an increase in LCGU in the perfused hemisphere but did not prevent lowering of LCGU in the contralateral hemisphere. Increased LCGU following osmotic opening of the BBB was not accompanied by a rise in local cerebral blood flow as measured by the iodoantipyrine method. Blood flow was, in fact, significantly decreased.
Bolus versus steady‐state infusion for determination of CSF outflow resistance
Authors: Sullivan Humbert G. , Miller J. Douglas , Griffith Richard L. , Carter Walter , Rucker Sarah 
Published: January 2005

AbstractFor rapid changes in cerebrospinal fluid volume an exponential relationship was demonstrated between CSF pressure and CSF volume in 15 cats. This relationship was valid over a CSF pressure range from 7 to 50 mm Hg and for acute increases of up to 9% of total CSF volume (∼ 13 ml for humans). Our data agree well with previous reports for the cat. A similar relationship has been shown in the dog and in humans. It has been claimed that, given the equations for CSF bulk flow and the exponential relationship between CSF pressure and CSF volume, one can calculate CSF outflow resistance by observing the decay of CSF pressure after a bolus injection into the CSF space. This claim was evaluated in an additional 18 cats. In these animals CSF outflow resistance calculated by the bolus method was compared with resistance calculated by a steady‐state infusion method over the CSF outflow resistance range of 74 to 293 mm Hg/ml min−1. Resistance calculated by the bolus method underestimated resistance calculated by the steady‐state method, and this underestimate grew larger with increasing resistance. The bolus technique is therefore not a valid method for determining CSF outflow resistance. The explanation offered for these results is that the decay of CSF pressure after a bolus injection into the CSF space occurs not only because of runoff of the injected volume of CSF but also because of “pressure relaxation” of the brain parenchyma around the CSF space. The phenomenon of pressure relaxation was not considered in developing the equation for calculation of CSF outflow resistance by the bolus technique. The time dependency of pressure relaxation allows for a fundamental element of hysteresis within the CSF space. A method of quantifying this element of hysteresis is suggested.
Cerebral color blindness: An acquired defect in hue discrimination
Authors: Pearlman Alan L. , Birch Jennifer , Meadows John C. 
Published: January 2005

AbstractIn contrast to the traditional view that striate visual cortex (area 17) is surrounded by two homogeneous cortical areas (areas 18 and 19), recent studies have shown that mammalian extrastriate visual cortex contains several anatomically and functionally distinct subregions. One such region, the V‐4 complex of the rhesus monkey, is highly specialized for the analysis of color information, suggesting that a lesion in a homologous region might produce a defect in color vision while sparing other visual functions.We have studied a patient whose clinical syndrome supports this suggestion: a 44‐year‐old man with normal color vision suffered two cerebral infarctions that produced first a right and then a left superior homonymous quadrantanopia and also caused prosopagnosia, topographical disorientation, and severely impaired color vision. Computed tomography demonstrated extensive lesions in both inferior occipital lobes in the territories of the lateral branches of the posterior cerebral arteries, involving the lingual and medial occipitotemporal gyri bilaterally; these gyri contain the inferior portion of striate cortex and segments of extrastriate visual cortex. The patient had no difficulty in giving the correct color names associated with common objects presented either verbally or in outline drawings. Standardized testing with the Farnsworth‐Munsell 100‐hue test, the Nagel anomaloscope, and a method that tests for just‐noticeable differences between monochromatic stimuli all showed that the patient's ability to distinguish one color from another was markedly impaired but not totally absent. In contrast, visual acuity, reading, visually guided eye movements, and stereopsis were normal. Cells in the V‐4 complex of monkey extrastriate cortex are highly specialized for distinguishing one color from another; the hue discrimination deficit that was demonstrated in this patient with cerebral color blindness indicates that a region or regions with similar function has been damaged.
Mucocutaneous eruptions due to antiepileptic drug therapy in children
Authors: Pollack Michael A. , Burk Peter G. , Nathanson Gerald 
Published: January 2005

AbstractThree children with bullous erythema multiforme and 1 with toxic epidermal necrolysis associated with antiepileptic drug therapy are described. One patient is unique because of seven mucocutaneous eruptions caused by three classes of antiepileptic drugs. Lymphocyte stimulation by antiepileptic drugs could not be demonstrated in the 2 patients in whom appropriate studies were performed, and no precipitating antibodies to antiepileptic drugs were found. Observation of four diagnostic and therapeutic principles, which are illustrated by the course of our patients, may reduce the incidence of life‐threatening mucocutaneous eruptions and simplify the long‐term management of individuals in whom such reactions occur.
Alzheimer neurofibrillary tangles in diseases other than senile and presenile dementia
Authors: Wisniewski Krystyna , Jervis George A. , Moretz Roger C. , Wisniewski Henryk M. 
Published: January 2005

AbstractA brief description is given of neurofibrillary changes of the paired helical filament type in a variety of chronic neurological diseases. These include subacute sclerosing panencephalitis, lead encephalopathy, tuberous sclerosis, Down syndrome, Hallervorden‐Spatz disease, and lipofuscinosis. In these conditions, with the exception of Hallervorden‐Spatz disease, neurofibrillary changes were previously recognized but paired helical filaments were identified only in some cases. Moreover, in the present series, the age of patients at death was often younger than in previously recorded cases.
The decreasing incidence of primary intracerebral hemorrhage: A population study
Authors: Furlan Anthony J. , Whisnant Jack P. , Elveback Lila R. 
Published: January 2005

AbstractThis population study describes the experience with primary intracerebral hemorrhage (PIH) in residents of Rochester, MN, for the 32‐year period from 1945 through 1976. The average annual age‐adjusted incidence rate for PIH was 12.1 per 100,000 population, and the incidence for all cases of spontaneous intracerebral hemorrhage was 15.2 per 100,000. The PIH rates were higher for males than for females, and they increased steadily with age. When patients on long‐term anticoagulant therapy were excluded, there was a steady decrease in the average annual age‐adjusted incidence rate for PIH in each succeeding 8‐year interval since 1945. Prehemorrhage hypertension, present overall in 89% of patients, was much more frequent and severe in the earlier years of the study. The frequency and severity of prehemorrhage hypertension also varied inversely with age in the population with PIH. The median age at the onset of PIH increased from 65 years for the period 1945 through 1952 to 71 years for 1969 through 1976.
Effect of phenytoin on bone and vitamin D metabolism
Authors: Bell Rodney D. , Pak Charles Y. C. , Zerwekh Joseph , Barilla Donald E. , Vasko Michael 
Published: January 2005

AbstractCalcium and vitamin D metabolism were evaluated in 5 adult epileptic patients before and during treatment with phenytoin. Significant decreases occurred in serum concentrations of calcium, albumin, and 25‐hydroxycholecalciferol. The decreases in serum calcium paralleled those in serum albumin. Significant increases occurred in serum alkaline phosphatase and 1α,25‐dihydroxycholecalciferol, in urinary hydroxyproline, and in the fractional gastrointestinal absorption of calcium. Urinary cyclic adenosine monophosphate and serum parathyroid hormone did not change.The results suggest that the bone disease resulting from phenytoin therapy may be associated with a deficiency of 25‐hydroxycholecalciferol and not of 1α,25‐dihydroxycholecalciferol, and that reduced gastrointestinal absorption of calcium or changes in parathyroid function may not be necessary for the development of bone disease.
Debrancher deficiency: Neuromuscular disorder in 5 adults
Authors: DiMauro Salvatore , Hartwig Geoffrey B. , Hays Arthur , Eastwood Abraham B. , Franco Richard , Olarte Marcelo , Chang Myung , Roses Allen D. , Fetell Michael , Schoenfeldt Richard S. , Stern Lawrence Z. 
Published: January 2005

AbstractFive patients, 4 men and 1 woman, had adult‐onset and slowly progressive weakness. There was distal wasting in 2, hepatomegaly in 3, and congestive heart failure in 2. Electromyography showed a mixed pattern with abundant fibrillations. Serum creatine phosphokinase was increased 5‐to 45‐fold. Blood glucose failed to respond to epinephrine or glucagon, and venous lactate did not rise after ischemic exercise. Muscle biopsy showed vacuolar myopathy affecting both fiber types. By electron microscopy the vacuoles corresponded to large pools of glycogen not limited by a membrane. Glycogen concentration was 3 to 5 times normal in muscle and 7 to 21 times normal in erythrocytes. In the presence of iodine, muscle glycogen showed a spectrum characteristic of phosphorylase‐limit‐dextrin. Debrancher activity was measured by a spectrophotometric assay and by a radioactive reverse reaction. The activity was lacking in muscle and erythrocytes of 4 patients according to both assays; in 1 patient the reverse reaction was not impaired.Though previously reported in only 5 patients, debrancher deficiency myopathy may not be rare and should be considered in the differential diagnosis of adult‐onset hereditary myopathies.
Paraneoplastic vasculitis of nerve: A remote effect of cancer
Authors: Johnson Peter C. , Rolak Loren A. , Hamilton Robert H. , Laguna Jose F. 
Published: January 2005

AbstractThe development of mononeuritis multiplex brought three men to medical attention weeks to months prior to the diagnosis of a malignancy. In each case, sural nerve biopsy exhibited active wallerian degeneration with vasculitis. Two of the men died and at autopsy were found to have clinically unrecognized oat cell carcinoma and carcinomatous sensory neuropathy with brainstem encephalitis. The third patient is still alive but was found to have a lymphoma and has subsequently developed a liposarcoma. The vasculitis in all 3 cases seems limited mainly to the peripheral nervous system. Mononeuritis multiplex due to vasculitis may represent a new remote effect of cancer on the nervous system.
Abnormal auditory evoked potentials in hereditary motor‐sensory neuropathy
Authors: Satya‐Murti Saty , Cacace Anthony T. , Hanson Peggy A. 
Published: January 2005

AbstractFrom a kinship with a dominantly inherited motor‐sensory neuropathy, we studied 2 brothers with brainstem auditory evoked potentials and behavioral audiometric tests. They had abnormal prolongation of I‐III interpeak intervals. Wave V was poorly developed. Conventional audiometric tests did not reveal a peripheral hearing loss. It is probable that their auditory nerves and spiral ganglia are undergoing a pathophysiological process analogous to that of their peripheral nerves.
Cytomegalovirus infection of the adult nervous system
Authors: Duchowny Michael , Caplan Louis , Siber George 
Published: January 2005

AbstractIn 2 patients, 1 with brachial plexus neuropathy and another with relapsing chronic encephalitis, the acute neurological syndrome was accompanied by fever, tachycardia, abnormal liver function, and atypical lymphocytosis. Cytomegalovirus (CMV) infection was documented in both patients by viral isolation and a fourfold rise in complement‐fixation titer. CMV may produce peripheral neuropathy, brachial plexus neuropathy, or an acute or chronic meningoencephalitis in previously healthy adults without immune deficiency.
Self‐limited granulomatous angiitis of the cerebellum
Authors: Beresford H. Richard , Hyman Roger A. , Sharer Leroy 
Published: January 2005

AbstractA middle‐aged woman presented with clinical evidence of rapidly progressive cerebellar dysfunction and had radiographic evidence of a cerebellar mass. Biopsy of the lesion disclosed granulomatous angiitis. She recovered completely, the only treatment being dexamethasone given for five days after surgery.
Does a defect of energy metabolism in the nerve fiber underlie axonal degeneration in polyneuropathies?
Authors: Spencer Peter S. , Sabri Mohammad I. , Schaumburg Herbert H. , Moore Cyril L. 
Published: January 2005

AbstractA number of chemically unrelated neurotoxic compounds and several types f metabolic abnormalities cause strikingly similar patterns of distal symmetrical polyneuropathy in humans and animals. Experimental studies with laboratory species have demonstrated that many toxic polyneuropathies are associated with distal and retrograde axonal degeneration occurring in vulnerable nerve fiber tracts in the central as well as the peripheral nervous system. This has been termed central‐peripheral distal axonopathy.Recent observations from the authors' laboratories regarding (1) the spatial‐temporal evolution of nerve fiber degeneration in experimental toxic neuropathies and (2) the inhibition of glycolytic enzymes by chemically unrelated neurotoxic compounds point to a common metabolic basis for many distal axonopathies. It is postulated that neurotoxic compounds deplete energy supplies in the axon by inhiniting nerve fiber enzymes required for the maintenance of energy synthesis. Resupply of enzymes from the neuronal soma fails to meet the increased demand for enzyme replacement in the axon, causing the concentration of enzymes to drop in distal regions. This leads to a local blockade of energy‐dependent axonal transport, which produces a series of pathological changes culminating in distal nerve fiber degeneration. The idea provides a working hypothesis with which to study the cause of inherited and acquired human and animal polyneuropathies.
Intracellular redox states under halothane and barbiturate anesthesia in normal, ischemic, and anoxic monkey brain
Authors: Sundt Thoralf M. , Anderson Robert E. , Michenfelder John D. 
Published: January 2005

AbstractCortical reflectance, mean arterial blood pressures, electroencephalograms, and cortical blood flow were continuously recorded together with fluorescence of reduced pyridine nucleotides (PN) at various carbon dioxide tensions before, during, and following middle cerebral artery occlusion in 10 squirrel monkeys receiving halothane or barbiturate anesthesia. Measurements were continued through a nitrogen breathing cycle and to death produced by anoxia. The anesthetic agent produced no detectable differences in PN fluorescence in cerebral tissue during ischemia and anoxia. The known cerebral protective action of barbiturates is apparently unrelated to the intracelular redox state.
The TO strains of Theiler's viruses cause “slow virus—like” infections in mice
Authors: Lipton Howard L. , Dal Canto Mauro C. 
Published: January 2005

AbstractIntracerebral inoculation of mice with tissue culture–adapted TO strains of Theiler's mouse encephalomyelitis viruses results in a clinical disease consisting of spastic paralysis due to demyelination after a lengthy incubation period. Thus, in effect, these ordinary picornaviruses are capable of causing a slow infection in their natural host, the mouse. In addition, through the use of tissue culture–adapted virus stocks, virus content in mouse tissues now can be accurately quantified by standard plaque assay.
Botulism, type A, and treatment with guanidine
Authors: Kaplan Jonathan E. , Davis Larry E. , Narayan Venkat , Koster John , Katzenstein David 
Published: January 2005

AbstractIn a double‐blind crossover study in which patients received placebo or active drug for varying periods, we evaluated the ability of guanidine hydrochloride (20 to 35 mg/kg per day perorally) to improve the rate of recovery in patients with moderate or severe botulism, type A, intoxication. Among 14 patients who received conventional botulism therapy, there was no improvement in recovery rate in those who received guanidine compared with the nontrated group. Individual patients in the treated group showed neither an acceleration in their rate of improvement when they received guanidine nor a regression in their progress when the drug was stopped. Individual patients, likewise, noted no subjective improvement when they received the drug compared with the placebo. Treatment with guanidine does not enhance recovery from botulism.
Ocular tilt reaction with peripheral vestibular lesion
Authors: Halmagyi G. Michael , Gresty Michael A. , Gibson William P. R. 
Published: January 2005

AbstractFollowing inadvertent destruction of the left vestibular labyrinth during stapedectomy, a patient developed a transient abnormality of posture consisting of leftward ocular counterrolling, leftward head tilting, and a right‐over‐left skew deviation. This postural pattern, known as the “ocular tilt reaction,” is the normal compensatory response of the dependent utricle to tilting. In this patient, the unopposed action of the intact right utricle was presumably responsible for the appearance of a normal leftward ocular tilt reaction.
Cytoarchitectonic abnormalities in developmental dyslexia: A case study
Authors: Galaburda Albert M. , Kemper Thomas L. 
Published: January 2005

AbstractA patient is reported who had a well‐documented history of developmental dyslexia. Reading difficulties were present in other male members of the immediate family. Gross anatomical and cytoarchitectonic analysis of the brain in serial sections showed a consistently wider left cerebral hemisphere, an area of polymicrogyria in the left temporal speech region, and mild cortical dysplasias in limbic, primary, and association cortices of the left hemisphere.
γ‐Hydroxybutyric acid is not a GABA‐mimetic agent in the spinal cord
Authors: Osorio Ivan , Davidoff Robert A. 
Published: January 2005

Abstractγ‐Hydroxybutyric acid (GHB), a pharmacologically active central nervous system constituent, has been postulated to function as a γ‐aminobtyric acid (GABA) agonist. This hypothesis was tested directly on GABAergic synapses in isolated, superfused frog spinal cord.Addition of GHB to the superfustate produced effects on primary afferent terminals that were distinctly different from the effects of GABA. Thus, although both compounds depressed dorsal root potentials, GHB hyperpolarized terminals while GABA depolarized the same structures. The GABA responses were antagonized by bicuculline and picrotoxin, but these alkaloids did not change GHB's actions. In addtion, GHB altered neither high‐affinity uptake by cord slices, nor potassium‐evoked release of tritiated GABA from them. GHB did not directly release GABA from spinal slices preloaded with [3H]GABA.These observations suggest that the central nervous system actions of GHB are not dependent upon its ability to activate GABAergic synapses or to modify GABAergic mechanisms.