bluuebunny/crossref_metadata_2025_split huggingface dataset:
Creutzfeldt‐Jakob disease: A case of 16 years' duration
Authors: Cutler Neal R. , Brown Paul W. , Narayan Tina , Parisi Joseph E. , Janotta Frank , Baron Henry 
Published: January 2005

AbstractA 46‐year‐old man with Creutzfeldt‐Jakob disease confirmed postmortem had a 16‐year course of very slowly progressing incoordination and mental deterioration, suggesting Alzheimer's disease. The disease course transformed abruptly into a 7‐week terminal phase of florid Creutzfeldt‐Jakob disease. Dementing illnesses of unknown cause were present in the patient's paternal lineage.
Central nervous system catecholamine metabolism in Korsakoff's psychosis
Authors: Martin Peter R. , Weingartner Herbert , Gordon Edna K. , Burns R. Stanley , Linnoila Markku , Kopin Irwin J. , Ebert Michael H. 
Published: January 2005

AbstractWe attempted to replicate previous reports of impaired central noradrenergic activity in patients with Korsakoff's psychosis. No differences were demonstrated in lumbar cerebrospinal fluid concentrations of norepinephrine or its major metabolite, 3‐methoxy‐4‐hydroxypheny glycol (MHPG), or in the contribution of plasma to cerebrospinal fluid free MHPG in 6 patients with clinically well characterized Korsakoff's psychosis and 8 age‐matched healthy, normal volunteers. Likewise, cerebrospinal fluid concentrations of the major metabolites of dopamine (homovanillic acid) and serotonin (5‐hydroxyindoleacetic acid) were the same in patients and controls.
Pulmonary lymphatic flow alterations during intracranial hypertension in sheep
Authors: Simon R. P. , Bayne L. L. 
Published: January 2005

AbstractThe role of intracranial hypertension in the genesis of neurogenic pulmonary edema was studied in 25 sheep; cardiopulmonary hemodynamics (aortic, pulmonary arterial, and left atrial pressures and cardiac output) and fluid and protein movement across the pulmonary capillary bed (efferent pulmonary lymph flow and lymph/plasma protein ratio) were monitored. Only when intracranial pressure was raised to equal the baseline mean systemic pressure (75 to 120 Torr) did we observe the expected Cushing response of increased aortic pressure, or any alteration in pulmonary hemodynamics or fluid movement. When pulmonary changes did occur, they included an increase in pulmonary arterial pressure of between 5 and 15 Torr without any notable rise in left atrial pressure, and a sustained doubling of the pulmonary lymph flow with no dilution of the lymph/plasma protein ratio. In 3 additional animals cerebral ischemia alone produced an elevation in systemic pressure (74 Torr over baseline) without change in pulmonary arterial pressure, left atrial pressure, or pulmonary lymph flow. Thus, intracranial hypertension and ischemia both affect systemic pressure, but only the elevated intracranial pressure is followed by changes in the pulmonary circuit. We suggest that these changes in pulmonary vascular pressure, independent of changes in left atrial pressure, produce increased pulmonary transcapillary fluid flux that may result in neurogenic pulmonary edema.
A quantitative model for the in vivo assessment of drug binding sites with positron emission tomography
Authors: Mintun Mark A. , Raichle Marcus E. , Kilbourn Michael R. , Wooten G. Frederick , Welch Michael J. 
Published: January 2005

AbstractWe propose an in vivo method for use with positron emission tomography (PET) that results in a quantitative characterization of neuroleptic binding sites using radiolabeled spiperone. The data are analyzed using a mathematical model that describes transport, nonspecific binding, in the brain. The model demonstrates that the receptor quantities Bmax (i.e., the number of binding sites) and KD−1 (i.e., the binding affinity) are not separably ascertainable with tracer methodology in human subjects. We have, therefore, introduced a new term, the binding potential, equivalent to the product BmaxKD−1, which reflects the capacity of a given tissue, or region of a tissue, for ligand‐binding site interaction. The procedure for obtaining these measurements is illustrated with data from sequential PET scans of baboons after intravenous injection of carrier‐added [18F]spiperone. From these data we estimate the brain tissue nonspecific binding of spiperone to be in the range of 94.2 to 95.3%, and the regional brain spiperone permeability (measured as the permeability–surface area product) to be in the range of 0.025 to 0.036 cm3/(s·ml). The binding potential of the striatum ranged from 17.4 to 21.6; these in vivo estimates compare favourably to in vitro values in the literature. To our knowledge this represents the first direct evidence that PET can be used to characterize quantitatively, locally and in vivo, drug binding sites in brain. The ability to make such measurements with PET should permit the detailed investigation of diseases thought to result from disorders of receptor function.
Elevated neutral protease activity in myelin from brains of patients with multiple sclerosis
Authors: Sato Shuzo , Quarles Richard H. , Brady Roscoe O. , Tourtellotte Wallace W. 
Published: January 2005

AbstractIncubation of human myelin at neutral PH resulted in the proteolytic conversion of the myelin‐associated glycoprotein to a lower molecular weight derivative (dMAG) and the degradation of basic protein. The formation of dMAG occured much more rapidly than the degradation of basic protein. The formation of dMAG and the degradation of basic protein both occured significantly more rapidly in myelin preparations purified from brains of patients with multiple sclerosis than in preparations from control brain. The results suggest that this neutral protease associated with myelin may function in the pathogenesis of demyelinating diseases such as multiple sclerosis.
Long‐term corticosteriod treatment of myasthenia gravis: Report of 116 Patients
Authors: Pascuzzi Robert M. , Coslett H. Branch , Johns T. R. 
Published: January 2005

AbstractOne hundred sixteen patients, aged 8 to 82 years, with myasthenia gravis were treated with prednisone, 6o to 80 mg daily, until the onset of improvement, followed by lower‐dose alternate‐day therapy of several years' duration. Of all patients, 80.2%achived wither remission(27.6%) or marked improvement (52.6%). Moderate improvement occurred in 14.7%, and 5.2% showed no improvement. Increasing age correlated with a favorable outcome, but sex, duration of illness prior to treatment, severity and distribution of weaskness at the time of onset of treatment, and presence of thymoma were not factors in the response to therapy.
Chromatin structure in dementia
Authors: McLachlan D. R. Crapper , Lewis P. N. , Lukiw W. J. , Sima A. , Bergeron C. , Boni U. 
Published: January 2005

AbstractNuclei extracted from neocortex of patients with Alzheimer's disease and treated with micrococcal nuclease release a population of dinucleosomes that contain an increase in the linker histones H1° and H1ºº. Five other degenerative brain diseases that clinically resemble Alzheimer's disease do not result in these changes, although Pick's disease is associated with an increase in H1 on dinucleosomes. Histones from nuclei of patients with Alzheimer's disease are also more resistant to salt‐induced release from chromatin than are those from age‐matched control subjects. These results support the hypothesis that an alteration in chromatin structure is a marker for Alzheimer's disease.
Effects of hypoxia‐ischemia on monoamine metabolism in the immature brain
Authors: Silverstein Faye , Johnston Michael V. 
Published: January 2005

AbstractWe measured acute changes in monoamine metabolites in corpus striatum of immature rat pups exposed to hypoxiaischemia, hypoxia alone, or total global ischemia. Carotid ligations and two hours of 8% oxygen environment in 7‐day‐old pups led to asymmetrical turning behavior, a 70% decrease in endogenous striatal dopamine levels, and a 125% increase in homovanillic acid (HVA) concentrations on the side of ligation. In contrast, hypoxia alone and total global ischemia alone were not associated with HVA level elevation. Elevation of HVA level with hypoxia‐ischemia showed a threshold effect between 1 and 1.5 hours, and this time course paralleled that for production of gross morphological changes in rats raised to maturity. The data suggest that dopamine release from striatal nerve terminals is associated with events causing brain injury during perinatal hypoxia‐ischemia. Tissue HVA in the animal model appears to be a quantitative marker for the effects of the insult on a population of nerve terminals.
Cerebrospinal fluid vasopressin and increased intracranial pressure
Authors: Sørensen Per Soelberg , Gjerris Flemming , Hammer Mogens 
Published: January 2005

AbstractCerebrospinal fluid and plasma vasopressin were measured in patients with cerebral disorders associated with varying levels of elevated intracranial pressure. The mean cerebrospinal fluid vasopressin concentration was significantly increased in patients with pseudotumor cerebri (2.0 ± 0.2 [SEM] pg/ml), intracranial tumor (2.3 ± 0.4 pg/ml), and intracranial hemorrhage (1.9 ± 0.3 pg/ml) compared with control patients (1.2 ± 0.1 pg/ml). A significant relationship was found between intracranial pressure and the cerebrospinal fluid vasopressin concentration within all groups of patients and in the whole sample as well (r = 0.79; < 0.001). In the groups of patients with intracranial tumor, hydrocephalus, and intracranial hemorrhage, some individuals showed plasma vasopressin concentratioons inappropriate to the corresponding plasma osmolality, but no relationship was found between intracranial pressure and plasma vasopressin concentration. It is suggested that increased intracranial pressure is a stimulus to centrally released vaso‐Pressin. The clinical importrance of increased cerebrospinal fluid vasopressin concentrations is still not known.
The functional anatomy of frontal lobe neglect in the monkey: Behavioral and quantitative 2‐deoxyglucose studies
Authors: Deuel Ruthmary K. , Collins Robert C. 
Published: January 2005

AbstractA syndrome of hemisensory neglect follows damage to frontal association areas in monkeys and humans. To study the syndrome we removed frontal association cortex from the right hemisphere of macaque monkeys. After operation, behavioral testing showed conditional deficits in visual, somatosensory, and motor responses contralateral to the lesion. Brains of animals were studied with [14C]2‐deoxyglucose autoradiography to evaluate functional neuroanatomical changes. Ipsilateral striatal and selected thalamic and midbrain nuclei exhibited depression of glucose utilization (10 to 60%). No consistent glucose utilization changes appeared in cortex or in primary motor or sensory pathways. Brains from unoperated control animals did not exhibit these changes, nor did brains from operated animals with behavioral recovery from neglect. We conclude that the symptoms of frontal lobe neglect in the monkey are the result of dysfunction within a widely distributed system of subcortical centers. The distribution of the dysfunction provides an explanation for some of the clinical features of neglect.
Ultrastructural, neurological, and glycosaminoglycan abnormalities in Lowe's syndrome
Authors: Wisniewski Krystyna E. , Kieras Fred J. , French Joseph H. , Houck George E. , Ramos Peter L. 
Published: January 2005

AbstractThe oculocerebrorenal syndrome (OCRS), Lowe's syndrome, is an X‐linked, recessive disease characterized by mental retardation, congenital corneal abnormalities and cataracts, growth failure, rickets, osseous abnormalities, renal dysfunction with periodic acidosis, hypotonia, and areflexia. Ultrastructural studies of skin biopsy specimens in three individuals with the disorder (aged 17, 9, and 8 years) revealed cytoplasmic, membrane‐bound, electron‐lucent vacuoles and some electron‐dense membranous inclusion bodies in fibroblasts and Schwann cells, as well as axonal degeneration and vascular changes. Computed tomographic scans evidenced brain atrophy. Urinary excretion of glycosaminoglycans (GAG) was four to five times greater than in normal controls. The predominant urinary GAG was a low‐sulfated chondroitin‐4‐sulfate; chondroitin‐6‐sulfate and heparan sulfate excretion levels were normal. A tenfold increase in urinary GAG excretion was found in one patient with oculocerebrorenal syndrome during periods of behavioral agitation. These findings suggest that the clinical stigmata of oculocerebrorenal syndrome may be related to a defect in GAG metabolism.
Startle epilepsy: A clinical study
Authors: Sàenz‐Lope E. , Herranz F. J. , Masdeu J. C. 
Published: January 2005

AbstractTwelve patients with perinatal anoxic encephalopathy, 1 with encephalitis, and 1 with Down's syndrome had startle epilepsy. Hemiparesis, startle‐induced seizures involving the hemiparetic side, focal electroencephalographic abnormalities, and unilateral lesions evident on computed tomographic scan were found in 6 patients, all of whom responded favorably to carbamazepine. The remaining 8 patients had severe intellectual impairment, bilateral motor deficits, generalized startle‐induced seizures, diffuse and lasting electroencephalographic abnormalities, and widespread cerebral lesions evident on computed tomographic scan. Of these, 2 patients with Lennox‐Gastaut syndrome responded to clonazepam, 4 others responded to valproic acid, and 2 others, with predominantly focal hemispheric lesions, improved on a regimen of carbamazepine.
Low T8 antigen density on lymphocytes in active multiple sclerosis
Authors: Reder Anthony T. , Antel Jack P. , Oger Joël J.‐F. , McFarland Thomas A. , Rosenkoetter Maureen , Arnason Barry G. W. 
Published: January 2005

AbstractThe median flurescence intensity of peripheral blood T8+ (T‐suppressor/cytotoxic) cells in patients with active (relapsing or progressive) or stable multiple sclerosis (MS) and in control subjects was determined with a fluorescence‐activated cell sorter using an indirect immunofluorescence assay. Median fluorescence intensity was singnificantly lower in patients with active MS than in controls. No differences in median fluorescence intensity were found between patients with stable MS and controls. Analysis of T8+ cells that had been modulated in vitro with OKT8 indicated that the resultant reduction in the median T8 antigen density per cell resulted in fewer T8+ cells being enumerated when the fluorescence‐activated cell sorter was used than when a fluorescence microscope was used. Reduced median T8 antigen density on cells as found in active MS may contribute to the apparent underrepresentation of circulating T‐suppressor cells in such patients and may be associated with reduced suppressor cell function.
Creutzfeldt‐Jakob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis
Authors: Brown Paul , Rodgers‐Johnson Pamela , Cathala Françoise , Gibbs Clarence J. , Gajdusek D. Carleton 
Published: January 2005

AbstractFive to 10% of patients with Creutzfeldt‐Jakob disease have a clinical course that extends for 2 years or more. In the present series 33 (9%) of 357 histopathologically verified cases, including 15 (7%) of 225 transmitted cases, fell into this long‐duration category, the longest transmitted case having had a 13‐year duration. As a group these cases were characterized by a higher familial representation (30%), a younger age at onset (average, 48 years), and lower frequencies of myoclonus (79%) and periodic electroencephalographic activity (45%) than occur in series of unselected cases. The clinical course most often showed a long, very slowly progressive terminal stage with both mental and physical deterioration. Although transmission of disease by primate inoculation periods and durations of illness in the long‐duration cases (64%) than in cases of shorter duration (88%), incubation periods and duration of illness in the inoculated animals bore no relation to the duration of illness in the patients. Clinical differentiation from other chronic dementing processes, particularly Alzheimer's disease, can prove impossible; however, the correct pathologica diagnosis was readily evident by light microscopic examination, and spongiosis was at least as prominent as gliosis in all but 4(15%) of the 27 patients studied postmortem, the same frequency found in case series not selected for length of illness.
Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndrome
Authors: Pavlakis Steven G. , Phillips Peter C. , DiMauro Salvatore , De Vivo Darryl C. , Rowland Lewis P. 
Published: January 2005

AbstractWe report on two patients who have a mitochondrial myopathy, encephalopathy, lactic acidosis, and recurrent cerebral insults that resemble strokes (MELAS). These two and nine other reported patients share the following features: ragged red fibers evident on muscle biopsy, normal early development, short stature, seizures, and hemiparesis, hemianopia, or cortical blindness. Lactic acidemia is a common finding. We believe that MELAS represents a distinctive syndrome and that it can be differentiated from two other clinical disorders that also are associated with mitochondrial myopathy and cerebral disease: Kearns‐Sayre syndrome and the myoclonus epilepsy ragged red fiber syndrome. Existing information suggests that MELAS is transmitted by maternal inheritance. The ragged red fibers suggest an abnormality of the electron transport system, but the precise biochemical disorders in these three clinical syndromes remain to be elucidated.
Peripheral beta‐adrenergic blockade treatment of parkinsonian tremor
Authors: Foster Norman L. , Newman Richard P. , Lewitt Peter A. , Gillespie Marjorie M. , Larsen T. Andreo , Chase Thomas N. 
Published: January 2005

AbstractThe effect of nadolol, a peripherally acting beta‐adrenergic blocker, on resting, postural, and intention tremor was examined in 8 patients with idiopathic Parkinson's disease whose motor symptoms, other than tremor, were well controlled with conventional medications. In a double‐blind, placebo‐controlled, crossover design, patients received 80 to 320 mg of nadolol for six weeks while continuing their previous therapeutic regimen. Accelerometer readings showed a 34% reduction (p < 0.025) in tremor distance, but no change in tremor frequency, during nadolol therapy. Maximum benefit was achieved with a dose of 240 mg, when resting tremor improved 54%, postural tremor 32%, and intention tremor 54%. Physician ratings and patient reports supported the accelerometer results. Nadolol appears to be a safe, effective adjunct to current dopaminergic and anticholinergic therapy for severe tremor in Parkinson's disease.
Diagnostic importance of immunological markers in lymphoma involving the central nervous system
Authors: Ezrin‐Waters Cheryl , Klein Michel , Deck John , Lang Anthony E. 
Published: January 2005

AbstractIn an elderly woman, lymphoma involved the central nervous system and unusual nonlymphoreticular sites. Cerebrospinal fluid cell surface marker studies revealed a clone of malignant B cells. Studies of peripheral blood demonstrated a systemic disturbance of immune regulation similar to Epstein‐Barr virus infections and the acquired immunodeficiency syndrome.
Alexander's law: Its behavior and origin in the human vestibulo‐ocular reflex
Authors: Robinson David A. , Zee David S. , Hain Timothy C. , Holmes Allistair , Rosenberg Lisa F. 
Published: January 2005

AbstractAlexander's law refers to the phenomenon in which the spontaneous nystagmus of a patient with a vestibular lesion is more intense when the patient looks in the quick‐phase than in the slow‐phase direction. Alexander's law was investigated in normal subjects as well as patients with vestibular lesions. During sinusoidal rotations of normal subjects, there was no trace of this phenomenon when subjects looked 25 degrees left and right in the dark. The phenomenon of Alexander's law is therefore created centrally and is not due to any mechanical properties of the orbit.During rotation at constant velocity in the dark, normal subjects did weakly demonstrate Alexander's law because of a mild gaze‐evoked nystagmus present in normal subjects in the dark. Gaze‐evoked nystagmus from a cerebellar lesion involved a pronounced demonstration of Alexander's law during rotatory nystagmus. In patients with a vestibular lesion and a mild spontaneous nystagmus in the dark that obeyed Alexander's law, the nystagmus reversed upon far gaze in the slow‐phase direction. We propose that in patients with a vestibular lesion, the phenomenon of Alexander's law is created by the sum of vestibular nystagmus and an abnormally large gaze‐evoked nystagmus that is consequent to the vestibular lesion. This conclusion has a number of neurological implications concerning the ways in which the nervous system attempts to compensate for vestibular lesions.
Autosomal recessive distal muscular dystrophy: A comparative study with distal myopathy with rimmed vacoule formation
Authors: Nonaka Ikuya , Sunohara Nobuhiko , Satoyoshi Eijiro , Teresawa Kenjiro , Yonemoto Kyozo 
Published: January 2005

AbstractTo clarify the clinical and morphological characteristics of distal muscular dystrophy, clinical and pathological material from 4 affected persons was compared with similar studies in 4 patients with distal myopathy with rimmed vacuole formation. Although these two forms of autosomal recessive distal myopathy with onset in young adulthood were highly similar in their clinical symptoms, histochemical and electron microscopic findings of muscles subjected to biopsy were quite different. The muscle abnormalities in distal muscular dystrophy were almost the same as those in Duchenne muscular dystrophy, showing massive fiber necrosis followed by active fiber regeneration. In contrast, distal myopathy with rimmed vacuole formation showed a progressive muscle fiber atrophy and loss, rimmed vacuoles in the sarcoplasm, and no apparent fiber necrosis or regeneration.
Crystalline encephalopathy: Cerebral immunoprotein deposits and isolated angiitis
Authors: Pezeshkpour G. , Stuart Thomas D. , Estridge M. N. 
Published: January 2005

AbstractBrain biopsy specimens from a 38‐year‐old woman with adversive seizures and bifrontal mass lesions evident on computed tomographic scans showed extracellular and intracellular deposits of crystallized protiens. These were morphologically identical to the immunoglobylin crystals seen in reactive or neoplastic plasma cells and by peroxidase‐antiperoxidase methods were found to contrain polyclonal immunoglobulins. In addition, severe angiitis of the intracerebral blood vessels was present.
Leptomeningeal metastases: Reduction in regional cerebral blood flow and cognitive impairment
Authors: Siegal Tali , Mildworf Bracha , Stein Daniel , Melamed Eldad 
Published: January 2005

AbstractTwenty‐six patients with recently diagnosed leptomingeal metastases underwent multiple diagnostic procedures in order to assess possible cerebral parenchymal involvement. Pretreatment studies included detailed neurological examination, computed tomographic scan, electroencephalgram, neuropsychological assessement, psychiatric evaluation, and measurement of regional cerebral blood flow (rCBF) using the xenon 133 inhalation technique. At diagnosis 50% of the patients had symptoms and signs of cerebral dysfunction. Computed tomographic scanning and electroencephalography detected abnormalities in 42% and 58%, respectively. Scoring was abnormally low on at least three neuropsychological tests in 93% of the patients. The mean rCBF was reduced in 88% of patients by 16.1 ± 2.5% (mean ± SEM; range, 8 to 45%) compared with age‐matched controls (p < 0.001).rCBF was reduced and cognition was impaired in patients with or without overt cerebral manifestations. Neuropsychological testing and rCBF measurement may be helpful in early detection of cerebral dysfunction in patients with leptomeningeal metastases.