bluuebunny/crossref_metadata_2025_split huggingface dataset:
Childhood brain tumors presenting as chronic uncontrolled focal seizure disorders
Authors: Blume Warren T. , Girivin John P. , Kaufmann John C. E. 
Published: January 2005

AbstractSixteen of 35 patients (46%) 21 years old or less who underwent surgical resection of a longstanding epileptic focus harbored tumors. The median duration of the seizure disorder prior to operation was 6.0 years for patients with and 7.6 years for those without tumor. Among the 35 patients, tumor was more common when intelligence and results of neurological examination were each normal, a plausible cause for uncontrolled seizures was lacking, and persistent focal delta activity occurred in a majority of electroencephalograms (EEGs). The type of seizures and the distribution of EEG spikes failed to distinguish patients with tumor from those without. Multifocal EEG spikes appeared in a majority of each group.
Toxic effect of phenytoin on developing cortical neurons in culture
Authors: Swaiman Kenneth F. , Neale Elaine A. , Schrier Bruce K. , Nelson Phillip G. 
Published: January 2005

AbstractStudies were undertaken to determine the effect of chronic phenytoin exposure on developing neurons. Cerebral cortex from 16‐day fetal mice was utilized to prepare primary dissociated cell cultures. Phenytoin weas added to the cultures 10 days after plating and the cultures were harvested on day 17. Cortical cultures were assayed for neuronal cell number by phase microscopy and for high‐affinity uptake of 3H‐labeled γ‐aminobutyric acid (GABA) by both radioautography and scintillation spectrometry.Neuronal cell counts demonstrated a highly significant decrement in the number of neurons in cultures exposed to phenytoin at 15, 25, and 50 μg/ml. 3H‐GABA‐labeled neurons constituted 13% of the neurons present in both control and phenytoin‐exposed cultures.These data indicate that phenytoin is toxic to cortical neurons in culture and that GABAergic neurons are affected to the same extent as the total neuronal population.
Serum mitochondrial aspartate aminotransferase in patients with polymyositis
Authors: Ogasahara Saburo , Takahashi Mitsuo , Kang Jin , Yorifuji Shiro , Wada Keiji , Hazama Takanori , Takeuchi Hiroaki , Tarui Seiichiro 
Published: January 2005

AbstractThe clinical significance of serum aspartate aminotransferase (GOT) isozymes was studied in 18 patients with polymyositis. Abnormally high levels of mitochondrial GOT (mGOT) (6.2 ± 1.2 IU/L, mean ± SEM; normal, <2.0 IU/L) and cytosol GOT (sGOT) (95 ± 21.6 IU/L; normal, <25 IU/L) were observed in sera. In polymyositic muscles, the sGOT level was significantly decreased but mGOT was not. The levels of serum sGOT and mGOT and the ratio of mGOT/tGOT before corticosteroid therapy correlated well with the severity of muscle weakness. Serial determination of CPK, sGOT, and mGOT during corticosteroid therapy revealed that mGOT most rapidly returned to normal. Exercise did not increase serum mGOT in polymyositis.
A dominantly inherited syndrome with continuous motor neuron discharges
Authors: Ashizawa Tetsuo , Butler Ian J. , Harati Yadollah , Roongta Suresh M. 
Published: January 2005

AbstractA distinct syndrome with continuous motor neuron discharges apparently developed in seven members of a single family, involving both sexes and spanning three generations. Persistent vermiform twitching and episodic stiffness predominantly in lower extremity muscles occurred in early childhood and tended to be less severe in adulthood. In 2 patients the clinical manifestations improved with oral phenytoin and carbamazepine but not with parenteral diazepam. Insertional activity was normal, and continuous, rhythmical, normal‐appearing muscle discharges were observed on electromyography. The cerebrospinal fluid levels of homovanillic acid and 5‐hydroxyindoleacetic acid were increased in the proband. The disappearance of continuous muscle discharges during spinal anesthesia and the lack of response to diazepam indicated generation of the discharges from the proximal portion of the motor unit.
Neuromuscular dysfunction and ultrastructural pathology in children with chronic cholestasis and vitamin E deficiency
Authors: Werlin Steven L. , Harb Joseph M. , Swick Herbert , Blank Ellen 
Published: January 2005

AbstractThe ultrastructural pathology of nerve and muscle and the neurological dysfunction in children with cholestatic liver disease and vitamin E deficiency have not been previously correlated. We studied two children with this syndrome. One child, 11 years of age, had severe hyporeflexia and decreased vibratory sense. Nerve conduction was delayed. The second child, 2 years of age, was neurologically normal.Both children showed ultrastructural evidence of damage to the sural nerve and accumulation of electron‐dense deposits in the muscle fibers. Abnormalities of the nerves included disruption of the myelin sheath and separation and degeneration of the inner and outer components of the Schmidt‐Lanterman incisure.
Removal of sedative‐hypnotic antiepileptic drugs from the regimens of patients with intractable epilepsy
Authors: Theodore William H. , Porter Roger J. 
Published: January 2005

AbstractSedative‐hypnotic antiepileptic drugs have potentially toxic effects, but their removal is often thought to be difficult and dangerous. We completely withdrew all barbiturates and benzodiazepines from 78 patients with intractable epilepsy (48 inpatients and 30 outpatients). Initially, 19 patients had plasma levels of sedative drugs above the therapeutic range; 28 were taking more than one of these drugs. Dosages of nonsedative antiepileptic drugs were adjusted to provide optimal seizure control.After 6 months of outpatient follow‐up, 69 patients remained on a nonsedative regimen: 35 (51%) showed improvement in both drug toxicity and seizure control, 13 (19%) in toxicity alone, 8 (12%) in seizure control alone; 12 (16%) were unchanged, and 1 was worse. Of 9 patients restarted on sedative antiepileptic drugs by their private physicians, 4 had more toxic symptoms than at discharge, 1 had more frequent seizures, 3 were unchanged, and 1, who had had a temporal lobectomy after drug withdrawal, had less frequent seizures.Sedative drugs are not necessary for optimal seizure control, even in intractable epilepsy, and they may be safely withdrawn.
Disease activity and emotional state in multiple sclerosis
Authors: Dalos Nancy P. , Rabins Peter V. , Brooks Benjamin R. , O'Donnell Pat 
Published: January 2005

AbstractThe prevalence and nature of emotional disturbance in patients with multiple sclerosis (MS) was studied prospectively in 64 MS patients and 23 spinal cord–injured (SCI) control patients by administration of the 28‐item subscale General Health Questionnaire (GHQ). MS patients in remission had a mean GHQ score of 4.4, and patients with acute exacerbation or progressive nonremitting MS had a mean score of 15.7, a significant difference (p < 0.001). The prevalence of emotional disturbance was 90% in exacerbating or progressing patients, 39% in stable patients, and 12% in SCI control patients. The presence of emotional disturbance was not related to age, sex, or other demographic variables, to duration or severity of disease, or to the degree of disability. In the group of MS patients in remission, somatic complaints, anxiety, and social dysfunction were more prevalent than symptoms of depression.
Long‐term prognosis in two forms of childhood epilepsy: Typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci
Authors: Loiseau P. , Pestre M. , Datigues J. F. , Commenges D. , Barberger‐Gateau C. , Cohadon S. 
Published: January 2005

AbstractFindings in children seen between 1955 and 1965 during the year of onset of typical absence seizures (90 patients) or rolandic epilepsy (79 patients) were analyzed by actuarial methods. One hundred and eighteen patients were followed for more than 15 years. Rolandic epilepsy is a true benign epilepsy ending with puberty. Although school and family problems are common during the acute stage of the disease, the social adaptability of such patients is excellent. We considered only typical absences occurring as a first epileptic sign in normal children. Myoclonic or atonic absences have a poor prognosis. Many patients with simple and automatic absences experience remission 15 years after withdrawal of medication. The overall cessation rate in those experiencing absences was only 57.5%, however, and 36% of patients developed tonic‐clonic seizures. Social adaptability was often inadequate. Simple and automatic absences (constituting a homogeneous group) are not truly a benign form of epilepsy, even though prognosis for those afflicated is better than that for those with other forms of primary generalized epilepsy.
Evidence that lack of deoxyribonucleic acid repair causes death of neurons in xeroderma pigmentosum
Authors: Robbins Jay H. , Polinsky Ronald J. , Moshell Alan N. 
Published: January 2005

AbstractXeroderma pigmentosum (XP) is an autosomal recessive disorder with hypersensitivity to the lethal effects of ultraviolet radiation caused by inherited defects in deoxyribonucleic acid (DNA) repair processes. Some patients with XP develop a primary neuronal degeneration which has been thought to result from unrepaired damage in neuronal DNA. Five years ago we reported that cultured skin fibroblasts from a 12‐yearold girl with XP, who then had only one major neurological abnormality of the disease, had a sensitivity to ultraviolet radiation intermediate between that of XP patients with numerous neurological abnormalities and those with none. Recent neurological studies reveal that she has a slowly but progressively developing sensorineural deafness as well as cerebellar and motor dysfunction typical of XP. The results support the postulate that defective DNA repair is associated with premature neuron death.
Amyotrophic lateral sclerosis: Alterations in neurotransmitter receptors
Authors: Whitehouse Peter J. , Wamsley James K. , Zarbin Marco A. , Price Donald L. , Tourtellotte Wallace W. , Kuhar Michael J. 
Published: January 2005

AbstractLoss of motor neurons is the primary pathological hallmark of amyotrophic lateral sclerosis. Drug and neurotransmitter receptors are neuronal markers and can be indicators of neuronal connectivity. Knowledge of alterations in receptors in amyotrophic lateral sclerosis should contribute to our understanding of normal spinal cord neurotransmitter systems as well as of the pathophysiology of amyotrophic lateral sclerosis. We therefore used a sensitive, light microscopic in vitro labeling receptor autoradiographic technique to map and quantitate muscarinic cholinergic, glycinergic, and benzodiazepine receptors in three levels of spinal cord from six patients with amyotrophic lateral sclerosis and six age and sex‐matched control patients. In control tissues, the receptor distributions were similar in the three levels of spinal cord and also similar to those found in previous studies with animals. In amyotrophic lateral sclerosis, major reductions in receptor densities were noted in Rexed layer IX, the region containing motor neurons. Reductions were noted in other laminae as well, particularly for muscarinic receptors. The changes in muscarinic receptors were caused solely by changes in high‐affinity agonist sites. Reductions in glycine and muscarinic receptors were highly correlated with the degree of motor neuron loss found in the amyotrophic lateral sclerosis patients. The findings in this study point out the usefulness of this receptor mapping technique in understanding the changes in neuronal populations that occur in the degenerative neurological diseases.
Limitations of ultrasound in detecting cerebral ischemic lesions in the neonate
Authors: Davis Suzanne L. Wilson , Lo Warren , Filly Roy A. 
Published: January 2005

AbstractUltrasound scans of three newborn infants with cerebral ischemic lesions demonstrated on computed tomographic scan showed unexpected changes in echogenicity of brain parenchyma in addition to lateralized mass effect. Areas of increased echogenicity were present adjacent to regions of infarction identified on computed tomographic scan. The findings suggest that caution should be exercised in the interpretation of echo‐dense areas in unusual locations for hemorrhage in infants at risk for cerebral ischemic lesions.
Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis
Authors: Bradley Walter G. , Good Paul , Rasool Chaudri G. , Adelman Lester S. 
Published: January 2005

AbstractPhrenic nerves of 11 patients with amyotrophic lateral sclerosis studied postmortem contained only 33% of the normal number of large myelinated fibers (9 controls; p < 0.001). In the phrenic nerves of these patients, there were 18% fewer large myelinated fibers in the distal segment than in the proximal segment (p < 0.025). The ratio of axonal circumference to myelin lamellae in large myelinated fibers in the distal segment was 34% greater than that in control fibers (p < 0.002). The proportion of acute axonal degeneration was the same at all levels (48.0 ± 13.7%). Sural nerves of 21 patients with amyotrophic lateral sclerosis had more acute axonal degeneration and 30% fewer myelinated fibers (p < 0.05) than controls; evidence of degeneration also extended to unmyelinated fibers. The amount of axonal transport of acetylcholinesterase in 9 sural nerves determined in vitro was reduced by 24% (p < 0.05) and the apparent transport rate was reduced by 44% (p < 0.01) compared with 4 controls. These findings show that in amyotrophic lateral sclerosis a small degree of dying‐back change and of distal axonal atrophy is superimposed on the degeneration of motor neuron cell bodies, and that the disease effects spread beyond the motor neurons.
Persistent vegetative state with high cerebral blood flow following profound hypoglycemia
Authors: Agardh C.‐D. , Rosén I. , Ryding E. 
Published: January 2005

AbstractA persistent vegetative state (severe dementia) developed in a 30‐year‐old man following hypoglycemic coma. Despite the poor clinical outcome, sensory evoked response recovered between 6 and 34 months after the insult. The cerebral blood flow level at rest after 34 months was slightly above the normal range. This finding contrasts with the low cerebral blood flow regularly reported in patients who are comatose or stuporous following severe brain hypoxia‐ischemia.
Antibody responses to measles virus and canine distemper virus in multiple sclerosis
Authors: Krakowka Steven , Miele James A. , Mathes Lawrence E. , Metzler Alfred E. , Vet Med 
Published: January 2005

AbstractAge‐matched serum and cerebrospinal fluid from 20 multiple sclerosis patients and 20 control patients with other neurological diseases were examined for antibodies to radiolabeled measles virus and canine distemper virus using an immunoprecipitation polyacrylamide gel technique. No evidence for reactivity to unique canine distemper virus‐virion polypeptides in the multiple sclerosis group was obtained. Competitive binding experiments with cerebrospinal fluid using labeled and unlabeled viral antigens failed to detect preferences in binding of antibodies for canine distemper virus versus measles virus. Cerebrospinal fluid samples tested for antiviral immunoglobulin M activity using both measles and canine distemper viruses showed no activity. The results of this study failed to implicate canine distemper virus directly as a cause of multiple sclerosis.
Autosomal dominant motor system degeneration in a black family
Authors: Cooper Joanna A. , Nakada Tsutomu , Knight Robert T. , Friedland Robert P. 
Published: January 2005

AbstractAutosomal dominant motor system degeneration has been described primarily in Portuguese families from the Azorean Islands. The symptoms include various combinations of ataxia, pyramidal and extrapyramidal signs, appendicular dystonias, tics, ophthalmoplegias, and peripheral neuropathies with amyotrophy. One non Portuguese, non‐Azorean black family has been described previously; this report describes a second such family affected by autosomal dominant motor system degeneration.
In vivo disturbance of the oxidative metabolism of glucose in human cerebral gliomas
Authors: Rhodes Christopher G. , Wise Richard J. S. , Gibbs Jeremy M. , Frackowiak Richard S. J. , Hatazawa Jun , Palmer Anthony J. , Thomas David G. T. , Jones Terry 
Published: January 2005

AbstractAbnormalities in the oxidative metabolism of glucose in human cerebral gliomas have been studied in seven patients using positron emission tomography. Measurements of regional cerebral blood flow and oxygen consumption were obtained using the oxygen‐15 steady‐state inhalation technique. Values of regional cerebral glucose consumption were obtained using fluorine 18–labeled 2‐fluoro‐2‐deoxy‐D‐glucose and a simplification of the method of Sokoloff. Functional values were obtained for regions of tumor and brain tissue in the middle cerebral artery territory of the contralateral cortex. Values of regional glucose consumption were calculated for both regions using a value of the lumped constant quoted for normal brain tissue (0.42).Tumor regional cerebral blood flow was comparable to that in the contralateral cortex, whereas regional cerebral oxygen consumption was depressed. This depression resulted in low tumor values of the fractional oxygen extraction ratio (0.21 + 0.07), indicating that oxygen supply exceeded the metabolic demand. In contrast, tumor regional cerebral glucose consumption was not depressed and regional glucose extraction ratios were similar for tumor and brain tissue. The metabolic uncoupling between regional oxygen consumption and regional glucose consumption (CMRO2/CMRGlu = 0.24 ± 0.07 ml of oxygen per milligram of glucose) is indicative of increased aerobic glycolysis.
Disturbance of oxidative metabolism of glucose in recent human cerebral infarcts
Authors: Wise Richard J. S. , Rhodes Christopher G. , Gibbs Jeremy M. , Hatazawa Jun , Palmer Tony , Frackowiak Richard S. J. , Jones Terry 
Published: January 2005

AbstractEight patients with recent cerebral hemispheric infarction were studied with positron emission tomography and the oxygen‐15 steady‐state inhalation and [18F]deoxyglucose techniques to obtain values of regional cerebral blood flow, oxygen consumption, and glucose metabolism. The Sokoloff equation, used to calculate glucose metabolism, was simplified to exclude the exponential terms containing the rate constants. A value of the lumped constant quoted for normal brain (0.42) was used for infarcted regions and contralateral hemisphere. Mean regional cerebral blood flow, oxygen consumption, and glucose metabolism were all significantly depressed within the infarcts compared with the mirror regions in the contralateral cerebral hemisphere. The mean fractional extraction of oxygen was low, indicating an adequate supply of oxygen for residual oxidative metabolism. Regional oxygen consumption and glucose metabolism were significantly correlated within the infarcts, but with a relationship of 2 moles of oxygen per mole of glucose—one‐third that in the contralateral hemisphere and in normal brain. Although these results suggest that the metabolizing tissue of a recent cerebral infarct utilizes aerobic glycolysis, caution about the validity of this pathophysiological observation is dictated by limitations in current positron emission tomographic tracer methodology.
Hemiparesis apparently due to central pontine myelinolysis following hyponatremia
Authors: Marra Thomas R. 
Published: January 2005

AbstractA 63‐year‐old woman was in an acute confusional state accompanied by rapidly progressive left hemiparesis, dysphagia, and dysarthria two weeks after an episode of diuretic‐induced hyponatremia. High‐resolution computed tomographic scanning disclosed a circumferential hypodense pontine lesion compatible with the diagnosis of central pontine myelinolysis. Although the patient improved clinically within six weeks, the computed tomographic abnormalities remained unchanged.
High blood glucose level on hospital admission and poor neurological recovery after cardiac arrest
Authors: Longstreth W. T. , Inui Thomas S. 
Published: January 2005

AbstractTo evaluate the relationship between blood glucose and neurological recovery after cardiac arrest, we retrospectively reviewed our experience with 430 consecutive patients resuscitated after out‐of‐hospital cardiac arrest. All these patients had received variable amounts of intravenous 5% glucose solutions before admission. Awakening patients, whose following commands or having comprehensible speech, were classified as to whether they suffered persistent neurological deficits. The mean blood glucose level on hospital admission was higher in 154 patients who never awakened than in 276 who did awaken (mean, 341 versus 262 mg per 100 milliliters; p <0.0005). Among the 276 who awakened, 90 patients with persistent neurological deficits had higher mean glucose levels on admission than did 186 without deficits (286 versus 251 mg per 100 milliliters; p < 0.02). These significant differences persisted after excluding all patients whose glucose levels were higher than 500 mg per 100 milliliters and after controlling for potentially confounding variables using multiple regression analysis.
Dysautonomia in Guillain‐Barré syndrome with dorsal root ganglioneuropathy, wallerian degeneration, and fatal myocarditis
Authors: Hodson Andrew K. , Hurwitz Barrie J. , Albrecht Renata 
Published: January 2005

AbstractAn acute Guillain‐Barré syndrome presenting as dysautonomia is described in a 12‐year‐old boy. The patient died of intractable cardiac arrhythmias and cardiac failure. A severe myocarditis with destruction of dorsal root ganglion cells and wallerian degeneration of dorsal roots and peripheral nerves was apparent postmortem. Segmental demyelination and inflammatory cellular infiltrations were not present at these sites.
Response of C‐6 glioma to isoproterenol and papaverine in vivo depends on β‐adrenergic receptor density
Authors: Chelmicka‐Schorr Ewa , Sportiello Michael G. , Atweh Samir F. , Arnason Barry G. W. 
Published: January 2005

AbstractRat C‐6 glioma serves as an experimental model for human glioma. C‐6 glioma cells carried to high culture passages in medium containing 10% fetal bovine serum when injected in vivo are unresponsive to treatment with the β‐adrenergic agonist isoproterenol and the phosphodiesterase inhibitor papaverine. When C‐6 glioma cells are kept in culture in serum‐containing medium, β‐adrenergic receptor density falls and, concomitantly, ability to accumulate cyclic adenosine monophosphate in response to stimulation with catecholamines declines. Responsiveness to treatment in vivo with a β‐adrenergic agonist was restored when C‐6 glioma cells were cultured in serum‐free defined medium prior to systemic injection into rats. Culturing of C‐6 glioma cells in serum‐free medium significantly increases the number of β‐ adrenergic receptors when compared with C‐6 glioma cells grown in serum‐containing medium.
Brain trace elements in Pick's disease
Authors: Ehmann W. D. , Alauddin M. , Hossain T. I. M. , Markesbery W. R. 
Published: January 2005

AbstractThe trace element content of the brain of two patients with Pick's disease examined postmortem was studied using instrumental neutron activation analysis. Results showed significant increases in chlorine, iron, manganese, sodium, and phosphorus and significant decreases in chromium, cesium, rubidium, and selenium and in the mean freeze‐dried to wet‐weight ratio for patients with Pick's disease compared with control patients. Brain zinc content was not elevated in the two patients, a finding that fails to support the hypothesis that elevated zinc levels play a role in the pathogenesis of Pick's disease.