bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cerebrospinal fluid IgG in childhood: The establishment of reference values
Authors: Rust Robert S. , Dodson W. Edwin , Trotter John L. 
Published: January 2005

AbstractA retrospective analysis of quantitative and qualitative immunoglobulin G (IgG) results from 253 children who were either medically and neurologically normal or highly unlikely to have abnormalities of CSF IgG is reported. Normal values in this reference population vary with age for CSF/albumin IgG ratio and CSF/serum IgG index and are significantly different from the adult reference values. The rate of false positivity is lower for quantitative values than for qualitative IgG determinations (oligoclonal bands).
Differential cognitive impairment in alzheimer's disease and huntington's disease
Authors: Brandt Jason , Folstein Susan E. , Folstein Marshal F. 
Published: January 2005

AbstractThe differentiation between cortical and subcortical dementias requires that the cognitive characteristics of dementias attributable to different causes be discriminable. For large samples of Alzheimer's disease and Huntington's disease patients, distinct cognitive profiles were obtained on the Mini‐Mental State Exam. The profile differences were independent of severity of dementia and were sufficiently robust to classify patients as Alzheimer's disease or Huntington's disease with 84% accuracy. The qualitative differences in cognitive functioning may also be typical of other cortical and subcortical dementias.
Long‐term duodenal infusion of levodopa for motor fluctuations in parkinsonism
Authors: Sage J. I. , Trooskin S. , Sonsalla P. K. , Heikkila R. , Duvoisin R. C. 
Published: January 2005

AbstractWe report the long‐term clinical responses of 2 parkinsonian patients with severe “on‐off” phenomena who have been receiving continuous duodenal levodopa (LD) infusions. The rate of LD infusion needed to keep the patients just “on” Decemberlined gradually during the initial 60 days of infusion and then remained constant. Total LD intake Decemberlined rapidly during the first several days and gradually for another 60 days, after which it remained stable. Motor fluctuations virtually disappeared in both patients during the time of the day when the pump was in use. This was accompanied by a Decemberline in the plasma LD concentration needed to just maintain the “on” stage in one patient.
Eales' disease presenting as stroke in the young adult
Authors: Gordon Marchk Forrest , Coyle P. K. , Golub Barry 
Published: January 2005

AbstractEales' disease is an uncommon idiopathic disorder characterized by retinal perivasculitis and recurrent vitreous hemorrhages in young males. Associated neurological involvement is rare. We report a 38‐year‐old man who presented with stroke attributed to Eales' disease.
Nondextrality and autoimmune disorders among relaives of language‐disabled boys
Authors: Urion David K. 
Published: January 2005

AbstractThe incidences of autoimmune diseases, nonright‐handedness, and premature graying of the hair have been said to be increased among the relatives of dyslexic persons. I undertook a prospective evaluation of the relatives of 100 language‐disabled boys seen in a tertiary hospital learning disabilities clinic. I established the incidence of these characteristics and compared the results with those obtained from the relatives of 100 boys referred to a neurology clinic for evaluation of seizures or muscle disease. The families of the language‐disabled boys were divisible into two groups, one with an increased incidence of these characteristics and one with a Decemberreased incidence as compared with the control group. This suggests a biological heterogeneity among these families, despite a similar phenotype among all the language‐disabled probands.
Hallervorden‐spatz syndrome: Clinical and magnetic resonance imaging correlations
Authors: Sethi K. D. , Adams R. J. , Loring D. W. , El Gammal T. 
Published: January 2005

AbstractTwo women with a presumptive diagnosis of Hallervor‐den‐Spatz syndrome had a combination of dystonia and parkinsonism. One had retinitis pigmentosa. Neuropsychological testing revealed Decemberreased verbal fluency and visuoconstructional and motor deficits. Magnetic resonance imaging performed with a high‐field‐strength unit (1.5 Tesla) showed striking abnormalities in the globus pallidus bilaterally (“eye‐of‐the‐tiger” sign). Magnetic resonance imaging may prove useful in the diagnosis of Hallervorden‐Spatz syndrome.
Depth electrode investigations in patients with bitemporal epileptiform abnormalities
Authors: So Norman , Gloor Pierre , Quesney L. Felipe , Jones‐Gotman Marilyn , Olivier André , Andermann Frederick 
Published: January 2005

AbstractFifty‐seven patients showing bitemporal independent epileptiform abnormalities on extracranial electroencephalograms (EEGs) in whom the epileptogenic zone could not be localized or lateralized by extracranial EEG and other noninvasive tests were investigated with stereotactic depth electrode recordings. In a majority of 44 patients (77%), seizures originated exclusively or with a strong predominance in one temporal lobe only. Of the remaining 13 patients (23%), 8 had seizures originating independently in either temporal lobe without significant lateralized predominance, and 5 had multiple seizure patterns, which were often diffuse. The patterns of seizure onset as recorded by depth electrodes tended to vary even in the same patient. Electrical stimulation studies and the determination of afterdischarge thresholds were of limited utility for lateralization of seizure onset.
Sleep abnormalities in progressive supranuclear palsy
Authors: Aldrich Michael S. , Foster Norman L. , White Roberta F. , Bluemlein Laurie , Ba Gregory Prokopowicz 
Published: January 2005

AbstractWe studied sleep patterns for three nights in 10 subjects with moderate to severe progressive supranuclear palsy and correlated the findings with disease severity using quantitative measures of motor, cognitive, and eye movement impairment. All subjects had severe insomnia, spending 2 to 6 hours awake per night; the mean time awake per night for the group was more than 4 hours. Sleep latency became shorter and the number of awakenings increased with greater motor impairment, and total sleep time declined as dementia worsened. These findings indicate that in progressive supranuclear palsy insomnia is related to disease severity. Insomnia associated with progressive supranuclear palsy appears to be worse than the insomnia of Parkinson's disease or Alzheimer's disease and may be due to degenerative changes in brain structures responsible for sleep maintenance.
The effect of MK‐801 on kindled seizures: Implications for use and limitations as an antiepileptic drug
Authors: Williamson John M. , Lothman Eric W. 
Published: January 2005

AbstractMK‐801 is a new drug that produces a noncompetitive blockade at the subclass of glutamate receptors activated by N‐methyl‐D‐asparate (NMDA). The antiepileptic properties of MK‐801 were studied using kindled seizures as a model of complex partial seizures with secondary generalization. A test protocol was employed that allowed: (1) examination of the efficacy of MK‐801 against several parameters that gauge different aspects of epiletogenesis; (2) determination of the time‐action profile of these effects; and (3) examination of the toxicity of MK‐801 in animals experiencing seizures. The drug was found to be potent against the spread of seizures but less effective against parameters linked to partial seizures. At the higher doses of the drug required to truncate hippocampal afterdischarges, considerable neurotoxicity was encountered. In addition, the antiepileptic effects of MK‐801 showed a use dependence so that, at a given time after the drug was administered, a greater suppression of seizures was noted if there had been preceding seizures in the presence of the drug than if there had not been. These findings indicate that there may be limitations to the clinical utility of MK‐801 as an antiepileptic agent and that the drug may provide greatest benefit when used for the suppression of seizure generalization and when seizures are closely spaced.
Do oligodendrocytes mediate iron regulation in the human brain?
Authors: Gerber Megan R. , Connor James R. 
Published: January 2005

AbstractWe used immunohistochemical studies to demonstrate that transferrin (the iron mobilization protein) and ferritin (the iron storage protein) are specifically localized in oligodendrocytes in gray and white matter of the human central nervous system. In addition, iron is also localized predominantly in oligodendrocytes. Oligodendrocytes have been well established as the cells responsible for myelin production in the central nervous system. The results of this study suggest that oligodendrocytes (or a subpopulation of oligodendrocytes) might have the additional function of mediating iron mobilization and storage in the central nervous system.
Abnormal pattern electroretinograms in patients with senile dementia of the alzheimer type
Authors: Trick Gary L , Barris Michael C , Bickler‐Bluth Michelle 
Published: January 2005

AbstractPatients with senile dementia of the Alzheimer type frequently have difficulty performing visual tasks. These difficulties may be due, at least partially, to degenerative changes in both the primary visual pathway and the visual association areas. To determine whether retinal ganglion cell dysfunction contributes to visual loss in senile dementia of the Alzheimer type, we tested a group of patients with this disease (n = 13) using the patten‐reversal electroretinogram to both low (4.0 reversals per second) and high (16.0 reversals per second) temporal frequency checkerboard patterns (1.0 degree checks). Significant amplitude reduction were noted for the patients relative to age‐matched control subjects (n = 30). In addition, the observed amplitude reduction were most pronounced for the high temporal frequency condition. Therefore, the results are consistent with retinal ganglion cell dysfunction and support the notion that optic nerve damage induced by senile dementia of the Alzheimer type preferentially affects the larger, faster‐conduction retinal ganglion cells along with their retinocortical projections.
Acetaminophen metabolism by cytochrome P450 monooxygenases in Parkinson's disease
Authors: Factor Stewart A. , Weiner William J. , Hefti Franz 
Published: January 2005

AbstractIt has been suggested that alterations in the activity of cytochrome P450 monooxygenases may play a role in the pathogenesis of Parkinson's disease, particularly in patients who had onset before the age of 40. We studied the P450‐mediated metabolism of acetaminophen to 3‐hydroxy‐acetaminophen in 26 patients with Parkinson's disease and in 18 control subjects. After subjects ingested 1,000 mg acetaminophen, urine was collected under controlled conditions. Acetaminophen and 3‐hydroxy‐acetaminophen were measured in the urine using newly developed high‐pressure liquid chromatography methods. The ratio of 3‐hydroxy‐acetaminophen to acetaminophen was calculated for each patient and no significant differences were observed in patients compared with control subjects. Abnormal metabolism was not observed in patients who had onset of Parkinson's diseae at or before the age of 40. In addition, no difference in metabolic activity was observed between the patients who were treated with levodopa/carbidopa and those who were not treated. These findings suggest that there are no alterations of P450‐mediated metabolism of acetaminophen in patients with Parkinson's disease.
Sympathetic skin responses are decreased and lymphocyte beta‐adrenergic receptors are increased in progressive multiple sclerosis
Authors: Karaszewski Joseph W. , Reder Anthony T. , Maselli Ricardo , Brown Margaret , Arnason Barry G. W. 
Published: January 2005

AbstractImmune abnormalities, including deficient CD8 lymphocyte‐mediated suppression, have been implicated in the progression of multiple sclerosis (MS). The peripheral sympathetic branch of the autonomic nervous system innervates the lymphoid organs and affects immune function. Animals with an ablated sympathetic nervous system develop more severe experimental allergic encephalomyelitis than control animals and exhibit an increased density of betaadrenergic receptors on their lymphocytes. Experimental allergic encephalomyelitis shares many features with MS. Accordingly, we investigated the psychogalvanic skin reflex in patients with rapidly progressive MS and found that 13 patients (57%) lacked this sympathetic‐mediated response. The density of beta‐adrenergic receptors on lymphocyte subsets was increased in progressive MS, most notably on the CD8 suppressor/cytotoxic subset. B lymphocytes had the greatest number of receptors with 12.1 ± 1.8 fmol/106 cells in control subjects and 18.7 ± 2.6 fmol/106 cells in patients with MS. CD8 lymphocytes possessed an intermediate number of receptors with 3.4 ± 0.4 fmol/106 cells in control subjects and 9.1 ± 1.6 fmol/106 cells in patients with MS. CD4 lymphocytes demonstrated the fewest receptors with 1.2 ± 0.1 fmol/106 cells in control subjects and 1.8 ± 0.4 fmol/106 cells in patients with MS. No differences in the affinity or function (cyclic adenosine monophosphate levels in response to 10−5 M (–)isoproterenol) of the adrenergic receptor were found when patients with progressive MS and control subjects were compared. Autonomic abnormalities in progressive MS and the increased beta‐adrenergic receptor density found on CD8 lymphocytes may be related.
Posthypoxic glucose supplement reduces hypoxic‐ischemic brain damage in the neonatal rat
Authors: Hattori Haruo , Wasterlain Claude G. 
Published: January 2005

AbstractWe evaluated the effect of posthypoxic glucose supplement in a neonatal hypoxic‐ischemic animal model. Seven‐day‐old rats underwent bilateral ligation of the carotid arteries, followed by exposure to an 8% oxygen atmosphere for 1 hour. The extent of hypoxic‐ischemic brain damage was assessed histologically 72 hours later. Gulcose load immediately after the end of the hypoxic exposure reduced the volume of neocortical infarction to 37% of the unsupplemented value, and attenuated ischemic damage in the striatum and the dentate gyrus. At the end of the hypoxic exposure, the brain level of glucose was 0.3 mmol/kg and the level of lactate, 9 mmol/kg. Glucose supplement produced a rapid rise in brain glucose level to 3 to 5 mmol/kg over the next 2 hours. Lactate in both brain and plasma gradually fell toward the baseline level during the first hour of recovery. Posthypoxic glucose supplement slightly retarded lactate restitution. At any period of this neonatal model, brain lactate levels did not exceed the toxic level, which is postulated to be responsible for cerebral infarction in adult ischemic models. These results illustrate the important role of glucose in the development of neonatal hypoxic‐ischemic encephalopathy and the fact that full cortical infarction can develop even if brain lactate levels are low.
NMDA antagonists potentiate antiparkinsonian actions of L‐dopa in monoamine‐depleted rats
Authors: Klockgether Thomas , Turski Lechoslaw 
Published: January 2005

AbstractSystemically administered N‐methyl‐D‐aspartate (NMDA) antagonists, MK‐801((+)5‐methyl‐10,11‐dihydro‐5H‐dibenzo(a, d)cyclohepten‐5,10‐imine maleate) and CPP(3‐[(±)‐2‐carboxypiperazin‐4‐y1]‐propyl‐1‐phosphonate), potentiate the ability of L‐dopa (L‐3,4‐dihydroxyphenylalanine) to reverse akinesia and to alleviate muscular rigidity in monoamine‐depleted rats. On the basis of these findings, it is proposed that NMDA antagonists may be beneficial as adjunctive treatment in the therapy of Parkinson's disease. CPP locally injected into the subthalamic nucleus, entopeduncular nucleus–the rat homologue of the internal pallidal segment–or substantia nigra pars reticulata of monoamine‐depleted rats stimulates locomotor activity and alleviates rigidity, whereas local microinjection of CPP into the neostriatum is ineffective. These results make it unlikely that the neostriatum is the site of the antiparkinsonian action of NMDA antagonists in monoamine‐depleted rats, whereas the subthalamic nucleus, internal pallidal segment, and substantia nigra pars reticulata appear to be important for the effects of NMDA antagonists.
Visual dysfunction in Alzheimer's disease: Relation to normal aging
Authors: Cronin‐Golomb Alice , Corkin Suzanne , Rizzo Joseph F. , Cohen Jennifer , Growdon John H. , Banks Kathleen S. 
Published: January 2005

AbstractIn patients with Alzheimer's disease (AD), compared with age‐matched and young healthy control subjects, visual deficits in the following functions were observed: color, stereoacuity, contrast sensitivity, and backward masking (homogeneous and pattern). Critical flicker fusion thresholds were normal, relative to age‐matched healthy subjects. For color, the majority of the errors were tritanomalous (blue axis). Color and stereoacuity deficits were unrelated to severity of dementia, in accordance with models of vision that describe these functions as modular rather than diffuse for cortical localization. Although contrast sensitivity was depressed throughout the frequency range in AD, more patients were impaired at low than at high spatial frequencies, contrasting with the observed normal aging pattern of high‐frequency loss. Healthy elderly subjects showed depressed critical flicker fusion thresholds and reduced contrast sensitivity at high frequencies, relative to the young group; differences between these groups were not found for the other vision tests. A subset of the AD group received detailed neuro‐ophthalmological examination, and no abnormalities were found. This finding, taken together with normal thresholds for critical flicker fusion, suggests that the widespread visual dysfunction reported here is more likely to be related to known pathological changes in primary visual and association cortex in AD than to changes in the retina or optic nerve.
Lymphoma, motor neuron diseases, and amyotrophic lateral sclerosis
Authors: Younger D. S. , Rowland L. P. , Latov N. , Hays A. P. , Lange D. J. , Sherman W. , Inghirami G. , Pesce M. A. , Knowles D. M. , Powers J. , Miller J. R. , Fetell M. R. , Lovelace R. E. 
Published: January 2005

AbstractWe studied 9 patients with motor neuron disease and lymphoma. The following several observations have not been recognized in the past: (1) Motor neuron syndromes are associated with either Hodgkin's disease or non‐Hodgkin's lymphoma. (2) The syndromes are not restricted to lower motor neuron disorders; 8 of 9 patients had definite or probable upper motor neuron signs as well, qualifying for the diagnosis of amyotrophic lateral sclerosis. Corticospinal tracts were affected in both postmortem examinations. (3) The combination of motor neuron disease and lymphoma is often accompanied by paraproteinemia (3 of 7 patients studied), increased cerebrospinal fluid protein content (6 of 9 patients), and cerebrospinal fluid oligoclonal bands (3 of 9 patients). (4) In 2 patients, asymptomatic non‐Hodgkin's lymphoma was found only because the discovery of paraproteinemia gave impetus to examine the bone marrow. (5) Patients with both upper and lower motor neuron signs (amyotrophic lateral sclerosis) may show physiological evidence of conduction block in peripheral nerves or autopsy abnormalities in peripheral nerves. The cause of this syndrome is not known. Both lymphoma and motor neuron disease could have a common cause, possibly a retroviral infection. The frequency of paraproteinemia suggests that an immunological disorder may play a role in the pathogenesis of the neurological disorder.
The predictive value of intrathecal immunoglobulin synthesis and magnetic resonance imaging in acute isolated syndromes for subsequent development of multiple sclerosis
Authors: Sharief Mohammad K. , Thompson Edward J. 
Published: January 2005

AbstractThe intrathecal synthesis of IgM in patients presenting with acute monosymptomatic syndromes may be relevant in predicting eventual progression to multiple sclerosis but has not been previously evaluated. We undertook a prospective 18‐month combined clinical, cerebrospinal fluid, and magnetic resonance imaging study of 45 patients who had presented with acute isolated lesions of brainstem and spinal cord to evaluate the predicive value of intrathecal synthesis of IgM and IgG (through the detection of cerebrospinal fluid oligoclonal bands) and magnetic resonance imaging brain lesions at presentation, for the subsequent progression to multiple sclerosis. Results indicate that the highest risk of developing multiple sclerosis is seen in patients who are positive for oligoclonal IgM and IgG bands and who have disseminated cerebral white matter magnetic resonance imaging lesions at the initial presentation. Oligoclonal IgM bands predict progression to multiple sclerosis more frequently than oligoclonal IgG or magnetic resonance imaging alone. Our results confirm and further extend previous reports, and indicate that detection of cerebrospinal fluid oligoclonal IgM bands at presentation is a valuable prognostic indicator in patients presenting with acute isolated brainstem or spinal cord syndromes.
Benign familial neonatal convulsions: Evidence for clinical and genetic heterogeneity
Authors: Ryan Stephen G. , Wiznitzer Max , Hollman Charlotte , Torres M. Cristina , Szekeresova Maria , Schneider Sandra 
Published: January 2005

AbstractThe gene for autosomal dominant “benign” familial neonatal convulsions, a transient, primary epilepsy of infancy, has recently been assigned to chromosome 20q. To determine whether this disorder is genetically heterogeneous, we performed linkage analysis in two previously unreported pedigrees with benign familial neonatal convulsions in which clinical heterogeneity was evident. There were 14 affected persons in the first family, and none had seizures (febrile or afebrile) after the age of 2 months. The second family had 13 affected individuals and 2 obligate carriers; seizures frequently did not remit until 6 to 24 months, febrile convulsions occurred in at least 2 patients, apparent audiogenic seizures occurred in 4 patients, and 1 individual had refractory epilepsy until late adolescence. Linkage studies with the chromosome 20 markers D20S19 and D20S20 were performed in both families. The resulting data favored linkage of the disease and marker loci in Family 2 by a maximum odds ratio of 45:1 at 6% recombination. In Family 1, however, the odds were greater than 20,000:1 against linkage at 10% recombination or less. We conclude that the syndrome of benign familial neonatal convulsions is clinically and genetically heterogeneous. Further study will be necessary to clarify the relationship between phenotype and genotype in this disorder.
High‐speed memory scanning in Parkmson's disease: Adverse effects of levodopa
Authors: Poewe W. , Berger W. , Benke Th. , Schelosky L. 
Published: January 2005

AbstractHigh‐speed memory scanning as assessed by the Sternberg paradigm was studied in 12 nondemented patients with fluctuating Parkinson's disease and 13 age‐matched healthy controls. Patients were first assesed before taking their morning dose of levodopa (“off”) and again after that dose had produced full clinical effect(“on”). Although motor components of the measured choice reaction time were slower in patients when off than in control subjects, memory scanning speed was not different. After levodopa (on), patients' motor time normalized, but cognitive processing speed became significantly slower when compared with previous performance in the off condition and with controls. Contrary to previous concepts of bradyphrenia in patients with Parkinson's diesase, these results indicate that dopaminergic stimulation can reduce cognitive processing speed.