bluuebunny/crossref_metadata_2025_split huggingface dataset:
Anticonvulsants, folate levels, and pregnancy outcome: A prospective study
Authors: Dansky Linda V. , Andermann Eva , Rosenblatt David , Sherwin Allan L. , Andermann Frederick 
Published: January 2005

AbstractFolate levels in serum and red cells, as determined by a microbiological assay using Lactobacillus casei, and plasma anticonvulsant concentrations were monitored concurrently in nonpregnant (50 subjects) and pregnant (49 pregnancies in 46 subjects) epileptic women. Twenty‐three (46%) nonpregnant women had subnormal serum folate levels and 4 nonpregnant women (8%) showed subnormal red cell folate levels. In pregnant women not taking folate supplements, the incidence of folate deficiency increased as the pregnancy advanced. Pregnant women taking folate supplements achieved normal or supranormal blood folate concentrations. In both nonpregnant and pregnant women, serum and red cell folate levels were inversely correlated with plasma concentrations of phenytoin and of phenobarbital, and with the number of anticonvulsants. In 49 pregnancies, there were 10 abnormal outcomes (20.4%): 4 spontaneous abortions (8.2%) and 6 children with major congenital malformations (12.2%). Blood folate levels were significantly lower in pregnancies with an abnormal outcome than in those with a normal outcome. The results suggest a dose‐response relationship among anticonvulsants, folate, and adverse pregnancy outcome.
Increased monomeric immunoglobulin A levels in tears from multiple sclerosis patients
Authors: Coyle P. K. , Sibony Patrick A. , Johnson Cynthia 
Published: January 2005

AbstractMucosal immunity and the secretory antibody are known to be important defense mechanisms against many common viruses. Tears were selected as a representative mucosal fluid, and immunoglobulin A (IgA) was examined by enzyme‐linked immunosorbent assay and immunoblot. Tears from 38 normal controls and patients with systemic or ocular diseases contained almost exclusively polymeric IgA. In contrast, almost 75% of 23 patients with multiple sclerosis had detectable monomeric IgA in addition to polymeric IgA. The functional importance of this alteration in the molecular form of secretory IgA remains to be determined.
Alzheimer's disease cells exhibit defective repair of alkylating agent—induced DNA damage
Authors: Robison Steven H. , Munzer J. Scott , Mrcp Rup Tandan , Bradley Walter G. 
Published: January 2005

AbstractThe most common cause of senile and presenile dementia is Alzheimer's disease, a disorder with an undetermined cause. A number of studies have indicated that neurons from patients with Alzheimer's disease have decreased ribonucleic acid levels and reduced protein synthesis. Recent studies using lymphoblasts from patients with Alzheimer's disease have indicated that these cells are more sensitive to deoxyribonucleic acid (DNA)–alkylating agents. We have used cell survival, unscheduled DNA synthesis, and alkaline elution to assess the capacity for DNA repair in skin fibroblasts from normal control subjects, control subjects with central nervous system disease, and patients with Alzheimer's disease. Our results indicate that the Alzheimer's disease cells, unlike normal cells, fail to repair methyl‐methane sulfonate–induced DNA damage. Both normal and Alzheimer's disease cells are able to ameliorate the effects of ultraviolet light. These results indicate that a specific pathway for DNA repair is affected in Alzheimer's disease. The repair defect may be related to the cause of the disease or may be the cause of the disease.
Heterogeneity in myasthenia gravis: HLA phenotypes and autoantibody responses in ocular and generalized types
Authors: Kida Kaichi , Hayashi Masatoshi , Yamada Itaru , Matsuda Hiroshi , Yoshinaga Junji , Takami Shunsai , Yashiki Shinji , Sonoda Shunro 
Published: January 2005

AbstractHLA phenotypes and autoantibody responses were studied in 71 Japanese patients with myasthenia gravis. HLA‐A2, Bw61, and DRw9 were associated with ocular myasthenia gravis (corrected p [CP] < 0.05 relative risk [RR] = 2.88; CP < 0.02, RR = 3.60; and CP < 0.001, RR = 4.63, respectively) and HLA‐DRw8 was associated with generalized myasthenia gravis (CP < 0.001, RR = 5.40). Neither HLA‐B8 nor DR3 was found in Japanese patients. The titer of antiacetylcholine receptor antibody (AChR Ab) and the incidence of autoantibodies other than AChR Ab were higher in patients with generalized myasthenia gravis than in those with the ocular type (2.77 ± 0.62 versus 0.17 ± 0.03 pmol/ml, p < 0.001; and 60.6 versus 29.0%, p < 0.02, respectively). Patients with a high titer of AChR Ab or with autoantibodies had an increased frequency of HLA‐DRw8 (CP < 0.02, RR = 4.61, and CP < 0.005, RR = 4.53, respectively); whereas patients with a low titer of AChR Ab or without autoantibodies had an increased frequency of HLA‐DRw9 (CP < 0.001, RR = 8.26, and CP < 0.005, RR = 4.08, respectively). These findings suggest that ocular and generalized myasthenia gravis might have different immunogenetic backgrounds.
Clonidine and Gilles de la Tourette's syndrome: Double‐blind study using objective rating methods
Authors: Goetz Christopher G. , Tanner Caroline M. , Wilson Robert S. , Susan V. Carroll , Como Peter G. , Shannon Kathleen M. 
Published: January 2005

AbstractClonidine has been suggested to be effective in Gilles de la Tourette's syndrome (GTS), but no double‐blind study has ever evaluated its effects using objective measures. Thirty patients with GTS completed a 6‐month placebo‐controlled crossover study of the effectiveness of clonidine. Videotapes were obtained at each 3‐week visit and were evaluated randomly at the end of the study for distribution, frequency, and severity of motor and vocal tics. Quantifiable psychometric examinations were performed as well. The use of clonidine did not significantly (p < 0.05) reduce motor tics, vocalizations, or behavior. The effect of a low dose (0.0075 mg/kg/day) was no different from that of a high dose (0.015 mg/kg/day); children's responses were no different from adults'; and those also receiving neuroleptic agents showed the same lack of efficacy as seen in patients on no other medication. Dosing schedule did not affect the objective ratings; scores from clonidine given twice a day were equivalent to those for three times a day.
Neocortical cell counts in normal human adult aging
Authors: Terry Robert D. , DeTeresa Richard , Hansen Lawrence A. 
Published: January 2005

AbstractFifty‐one brains from clinically and neuropathologically normal individuals ranging in age from 24 to 100 years were studied to determine what changes occur in neocortical neuroectodermal cell populations as a function of normal aging. Twenty‐m̈‐thick sections from the midfrontal, superior temporal, and inferior parietal areas were examined with an image‐analysis apparatus with combined manual and automatic editing capacity. Neuroectodermal cells were counted, measured, and assigned to one of ten categories, which were later summarized in three: large neurons (<90 m̈2), small neurons (41 to 90 m̈2), and glia (5 to 40 m̈2). Determinations were also made of brain weight, cortical thickness, neuronal density, neuron‐‐glia ratio, and percentage of cell area. The results showed statistically significant age‐related decrements in the following values: brain weight, cortical thickness in the midfrontal and superior temporal areas, large neurons in all three areas, and the neuron‐‐glia ratio in the midfrontal and inferior parietal areas. The total number of neurons, neuronal density, and percentage of cell area were all unchanged. Increasing with age were the number of small neurons in the midfrontal cortex and glia in the midfrontal and superior temporal areas. The following conclusions were drawn: (1) Aging affects the frontal and temporal lobes more than the parietal; the salient change is shrinkage of large neurons with consequently increasing numbers of small neurons; (2) constant neuronal density coupled with diminished cortical volume (decreased brain weight and cortical thinning) indicate that there is some neuronal loss with age, but it is of much lesser magnitude than previously supposed; and (3) the number of glia increases with age.
The magnetic field of complex partial seizures agrees with intracranial localizations
Authors: Sutherling William W. , Crandall Paul H. , Engel Jerome , Darcey Terrance M. , Cahan Leslie D. , Barth Daniel S. 
Published: January 2005

AbstractThe magnetoencephalogram (MEG) was recorded during 63 complex partial seizures in 4 patients. The MEG showed large biomagnetic signals occurring at the same time as discharges recorded from scalp electroencephalogram (EEG). These MEG signals had the same morphology and frequency as the discharges from the EEG. The location of the seizure focus was verified by depth electrode recordings in 2 patients and by lesions shown on computed tomographic scan and magnetic resonance imaging in the other 2. In each patient, MEG localization estimates were consistent with the location of the seizure focus shown by other methods. When seizures were recorded repeatedly and mapped with a single‐channel magnetometer placed at different scalp locations in a single patient, the MEG localization agreed with the electrographic seizure focus localized from depth electrodes. In the maps, the MEG resolved an ambiguity in the scalp EEG and therefore increased the confidence of localization. MEG recordings of seizures may help localize epileptic foci noninvasively.
No sex‐related differences in human corpus callosum based on magnetic resonance imagery
Authors: Oppenheim Jeffrey S. , Lee Benjamin C. P. , Nass Ruth , Gazzaniga Michael S. 
Published: January 2005

AbstractRecent studies have reported sex‐related differences in the morphology of the human corpus callosum in cadaver brains. To further investigate these reports, sagittal magnetic resonance image scans from 40 male and 40 female subjects were used to compare callosal morphology. Relative callosal measurements were calculated by morphometric analysis. Significant sex‐related differences were not found for callosal areas, maximal callosal width, or callosal curvature. These results indicate the need for further study before claims of sex‐related differences can be accepted.
Cholinergic‐dependent cognitive deficits in Parkinson's disease
Authors: Dubois Bruno , Danzé François , Pillon Bernard , Cusimano Guliana , Lhermitte François , Agid Yves 
Published: January 2005

AbstractIn a double‐blind cross‐over study, the effects of a subthreshold dose of scopolamine (0.25 mg) on memory were compared in 32 control subjects and 32 parkinsonian patients who were without any sign of intellectual and mnemic impairment. Although the scores of the controls in the memory test battery showed no deterioration after the administration of scopolamine, the same dose resulted in significantly reduced memory performance in parkinsonian patients in two tests which involved the recognition of meaningless drawings. The selective vulnerability of parkinsonian subjects without cognitive impairment to a subthreshold dose of scopolamine suggests the existence of an underlying alteration of central cholinergic transmission. The neuropsychological findings in our study agree with postmortem biochemical data, which showed decreased cortical choline acetyltransferase activity in all parkinsonian patients, suggesting the existence of neuronal compensation in parkinsonian patients who are without cognitive impairment.
Mononeuritis multiplex in Waldenström's macroglobulinemia
Authors: Lamarca Josep , Casquero Pilar , Pou Adolf 
Published: January 2005

AbstractWe describe a patient with mononeuritis multiplex who had cranial nerve involvement in association with Waldenström's macroglobulinemia. Sural nerve biopsy showed abundant endoneurial granulofibrillary deposits related to microangiopathic changes. These changes were distributed in a multifocal pattern among the fascicles. Immunohistochemistry demonstrated the IgM nature of the deposit located in the endoneurial interstitium.
Phenylethanolamine N‐methyltransferase activity is decreased in Alzheimer's disease brains
Authors: Burke William J. , Chung Hyung D. , Nakra B. R. S. , Grossberg George T. , Joh Tong H. 
Published: January 2005

AbstractPhenylethanolamine N‐methyltransferase (PNMT) is the rate‐limiting enzyme involved in the synthesis of epinephrine and a specific marker for epinephrine neurons. We have previously described the stability of this enzyme in a variety of pre‐ and postmortem conditions in human brain autopsy specimens and demonstrated its presence in areas of the human brain that are associated with memory and attention. We now report on 5 patients with Alzheimer's disease (AD) who had a Decemberrease in PNMT activity of 37 to 48% in areas of the brain affected by the disease but not in the cerebellum, an area of the brain unaffected by the disease. The degree of Decemberrease in PNMT activity in the hippocampus correlated significantly with the degree of dementia. We have provided direct immunochemical evidence that the Decemberrease in PNMT activity in AD is due to the loss of this specific enzyme protein.
Carbamazepine and cardiac conduction disturbances
Authors: Benassi Enrico , Bo Gian‐Paolo , Cocito Leonardo , Maffini Maura , Loeb Carlo 
Published: January 2005

AbstractCarbamazepine‐induced cardiac conduction disturbance is reported in 2 patients suffering from epilepsy. The cardiac defects disappeared in both patients after carbamazepine was discontinued, and reappeared in 1 patient after treatment was resumed.
A rating scale for amyotrophic lateral sclerosis: Description and preliminary experience
Authors: Appel V. , Stewart S. S. , Smith G. , Appel S. H. 
Published: January 2005

AbstractA rating scale has been developed to provide a quantitative estimate of clinical status and disease progression in amyotrophic lateral sclerosis (ALS). This scale includes assessment of swallowing, speech, and respiratory function, and both strength and function of upper and lower extremity musculature. The evaluation is relatively simple to perform and yields reproducible data for both a total ALS score and a score for each group of functions tested. A score of 30 points is normal; 164 points indicates maximal dysfunction. The total ALS score increased in a linear fashion in each of 74 patients followed for at least one year. Among patients, the rate of disease progression varied twenty‐fold, with a continuous distribution from the slowest to most rapid course. Thirty‐four percent of patients exhibited a rapid change of greater than 48 points in the year, predicting progression to a terminal stage in less than two years; 19% of patients exhibited a slow change of less than 13 points in one year, predicting progression to a terminal stage over at least five years. This ALS scoring system should permit more accurate assessment of drug efficacy in clinical trials ánd correlation of rates of progression with clinical variables.
The natural history of Tourette syndrome: A follow‐up study
Authors: Erenberg Gerald , Cruse Robert P. , David Rothner A. 
Published: January 2005

AbstractInitial reports described Tourette syndrome as a lifelong disorder. Since then, others have noted that some patients experience remissions during late adolescence. To examine this issue, we sent questionnaires to 99 patients with Tourette syndrome who were 15 to 25 years old. The majority of the 58 respondents indicated that they had fewer tics as they reached late adolescence or young adulthood. Although most reported associated behaviour or learning problems, the majority felt they were coping well. The long‐term outcome in many patients with Tourette syndrome may be more optimistic than previously reported.
Familial cases of HTLV‐I—associated myelopathy
Authors: Miyai Ichiro , Saida Takahiko , Fujita Masashi , Kitahara Yoshisuke , Hirono Nobutsugu 
Published: January 2005

AbstractWe studied two familial cases of human T‐lymphotropic virus type I (HTLV‐I)—associated myelopathy from the Kii Peninsula, an area of endemic adult T‐cell leukemia‐lymphoma (ATLL) in Japan. Incidence of familial clustering of HTLV‐I—associated myelopathy was about 20%. Type C retrovirus was isolated from cultured cerebrospinal fluid and peripheral blood lymphocytes in both cases. Modes of transmission seem to be similar to those described in ATLL, although there are no reports of both HTLV‐I—associated myelopathy and ATLL occurring in the same family. We suggest three possibilities: (1) that the virus associated with HTLV‐I—associated myelopathy is different from the virus causing ATLL, although they seem to be morphologically and immunologically similar; (2) that HTLV‐I—associated myelopathy may be determined by the ATLL‐causing virus plus a specific genetic background; and (3) some combination of factors 1 and 2.
Marked loss of cerebral galactolipids in Pick's disease
Authors: Scicutella Angela , Davies Peter 
Published: January 2005

AbstractStudies with two monoclonal antibodies reactive with galactolipids suggest that there is a marked loss of these compounds in the brains of patients with Pick's disease. Quantitative assays, immunocytochemical studies, and thinlayer chromatography confirm that both galactocerebrosides and sulfatides are selectively lost in this condition. This finding appears to be specific for Pick's disease, distinguishing it from Alzheimer's disease and other dementing disorders.
Soluble IL‐2 receptors in acute and subacute encephalitis
Authors: Boutin B. , Matsuguchi L. , Lebon P. , Ponsot G. , Artbuis M. , Nelson D.‐L. 
Published: January 2005

AbstractElevated levels of soluble interleukin‐2 receptors were present in the serum from patients with acute primary and postinfectious encephalitis and subacute sclerosing panencephalitis. In addition, soluble interleukin‐2 receptors were detected in the cerebrospinal fluid from patients with acute primary encephalitis. Their presence in the cerebrospinal fluid was not explained by damage to the blood—brain barrier and our data attest to their local origin. This suggests that it may be possible in certain neurological diseases to detect cerebral Tlymphocyte activation through a specific marker in the cerebrospinal fluid.
Anatomy of cholinesterase inhibition in Alzheimer's disease: Effect of physostigmine and tetrahydroaminoacridine on plaques and tangles
Authors: Mesulam M.‐Marsel , Geula Changiz , Asuncion Morán M. 
Published: January 2005

AbstractThe histochemical distribution of cholinesterases in the cerebral cortex and their response to cholinesterase inhibitors such as physostigmine and tetrahydroaminoacridine (THA) were investigated in brains from patients with Alzheimer's disease and control subjects. In the temporal neocortex of the control subjects, most of the cholinesterase activity was located within axons and cell bodies belonging to cholinergic pathways. In keeping with their well‐known cholinomimetic effects, physostigmine and THA effectively inhibited this cholinesterase activity. Cholinesterase‐containing normal axons (and in some cases cells) were severely depleted in the cerebral cortex of patients with Alzheimer's disease. Although the cerebral cortex of these patients continued to display abundant cholinesterase activity, the location of this enzyme was largely shifted to the neuritic plaques and neurofibrillary tangles. In fact, the majority of these pathological structures demonstrated intense acetylcholinesterase and butyrylcholinesterase activities. Physostigmine and THA were potent inhibitors of these plaque‐ and tangle‐bound cholinesterases as well. In patients with Alzheimer's disease, cholinesterase inhibitors would therefore appear to have a major and widespread effect directly upon the enzymatic activity of plaques and tangles. Consequently, the clinical effects of anticholinesterases in Alzheimer's disease may be based on mechanisms that are different from those that apply to the normal brain.
The metabolic pathology of the AIDS dementia complex
Authors: Rottenberg D. A. , Moeller J. R. , Strother S. C. , Sidtis J. J. , Navia B. A. , Dhawan V. , Ginos J. Z. , Price R. W. 
Published: January 2005

AbstractA progressive dementing illness, the AIDS dementia complex (ADC) is the most frequent neurological complication of the acquired immunodeficiency syndrome. Characteristic alterations in regional cerebral metabolic rate for glucose (rCMRGlc), associated with the presence or progression of ADC, were demonstrated by {18F} fluorodeoxy glucose/positron emission tomography in 9 of 12 patients with ADC compared with 18 normal volunteer subjects. In these 9 patients, two distinct patterns of regional metabolic activity were highly correlated with intersubject gray matter rCMRGlc variation and with disease severity as assessed by neuropsychological testing. Relative subcortical (thalamus and basal ganglia) hypermetabolism was characteristic of early ADC, and disease progression was accompanied by cortical and subcortical gray matter hypometabolism.
Efficiency of plasma exchange in Guillain‐Barré syndrome: Role of replacement fluids
Authors:   French Cooperative Group on Plasma Exchange in Guillain‐Barrk Syndrome
Published: January 2005

AbstractThe goals of this multicenter controlled trial were: (1) to study the short‐term effect of plasma exchange in the Guillain‐Barré syndrome when applied alone within 17 days of onset of the disease; and (2) to compare two replacement fluids, diluted albumin and fresh frozen plasma (FFP) with regard to efficacy and morbidity. Two hundred twenty patients were included, 111 in the control group, 109 in the plasma exchange group, 57 of whom were assigned to receive albumin and 52 to receive fresh frozen plasma. Treatment consisted of four plasma exchanges of two plasma volumes each, initiated on the day of randomization and repeated on alternate days. Significant short‐term benefits appeared in the group that received plasma exchange as demonstrated by the reduction in the proportion of patients who required assisted ventilation after randomization, and the decrease in time before beginning weaning from ventilator, time to onset of motor recovery, and time to walk with and without assistance. No statistically significant difference was found between the group that received albumin and the group that received fresh frozen plasma. Incidents during exchanges and complications related to the plasma exchange were more frequent in patients who received fresh frozen plasma. Plasma exchange is beneficial in Guillain‐Barré syndrome when it is carried out early in the course of the disease. Given its risks and the lack of its clear superiority over albumin, however, we recommend that fresh frozen plasma be abandoned in the treatment of Guillain‐Barré syndrome.
Ultralow vestibuloocular reflex time constants
Authors: Baloh R. W. , Beykirch K. , Tauchi P. , Yee R. D. , Honrubia V. 
Published: January 2005

AbstractWe report detailed oculomotor studies in 3 patients with central nervous system lesions and markedly decreased time constants (< 2 seconds) of the vestibuloocular reflex (VOR). In 1 patient with Chiari type I malformation, serial measurements over 3 years documented a progressive decrease in the duration of postrotatory nystagmus (100 deg/sec steps, acceleration 140 deg/sec2) until finally there was no sustained nystagmus. At this time, the patient had no response to caloric stimulation or to sinusoidal rotation below 0.2 Hz but normal gain (peak slow‐phase eye velocity/peak chair velocity) above 0.4 Hz (phase lead increased). Gaze holding, saccades, smooth pursuit, and optokinetic nystagmus were normal, but optokinetic‐after‐nystagmus disappeared. The other 2 patients (combined brainstemcerebellar atrophy) had impaired gaze holding, abnormal smooth pursuit and optokinetic nystagmus, and absent optokinetic‐after‐nystagmus. VOR gain to step and high‐frequency sinusoidal stimuli was increased. The neural mechanism that normally prolongs the VOR time constant may have reduced it in our patients.