bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical significance of cerebrospinal fluid tests for neurosyphilis
Authors: Davis Larry E. , Schmitt Joseph W. 
Published: January 2005

AbstractFrom 1978 to 1987, 1,665 cerebrospinal fluid (CSF) fluorescent treponemal antibody absorption (CSF‐FTA‐ABS) tests were performed as the screening procedure for neurosyphilis. The CSF samples from 48 patients were reactive, and the medical history and results of the CSF–Venereal Disease Research Laboratory test (CSF‐VDRL) for syphilis for 38 of these patients were reviewed. Likely active neurosyphilis was diagnosed if the patient had a reactive CSF‐FTA‐ABS test, recent onset of neurological signs consistent with neurosyphilis, abnormal CSF, and no other recognized cause for the neurological illness. Fifteen patients were so classified. Four had a reactive CSF‐VDRL test. The specificity of the CSF‐VDRL in diagnosing likely active neurosyphilis was 100%, but the sensitivity was only 27%. The insensitivity of the CSF‐VDRL test limits its usefulness as a screening test for neurosyphilis. The CSF‐FTA‐ABS test appears more sensitive for screening but is less specific than the CSF‐VDRL test in distinguishing currently active neurosyphilis from past syphilis. These findings imply that clinical judgment is still essential in establishing the diagnosis of active neurosyphilis.
Speech manifestations in lateralization of temporal lobe seizures
Authors: Gabr M. , Lüders H. , Dinner D. , Morris H. , Wyllie E. 
Published: January 2005

AbstractTo evaluate the role of speech manifestations in lateralization of temporal lobe seizures, we reviewed videotapes of 100 complex partial seizures in 35 patients who underwent temporal lobectomy for intractable epilepsy. All patients had prolonged electroencephalographic video monitoring with scalp and subdural electrodes, and their speech dominance was determined with an intracarotid amobarbital test. Speech manifestations were observed in 79 seizures and were classified as vocalization, normal speech, or abnormal speech. Vocalization of sounds without speech quality occurred ictally in 48.5% of patients. Normal speech (identifiable speech) occurred ictally in 34.2% of patients. Abnormal speech (speech arrest, dysphasia, dysarthria, and nonidentifiable speech) occurred in 51.4% of patients, either ictally or postictally. Of all the above speech manifestations, only postictal dysphasia and ictal identifiable speech had significant lateralizing value: 92% of patients with postictal dysphasia had their seizures originating from the dominant temporal lobe (p < 0.001), and 83% of those with ictal identifiable speech had their seizures from the nondominant side (p = 0.013). This study shows that speech manifestations are common in complex partial seizures of temporal lobe origin and can provide an excellent clinical tool for lateralization of seizure onset.
The prevalence of primary sjögren's syndrome in a multiple sclerosis population
Authors: Noseworthy J. H. , Bass B. H. , Vandervoort M. K. , Ebers G. C. , Rice G. P. A. , Weinshenker B. G. , McLay C. J. L. , Bell D. A. 
Published: January 2005

AbstractA central nervous system illness closely resembling multiple sclerosis has been described in patients with primary Sjögren's syndrome. From these reports, the estimated prevalence of this syndrome could be similar to that of multiple sclerosis in a high‐frequency area. We evaluated 192 consecutive patients in our multiple sclerosis clinic to determine if such cases had gone unrecognized. We did not find any patients with clinical or serological evidence of primary Sjögren's syndrome. We conclude that it is rare for neurological manifestations of Sjögren's syndrome to mimic multiple sclerosis in our population.
Immunocytochemical characterization of neurofibrillary tangles in amyotrophic lateral sclerosis and parkinsonism‐dementia of guam
Authors: Shankar Susarla K. , Yanagihara Richard , Garruto Ralph M. , Grundke‐Iqbal Inge , Kosik Kenneth S. , Gajdusek D. Carleton 
Published: January 2005

AbstractCryostat‐cut sections of formalin‐fixed and unfixed hippocampus from 23 Guamanian Chamorros with clinically and neuropathologically verified amyotrophic lateral sclerosis (ALS) (8 cases) and parkinsonism‐dementia (PD) (15 cases) and from 12 neurologically normal Guamanians (5 with and 7 without neurofibrillary degeneration) were evaluated by the immunoperoxidase technique, using monoclonal antibodies against phosphorylated neurofilament, human fetal microtubule‐associated protein tau, and paired helical filaments. On immunostaining, all three antibodies showed intracellular tangles in the hippocampal neurons of patients with ALS, patients with PD, and in neurologically normal Guamanians with neurofibrillary pathology, but the correlation of immunostaining between these antibodies was not absolute. Extracellular or ghost tangles were immunostained only with the antibody against paired helical filaments. Our immunocytochemical data indicate that the antigenic composition of neurofibrillary tangles in Guamanian ALS and PD is similar to that of Alzheimer's disease, suggesting a common pathogenetic pathway for neurofibrillary tangle formation in these neurodegenerative disorders.
Transient neonatal hyperglycinemia
Authors: Schiffmann Raphael , Kaye Edward M. , Willis John K. , Africk Diane , Ampola Mary 
Published: January 2005

AbstractTwo patients with neonatal seizures and subsequent normal neurological development were found to have nonketotic hyperglycinemia. In both patients, hyperglycinemia resolved at 6 weeks of age. After cerebrospinal fluid glycine levels were normalized, the seizures stopped completely in one child and were markedly improved in the other. The possible mechanisms for the hyperglycinemia are discussed.
Differential regulation of γ‐aminobutyric acid receptor channels by diazepam and phenobarbital
Authors: Twyman Roy E. , Rogers Carl J. , Macdonald Robert L. 
Published: January 2005

AbstractThe anticonvulsant activity of diazepam and phenobarbital may be mediated in part by enhancement of inhibition involving γ‐aminobutyric acid (GABA). While both diazepam and phenobarbital increase GABA receptor chloride current, they may have different mechanisms of action, since they bind to different sites on the GABA receptor—chloride channel complex. We used the patch clamp technique to compare the effects of diazepam and phenobarbital on single GABA receptor currents. Outside‐out patches were obtained from mouse spinal cord neurons grown in cell culture for 2 to 4 weeks. GABA (2 μM) evoked single channel currents that occurred as single brief openings or in bursts of multiple openings. Diazepam (20 nM) and phenobarbital (500 μM) both increased the GABA receptor current by increasing mean open time without altering channel opening frequency. However, the temporal grouping of openings into bursts suggested that the enhancement occurred via different mechanisms. Diazepam increased the frequency of bursting GABA receptor currents with minimal effect on the duration of bursts. Phenobarbital increased the duration of bursting GABA receptor currents without altering the frequency of bursts. These results suggest that diazepam binds to a site that may enhance single channel burst frequency by increasing the affinity of GABA binding, while phenobarbital may stabilize the bursting open state of the channel by binding to a different modulatory site at or near the chloride channel.
Decreased adhesiveness of alzheimer's disease fibroblasts: Is amyloid β‐protein precursor involved?
Authors: Uéda Kenji , Cole Gregory , Sundsmo Mary , Katzman Robert , Saitoh Tsunao 
Published: January 2005

AbstractThe adhesiveness of fibroblasts derived from Alzheimer's disease (AD) patients to a plastic substratum was assessed as the proportion of cells attached to a plastic dish bottom after a 30‐minute incubation in culture medium of cells dissociated in ethylenediaminetetraacetic acid and was compared with the adhesiveness of normal fibroblasts from non‐AD controls. It was found that the normal fibroblasts adhered better to plastic than did AD cells. This reduced adhesiveness was observed in fibroblasts derived from both sporadic and familial AD patients. Because of the possible involvement of amyloid β‐protein precursor (ABPP) in the process of cellular adhesion, the amount of ABPP messenger RNA was measured in these fibroblasts and was found to be decreased in the familial AD fibroblasts, although not in cells from sporadic AD patients. Furthermore, there were fewer molecules detected by an anti‐ABPP antibody in conditioned media from familial AD fibroblasts as compared with media from control fibroblasts. Therefore, although it is possible that the reduced adhesiveness of familial AD fibroblasts may result from genetic deficits in ABPP gene expression, the reduced adhesiveness of sporadic AD fibroblasts most likely results from certain deficits in molecules other than the ABPP.
Lambert‐eaton myasthenic syndrome: Immunoglobulin G inhibition of Ca2+ flux in tumor cells correlates with disease severity
Authors: Lang B. , Vincent A. , Murray N. M. F. , Newsom‐Davis J. 
Published: January 2005

AbstractWe compared the effects of Lambert‐Eaton myasthenic syndrome (LEMS) immunoglobulin G (IgG) obtained from patients with and without small‐cell lung carcinoma (SCLC) on voltage‐gated (K+‐stimulated) 45Ca2+ flux in cell lines derived from a human SCLC (MAR10) and from a rat pheochromocytoma (PC12) and related these to electromyographic indexes of clinical severity. Control IgG was obtained from patients with other neurological disorders or healthy individuals. Inhibition of Ca2+ flux by LEMS IgG was time and dose dependent. The flux was significantly reduced in MAR10 cells grown in either SCLC‐LEMS IgG (0.38 nmol/106 cells; p < 0.001) or non‐SCLC‐LEMS IgG (0.35 nmol/106 cells; p < 0.001), compared with that in MAR10 cells grown in control IgG (0.7 nmol/106 cells). Similar significant reductions were also observed in PC12 cells. The reduction in amplitude of the resting compound muscle action potential in the LEMS patients correlated positively (r = 0.70; p = 0.007) with the inhibition of Ca2+ flux in MAR10 cells by their IgG. These results strongly support the view that IgG autoantibodies that can inhibit Ca2+ flux in SCLC cells are responsible for the disorder of transmitter release at motor nerves in SCLC‐associated LEMS.
Transient ocular motor paresis associated with acute internal carotid artery occlusion
Authors: Wilson W. Bruce , Leavengood John M. , Ringel Stephen P. , Bott Allen D. 
Published: January 2005

AbstractWhile sudden monocular blindness and occlusion of the central retinal artery associated with acute thrombosis of the internal carotid artery are well reported, concurrent unilateral ophthalmoparesis is not. We studied 3 adult men who did not have other major signs of vascular disease but who exhibited the complete syndrome. The initial paresis of the oculomotor, trochlear, and abducens nerves varied in each individual and slowly cleared over days to weeks in all, but vision did not return in any. Each had mild‐to‐moderate signs of hemispheric dysfunction. In each, the thrombus extended from the origin of the internal carotid to its intracranial bifurcation into the anterior and middle cerebral arteries. It also occluded the proximal one‐half to two‐thirds of the ophthalmic artery. None had evidence of vasculitis or compromise of the posterior circulation.
Development of dementing illnesses in an 80‐year‐old volunteer cohort
Authors: Katzman R. , Aronson M. , Fuld P. , Kawas C. , Brown T. , Morgenstern H. , Frishman W. , Gidez L. , Eder H. , Ooi W. L. 
Published: January 2005

AbstractWe have prospectively followed over a 5‐year period 434 volunteers who were at intake ambulatory, functional, presumably nondemented, and between 75 and 85 years of age. Fifty‐six (an incidence of 3.53 per 100 person‐years at risk) developed a progressive dementia: 32 met diagnostic criteria for Alzheimer's disease (AD) (an incidence of 2.0 per 100 person‐years at risk), 15 had vascular or mixed dementia, and 9 had other disorders or remain undiagnosed. New cases of dementia were as common as myocardial infarction and twice as common as stroke. Risk factors for both dementia and AD were age (over 80) and gender (female); other reported risk factors such as family history, prior head injury, thyroid disease, maternal age, and smoking were not risk factors for AD in this elderly cohort. Prior stroke was the major risk factor for vascular or mixed dementia; diabetes and left ventricular hypertrophy but not a history of hypertension per se were also risk factors for vascular dementia. The major predictor of the development of AD was the mental status score on entry. The 58.5%f of the cohort who made zero to two errors on a 33‐item mental status test had a less than 0.6% per year chance of developing AD, whereas the 16% of the cohort with five to eight errors on this test developed AD at a rate of over 12% per year. Thus, it is possible to identify a large cohort of 80‐year‐olds who are at low risk for AD and a smaller cohort at very high risk.
The effect of intraocular 6‐hydroxydopamine on retinal processing of primates
Authors: Ghilardi Maria Felice , Marx Marcia S. , Bodis‐Wollner Ivan , Camras Carl B. , Glover Andrew A. 
Published: January 2005

AbstractIn previous studies, we showed that in the monkey, systemically administered N‐methyl, 4‐phenyl, 1‐2‐3‐6 tetrahydropyridine (MPTP) produces a chronic parkinsonian syndrome accompanied by spatial frequency‐dependent abnormalities in both the pattern electroretinogram and visual evoked potential. We describe the effect of intravitreally administered 6‐hydroxydopamine (6‐OH‐DA) on the pattern electroretinogram and pattern visual evoked potential of 3 aphakic monkeys. Because of the aphake condition, several complexities of intravitreal injection of 6‐OH‐DA could be avoided. Nevertheless, following 6‐OH‐DA treatment, both the phase and the amplitude of pattern electroretinogram and pattern visual evoked potential became abnormal. This abnormality was most pronounced for the higher spatial frequencies (2.5 and 3.5 cycles per degree), whereas lower spatial frequencies (0.5 and 1.2 cycles per degree) were less impaired. The effects of systemically administered MPTP on pattern electroretinogram and pattern visual evoked potential are similar to the effects of intravitreal injections of 6‐OH‐DA, suggesting that a retinal catecholaminergic system plays an important role in pattern vision of primates.
Catecholamine depletion produces irrepressible saccadic eye movements in normal humans
Authors: Tychsen Lawrence , Sitaram N. 
Published: January 2005

AbstractTo determine the effect of catecholamine depletion on ocular motor pathways in humans, we studied the eye movements of 3 normal subjects who received the drug metyrosine (alpha‐methylparatyrosine). This drug temporarily depleted dopamine and norepinephrine, as measured by a reduction in the metabolite, 3‐methoxy‐4‐hydroxy‐phenylethyleneglycol (MHPG). Saccadic, pursuit, and vestibulo‐ocular eye movements were recorded using infrared oculography with subjects both on placebo and on metyrosine. The most consistent effect observed with metyrosine was an increase in the amplitude and frequency of saccadic intrusions during fixation and pursuit. Two of the 3 subjects also had shortened time constants for the vestibulo‐ocular reflex, attributable in part to the sedative effect of catecholamine depletion. The increase in saccadic intrusions implies that catecholamines modulate the activity of a subpopulation of suppressor motor neurons in the human brainstem.
Assessment of genetic risk for alzheimer's disease among first‐degree relatives
Authors: Farrer Lindsay A. , O'Sullivan Dianne M. , Cupples L. Adrienne , Growdon John H. , Myers Richard H. 
Published: January 2005

AbstractLife table methods were used to determine the relative risk of Alzheimer's disease (AD) in relatives of index cases with AD. Risk of AD was assessed in 967 first‐degree relatives of 128 probands with clinically diagnosed AD and 572 first‐degree relatives of a control group consisting of 84 subjects with Parkinson's disease (PD). Using a method that weights likelihood of correct diagnosis of AD, cumulative risk among AD relatives by age 93 was 24%, whereas relatives of the PD probands had a weighted risk of 16% by age 90. Overall, the total lifetime risk of developing dementia was similar among first‐degree relatives of patients with AD and those of patients with PD. The age‐specific risks were much different, however; from the age of 65 years to the age of 80 years, relatives of patients with AD had a twofold to fourfold increased risk of dementia. Equal risks were found for parents and siblings and for male and female relatives after adjustment for sex‐specific patterns of survivorship. Relatives of probanwith early‐onset (> 67 years) and late‐onset (> 67 years) AD had equivalent risks of developing illness. Because onset age was found to cluster in families, the apparent increase in cumulative incidence for dementia in relatives of early‐onset cases was likely due to relatives of the late‐onset cases dying before developing illness. Our interpretation of these data is that a proportion of AD cases is caused by a fully penetrant gene transmitted as an autosomal dominant with age‐dependent penetrance, whereas other cases, including some with multiple affected members, are clinically indistinguishable and of nongenetic cause.
Decrease of suppressor inducer (cd4+ 2h4+) t cells in multiple sclerosis cerebrospinal fluid
Authors: Chofflon Michel , Weiner Howard L. , Morimoto Chikao , Hafler David A. 
Published: January 2005

AbstractT‐lymphocytes in the cerebrospinal fluid of patients with multiple sclerosis are predominantly CD4+ (inducer) as opposed to CD8+ (suppressor/cytotoxic) T cells. The CD4+ lymphocytes can be subdivided into populations that express high densities of the CDw29 (4B4) determinant and have helper inducer function or express high densities of CD45R (2H4) determinant and have suppressor inducer function. In the present study, we characterized the nature of these CD4+ T cells in the cerebrospinal fluid of patients with multiple sclerosis by performing flow cytometric analysis on paired samples of blood and cerebrospinal fluid. There were significantly lower percentages of CD4+ 2H4+ T cells in the cerebrospinal fluid than in the peripheral blood (p = 0.001, paired t test). In contrast, there were increased percentages of helper inducer (CD4+ 4B4+) T cells in the cerebrospinal fluid (p = 0.001, paired t test), compared with the peripheral blood. Analysis of subjects with other inflammatory disorders of the central nervous system did not show significant decreases in CD4+ 2H4+ T cells in cerebrospinal fluid, though in some, decreases were also observed. These results indicate that the CD4+ T cells in the cerebrospinal fluid of patients with multiple sclerosis are predominantly helper inducer, as opposed to suppressor inducer T cells, and that the relative decrease of suppressor inducer cells in the peripheral blood of multiple sclerosis patients is not due to their migration to the cerebrospinal fluid. Furthermore, the increased numbers of helper inducer cells in the cerebrospinal fluid may contribute to local autoimmune processes in the central nervous system compartment of multiple sclerosis patients.
The effect of carbamazepine on cerebral glucose metabolism
Authors: Theodore William H. , Bromfield Edward , Onorati Linda 
Published: January 2005

AbstractWe used positron emission tomography with 18F‐2‐deoxy‐D‐glucose to study the effect of carbamazepine on local cerebral metabolic rate for glucose (lCMRGlc) in 9 patients with complex partial seizures. Twenty regions of interest were evaluated. Seven control patients had serial scans without a drug change. Metabolic rates were significantly (p ± 0.05) lower in patients on carbamazepine in 6 of 20 regions of interest (3 left cerebral hemisphere, 3 right). Mean lCMRGlc was 7.4 ± 2.0 mg/min/100 in patients on carbamazepine and 8.8 ± 2.5 in patients off carbamazepine (p < 0.00005; cutoff level for 180 comparisons: 0.00027). The mean (± SEM) difference in 1CMRGlc between scans was 12 ± 2%. No significant changes in lCMRGlc on serial scans were detected in any of the 20 regions for the control group. The mean (± SEM) variation for control regions of interest was 1 ± 1%. This study showed that carbamazepine depresses cerebral glucose metabolism as much as phenytoin does, but much less than phenobarbital does. The difference in effect on lCMRGlc may be related to drug mechanisms of action, as well as to effects on memory, learning, mood, and behavior.
Apraxia of eyelid opening secondary to right hemisphere infarction
Authors: Johnston James C. , Rosenbaum Daniel M. , Picone Carmela M. , Grotta James C. 
Published: January 2005

AbstractA variety of eyelid movement abnormalities have been attributed to lesions of the central nervous system. Apraxia of lid movements, and especially of lid opening, has received the least attention. We present 2 cases of lid opening apraxia and propose that this abnormality may be due to right hemisphere dysfunction.
Parkinsonism and amiodarone therapy
Authors: Werner Elke G. , Olanow C. Warren 
Published: January 2005

AbstractWe report a case of reversible, dose‐related parkinsonian tremor in a patient taking amiodarone. In previous writings by others, basal ganglia dysfunction associated with amiodarone was dose related and reversibility was inversely related to duration of therapy. Patients receiving amiodarone are at risk for the development of basal ganglia dysfunction which may persist if the drug is not discontinued or the dosage reduced.
Amaurosis fugax and ocular infarction in adolescents and young adults
Authors: Tippin Jon , Corbett James J. , Kerber Richard E. , Schroeder Erwin , Thompson H. Stanley 
Published: January 2005

AbstractBecause the cause and natural history of amaurosis fugax and ocular infarction are unknown in most younger patients, we reviewed the records of 83 patients who had become symptomatic before the age of 45. Cerebral transient ischemic attacks had occurred in 9 of these patients but no case of stroke was found. A striking feature of these patients was that 41° had headache or orbital pain accompanying their amaurotic spells and an additional 25.3° had severe headaches independent of the visual loss. Results of laboratory studies were rarely abnormal and echocardiography disclosed that only 1 patient had previously unknown heart disease. Mitral valve prolapse was detected in 6.5°, a figure similar to that expected for the general population. Of the original 83 patients, 42 were reexamined after a mean period of 5.8 years. None of the patients in this group had had a stroke, and the clinical status at follow‐up was not found to correlate with the duration of the visual loss (amaurosis fugax versus ocular infarction), frequency (single versus recurrent episodes), sex, presence of headache or heart disease, cigarette smoking, use of oral contraceptives, or abnormal findings on echocardiograms or laboratory studies. We conclude that amaurosis fugax and ocular infarction occurring in the younger patient are probably associated with a more benign clinical course than that seen in older persons and that migraine is a likely cause for the episodes of visual loss in a majority of this group. Because of this, we believe that a conservative approach to the evaluation of such patients seems warranted.