bluuebunny/crossref_metadata_2025_split huggingface dataset:
Incidence and clinical features of trigeminal neuralgia, Rochester, Minnesota, 1945–1984
Authors: Katusic Slavica , Beard C. Mary , Bergstralth Erik , Kurland Leonard T. 
Published: January 2005

AbstractThe overall crude incidence rate of trigeminal neuralgia per 100,000 population in Rochester, Minnesota, for 1945 through 1984 was 4.3 for both sexes combined. The age‐adjusted (to total 1980 US population) rate for women (5.9) was significantly higher than that for men (3.4). Annual incidence rates increased significantly with age in both women and men. Data based on evidence in the medical records suggest that trigeminal neuralgia is a rather rare and unpredictable disease: The number of episodes varies from 1 to 11, and length of episode from 1 day to 4 years. This study indicates an elevated relative risk associated with hypertension (both overall and among women in the cohort). Also, prevalence of multiple sclerosis was significantly greater among the group of 75 patients who had trigeminal neuralgia. Since the data on treatment in this study do not correspond to a randomized clinical trial, they are presented as empirical results only and show no single treatment of choice for trigeminal neuralgia.
Immunological studies in tropical spastic paraparesis
Authors: Jacobson Steven , Gupta Ajay , Mattson David , Mingioli Elizabeth , McFarlin Dale E. 
Published: January 2005

AbstractTropical spastic paraparesis (TSP) and other chronic‐progressive myelopathies have been clearly associated with increased serum and cerebrospinal fluid antibody titers to human T‐lymphotropic virus type I (HTLV‐I). However, little is known about the cellular immune function in TSP. In the present study, activated T lymphocytes were found in the peripheral blood of patients with TSP. Specifically, there were increased numbers of large CD3+ cells that also expressed HLA‐DR and interleukin‐2–receptor molecules. A significantly elevated spontaneous lymphoproliferative response was demonstrated in all patients tested. Generation of measles virus–specific cytotoxic T‐cell response was reduced in 4 of 4 patients. This was similar to previous findings in patients with multiple sclerosis. However, unlike multiple sclerosis, reduced generation of cytotoxic T‐cell response to influenza and mumps viruses was observed in 2 of 4 patients. These observations confirm further the strong association between TSP and an HTLV‐I–like virus and suggest that the observed abnormalities of the cellular immune response in TSP are related to infection of lymphocytes by the retrovirus.
Age‐related changes in the human nigrostriatal dopaminergic system
Authors: De Keyser Jacques , Ebinger Guy , Vauquelin Georges 
Published: January 2005

AbstractA deficiency of dopamine in the striatum may contribute to the decline in motor function associated with aging. We examined the effect of aging on the densities of the D1 and D2 dopamine receptors, their high‐agonist affinity sites, and the dopamine uptake sites in postmortem human putamen (n = 32; age range, 19–88 years). With aging a steady decrease was seen in dopamine uptake sites (p < 0.001), confirming previous morphometric and biochemical data of an age‐related loss of nigrostriatal axons. In contrast, the concentrations of the D1 and D2 dopamine receptors and their high‐agonist affinity sites, which are believed to represent the functionally active receptors, were not affected by the aging process. These results may have implications for the pharmacological treatment of the age‐related decline in motor function.
Degeneration of spinocerebellar neurons in amyotrophic lateral sclerosis
Authors: Williams Celia , Kozlowski Mark A. , Hinton David R. , Miller Carol A. 
Published: January 2005

AbstractThe selective involvement of spinocerebellar neurons in sporadic amyotrophic lateral sclerosis was investigated using two monoclonal antibodies that have neuronal subset specificity in human spinal cord. In normal control subjects, monoclonal antibody 6A2 showed specificity for neurons of the dorsal nucleus of Clarke, the cells of origin of the dorsal spinocerebellar tract. Immunoreactive neurons were also observed in locations corresponding to the central cervical nucleus and spinal border region, containing neurons of the cervicospinocerebellar and ventral spinocerebellar tracts, respectively. The latter two neuronal subsets are indistinguishable from surrounding neurons when conventional histological stains are used. Antigen 6A2 was distributed on surfaces of neuronal somas and proximal neurites and extended into the extracellular space. A second antibody, monoclonal antibody 44.1, labeled the cytoplasm of neuronal somas and neurites, including all monoclonal antibody 6A2‐reactive cells and alpha motoneurons. In spinal cords of all 5 patients with amyotrophic lateral sclerosis, monoclonal antibody 6A2 reactivity in the majority of spinocerebellar neurons was absent or localized to the somal cytoplasm, which still stained with monoclonal antibody 44.1. In more severely involved tissues, there was loss of some spinocerebellar neurons and a corresponding loss of monoclonal antibody 44.1 reactivity. These findings confirm involvement of the spinal cord components of the spinocerebellar system at all levels in sporadic amyotrophic lateral sclerosis and suggest that some surface molecules are modified during the degenerative process.
DNA length polymorphism 5′ to the myelin basic protein gene is associated with multiple sclerosis
Authors: Boylan Kevin B. , Takahashi Naoki , Paty Donald W. , Sadovnick Adele D. , Diamond Marc , Hood Leroy E. , Prusiner Stanley B. 
Published: January 2005

AbstractA site of DNA polymorphism linked to the myelin basic protein gene, identified as restriction fragment length polymorphism, was analyzed in a population‐based study comparing patients with clinically definite multiple sclerosis (MS) and population‐matched control subjects. A 0.9‐kilobase (kb) genomic DNA fragment (EcoG) encompassing the first exon of the human myelin basic protein gene, located on the long arm of chromosome 18, identified ten alleles arising from a region of DNA, 1.5 kb 5′ to the myelin basic protein gene first exon coding region. Produced by RsaI digests and ranging in length from 2.05 to 2.15 kb, these alleles vary in size by up to 100 base pairs due to insertion or deletion, or both, from a 1‐kb length of repetitive DNA. Allele frequencies among 65 patients with MS were compared with those of 63 control subjects. Chi square for these data was significant (p<0.001), largely due to a preponderance in the patients with MS of alleles in the 2.14‐ to 2.15‐kb range. Comparison of the numbers of patients with MS and control subjects bearing specific alleles showed that 45% of the patients carried at least one allele of 2.14 to 2.15 kb as opposed to 19% of control subjects (p<0.005). These data, while preliminary, suggest that patients with MS differ from population‐matched control subjects with respect to DNA polymorphism linked to the myelin basic protein gene. Although no pathogenic relationship between this polymorphism and MS can be presupposed, this finding raises the possibility that the myelin basic protein gene or some other myelin basic protein‐linked locus may be a factor in susceptibility to MS.
Microvascular changes in early and advanced dermatomyositis: A quantitative study
Authors: Emslie‐Smith Alison M. , Engel Andrew G. 
Published: January 2005

AbstractIn adult dermatomyositis 10 muscle specimens with no or minimal histological alterations were compared with 7 that showed typical alterations. Five specimens from patients with inclusion body myositis, 5 from patients with polymyositis, and 8 from normal subjects served as controls. Vascular endothelium, visualized with the lectin Ulex europaeus agglutinin I, and complement membrane attack complex were demonstrated in the same cryostat sections by paired immunofluorescence. Large randomly selected fields were analyzed to determine the number of capillaries per square millimeter of fiber area (capillary density), per 1,000‐m̈m2 area of each muscle fiber (capillary index), and in 100 × 100m̈m grid squares. In dermatomyositis specimens with minimal structural alterations there was focal capillary depletion, the capillary density was significantly reduced, and the frequency distributions of the capillary index and grid count were shifted to the left. In advanced dermatomyositis specimens, the findings were similar but more severe. In both kinds of specimens, clusters of capillaries reacted for complement membrane attack complex. The 2 patients with the highest proportion of vessels positive for membrane attack complex had a fulminant and fatal course. In polymyositis and inclusion body myositis specimens, the capillaries had a normal overall density and none reacted for membrane attack complex. The findings imply that the capillaries are an early and specific target of the disease process in dermatomyositis.
Striatal and nigral neuron subpopulations in rigid Huntington's disease: Implications for the functional anatomy of chorea and rigidity‐akinesia
Authors: Albin Roger L. , Reiner Anton , Anderson Keith D. , Penney John B. , Young Anne B. 
Published: January 2005

AbstractNeuropeptide immunohistochemistry was used to test several hypotheses of the anatomical bases of chorea and rigidity‐akinesia. To test the hypothesis that elevated concentration of striatal somatostatin causes chorea, we visually compared the density of striatal neurons containing somatostatin and neuropeptide Y in brains affected by choreic or rigid‐akinetic Huntington's disease (HD). The density of these neurons was elevated in both rigid‐akinetic and choreic HD specimens with an apparently normal total number of these neurons, indicating that elevated somatostatin concentration, by itself, does not lead to chorea. We tested the hypothesis that rigid‐akinetic HD results from deficient dopaminergic nigrostriatal neurotransmission by examining tyrosine hydroxylase–immunoreactive (TH‐IR) neurons in the substantia nigra. In rigid‐akinetic HD brains, there was no obvious reduction of nigral TH‐IR neurons, indicating that rigid‐akinetic HD is probably not due to loss of nigral dopaminergic neurons. Finally, we also examined the status of striatal projection neurons and found near total loss of all striatal neurons projecting to the lateral globus pallidus, medial globus pallidus, and substantia nigra in brains affected by rigid‐akinetic HD in contrast to the preservation of neurons projecting to the medial globus pallidus in choreic HD. These results are consistent with the hypothesis that chorea results from preferential loss of striatal neurons projecting to the lateral globus pallidus and that rigid‐akinetic HD is a consequence of the additional loss of striatal neurons projecting to the medial segment of the pallidum.
The prevalence of dementia and Alzheimer's disease in Shanghai, China: Impact of age, gender, and education
Authors: Zhang Mingyuan , Katzman Robert , Salmon David , Jin Hua , Cai Guojun , Wang Zhengyu , Qu Guangya , Grant Igor , Yu Elena , Levy Paul , Klauber Melville R. , Liu William T. 
Published: January 2005

AbstractWe report the prevalence rates for dementia and Alzheimer's disease (AD) obtained from a probability sample survey of 5,055 noninstitutionalized older persons in Shanghai, China. A two‐stage procedure was used for case finding and case identification. A Chinese version of the Mini‐Mental State Examination was used to determine cases of possible dementia. Three different cutoff points on this mental status test were used depending on the respondent's level of education. Clinical evaluations, based on functional assessments and psychiatric inverview, medical and neurological examinations, three standardized mental status tests, and a selected group of psychometric tests, were made in the second stage of the study to ascertain the clinical diagnoses of dementia and AD utilizing the Diagnostic and Statistical Manual for Mental Disorders, edition 3 and National Institute of Neurological and Communicative Disorders and Stroke–Alzheimer's Disease and Related Disorders Association criteria, respectively. The prevalence rate of dementia in persons 65 years and older was 4.6%. Clinically diagnosed AD accounted for 65% of the subjects with dementia. These findings indicate that the prevalence of dementia in Shanghai is very much higher than figures published earlier for China and Japan, and at the lower part of the range of values reported for community residents in the United States and other Western countries, but less than half of that reported in the recently published survey of the elderly in East Boston. Increasing age, gender (female), and low education are each highly significant and independent risk factors for dementia. One hypothesis to explain the increased prevalence in elderly women who had received no formal education invokes the possibility of an effect of early deprivation, perhaps lowering brain %reserve,% allowing the symptoms of dementia to appear at an earlier date during disease progression.
Normocalcemic tetany abolished by calcium infusion
Authors: Day John West , Parry Gareth J. 
Published: January 2005

AbstractTetany is a disorder of increased neuronal excitability usually associated with hypocalcemia. We studied a patient with typical tetanic cramps that responded to intravenous infusion of calcium despite normal serum concentrations of total and ionized calcium. Three generations were affected by similar symptoms, suggesting that this is a dominantly inherited disorder of neuronal hyperirritability.
Intracranial hypertension associated with unruptured cerebral arteriovenous malformations
Authors: Chimowitz Marc I. , Little John R. , Awad Issam A. , Sila Cathy A. , Kosmorsky G. , Furlan Anthony J. 
Published: January 2005

AbstractOnly 6 patients with intracranial hypertension associated with unruptured cerebral arteriovenous malformations have been reported. We report 6 additional patients seen at the Cleveland Clinic during the past 10 years. The average age was 28 years (range, 19–44 years); 4 were women. Symptoms and signs included papilledema (6 patients), headache (6), transient nonepileptic focal symptoms (4), visual obscurations (3), ipsilateral carotid or ocular bruits (3), abnormal visual fields (3), focal seizures (2), and progressive visual loss (1). Enhanced computed tomography (CT) or magnetic resonance imaging (MRI) demonstrated the malformations in all 6 patients. The malformations were large, supplied by the branches of the middle and anterior cerebral arteries, with the posterior cerebral artery contributing in 3 patients, and all drained into the superior sagittal sinus. Associated venous obstruction was seen in 2 patients. Four patients underwent excision of the arteriovenous malformation, with resolution of papilledema in all 4. Measurements of cortical arterial and venous pressures during surgery in 3 patients showed decreased feeding artery pressures and elevated draining vein pressures, which normalized after removal of the malformation. Treatment in the 2 remaining patients consisted of medical therapy (acetazolamide, furosemide, steroids) alone in 1 patient, and in conjunction with proton beam radiation in the other. Papilledema resolved in the former patient, but the patient receiving proton beam radiation still had papilledema 2 years later. Intracranial hypertension associated with unruptured cerebral arteriovenous malformations occurs in young patients with high flow malformations that drain into the superior sagittal sinus, and is likely the result of increased cortical venous and superior sagittal sinus pressure. Excision of the malformation effectively reduces the intracranial pressure.
Mechanism of muscle wasting in myotonic dystrophy
Authors: Griggs Robert C. , Jozefowicz Ralph , Kingston William , Nair K. Sreekumaran , Herr Barbara E. , Halliday David 
Published: January 2005

AbstractMyotonic dystrophy is associated with progressive muscular atrophy. In order to determine the mechanism of muscle wasting in this condition, we measured fractional mixed skeletal muscle protein synthesis in the postabsorptive state in 8 patients with myotonic dystrophy, and compared the results with those of 10 normal subjects. Fractional muscle protein synthesis was determined by measuring the increment of 13C leucine in mixed skeletal muscle protein obtained by needle biopsy from the quadriceps muscle during a primed‐continuous infusion of L‐(1‐13C) leucine. We used plasma 13C α‐ketoisocaproate (representing intracellular leucine labeling) as the precursor pool for the calculation of fractional muscle protein synthesis and leucine kinetics. Fractional muscle protein synthesis was depressed in the patients with myotonic dystrophy (28% decrease, p < 0.02). Leucine flux, leucine oxidation, and the nonoxidative portion of leucine flux were not different between the patients with myotonic dystrophy and the normal control subjects. Muscle atrophy in myotonic dystrophy reflects a selective decrease in muscle protein synthesis without any similar decrease in nonmuscle protein synthesis. This decrease may result from an impaired end‐organ response to anabolic hormones or substrates.
Antibody response to rubella virus structural proteins in multiple sclerosis
Authors: Nath Avindra , Wolinsky Jerry S. 
Published: January 2005

AbstractThe antibody response to the structural proteins of rubella virus was studied in patients with multiple sclerosis (MS). Irrespective of the antibody titer to whole rubella virus, the relative proportion of the IgG response to the surface glycoprotein E1 was diminished, and that to the surface glycoprotein E2 was elevated in MS patients when compared to a matched control population of normal healthy individuals or a group of patients with systemic lupus erythematosus and other collagen vascular diseases. No difference was observed in the response to the core protein of rubella virus on comparing the MS and normal control groups. This divergence in the relative antibody response to the viral surface proteins suggests that the vigorous antibody response to rubella virus reported in MS is not simply an expression of a nonspecific polyvalent B‐cell response.
Detection of the anti‐Hu antibody in the serum of patients with small cell lung cancer—A quantitative western blot analysis
Authors: Dalmau Josep , Furneaux Henry M. , Gralla Richard J. , Kris Mark G. , Posner Jerome B. 
Published: January 2005

AbstractWe looked for the presence of the anti‐Hu antibody in the sera from 50 normal subjects; 44 patients with small cell lung cancer, not associated with paraneoplastic disease; and 25 patients with small cell lung cancer associated with paraneoplastic sensory neuropathy, encephalomyelitis, or both. Using the avidin‐biotin immunoperoxidase method and a highly sensitive quantitative Western blot analysis, the anti‐Hu antibody was not detected in the 50 normal human sera. Seven of the 44 patients with small cell lung cancer but no paraneoplastic syndrome had detectable levels (average titer, 76 U/ml) of anti‐Hu antibody on Western blot. These levels are significantly lower than the average titer of the 25 patients who had small cell lung cancer and paraneoplastic sensory neuropathy or encephalomyelitis (average titer, 4,592 U/ml). In the group with nonparaneoplastic small cell lung cancer (low anti‐Hu titer) there was a predominance of women (5 women: 2 men), and all patients had “limited” disease when diagnosed. In the antibody‐negative group the sex ratio was 16 women to 21 men and 51% of the patients had “extensive” disease. None of the 7 patients with a low‐titer anti‐Hu antibody developed a paraneoplastic syndrome by the time of writing. The anti‐Hu antibody appears, when present, to be a good marker for small cell lung cancer and, when present at high titer, for small cell lung cancer associated with a paraneoplastic syndrome.
Increase in differentiated type of T lineage cells in the myasthenic thymus: Two‐color fluorocytometric analysis
Authors: Fujii Naoki , Itoyama Yasuto , Goto Ikuo 
Published: January 2005

AbstractWe made use of two‐color flow cytometry to examine thymic lymphoid cells from patients with myasthenia gravis. The CD4+CD8− cells and the CD4−CD8+ cells were significantly increased in the thymus from the patients, compared with findings in the control samples. Conversely, the CD4+CD8+ cells were significantly decreased, Significant increases in CD1−CD3+ cells and significant decreases in CD1+CD3− cells were noted. The percentage of CD45R+ cells also increased. There were no increases in activated T lymphocytes, defined as CD4+DR+ cells, CD8+DR+ cells, or IL2R+ cells. We conclude that the proportion of T lineage lymphocytes that are differentiated is increased in the thymus of patients with myasthenia gravis, and could reflect accelerated differentiation.
Increased ratio of quinolinic acid to kynurenic acid in cerebrospinal fluid of D retrovirus—infected rhesus macaques: Relationship to clinical and viral status
Authors: Heyes Melvyn P. , Mefford Ivan N. , Quearry Bonnie J. , Dedhia Manish , Lackner Andrew 
Published: January 2005

AbstractIncreased concentrations of excitotoxin quinolinic acid in cerebrospinal fluid (CSF) are associated with infection with the human immunodeficiency virus (HIV‐1) and have been implicated in the pathogenesis of the acquired immune deficiency syndrome (AIDS) dementia complex. In the present study, inoculation of macaques with D/1/California, an immunosuppressive serotype 1 type D retrovirus, was associated with acute and chronic increases in CSF and serum quinolinic acid concentrations in macaques that had developed SAIDS, a simian disease analogous to AIDS in humans–particularly macaques with demonstrable opportunistic infections. Kynurenic acid, an antagonist of excitatory amino acid receptors as well as the excitotoxic effects of quinolinic acid, was also increased in the CSF of SAIDS macaques, but to a significantly lesser degree than was quinolinic acid (kynurenic acid, 1.8‐fold; quinolinic acid, 15.6‐fold). CSF quinolinic acid, but not kynurenic acid, was also increased in viremic macaques with SAIDS‐related complex (2.4‐fold) and asymptomatic virus positive carriers (3.4‐fold). Macaques that had recovered from D/1/California infection and were antibody positive and virus negative had normal CSF quinolinic acid and kynurenic acid concentrations. Increased activity of indoleamine‐2,3‐dioxygenase, the first enzyme of the kynurenine pathway, was indicated in the macaques with SAIDS by reduced serum L‐tryptophan and elevated serum L‐kynurenine concentrations. Macaques infected with D/1/California may provide a primate model for investigation of the mechanisms involved in increases in CSF quinolinic acid in retrovirus and other infectious diseases, including HIV‐1. It remains to be determined whether the increased CSF quinolinic acid concentrations and the increased ratio of quinolinic acid to kynurenic acid have neurological significance or are a useful “marker” of infection.
Cerebrospinal fluid choline levels are decreased in Parkinson's disease
Authors: Manyam Bala V. , Giacobini Ezio , Colliver Jerry A. 
Published: January 2005

AbstractWe examined acetylcholinesterase (AChE) activity and choline levels in cerebrospinal fluid (CSF) in 16 patients with idiopathic Parkinson's disease and 9 control subjects of corresponding age: 8 were untreated Parkinson's patients; 4 were treated with carbidopa‐levodopa (100/1,000 mg/day) for 20 ± 3 months; and 4 were treated with carbidopa‐levodopa (110/1,100 mg/day) for 28 ± 18 months plus amantadine (220 mg/day) for 16 ± 8 months. CSF choline levels (nmol/ml) were 2.97 ± 0.79 (control subjects); 1.31 ± 0.29 (untreated patients); 1.00 ± 0.29 (carbidopa‐levodopa treated); and 1.26 ± 0.19 (carbidopa‐levodopa/amantadine treated). Choline levels were significantly lower in untreated and treated patients compared to control subjects (p = 0.0001). AChE activity did not differ in Parkinson's disease patients as compared to control subjects. The reduced level of choline in CSF may reflect a deficit in choline transport into the brain or a decrease of choline‐phospholipid output from the brain.