bluuebunny/crossref_metadata_2025_split huggingface dataset:
Topical lidocaine gel relieves postherpetic neuralgia
Authors: Rowbotham Michael C. , Davies Pamela S. , Fields Howard L. 
Published: January 2005

AbstractPostherpetic neuralgia (PHN) following herpes zoster is a common and disabling neuropathic pain syndrome. In a double‐blind, three‐session study, 5% lidocaine gel or vehicle was applied simultaneously to both the area of pain and to the contralateral mirror‐image unaffected skin. In the local session, lidocaine gel was applied to the painful skin area. In the remote session, lidocaine gel was applied to mirror‐image skin. In the placebo session, vehicle was applied bilaterally. For cranial PHN, gel was applied without occlusion for 8 hours. For limb or torso PHN, gel was applied under occlusion for 24 hours. The 16 subjects with cranial PHN reported pain relief significantly favoring local drug application at 30 minutes, 2, 4, and 8 hours. The 23 subjects with torso or limb PHN reported significantly lower pain intensity with local drug application at 8 hours and both pain relief and reduced pain intensity at 24 hours. Remote lidocaine application to mirror‐image skin was no different from placebo. No systemic adverse effects were reported and blood levels did not exceed 0.6 μg/ml. Topical application of 5% lidocaine gel relieves PHN pain by a direct drug action on painful skin.
Concentrations of amyloid β protein in cerebrospinal fluid of patients with alzheimer's disease
Authors: Van Gool W. A. , Walstra G. J. M. , Bolhuis P. A. , Kuiper M. A. , Wolters E. Ch 
Published: January 2005

AbstractDeposition of fibrillar amyloid β protein (Aβ) is increased in brains of patients with Alzheimer's disease. Concentrations of Aβ were measured in cerebrospinal fluid with an enzyme‐linked immunosorbent assay in 10 neurological patients free from neurodegenerative disease, 28 patients with Parkinson's disease, and 18 patients with probable Alzheimer's disease. Levels of Aβ in cerebrospinal fluid were not significantly different among these groups. This observation suggests that concentrations of soluble Aβ in cerebrospinal fluid as measured in this study do not reflect the amount of fibrillar, aggregated Aβ in the brain of patients with Alzheimer's disease.
Proton magnetic resonance spectroscopic imaging in patients with frontal lobe epilepsy
Authors: Garcia Paul A. , Laxer Kenneth D. , van der Grond Jeroen , Hugg James W. , Matson Gerald B. , Weiner Michael W. 
Published: January 2005

AbstractProton magnetic resonance spectroscopic imaging (1H MRSI) has demonstrated decreased N‐acetyl compounds (NA) in the epileptogenic hippocampus in patients with temporal lobe epilepsy. We studied 8 patients with frontal lobe epilepsy and found mean NA/creatine (Cr) in the epileptogenic frontal lobe decreased by 27% compared with that of the contralateral homologous region (1.81 ±0.36 vs 2.49 ± 0.60, p < 0.008). In every patient, NA/Cr was decreased in the epileptogenic region by at least 5%. These findings suggest that 1H MRSI may be useful in the presurgical evaluation of patients with frontal lobe epilepsy.
JC virus DNA in cerebrospinal fluid of human immunodeficiency virus—infected patients: Predictive value for progressive multifocal leukoencephalopathy
Authors: McGuire Dawn , Barhite Steven , Hollander Harry , Miles Michael 
Published: January 2005

AbstractProgressive multifocal leukoencephalopathy (PML) is a lytic infection of oligodendrocytes by the human papovavirus JC. Patients with defects in cell‐mediated immunity are at risk for active disease: a usually lethal demyelination of the brain. PML develops in at least 4% of patients with the acquired immunodeficiency syndrome (AIDS). Definitive diagnosis currently requires brain biopsy. Previous attempts to detect JC virus DNA by polymerase chain reaction in cerebrospinal fluid of PML patients, particularly those with human immunodeficiency virus type 1 (HIV ‐ 1) infection, have been of low sensitivity. In the present study, cerebrospinal fluid was assayed by polymerase chain reaction from 26 HIV‐1‐positive patients with PML, 114 HIV‐1‐positive control subjects, and 16 control subjects who were HIVv1 negative or were without risk factors for HIV disease. Polymerase chain reaction conditions were optimized to detect a single copy of viral DNA in 50 μl of cerebrospinal fluid. Specificity of the polymerase chain reaction product was confirmed by size on gel electrophoresis and Southern blot hybridization. JC virus DNA was detected in 24 of 26 samples from patients with PML: 8 of 8 with tissue diagnosis and 16 of 18 with strong clinical and magnetic resonance imaging evidence of PML. Among control subjects, 11 of 130 samples were positive for JC virus: 10 of 114 samples from HIV‐infected patients and one from an HIVvnegative patient with risk factors for PML and an unexplained hemiparesis. Overall senstivity was 92% (24/26); specificity was, at minimum, 92% (119/130). Treatments for PML are now in clinical trials. This assay provides a safe, inexpensive means of establishing the diagnosis of a potentially treatable condition. Furthermore, our data suggest that a subgroup of presymptomatic patients who may be at high risk for PML can be identified. Such patients may be particularly suitable for prophylactic interventions.
Pontine ischemic rarefaction
Authors: Pullicino Patrick , Ostrow Peter , Miller Lucia , Snyder Wendy , Munschauer Frederick 
Published: January 2005

AbstractTo investigate apparently asymptomatic, bilateral symmetrical predominantly pontine hyperintensities (PHI) on magnetic resonance imaging (MRI) scans in elderly patients, we examined the pons histopathologically in two brains of elderly hypertensives with PHI, and in three without PHI, on postmortem MRI scans. We also reviewed 85 serial in vivo MRI scans of patients over 60 and compared scan findings, vascular risk factors, and clinical symptoms between patients with PHI and a control group. A subcortical arteriosclerotic encephalopathy (SAE)‐like pathology was present in the pons in only the two autopsy brains with PHI and corresponded with the location of PHI on the postmortem MRI scans and with the most frequent sites of PHI on in vivo scans. SAE also involved the hemispheric white matter in one of the autopsy brains. Five of 16 (31%) patients with, and 4 of 69 (6%) without, PHI on in vivo MRI scans had marked periventricular hyperintensity (PVHI) compatible with SAE (p = 0.01). We conclude that an SAE‐like pathology may be seen in the pons in elderly hypertensives and this pathology is probably the cause of PHI seen on MRI scans of patients over 60 years of age.
X‐linked vacuolated myopathy: Complement membrane attack complex on surface membrane of injured muscle fibers
Authors: Villanova Marcello , Louboutin Jean Pierre , Chateau Danielle , Eymard Bruno , Sagniez Marguerite , Toméa Fernando M. S. , Fardeau Michel 
Published: January 2005

AbstractWe describe a probable recessive X‐linked myopathy characterized by the presence of vacuolated muscle fibers. Four males and their shared maternal grandfather were affected. Clinical characteristics include juvenile onset, very slow progression, and predominant proximal muscle involvement. The clinical picture and the morphological findings are compared with those previously described in a family. By immunofluorescence, all histologically abnormal muscle fibers, in particular those vacuolated, showed a strong deposition of the complement C5b‐9 membrane attack complex over the whole muscle fiber surface. Weak immunostaining for membrane attack complex was also found in endomysial capillaries and perimysial vessel walls. Muscle fibers showed sarcolemmal immunolabeling with anti–major histocompatibility complex I, which was also present on the margins of many vacuoles. All vacuoles were stained by antidystrophin antibody, which colocalized in most of them with antilaminin immunostaining. Taken together, these results suggest that the deposition of membrane attack complex on the damaged cell surface membrane could be important in the pathogenesis of this muscle disorder, and that the membrane‐bounded vacuoles could be a consequence of sarcolemmal invagination.
Retinotopic and directional defects in motion discrimination in humans with cerebral lesions
Authors: Barton Jason J. S. , Sharpe James A. , Raymond Jane E. 
Published: January 2005

AbstractWe investigated the discrimination of motion direction in peripheral and central vision in 23 patients with unilateral cerebral hemispheric lesions on computed tomography or magnetic resonance imaging. We used random dot cinematograms that determined a percent coherence motion threshold for 16 points in the peripheral field and for four directions separately in central vision. We measured asymmetry of right‐ versus left‐field peripheral discrimination (retinotopic defects) and asymmetry of central discrimination for rightward versus leftward motion (directional defects), compared with normal subjects. Five patients had directional asymmetries of foveal motion perception, all worse for motion toward the side of their lesions. One patient had a bidirectional defect for the perception of horizontal motion. For 3 of these 6, the average of all horizontal and vertical motion discrimination thresholds was also elevated. Two had contralateral retinotopic defects. One of these also had an ipsidirectional foveal defect, but the other did not. The remaining 5 patients with ipsidirectional foveal defects had hemianopias that precluded testing for coexistent retinotopic defects. The lesions of the 6 patients with ipsidirectional defects overlapped in white matter underlying the lateral temporo‐occipital cortex, at the junction of Brodmann areas 19 and 37. In contrast, lesions of patients without directional defects spared this region.
Studies of the coding region of the neuronal glutamate transporter gene in amyotrophic lateral sclerosis
Authors: Meyer T. , Lenk U. , Küther G. , Weindl A. , Speer A. , Ludolph A. C. 
Published: January 2005

AbstractStudies of the coding region of the neuronal glutamate transporter of 6 amyotrophic lateral sclerosis (ALS) patients and 10 controls show an identical pattern of four reported amino acid variations. No mutations and polymorphisms were detected in 5 sporadic ALS patients and a single patient with the familial form of the disease.
Neuroimaging and cerebrospinal fluid cytology in the diagnosis of leptomeningeal metastasis
Authors: Freilich Ronnie J. , Krol George , Deangelis Lisa M. 
Published: January 2005

AbstractThe diagnosis of leptomeningeal metastasis is often difficult and usually requires the demonstration of malignant cells in the cerebrospinal fluid. Neuroimaging, however, may establish or support the diagnosis in some patients. Radiographic abnormalities consistent with or suggestive of leptomeningeal metastasis include leptomeningeal, subependymal, dural, or cranial nerve enhancement; superficial cerebral lesions; and communicating hydrocephalus. We evaluated 137 cancer patients with clinical symptoms suspicious for leptomeningeal metastasis with neuroimaging or cerebrospinal fluid cytology or both. Neuroimaging findings were abnormal in 70 of 128 tested patients; cytology was performed in 58 of these 70 and the results were positive in 37. Conversely, cytological findings were positive in 53 of 115 tested patients; neuroimaging was performed in 49 of these 53 and the findings were abnormal in 37 (26/29 solid tumors and 11/20 hematological tumors). Of the total series of 137 patients, leptomeningeal metastasis was diagnosed in 77; in 24 (31%) the diagnosis was made on the basis of clinical picture and abnormal neuroimaging alone. Neuroimaging is a valuable tool in the investigation of leptomeningeal metastasis in the cancer population, and the presence of typical clinical features together with appropriate neuroimaging abnormalities is adequate to make the diagnosis of leptomeningeal metastasis even if cerebrospinal fluid cytological results are negative.
Molecular and clinical correlations in spinocerebellar ataxia type 3 and Machado‐Joseph disease
Authors: Matilla Toni , McCall Alanna , Subramony S. H. , Zoghbi Huda Y. 
Published: January 2005

AbstractThe autosomal dominant spinocerebellar ataxias are clinically and genetically a heterogeneous group of neurodegenerative disorders. Genetic studies have classified some of these disorders based on the mapping of their respective genes. The gene for Machado‐Joseph disease, one type of spinocerebellar ataxia, has been mapped to the long arm of chromosome 14q24.3‐ter. The gene for another spinocerebellar ataxia, which is clinically distinct from Machado‐Joseph disease, has been also localized to the same region on 14q and has been named type 3 spinocerebellar ataxia. Recently, expansions of a CAG trinculeotide repeat in a novel gene on chromosome 14q32.1 were shown in 11 patients affected with Machado‐Joseph disease. In this study, we analyzed the DNA samples from 103 individuals representing 42 independent families with dominantly inherited ataxia to determine whether any had the Machado‐Joseph disease mutation. The Machado‐Joseph disease CAG expansion was detected in 5 of these 42 families. Sixteen affected individuals displayed a normal allele containing 14 to 31 CAG repeats and an expanded allele ranging between 66 and 79 CAG repeats. Seven asymptomatic individuals showed an allele ranging between 67 and 80 CAG repeats. Two of these families had a phenotype consistent with Machado‐Joseph disease while the other 3 had clinical features of type 3 spinocerebellar ataxia. These data suggest that a single locus at 14q32.1 is responsible for two forms of spinocerebellar ataxia, spinocerebellar ataxia type 3 and Machado‐Joseph disease, and that this locus may account for approximately 11% of this group of dominantly inherited ataxias.
Oligodendroglial development in human fetal cerebrum
Authors: Rivkin Michael J. , Flax Jonathan , Mozell Robin , Osathanondh Rapin , Volpe Joseph J. , Villa‐Komaroff Lydia 
Published: January 2005

AbstractWe have successfully established mixed glial cell primary cultures prepared from individual fetal human brains (15–18 weeks' gestation in age). Cultures were maintained for as long as 3 months in either 10% fetal calf serum (FCS) or serum‐free chemically defined medium (CDM). By morphological and immunohistochemical criteria, the precursor cell for human oligodendrocytes (O‐2A cell) was identified. This cell exhibited the bipolar morphology and A2B5‐positive (A2B5+) immunoreactivity typical of the O‐2A precursor cell. With time in culture, cells possessing a stellate morphology appeared, some of which stained with the O4 antibody, indicative of cell differentiation in the oligodendroglial lineage. At yet older culture age, arborized cells bearing the O1 (galactocerebroside, GC) immunohistochemical marker and displaying the morphological characteristics typical of more mature oligodendrocytes were found, confirming their oligodendroglial identity. Oligodendroglial differentiation was supported best by CDM rather than FCS. To complement these observations, double immunofluorescent studies were performed on parietal sections from human fetal brains at 20 to 22 weeks of gestation. Bipolar A2B5+, multipolar A2B5+/O4+, and arborized A2B5−/O1+ cells were found, thus confirming the presence of oligodendrocytes in human fetal brain at this stage of prenatal development and consistent with the observations made in cell culture.
A case—control study of apolipoprotein E genotypes in Alzheimer's disease associated with Down's syndrome
Authors: van Gool W. A. , Evenhuis H. M. , van Duijn C. M. ,   Behalf of the Dutch Study Group on Down's Syndrome and Ageing
Published: January 2005

AbstractThe prevalence of clinical signs and neuropathological findings of Alzheimer's disease (AD) is high in Down's syndrome (DS). In the general population, the apolipoprotein E (ApoE) ϵ4 isoform is an important risk Marchker for AD. We studied the allelic frequencies of ApoE in 26 DS cases fulfilling clinical diagnostic criteria for AD and in 26 DS controls matched for age, sex, and premorbid level of mental retardation. A meta‐analysis of data available in the literature was used for comparison with allele frequencies in other AD and control populations. ApoE type 2, 3, or 4 allele freqeuencies were not significantly different in AD–DS cases and DS controls. The ApoE ϵ4 frequency in DS cases with AD (0.14; CI, 0.06–0.26) was significantly lower than in any other AD population studied so far and it is within the range of nondemented controls from the general population. These findings suggest that ApoE ϵ4 does not significantly affect the pathogenesis of AD in DS patients.
Intracerebral distribution of infectious amyloid protein in spongiform encephalopathy
Authors: Brown P. , Kenney K. , Little B. , Ironside J. , Will R. , Cervenáková L. , Bjork R. J. , San Marchtin R. A. , Safr J. , Roos R. , Haltia M. , Gibbs C. J. , Gajdusek D. C. 
Published: January 2005

AbstractWe studied the regional distribution of infectious amyloid protein by western immunoblots of brain tissue extracts from 37 patients with different forms of spongiform encephalopathy, i.e., 16 sporadic cases, 18 familial cases with a variety of mutations, and 3 iatrogenic cases. In sporadic and familial Creutzfeldt‐Jakob disease, amyloid protein concentrations were usually highest in the frontotemporal regions of the cerebral cortex, whereas iatrogenic Creutzfeldt‐Jakob disease and Gerstmann‐Sträussler‐Scheinker syndrome had as high or higher concentrations in the deep cerebral nuclei and cerebellum. As a group, familial cases had lower amyloid protein concentrations than either sporadic or iatrogenic cases, and fatal familial insomnia patients had the lowest concentrations found in any form of disease. This hierarchy of amyloid protein concentrations corresponds to the experimental transmission rates observed for each form of disease and is consistent with the concept that the protein molecule is an integral component of the infectious agent. Regional amyloid protein pattern analysis of brain and spinal cord may help to distinguish sporadic from environmentally acquired infections, as for example, cases of human disease suspected to have arisen from exposure to sheep or cows infected with scrapie or bovine spongiform encephalopathy.
Enhanced endothelial cell adhesion of human cerebrospinal fluid lymphocytes
Authors: Elfont Robert M. , Griffin Diane E. , Goldstein Gary W. 
Published: January 2005

AbstractLymphocyte migration into a tissue depends on properties of both the lymphocyte and the tissue's vascular endothelium. The central nervous system (CNS) possesses a specialized microvasculature and lymphocytes appear to enter the CNS less readily than peripheral tissues. We investigated whether those lymphocytes that interact with the CNS, as represented by cerebrospinal fluid (CSF)‐derived lymphocytes, express adhesive properties distinct from peripheral blood lymphocytes (PBLs). Adhesion of human lymphocytes to bovine endothelial cell monolayers was quantitated microscopically. A greater number of PBLs adhered to aortic than to retinal endothelial cell cultures (e.g., 10.9 ± 0.6 and 4.5 ± 0.2, respectively; p = 0.0023). Preincubation of either endothelial cell type with tumor necrosis factor‐α (TNF‐α) enhanced lymphocyte adhesion. Activation of PBLs with concanavalin A or phytohemagglutinin increased endothelial cell adhesion and the effect was additive with that of TNF‐7alpha;. The number of CSF lymphocytes adhering to endothelial cell cultures (retinal, 67.5 ± 9.0; aortic, 83.7 ± 10.6) was more than 10 times the number of PBLs (retinal, 5.4 ± 0.8; aortic, 8.0 ± 1.3; p < 0.0001). CSF lymphocytes did not, however, adhere preferentially to CNS‐derived endothelial cell cultures. These results suggest that CSF may be enriched, compared with peripheral blood, in its content of surveillance lymphocytes, but that these cells might enter target tissues nonspecifically.
An adenoviral vector can transfer lacZ expression into schwann cells in culture and in sciatic nerve
Authors: Shy Michael E. , Tani Mari , Shi Yi‐Jun , Whyatt Shelley A. , Chbihi Taibi , Scherer Steven S. , Kamholz John 
Published: January 2005

AbstractAlthough a number of genetic defects in the P0, peripheral myelin protein‐22, and connexin‐32 genes recently were shown to cause the demyelinating forms of Charcot‐Marie‐Tooth disease, there is yet no effective treatment for these patients. Recent studies showed that replication defective adenoviral vectors can efficiently introduce genes into muscle, brain, lung, and other tissues, suggesting that this vector system may be useful for the treatment of a number of genetic diseases. In this work, we demonstrated that a replication deficient adenovirus expressing the Escherichia coli β‐galactosidase gene (AdCMVLacZ) can introduce genes into Schwann cells, in culture as well as in sciatic nerve. Schwann cells cultured at a multiplicity of infection of 250:1 did not demonstrate cytopathic effects. Following injection of AdCMVLacZ into sciatic nerve of rats, lacZ‐expressing, myelinating Schwann cells could be detected for at least 45 days. These data suggest that in the future, these vectors may be useful both in perturbing Schwann cell gene expression and in designing therapies for the treatment of Charcot‐Marie‐Tooth disease.
Deficiency of brain synaptic dystrophin in human duchenne muscular dystrophy
Authors: Kim Tae‐Wan , Wu Kuo , Black Ira B. 
Published: January 2005

AbstractDuchenne muscular dystrophy (DMD) is characterized by a defect in dystrophin, a high molecular weight protein that is located predominantly in muscle, but which has been detected in brain. Brain dystrophin has been localized to the synapse, in the postsynaptic density (PSD), and is absent in the mdx mouse, an animal model of human DMD. To define the potential pathogenic role of dystrophin deficiency in cognitive impairment, we examined the protein in human DMD brain. The 427‐kd dystrophin was absent in the PSD from DMD brain, but was normally expressed in the brain from an age‐matched control subject. Our findings indicate that dystrophin is deficient in human DMD cortical synapses and provide a potential pathogenic mechanism for cognitive impairment.
AIDS dementia complex and HIV‐1 brain infection: Clinical‐virological correlations
Authors: Brew Bruce J. , Rosenblum Marc , Cronin Kathy , Price Richard W. 
Published: January 2005

AbstractTo evaluate the presence and distribution of central nervous system infection by human immunodeficiency virus type 1 (HIV‐1), we used immunohistochemical methods to map the HIV‐1 p24 core protein in the brains of 55 autopsied patients with acquired immunodeficiency syndrome (AIDS). In a subset of 40 of these patients who had undergone antemortem neurological evaluation of the AIDS dementia complex (ADC), we analyzed the relation between the severities of the viral infection and clinical dysfunction. Viral antigen was detected in macrophages and cells with morphological and immunohistochemical characteristics of microglia as well as multinucleated cells. The distribution of antigen‐positive cells preferentially involved certain deep brain structures, especially the globus pallidus, other basal ganglia nuclei, and the central white matter. Overall, the presence and frequency of infected cells were highly correlated with the histological findings of multinucleated‐cell encephalitis and in general with the clinical ADC stage. However, infection was often more limited than might be “anticipated” from the severity of patients' clinical dysfunction: Only 61% of patients with at least ADC stage 1 had detectable antigen and of these only approximately 30% of the brain sections were antigen positive. These results suggest a pathogenetic model of ADC where virus‐ or cell‐coded toxins amplify the effect of limited brain infection.