bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effects of treatment on long‐term survivors with malignant astrocytomas
Authors: Imperato Joseph P. , Paleologos Nina A. , Vick Nicholas A. 
Published: January 2005

AbstractWe reviewed the records of 160 consecutive patients with glioblastoma and anaplastic astrocytoma to evaluate the long‐term consequences of radiation therapy and chemotherapy. We defined long‐term survivors as those patients with glioblastoma or anaplastic astrocytoma who lived at least 100% longer than median survival of historical controls, for example, 2 years for patients with glioblastoma and 4 years for patients with anaplastic astrocytoma. There were 9 (5.6%) long‐term survivors. Three (30%) became demented and died without evidence of tumor recurrence. One, after survival of 10 years, died of tumor recurrence. Of the remaining survivors, 2 (22%) have significantly impaired short‐term memory function and other neurological deficits such as gait apraxia. Three (30%) can function independently. It is likely but cannot be proved that it is radiotherapy and not chemotherapy that is the causal factor of this dismal therapeutic outcome. Our study suggests restraint in the use of radiotherapy for patients with brain tumors that have more favorable prognoses than glioblastomas and anaplastic astrocytomas, such as low‐grade astrocytomas and oligodendrogliomas.
Correlation of phasic muscle strength and corticomotoneuron conduction time in multiple sclerosis
Authors: van der Kamp W. , de Noordhout A. Maertens , Thompson P. D. , Rothwell J. C. , Day B. L. , Marsden C. D. 
Published: January 2005

AbstractCentral motor conduction times for the adductor pollicis muscle, the twitch force of that muscle to scalp magnetic motor cortex stimulation, and the maximum force of phasic voluntary contraction of the same muscle were measured in 15 patients with multiple sclerosis. Two tests of manual dexterity of the same hand also were studied: the Purdue pegboard test, and the maximal frequency of a scissors movement of the thumb and index finger. The patients had normal strength or minimal weakness of the intrinsic muscles of the hand on clinical examination. The mean central motor conduction times for the adductor pollicis muscle for the patients were longer than normal, the peak twitch force of the adductor pollicis muscle evoked by cortical stimulation and the maximum force of a phasic voluntary contraction of the adductor pollicis muscle were smaller than normal. There were strong correlations between all these measures. Central motor conduction time in the patients was inversely correlated with voluntary phasic force and the twitch force after cortical stimulation. That is, the longer the central motor conduction time, the weaker the force. Prolonged central motor conduction time is likely to be accompanied by conduction block in corticomotoneuron pathways. The correlation of central motor conduction time with voluntary phasic force and the twitch force most likely reflects the degree of conduction block and temporal dispersion rather than delay in conduction per se. These results indicate that objective assessments of phasic muscle strength may reveal correlations with central motor conduction time that are not evident on conventional clinical examination which assesses tonic muscle contraction strength. There also was some correlation of central motor conduction time, twitch force, and voluntary phasic force with the performance of tests of manual dexterity. Since the voluntary phasic strength and twitch force to cortical stimulation were similarly weakened, the findings would also be consistent with the cortical stimulus and voluntary contraction using the same descending motor tracts.
Developmental regulation of human truncated nerve growth factor receptor
Authors: DiStefano Peter S. , Clagett‐Dame Margaret , Chelsea Diane M. , Loy Rebekah 
Published: January 2005

AbstractMonoclonal antibodies (designated XIF1 and IIIG5) recognizing distinct epitopes of the human truncated nerve growth factor receptor (NGF‐Rt) were used in a two‐site radiometric immunosorbent assay to monitor levels of NGF‐Rt in human urine as a function of age. Urine samples were collected from 70 neurologically normal subjects ranging in age from 1 month to 68 years. By using this sensitive two‐site radiometric immunosorbent assay, NGF‐Rt levels were found to be highest in urine from 1‐month old subjects. By 2.5 months, NGF‐Rt values were half of those seen at 1 month and decreased more gradually between 0.5 and 15 years. Between 15 and 68 years, urine NGF‐Rt levels were relatively constant at 5% of 1‐month values. No evidence for diurnal variation of adult NGF‐Rt was apparent. Pregnant women in their third trimester showed significantly elevated urine NGF‐Rt values compared with age matched normals. Affinity labeling of NGF‐Rt with 125I‐NGF followed by immunoprecipitation with ME20.4‐IgG and gel autoradiography indicated that neonatal urine contained high amounts of truncated receptor (Mr = 50 kd); decreasingly lower amounts of NGF‐Rt were observed on gel autoradiograms with development, indicating that the two‐site radiometric immunosorbent assay correlated well with the affinity labeling technique for measuring NGF‐Rt. NGF‐Rt in urines from 1‐month‐old and 36‐year‐old subjects showed no differences in affinities for NGF or for the monoclonal antibody IIIG5. These data show that NGF‐Rt is developmentally regulated in human urine, and are discussed in relation to the development and maturation of the peripheral nervous system. The results obtained in this study establish the basis for studying NGF‐Rt regulation during the course of abnormal development, regeneration, and neurodegeneration.
Localization of 3H‐Dihydroergotamine‐binding sites in the cat central nervous system: Relevance to migraine
Authors: Goadsby Peter J. , Gundlach Andrew L. 
Published: January 2005

AbstractDihydroergotamine (DHE) is the treatment of choice in aborting the acute attack of migraine. Although its efficacy has been known for 40 years, its mechanism of action is still disputed. Data regarding the site of action of dihydroergotamine may provide an insight into its mechanism of action and thus identify a locus of potentially abnormal pathophysiology in migraine. By using in vitro and ex vivo autoradiographic techniques, the localization of specific binding sites for 3H‐dihydroergotamine in the cat brain has been examined. Binding was seen in the dorsal horn of the cervical spinal cord, in the medulla, associated with the nucleus of the tractus solitarius, area postrema, and descending spinal trigeminal nucleus, and in the mesencephalon and the cerebral cortex. The highest density of binding sites was found in the dorsal and medial raphe nuclei of the midbrain. Furthermore, these same brain regions were also labeled after intravenous administration of 3H‐dihydroergotamine. It is important that the brain areas specifically labeled are key nuclei involved in cranial pain transmission, suggesting that dihydroergotamine may act at these central sites in migraine.
Quinolinic acid catabolism is increased in cerebellum of patients with dominantly inherited olivopontocerebellar atrophy
Authors: Kish Stephen J. , Du Fu , Parks Deborah A. , Robitaille Yves , Ball Melvyn J. , Schut Lawrence , Hornykiewicz Oleh , Schwarcz Robert 
Published: January 2005

AbstractWe measured the activities of the enzymes responsible for the metabolism of the excitotoxin quinolinic acid, 3‐hydroxyanthranilate oxygenase and quinolinic acid phosphoribosyltransferase, in autopsied brain of 11 patients with olivopontocerebellar atrophy. In cerebellar cortex, severe Purkinje cell loss was evident but with relative preservation of granule cells. As compared with the control subjects (n = 14), mean activity of 3‐hydroxyanthranilate oxygenase was normal in cerebellar cortex from the patients with olivopontocerebellar atrophy, whereas quinolinic acid phosphoribosyltransferase activity was markedly increased (+ 92%, p < 0.02). No significant changes in enzyme activities were found in samples from occipital cortex. Increased quinolinic acid phosphoribosyltransferase activity may represent a mechanism, in the degenerating cerebellum, to protect quinolinic acid–sensitive granule cells in patients with olivopontocerebellar atrophy.
Intraspinal transplants
Authors: Tessler Alan 
Published: January 2005

AbstractTransplants of embryonic central nervous system tissue have long been used to study axon growth during development and regeneration, and more recently to promote recovery in models of human diseases. Transplants of embryonic substantia nigra correct some of the deficits found in experimental Parkinson's disease, for example, by mechanisms that are thought to include release of neurotransmitter and reinnervation of host targets, as well as by stimulating growth of host axons. Similar mechanisms appear to allow intraspinal transplants of embryonic brainstem to reverse locomotor and autonomic deficits due to experimental spinal cord injuries. Embryonic spinal cord transplants offer an additional strategy for correcting the deficits of spinal cord injury because, by replacing damaged populations of neurons, they may mediate the restoration of connections between host neurons. We have found that spinal cord transplants permit regrowth of adult host axons resulting in reconstitution of synaptic complexes within the transplat that in many respects resemble normal synapses. Transplants of fetal spinal cord may also contribute to behavioral recovery by rescuing axotomized host neurons that otherwise would have died. Electrophysiological and behavioral investigations of functional recovery after intraspinal transplantation are preliminary, and the role of transplants in the treatment of human spinal cord injury is uncertain. Transplants are contributing to our understanding of the mechanisms of recovery, however, and are likely to play a role in the development of rational treatments.
Genetic mapping of “Lubag” (X‐linked dystonia‐parkinsonism) in a filipino kindred to the pericentromeric region of the X chromosome
Authors: Wilhelmsen Kirk C. , Weeks Daniel E. , Nygaard Torbjoern G. , Moskowitz Carol B. , Rosales Raymond L. , Dela Paz Daniel C. , Sobrevega Eufemio E. , Fahn Stanley , Gilliam T. Conrad 
Published: January 2005

Abstract“Lubag” is an X‐linked disorder causing dystonia and parkinsonism that has only been described in families from the Philippines, Principally from the island of Panay. We have established linkage between the disease phenotype “lubag” and DNA markers which span the Xp11.22‐Xq21.3 region by using a large Filipino family with 8 affected men in three generations. These DNA markers define an interval of about 20 centimorgans in the pericentromeric region of the X chromosome as the most likely site of the disease locus XDPD (X‐linked dystonia‐parkinsonism). XDPD has a maximum multipoint log likelihood ratio score (Zmax) of about 4.6 over the interval from Xq12 to Xq21.31 (DXS159‐DXYS1X). The co‐occurrence of dystonia and parkinsonism in lubag and in other known disorders suggests there may be a common pathogenetic mechanism. Identification of the genetic defect in this family may provide an important clue toward understanding the pathogenesis and pathophysiology of both dystonia and parkinsonism.
Human microglial cells: Characterization in cerebral tissue and in primary culture, and study of their susceptibility to HIV‐1 infection
Authors: Peudenier Sylviane , Hery Christiane , Montagnier Luc , Tardieu Marc 
Published: January 2005

AbstractNeuropathological studies have shown that human immunodeficiency virus type 1‐infected cells within the brain express several markers characteristic of macrophages and could either be microglial cells, or monocytes invading the CNS, or both. To better define the target cells of human immunodeficiency virus type 1 within the brain, we have studied human microglial cells, both in vivo and in vitro, and compared them to monocytes for their antigenic markers and their susceptibility to human immunodeficiency virus type 1 infection. Brain‐derived macrophages were isolated from primary cortical and spinal cord cultures obtained from 8 to 12‐week‐old human embryos. The isolated cells presented esterase activity, phagocyted zymosan particles, expressed several (Fc receptors, and CD68/Ki‐M7 and CD11b/CR3 receptors) of the macrophagic antigenic markers, and appeared to be resident microglial cells from human embryonic brain. Conversely, brain‐derived macrophages did not express antigens CD4, CD14, or CD68/Ki‐M6, which are easily detected on freshly isolated monocytes. Using these antigenic differences between isolated microglial cells and monocytes, we have observed that two populations of macrophages could be individualized. In the normal adult brain, microglial cells were numerous in both the gray and the white matter. The infrequent cells sharing antigens with monocytes were found almost exclusively around vessels. In 8 to 12‐week‐old human embryos, microglial cells were found in both the parenchyma and the germinative layer. Cells sharing antigens with monocytes were only found at the top of and inside the germinative layer. In brain tissue from patients with human immunodeficiency virus type 1 encephalitis, cells sharing antigens with monocytes are abundant not only around the vessels but also in the parenchyma. In double‐labeling experiments, human immunodeficiency virus type 1‐infected cells showed monocyte antigens. Finally, microglial cells also differ from monocytes in their in vitro susceptibility to human immunodeficiency virus type 1 infection; after stimulation by r‐TNFα or GmCSF, monocytes but not microglial cells can replicate human immunodeficiency virus type 1. This in vitro difference in human immunodeficiency virus type 1 susceptibility between monocytes and microglial cells together with the presence of monocytic antigens within the brain tissue of human immunodeficiency virus type 1‐infected patients suggest that human immunodeficiency virus type 1‐infected cells within the brain are either monocytes that have crossed the blood‐brain barrier and spread through the tissue or perivascular microglial cells that, after phagocyting infected blood lymphocytes, subsequently contain viral antigen and migrate to brain tissue.
Glutamate‐, glutaminase‐, and taurine‐immunoreactive neurons develop neurofibrillary tangles in Alzheimer's disease
Authors: Kowall Neil W. , Beal M. Flint 
Published: January 2005

AbstractAlthough formation of neurofibrillary tangles is a major pathological feature of Alzheimer's disease (AD), the neurotransmitter content of neurofibrillary tangle‐bearing neurons has not been well characterized. We studied the hippocampus of 6 patients with pathologically verified AD and 6 control subjects using a monoclonal antibody to glutamyl‐glutamate and polyclonal antisera against glutaminase and taurine. In normal hippocampus, gluamate and glutaminase stained pyramidal neurons in the cornu ammonis (CA) fields and the subiculum, as well as the dentate granule cells. Fiber staining was better seen with glutamate antisera, which in AD specimens showed reduced numbers of glutamate‐immunoreactive fibers in the molecular layer of the dentate gyrus. In AD specimens, glutamate‐ and glutaminase‐immunoreactive pyramidal neurons in the hippocampal CA fields were decreased in number and remaining neurons showed irregular shortened and disorganized dendritic fields. Taurine immunoreactivity was localized to a subset of hippocampal pyramidal neurons, which showed similar degenerative changes in AD specimens. Glutamate‐, glutaminase‐, and taurine‐stained neurons were found to contain neurofibrillary tangles using either double immunofluorescence with tau antisera, double immunoperoxidase stains, or silver and thioflavine S counterstains. These studies show that two distinct neurochemically defined populations of pyramidal neurons in allocortex frequently show degenerative changes and develop neurofibrillary tangles in AD.
Increased HTLV‐I proviral DNA in HTLV‐I–associated myelopathy: A quantitative polymerase chain reaction study
Authors: Kira Jun‐ichi , Koyanagi Yoshio , Yamada Takeshi , Itoyama Yasuto , Goto Ikuo , Yamamoto Naoki , Sasaki Hiroyuki , Sakaki Yoshiyuki 
Published: January 2005

AbstractUsing the polymerase chain reaction, we quantitated the amount of human T‐lymphotropic virus type I (HTLV‐I) proviral DNA in peripheral blood mononuclear cells from 18 patients with HTLV‐I–associated myelopathy/tropical spastic paraparesis; 17 HTLV‐I carriers without HTLV‐I–associated myelopathy/tropical spastic paraparesis, with or without other autoimmune or inflammatory diseases; and 19 seronegative control subjects. The HTLV‐I proviral DNA was 10‐ to 100‐fold higher in the patients and in the HTLV‐I carriers without HAM/TSP who had autoimmune or inflammatory diseases than in the carriers without autoimmune or inflammatory diseases. The patients who had had onset of myelopathy at a younger age (15 to 39 years) had an extremely high level of HTLV‐I proviral DNA in the early phase, as compared with findings in those with a late onset of myelopathy (at 44 to 61 years). The large increase in HTLV‐I proviral DNA in peripheral blood mononuclear cells is presumably closely related to the development of autoimmune or inflammatory processes in HTLV‐I carriers, including HTLV‐I–associated myelopathy/tropical spastic paraparesis.
Peripheral neuropathy after chronic endoneurial ischemia
Authors: Sladky John T. , Tschoepe Raymond L. , Greenberg Joel H. , Brown Mark J. 
Published: January 2005

AbstractWe have developed a method for producing chronic regional nerve ischemia in rats by creating proximal limb arteriovenous shunts. This procedure results in a 50 to 75% reduction in endoneurial blood flow within the distal sciatic nerve as measured by the iodoantipyrine method. Nerve conduction velocities in sciatic nerves ipsilateral to the shunt fell by 25 to 30% within 2 weeks after creation of the shunt and did not recover for up to 10 months after the procedure. Morphological studies of the ischemic nerves showed structural abnormalities at nodes of Ranvier and mild axonal atrophy. Neither segmental demyelination nor axonal degeneration were evident. These results indicate that reduced endoneurial blood flow, insufficient to cause infarction, may result in measureable functional and morphological abnormalities in peripheral nerves.
Calcium channel autoantibodies in the lambert‐eaton myasthenic syndrome
Authors: Leysm Katherine , Lang Bethan , Johnston Irene , Newsom‐Davis John 
Published: January 2005

AbstractWe have tested 36 patients with the Lambert‐Eaton myasthenic syndrome for serum antibodies to voltage‐gated calcium channels by using an immunoprecipitation assay with [125I] ω‐conotoxin–labeled voltage‐gated calcium channels extracted from a human neuroblastoma cell line, SKN‐SH. Forty‐four percent of these patients had significant levels of antibody (30–1,466 pM) compared with healthy control individuals (< 15 pM). The incidence of positive sera in patients without associated small cell lung carcinoma (61%) was greater than in those patients with small cell lung carcinoma (28%). Results correlated strongly with results obtained using voltage‐gated calcium channels extracted from the small cell lung carcinoma line, MAR5. Anti–voltage‐gated calcium channel antibody titers did not correlate with disease severity across individuals, but longitudinal studies in 2 patients receiving immunosuppressive therapy showed a clear inverse relation between antibody titer and an electromyographic index of disease severity. The incidence of positive sera among patients with other neurological disorders was not significant, but 8 of 12 patients with rheumatoid arthritis or systemic lupus erythematosus had raised titers (30–82 pM). We conclude that the antibodies detected in this assay are heterogeneous and that some of them are likely to be implicated in this disorder of neuromuscular transmission. The assay should prove useful as an additional diagnostic aid in patients with Lambert‐Eaton myasthenic syndrome.
Magnetic resonance imaging of Creutzfeldt‐Jacob disease
Authors: Milton Walter J. , Atlas Scott W. , Lavi Ehud , Mollman Joan E. 
Published: January 2005

AbstractWe report a biopsy‐diagnosed patient with Creutzfeldt‐Jacob disease showing on magnetic resonance images bilateral increased signal intensity in the basal ganglia on long repetition time images. Creutzfeldt‐Jacob disease (CJD) is a degenerative process of the brain, induced by a novel infectious agent, and is usually characterized by a rapidly progressive dementia. We report a biopsydiagnosed patient with CJD with a distinctive magnetic resonance (MR) apperance.
Selective impairment of smooth‐pursuit eye movements due to an ischemic lesion of the basal pons
Authors: Thier Peter , Bachor Alexander , Faiss Jürgen , Dichgans Johannes , Koenig Eberhard 
Published: January 2005

AbstractVoluntary and reflex‐like eye movements were measured in a patient with an ischemic lesion of the right basal pons. Ipsilateral smooth‐pursuit eye movements were predominantly impaired and interrupted by saccades. This profound smooth‐pursuit deficit contrasted with only minor abnormalities of visually guided saccades and the vestibulo‐ocular reflex. A selective disturbance of smooth‐pursuit eye movements due to a lesion of the basal pons in this patient concurs with recent work in monkeys suggesting that smooth‐pursuit eye movements are mediated by a parietooccipito‐ponto‐cerebellar pathway.
Coculture with autologous myotubes of cytotoxic T cells isolated from muscle in inflammatory myopathies
Authors: Hohlfeld Reinhard , Engel Andrew G. 
Published: January 2005

AbstractT‐cell lines were expanded from muscle of 10 patients with polymyositis, 5 with inclusion body myositis, 5 with dermatomyositis, and 5 with other muscle diseases. All cell lines uniformly expressed T‐cell antigens, but not natural killer cell or B‐cell antigens. The proportion of helper (CD4+) and cytotoxic (CD8+) T cells in the expanded lines was variable and showed no correlation with the diagnosis. Sixteen cell lines (6 polymyositis, 4 inclusion body myositis, 5 dermatomyositis, 1 other muscle disease) consisted predominantly of CD8+ T cells. None of these lines displayed natural killer‐like cytotoxicity but all were capable of lectin‐dependent cytotoxicity. Three of 6 polymyositis, 1 of 4 inclusion body myositis, and 1 of 5 dermatomyositis lines showed low but statistically significant cytotoxicity against autologous myotubes (6 to 27% specific 51Cr release; effector‐target ratio, 20:1). The results demonstrate that functionally competent cytotoxic T cells can be expanded from muscle affected by inflammatory myopathies and are consistent with the hypothesis that some cytotoxic T cells recognize an autoantigen on myotubes. Further studies of this experimental system may define the molecular mechanism of T cell‐mediated muscle fiber injury and may help to identify the relevant antigens.
Lhermitte‐duclos disease and cowden disease: A single phakomatosis
Authors: Padberg George W. , Schot Johannes D. L. , Vielvoye G. Jan , Bots Gerard Th. A. M. , De Beer Frits C. 
Published: January 2005

AbstractTwo unrelated patients with macrocephaly, seizures, and mild cerebellar signs had a dysplastic gangliocytoma of the cerebellum (Lhermitte‐Duclos disease). Both also had autosomal dominant Cowden disease as evidenced by facial, oral, and acral papules. In the two families, 9 sibs demonstrated the mucocutaneous lesions, thyroid disease, breast tumors, and ovarian tumors compatible with the diagnosis of Cowden disease. Some of the sibs also showed various degrees of neurological signs such as macrocephaly, mental retardation, seizures, tremor, and dysdiadochokinesia. Magnetic resonance imaging scans of sibs of one family demonstrated megalencephaly and other mild abnormalities. The occurrence of these two rare disorders in single patients is more than a coincidence, and the clinical findings in the combined condition establishes it as a new phakomatosis.