bluuebunny/crossref_metadata_2025_split huggingface dataset:
Isolation of P2 protein–reactive T‐cell lines from human blood
Authors: Burns James , Krasner L. Jill , Rostami Abdolmohammad , Pleasure David 
Published: January 2005

AbstractHuman T‐cell lines reactive with the peripheral nerve myelin, protein, P2 protein, were isolated from the peripheral blood of 4 normal persons and 1 patient with Guillain‐Barré syndrome. These predominantly helper phenotype T‐cell lines were isolated and maintained in vitro by antigen stimulation followed by culture with interleukin 2. Myelin basic protein–reactive T cells were also isolated in parallel from the same subjects as antigen specificity controls. T cells recognizing myelin basic protein did not respond to P2 protein, nor did P2‐reactive cells respond to myelin basic protein. These findings suggest that a potential for autoimmune reactivity with peripheral nervous system myelin antigens may exist for both normal persons and some patients with neurological disease.
Cortical potentials related to voluntary and passive finger movements recorded from subdural electrodes in humans
Authors: Lee B. I. , Lüders H. , Lesser R. P. , Dinner D. S. , Morris H. H. 
Published: January 2005

AbstractMovement‐related potentials were recorded from subdural electrodes placed on the precentral and postcentral cortex in 3 patients undergoing operation for intractable epilepsy. With self‐initiated index finger movement, a negative potential of 25 to 50 microvolts in amplitude, preceding onset of the electromyographic activity by 60 to 95 ms (or onset of movement by 150 to 230 ms), was recorded from the hand somatosensory postrolandic area in all 3 patients. A similar potential preceding the movement was recorded from the precentral hand motor area in one subject who was the only patient in whom the precentral electrodes were placed on the hand motor area. Following active and passive movements, a clearly defined positivity (18 to 32 ms after a photometer trigger) that reversed phase across the central fissure was recorded. The premovement potentials are most probably generated by pyramidal tract neurons and motor‐function‐related neurons located in the post‐ and prerolandic areas. The postmovement positivity is most probably due to short‐latency kinesthetic reafferent activation of the posterior bank of the central fissure (equivalent to P2 of the somatosensory evoked potentials).
Neurological manifestations in xeroderma pigmentosum
Authors: Mimaki Takashi , Itoh Naoyuki , Abe Jiro , Tagawa Tetsuzo , Sato Kenji , Yabuuchi Hyakuji , Takebe Hiraku 
Published: January 2005

AbstractXeroderma pigmentosum is an unusual neurocutaneous disorder. Recent studies have classified patients with xeroderma pigmentosum into 10 groups by somatic cell hybridization methods. In this report we describe 32 patients with Group A xeroderma pigmentosum, including 1 patient with an atypical case, who were assessed for neurological complications. Of these patients, 17 had microcephaly, 13 short stature, and 21 mental retardation. In patients over 7 years of age, sensorineural deafness and spinocerebellar signs such as nystagmus, dysarthria, tremor, and ataxia were frequently observed; no patients below 7 years of age had such neurological complications. Electroencephalographic studies revealed abnormal slow and low voltage background activity. Two patients had focal abnormal discharges, one of whom developed versive seizures. Cranial computed tomographic scans revealed abnormalities, including ventricular dilatation, cerebral atrophy, cerebellar and brainstem atrophy, and cranial bone thickening. A patient with an atypical case of Group A xeroderma pigmentosum had less skin and neurological involvement, and higher levels of postultraviolet colony‐forming ability and host cell reactivation than did a typical Group A case. It is possible that these less severe cytological findings are responsible for the less severe skin lesions and neurological complications noted clinically.
Primary central nervous system lymphoma related to Epstein‐Barr virus in a patient with acquired immune deficiency syndrome
Authors: Rosenberg Neil L. , Hochberg Fred H. , Miller George , Kleinschmidt‐DeMasters B. K. 
Published: January 2005

AbstractThe study of a patient suggested a relationship between Epstein‐Barr virus infection and primary lymphoma of the central nervous system in the acquired immune deficiency syndrome. Deoxyribonucleic acid preparations from tumor tissue contained 30 to 100 copies of Epstein‐Barr virus genome per cell when hybridized with a probe consisting of the Bam‐HI K fragment of Epstein‐Barr virus strain FF41. This hybridization study suggests that induction of this patient's central nervous system lymphoma was related to Epstein‐Barr virus infection.
Phenytoin: Mechanisms of its anticonvulsant action
Authors: Yaari Yoel , Selzer Michael E. , Pincus Jonathan H. 
Published: January 2005

AbstractPhenytoin is a major anticonvulsant drug that is very effective in controlling a wide variety of seizure disorders while impairing neurological function little, if at all. Early work suggested the hypothesis that the drug's effects were due to a selective block of high‐frequency neuronal activity. This theory is reevaluated in the light of accumulated observations on the effects of phenytoin in many neuronal and synaptic preparations. Most of these observations can be explained by a use‐ and frequency‐dependent suppression of the sodium action potential by phenytoin, with a consequent filtering out of sustained high‐frequency neuronal discharges and synaptic activity. The molecular mechanism for this is a voltage‐dependent blockade of membrane sodium channels responsible for the action potential. Through this action, phenytoin obstructs the positive feedback that underlies the development of maximal seizure activity, while normal brain activity, proceeding at lower neuronal firing rates, is spared its depressant action. Other mechanisms of action that may contribute to the drug's efficacy and selectivity are also discussed.
The incidence and prevalence of multiple sclerosis in Newfoundland and Labrador, 1960–1984
Authors: Pryse‐Phillips W. E. M. 
Published: January 2005

AbstractA study of the incidence and prevalence of multiple sclerosis on the island of Newfoundland between 1960 and 1984 has yielded a current overall prevalence rate of 55.2 per 100,000, but wide variation is noted in the rates for different parts of the island (range, 15.9 to 105.0 per 100,000). The annual incidence rates for St. John's and the Avalon region of Newfoundland show cyclical variation on an approximate five‐ or six‐year cycle. Suggestive evidence for a temporal link between varying incidence rates in these areas and recurrent local outbreaks of canine distemper was obtained.
Epitopes of immunoreactive myelin basic protein in human cerebrospinal fluid
Authors: Whitaker John N. , Gupta Manjula , Smith Olive F. 
Published: January 2005

AbstractTo define in more detail the features of the immunoreactive myelin basic protein (MBP) present in cerebrospinal fluid (CSF) of humans following acute injury to central nervous system myelin, the epitopes of MBP recognized by three different antisera, each capable of detecting immunoreactive MBP in CSF, were examined. All three antisera reacted well with human MBP and human MBP peptide 45–89. Only in radioimmunoassays in which the MBP peptide 45–89 served as the radioligand could clearly elevated values of immunoreactive MBP be measured in CSF specimens from 5 patients with multiple sclerosis during or immediately after an exacerbation. The two antisera that reacted well with MBP peptide 80–89 resulted in higher levels of immunoreactive MBP measured in CSF. An epitope present in human MBP peptide 80–89 but sharing a conformation with both MBP and MBP peptide 45–89 is present in CSF following acute central nervous system myelin damage in multiple sclerosis.
HLA study of Japanese patients with Crutzfeldt‐Jakob disease: Significant association with HLA‐DQw3
Authors: Kuroda Yasuo , Kaneoka Hidetoshi , Shibasaki Hiroshi , Kume Shuji , Yamaguchi Masaya 
Published: January 2005

AbstractTo demonstrate genetic susceptibility to Creutzfeldt‐Jakob disease (CJD), 10 Japanese patients with pathologically proved CJD and 6 patients with clinically definite CJD were tissue typed for 48 class I and 14 class II human leukocyte antigen (HLA) specificities. The patients were scattered throughout Japan and there were no consanguineous relationships. Among 62 HLA specificities, HLA‐DRw53 and DQw3 were found in all pathologically proved CJD cases. When the frequencies of the HLA antigens in the 16 CJD cases were compared to those of normal Japanese, a highly significant (p < 0.005) increase was found for HLA‐DQw3 in the CJD group. The present findings provide evidence for genetic control of susceptibility to CJD in humans.
Atypical presentation and neuropathological studies in 3‐hydroxy‐3‐methylglutaryl–CoA lyase deficiency
Authors: Zoghbi Huda Y. , Spence J. Edward , Beaudet Arthur L. , O'Brien William E. , Goodman Clay J. , Gibson Kenneth M. 
Published: January 2005

AbstractA 6½‐month‐old male offspring of consanguineous Egyptian parents was first seen because of fever, somnolence, vomiting, right focal motor seizures, right hemiparesis, elevated transaminase levels, hyperammonemia, and acidosis. A computed tomographic scan of the head suggested swelling of the left cerebral hemisphere, and an electroencephalogram indicated left frontotemporal abnormalities, but brain biopsy demonstrated diffuse white matter spongiosis and gliosis. Subsequently, urine organic acid analysis and enzyme assays were diagnostic of 3‐hydroxy‐3‐methylglutaryl–coenzyme A lyase deficiency.
Localization and effects of neuropeptide Y, vasoactive intestinal polypeptide, substance P, and calcitonin gene–related peptide in human temporal arteries
Authors: Jansen Inger , Uddman Rolf , Hocherman Max , Ekman Rolf , Jensen Kai , Olesen Jes , Srienholm Pia , Edvinsson Lars 
Published: January 2005

AbstractNerve fibers containing neuropeptide Y (NPY), vasoactive intestinal polypeptide (VIP), substance P (SP), and calcitonin gene–related peptide (CGRP) were seen in the adventitia or at the adventitia–media border of the human temporal artery. Pharmacological experiments on isolated temporal artery segments revealed that NPY potentiated the vasoconstrictor responses to noradrenaline, but had no vasoconstrictor ability or only a small vasoconstrictor ability per se. VIP, peptide histidine methionine 27 (PHM‐27), SP, neurokinin A (NKA), and CGRP potently relaxed vessels precontracted by prostaglandin F2α, the relative potency being CGRP > SP > NKA  VIP  PHM‐27. The amount of relaxation varied between 67 and 91% of the prostaglandin F2α–induced contraction. The peptide effects were not antagonized by classic adrenergic or cholinergic blockers, suggesting interactions via separate receptor sites.
Acute high‐dose methotrexate neurotoxicity in the rat
Authors: Phillips Peter C. , Thaler Howard T. , Berger Claudia A. , Fleisher Martin , Wellner Daniel , Allen Jeffrey C. , Rottenberg David A. 
Published: January 2005

AbstractIntravenous high‐dose methotrexate chemotherapy may produce acute, subacute, or chronic neurotoxicity in patients with cancer. Acute encephalopathies following high‐dose methotrexate treatment are recognized with increasing frequency. This study describes a model of acute high‐dose methotrexate neurotoxicity in the rat characterized by a profound dose‐dependent depression of cerebral glucose metabolism in association with behavioral and electroencephalographc abnormalities. Alterations in the amino acid profile, similar to those described in cancer patients after high‐dose methotrexate treatment, were observed in the absence of biochemical evidence of systemic organ toxicity. This model facilitates the study of the biochemical mechanisms of antifolate neurotoxicity in humans and permits the evaluation of potential therapeutic interventions.
Subclasses of event‐related potentials: Response‐locked and stimulus‐locked components
Authors: Goodin Douglas S. , Aminoff Michael J. , Mantle Mary M. 
Published: January 2005

AbstractThe manner in which information processing proceeds and the relationship of the components of the cerebral “event‐related” evoked potential (ERP) to the presumed stages of such processing are uncertain. We studied these relationships by recording long‐latency evoked potentials from the scalp in conjunction with electromyographic (EMG) recording in experiments in which subjects were required to make a motor response to one of two auditory stimuli. The cerebral response was averaged from the tone onset (stimulus‐synchronized average) and the EMG onset (response‐synchronized average). We found that N2 was quite distinct from the other ERP components (P165 and P3) in its lack of a close relationship to the motor response required in a sensory discrimination task. This suggests that, contrary to general belief, the P165‐N2‐P3 components of the ERP are not generated in an orderly sequence, but rather are generated separately and are possibly related to different cognitive processes.
Cell surface markers for diagnosis of central nervous system involvement in lymphoproliferative diseases
Authors: Ernerudh Jan , Olsson Tomas , Berlin Gösta , Gustafsson Bertil , Karlsson Hans 
Published: January 2005

AbstractTo diagnose lymphoproliferative central nervous system (CNS) involvement we have used monoclonal antibodies in an immunocytochemical method for differentiation of cells in cerebrospinal fluid (CSF) and peripheral blood. The cell distribution in 9 patients with B‐cell lymphoma and 7 patients with chronic lymphatic leukemia was compared to that in a group of patients with aseptic meningitis. Most patients with neoplastic CNS involvement showed a high proportion of CSF B cells (OKB2+ and/or OKB7+) and a concurrently low proportion of CSF T cells (anti‐Leu 1 +). Proliferating cells expressing transferrin receptor (OKT9 labeled) were increased in the CSF of 2 patients with neoplastic CNS involvement. In 2 patients with infectious CNS complications, the cell distribution in CSF did not differ from that in patients with aseptic meningitis. Patients with leukemia who had no CNS symptoms, and also 1 patient with meningitis and blood—brain barrier damage, showed a normal cell distribution in CSF despite high B‐cell numbers in the peripheral blood. This indicates a selective passage of leukocytes into the CNS and/or local proliferation.
4‐Aminopyridine improves clinical signs in multiple sclerosis
Authors: Stefoski D. , Davis F. A. , Faut M. , Schauf C. L. 
Published: January 2005

AbstractTwelve temperature‐sensitive male patients with multiple sclerosis and 5 normal men were monitored before, during, and after the intravenous injection of 7 to 35 mg of 4‐aminopyridine (4‐AP) in 1‐to 5‐mg doses, every 10 to 60 minutes. Static quantitative perimetry, flicker‐fusion frequency, visual acuity, and videotaped neurological examinations were performed. Ten of the 12 patients showed mild to marked improvement. Vision improved in 7 patients, oculomotor function in 5, and motor function (power, coordination, gait) in 5. Improvements developed gradually within minutes of drug injection at doses as low as 2 mg, and gradually reversed around 2 to 4 hours after the peak drug effect. No effects were observed in 5 patients given saline injections. No serious side effects occurred in either the normal subjects or the patients receiving 4‐AP. It is concluded that 4‐AP lessens multiple neurological deficits in multiple sclerosis and, furthermore, that the K+ channel is functional in demyelinated central nervous system axons in humans. The improvements with 4‐AP are substantial enough to be of transient therapeutic benefit in selected patients.
Irradiation of meningiomas of the intracranial anterior visual pathway
Authors: Kupersmith Mark J. , Warren Floyd A. , Newall Joseph , Ransohoff Joseph 
Published: January 2005

AbstractTwenty patients with progressive visual loss caused by intracranial meningiomas involving the anterior visual pathway were treated with irradiation alone (n = 4) or with irradiation combined with tumor excision (n = 16). Improvement in visual performance occurred in 13 patients; 2 patients experienced temporary improvement; and 5 others maintained stable visual function for up to 9.5 years (mean follow‐up, 4.0 years). Radiotherapy seemed to have no effect in 2 patients. One patient experienced temporary improvement, but eventually died from spread of tumor. Only one definite complication of radiotherapy occurred and was related to excessive radiation dose (greater than 7,000 cGy). Irradiation in the range of 5,000 to 5,000 cGy was effective either alone or in combination with surgical excision. Serial assessment of visual acuity and fields allowed us to demonstrate and quantify improvement in our patients following radiotherapy; in contrast, follow‐up computed tomographic studies rarely demonstrated reduced tumor size.
Cerebrospinal fluid monoaminergic metabolites are elevated in adults with Down's syndrome
Authors: Kay Arthur D. , Schapiro Mark B. , Riker Amy K. , Haxby James V. , Rapoport Stanley I. , Cutler Neal R. 
Published: January 2005

AbstractUnder conditions of rest and a low monoamine diet, brain monoamine activity was examined in young (<35 years) and old (>35 years) adults with Down's syndrome and in control subjects by measuring the cerebrospinal fluid (CSF) and plasma concentrations of the neurotransmitter norepinephrine, and of 5‐hydroxyindoleacetic acid (5‐HIAA), homovanillic acid (HVA), and 3‐methoxy 4‐hydroxyphenylglycol (MHPG), the respective metabolites of the neurotransmitters serotonin, dopamine, and norepinephrine. There were no age‐related differences in metabolite concentrations in either the Down's syndrome or control subjects. CSF concentrations of 5‐HIAA, HVA, and norepinephrine were significantly higher in young subjects with Down's syndrome as compared with young controls, and CSF concentrations of 5‐HIAA and norepinephrine were significantly higher, by twofold or more, in old subjects with Down's syndrome as compared with older controls. The results suggest that monoamine turnover and brain functional activity involving monoamines is elevated in Down's syndrome, and that the early neuropathological changes in the disorder are not associated with a monoamine deficit.
IgG heavy‐chain (Gm) allotypes in demyelinating polyneuropathy associated with MAG‐binding monoclonal IgM autoantibodies
Authors: Kahn Sidney N. , Pandey Janardan P. 
Published: January 2005

AbstractIgG heavy‐chain (Gm) allotypes were determined in 11 Caucasian patients with the syndrome of demyelinating polyneuropathy associated with monoclonal IgM autoantibodies with affinity for myelin‐associated glycoprotein. The frequency of Gm phenotypes in the patient group was significantly different from that seen in a normal Caucasian population.
Postherpetic gustatory flushing and sweating
Authors: Drummond Peter D. , Boyce Geoffrey M. , Lance James W. 
Published: January 2005

AbstractAn 11‐year‐old girl who had suffered right facial herpes zoster at the age of 6 years was left with anesthetic scars in the distribution of the third division of the trigeminal nerve. Since then, certain tastes provoked flushing and sweating localized to the scarred areas, lasting for 10 to 15 minutes after a latency of a few seconds. The response was evoked most readily from the ipsilateral posterior section of the tongue and was virtually abolished by local administration of anesthesia to the tongue. It remained unaltered after blockade of the sphenopalatine and stellate ganglia but was diminished by blockade of the mandibular nerve. Thermoregulatory sweating and flushing were diminished in the scarred areas. Patchy destruction of sympathetic fibers, which are known to accompany peripheral trigeminal nerve branches, and reinnervation of the affected areas by parasympathetic fibers that normally mediate salivation may explain the phenomenon. It is thus analogous to the gustatory flushing and sweating that may follow damage to the auricultemporal nerve in the region of the parotid gland (Frey's syndrome).
3‐O‐Methyldopa and the response to levodopa in Parkinson's disease
Authors: Nutt John G. , Woodward William R. , Gancher Stephen T. , Merrick Dawn 
Published: January 2005

AbstractPlasma 3‐O‐methyldopa (3OMD) concentrations in parkinsonian patients treated with levodopa on a long‐term basis reflect daily levodopa dosage and do not vary markedly during the day. Oral challenges with 3OMD reduce the clinical response to levodopa infusions, but 3OMD is no more potent than phenylalanine in this regard. These observations, plus the fact that 3OMD makes a small contribution to the total concentration of large neutral amino acids competing with levodopa for transport at the blood‐‐brain barrier, support the contention that 3OMD is not an important determinant of clinical response to levodopa.
Lambert‐Eaton myasthenic syndrome: II. Immunoelectron microscopy localization of IgG at the mouse motor end‐plate
Authors: Fukuoka Tadahiro , Engel Andrew G. , Lang Bethan , Newsom‐Davis John , Vincent Angela 
Published: January 2005

AbstractThe autoimmune origin of the Lambert‐Eaton myasthenic syndrome (LEMS) was documented by passive transfer of its electrophysiological features from humans to mice with IgG. Freeze‐fracture electron microscopy has demonstrated a loss of active‐zone particles in human LEMS and in its mouse passive transfer model. These data imply that the active zones are targets of the pathogenic LEMS autoantibodies. Immunolocalization of the antibodies has been hindered, however, by a paucity of active‐zone particles (about 50/μm2 normally and still lower in LEMS) and by diffusion artifacts in the immunoperoxidase method. To obviate these problems, we employed sensitive avidin–biotin detection systems, both peroxidase and ferritin labels, and quantitative immunoelectron microscopy and end‐plate morphometry. We compared mice treated with LEMS IgG, control IgG, and no IgG. In all mice, nonspecific background staining was found in the basal lamina covering the muscle fibers and Schwann cells. When a single 10‐mg dose of IgG was injected intravenously, IgG samples from 12 patients produced significant immunostaining of the mouse active zones; from 7 patients they did not. Higher doses of intraperitoneally injected IgG (20 mg, three times a day for 2 days, or 10 mg/day for 15 days) from each of 4 patients (3 of whose IgG previously transferred LEMS to mice) caused significant immunostaining of mouse active zones: (1) the mean density (no./μm presynaptic membrane length) of positive active zones was 0.91 in the immunoferritin study and 0.72 in the immunoperoxidase study (control values, 0.12 and 0.02); and (2) 43% of the ferritin particles in the primary cleft were concentrated at the active zones and the rest were scattered randomly (control value, 5.3%). The findings indicate that LEMS IgG binds to the active zones of the presynaptic membrane.