bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ergot alkaloids block neurogenic extravasation in dura mater: Proposed action in vascular headaches
Authors: Saito Kiyoshi , Marchkowitz Stephen , Moskowitz Michael A. 
Published: January 2005

AbstractAlthough the ergot alkaloids (ergots) are useful drugs for the acute treatment of migraine headaches, their mechanism of action remains obscure. When administered to rats in clinically relevant doses, ergots blocked the development of neurogenic plasma extravasation in dura mater. Plasma extravasation was induced by depolarization of perivascular axons following capsaicin injection or unilateral electrical stimulation of the trigeminal nerve. The ergot action could not be accounted for by vasoconstriction alone because neurogenic plasma leakage was not blocked by angiotensin or phenylephrine. Furthermore, ergots did not block plasma extravasation induced by administering sensory neuropeptides that mediate enhanced permeability. We propose that the therapeutic effects of ergots in vascular headaches may result from peripheral blockade of small fiber (C or A‐delta)‐dependent neurogenic inflammation within the dura mater.
Metabolic changes in rabbit spinal cord after trauma: Magnetic resonance spectroscopy studies
Authors: Vink Robert , Noble Linda J. , Knoblach Susan M. , Bendall M. Robin , Faden Alan I. 
Published: January 2005

AbstractCombined phosphorus and proton magnetic resonance spectroscopy (MRS), using double‐tuned surface coils, was used to monitor certain metabolic changes in the L‐3 spinal segment of anesthetized rabbits prior to and following experimental spinal cord trauma. Following severe trauma, resulting in spastic paraplegia, there was a delayed and progressive accumulation of lactic acid, a decline in intracellular pH, and a loss of high‐energy phosphates. Maximal alterations occurred between 2 and 3 hours after the trauma, with little further change by 4 hours. Histological examination 2 weeks after trauma showed tissue necrosis and cavitation. These findings support the concept of secondary tissue injury after spinal cord trauma and suggest that early changes in metabolism, as shown by MRS, may predict irreversible tissue damage.
Phenobarbital rheumatism in patients with brain tumor
Authors: Taylor Lynne P. , Posner Jerome B. 
Published: January 2005

AbstractWe reviewed the charts of 429 patients with primary brain tumors treated at Memorial Sloan‐Kettering Cancer Center to determine the frequency, clinical characteristics, and provocative factors involved in the development of the shoulder‐hand syndrome. Five percent (20/429) of patients developed this syndrome, which was strongly associated with phenobarbital use (p < 0.0001) and often produced more short‐term disability than the brain tumor itself.
Neuronal degeneration and neurofilament accumulation in the trigeminal ganglia in creutzfeldt‐jakob disease
Authors: Guiroy Don C. , Shankar Susarla K. , Gibbs Clarence J. , Messenheimer John A. , Das Sarala , Gajdusek D. Carleton 
Published: January 2005

AbstractWe report the pathological and immunohistochemical changes in the first‐order neurons in the trigeminal ganglia in Creutzfeldt‐Jakob disease (CJD). Degenerative changes consisted of cytoplasmic vacuolation and fenestration, abundant satellite cells, neurofilament accumulation in neurons, and axonal dystrophy with spheroid formation and torpedolike structures arising from the neuronal cytoplasm. Dystrophic axons, axonal spheroids, and some ganglion cells were labeled with monoclonal antibodies to a phosphorylated epitope of neurofilaments (200 kDa). Polyclonal antibodies to purified scrapie‐associated fibril/prion protein (molecular weight 27–30 kDa) extracted from scrapie‐infected hamster brains, as well as polyclonal and monoclonal antibodies to a synthetic 15–amino acid polypeptide of the 27‐ to 30‐kDa protein, demonstrated variable immunoreactivity with degenerating neurons in the CJD cases, but not in the controls. Furthermore, some of the satellite cells and dystrophic axons were stained by the antibodies to the synthetic peptide. These data indicate that the first‐order neurons of the trigeminal ganglia may form a route by which the CJD agent may travel from the brain to the periphery or vice versa. As in other chronic neurodegenerative diseases, disturbances of neuroaxonal transport seem to occur in CJD.
Absences in juvenile myoclonic epilepsy: A clinical and video‐electroencephalographic study
Authors: Panayiotopoulos Chrysostomos P. , Obeid Tahir , Waheed Ghazala 
Published: January 2005

AbstractWe report a prospective clinical and electroencephalographic study of 19 patients with juvenile myoclonic epilepsy and absence seizures. Absences began 1 to 9 (4.5 ± 2.5) years before myoclonic jerks and generalized tonic‐clonic seizures. Clinical manifestations during the absence ictus showed great variation, ranging from subtle or no overt features to severe impairment of consciousness, and severity was age related. Simple and complex absence seizures can occur in the same patient. The electroencephalographic features were distinct, with many interictal discharges, fragmentation of the paroxysms, and frequent polyspikes of varying numbers and amplitude for each spike‐slow wave component. The combined clinical‐electroencephalographic manifestations were characteristic and allow differentiation of absences in juvenile myoclonic epilepsy from typical absence seizures in other epileptic syndromes.
Lymphocyte subsets in the cerebrospinal fluid in active multiple sclerosis
Authors: Salonen Reijo , Llonen Jorma , Jägerroos Henrik , Syrjälä Hannu , Nurmi Tapio , Reunanen Mauri 
Published: January 2005

AbstractWe studied the relative number of lymphocyte subsets in the cerebrospinal fluid (CSF) of patients with active multiple sclerosis. The cells were double‐labeled with monoclonal antibodies and were studied using a fluorescence‐activated cell analyzer. The number of Leu2+ Leul5+ cells and Leu3+ Leul8+ cells was markedly reduced in the CSF but not in the peripheral blood of the patients. The number of Leu3+ Leu18+ cells was reduced also in the CSF of control patients (patients with other inflammatory or infectious neurological diseases).
Cytochrome C Oxidase—deficient myogenic cell lines in mitochondrial myopathy
Authors: Shimoizumi Hideo , Momoi Mariko Yoshida , Ohta Shigeo , Kagawam Yasuo , Momoi Takashi , Yanagisawa Masayoshi 
Published: January 2005

AbstractIn a patient with mitochondrial myopathy, the defect of cytochrome
ϵ oxidase activity was restricted to some muscle fibers. To isolate cell lines with or without oxidase activity from a single muscle sample, primary cultured cells were transformed by replication origin–defective simian virus 40, and then cloned. The clones were examined by cytochemical staining for cytochrome ϵ oxidase activity. Eight myogenic clones were completely devoid of activity, while the other myogenic and nonmyogenic clones were not. Deficiency of cytochrome ϵ oxidase was stable in culture for at least a year after serial passaging. The amount of mitochondrial DNA in cytochrome ϵ oxidase—deficient cells was the same as in control cells, and no deletion in the mitochondrial DNA was detected. Protein synthesis in mitochondria of the subunits of cytochrome ϵ oxidase and subunit 6 of the ATP synthase complex was markedly decreased, whereas synthesis of the other subunits encoded by mitochondrial DNA was normal. These cloned cell lines provide an excellent system for clarifying the cause of mitochondrial myopathy and for investigating nuclear‐mitochondrial genetic interaction.
Induction of aromatic‐l‐amino acid decarboxylase by decarboxylase inhibitors in idiopathic parkinsonism
Authors: Boomsma F. , Meerwaldt J. D. , Veld A. J. Manin't , Hovestadt A. , Schalekamp M. A. D. H. 
Published: January 2005

AbstractWe evaluated the effect of administration of L‐dopa, alone or in combination with a peripheral decarboxylase inhibitor, on plasma levels of aromatic‐L‐amino acid decarboxylase (ALAAD). After single‐dose administration of L‐dopa plus benserazide (Madopar) in healthy subjects and in chronically treated patients with parkinsonism, plasma ALAAD followed for 2 to 3 hours fell, but returned to predosing levels within 90 minutes. Four groups of patients with idiopathic parkinsonism were studied during chronic treatment: Group I, no L‐dopa treatment (n = 31); Group II, L‐dopa alone (n = 15); Group III, L‐dopa plus benserazide (n = 28); and Group IV, L‐dopa plus carbidopa (Sinemet, n = 30). Plasma ALAAD 2 hours after dosing was normal in Groups I and II. ALAAD was increased threefold in Groups III and IV, suggesting induction of ALAAD by the coad‐ministration of a peripheral decarboxylase inhibitor. In a study of 3 patients in whom L‐dopa/benserazide was started, plasma ALAAD rose gradually over 3 to 4 weeks. Further detailed pharmacokinetic studies of L‐dopa, dopamine, and ALAAD in plasma and cerebrospinal fluid are required to determine if the apparent ALAAD induction by a peripheral decarboxylase inhibitor may be related to the loss of clinical efficacy of combination therapy in some patients and how it is related to end‐of‐dose deterioration and on‐off phenomena.
Altered glucose metabolism in microvessels from patients with Alzheimer's disease
Authors: Marcus David L. , de Leon Mony J. , Goldman Jeffrey , Logan Jean , Christman David R. , Wolf Alfred P. , Fowler Joanna S. , Hunter Kevin , Tsai Jir , Pearson John , Freedman Michael L. 
Published: January 2005

AbstractMicrovessels isolated from temporal of cortex of patients with Alzheimer's disease showed decreased uptake of glucose when compared with vessels from age‐matched or young control subjects. This was due to decreased hexokinase activity in the Alzheimer samples, as determined by ion exchange chromatography. This finding was confirmed independently by determination of the phosphorylation constant for hexokinase, K3, using positron emission tomography. The results suggest that Alzheimer's disease may result from a global defect in brain energy metabolism.
Tomaculous neuropathy presenting as acute recurrent polyneuropathy
Authors: Joy Juan L. , Oh Shin J. 
Published: January 2005

AbstractTomaculous neuropathy (TN) is classically associated with the inherited, recurrent focal neuropathies. We report a case of TN manifesting as an acute recurrent polyneuropathy. A 28‐year‐old woman had 2 episodes of acute, ascending, symmetrical sensorimotor deficit. Teased nerve‐fiber preparation confirmed the presence of TN. Extensive investigations failed to reveal other cause for her symptoms. We believe that this case is unique and broadens the clinical spectrum of TN.
Increases in helper induced T cells and activated T cells in HTLV‐I–associated myelopathy
Authors: Itoyama Yasuto , Kira Jun‐ichi , Fujii Naoki , Goto Ikuo , Yamamoto Naoki 
Published: January 2005

AbstractUsing two‐color flow cytometric analysis, we studied peripheral blood lymphocyte subsets in 15 patients with human T‐cell lymphotropic virus type I–associated myelopathy. The percentage of CD 4+4B4+ cells (helper inducer T cell) was significantly increased in the patients with the myelopathy, compared with 16 healthy control subjects who were seronegative for human T‐cell lymphotropic virus type I. However, there was no difference in the percentage of CD4+2H4+ cells (suppressor inducer T cell) between the two groups. The ratio of CD4+4B4+ cells to CD4+ cells and CD4+4B4+ to CD4+ 2H4+ cells was also elevated in these patients. The percentage of CD4+DR+ cells and CD8+DR+ cells, both of which are phenotypically activated T cells, and the ratio of CD4+DR+ cells to CD4+ cells and of CD8+DR+ cells to CD8+ cells are also increased in the patients, compared with the control subjects. The percentage of Cd4+4B4+ cells showed positive correlations with values of spontaneous proliferation of peripheral blood lymphocytes and serum IgG level in patients with the myhelopathy.
Hyperkalemic periodic paralysis in Gordon's syndrome: A possible defect in atrial natriretic peptide function
Authors: Pasman J. W. , Gabreëls F. J. M. , Semmekrot B. , Renier W. O. , Monnens L. A. H. 
Published: January 2005

AbstractWe present the case of a 14‐year‐old boy who had secondary hyperkalemic periodic paralysis caused by Gordon's syndrome. This syndrome consists of hypertension, tubular acidosis, and hyperkalemia with normal glomerular filtration rate. The pathophysiological mechanism is still unknown. Pathophysiological studies suggest that in this disorder the kidney lacks sensitivity to atrial natriretic peptide. After treatment with hydrochlorothiazide, serum potassium and plasma aldosterone values, plasma rennin activity, and blood pressure became normal and the attacks of periodic paralysis disappeared.
Neurotrophic molecules
Authors: Snider W. D. , Johnson E. M. 
Published: January 2005

AbstractNeurotrophic molecules have a profound influence on development events such as naturally occurring cell death, differentiation, and process outgrowth. Despite their Striking effects on developing neurons, a role for these molecules in the pathogenesis or therapy of neurological disease has not yet been defined. However, a variety of recent advances promise to provide the techniques necessary to asses the potential relevance of neurotrophic molecules to clinical neurology. In this article we review recent investigations into the biological effects, regulation of production, and mechanisms of action of the best characterized trophic molecule, nerve growth factor. In addition we review studies characterizing brain‐derived neurotrophic factor and other putative neurotrophic molecules. Finally, we discuss how pharmacological effects of these molecules may be relevant to the therapy of disease states as well as neural regeneration.
Abnormal systemic metabolism of iron, porphyrin, and calcium in fahr's syndrome
Authors: Beall Steven S. , Patten Bernard M. , Mallette Lawrence , Jankovic Joseph 
Published: January 2005

AbstractStriopallidodentate calcinosis (Fahr's disease) is characterized clinically by seizures, rigidity, and dementia and pathologically by mineral deposition in the basal ganglia, dentate nucleus, and cerebral cortex. Disorders of iron and calcium‐phosphate metabolism are thought to play a role in its pathogenesis. We present the case of a patient with familial striopallidodentate calcinosis who had porphyria cutanea tarda, refractory anemia, and pseudohypoparathyroidism type 2. The serum level of ferritin was markedly increased, serum iron and iron‐binding capacity were below normal, and at autopsy she had deposition of iron in liver, spleen, bone marrow, and brain. She showed intermittent mild hypocalcemia, increased serum values of parathyroid hormone, elevated renal tubular reabsorption of phosphate, and low serum levels of 1,25‐dihydroxyvitamin D, suggesting blunted renal responsiveness to endogenous parathyroid hormone. Pseudohypoparathyroidism type 2 was confirmed by infusion of synthetic parathyroid hormone, which gave a normal urinary cyclic adenosine monophosphate response, but a blunted phosphaturic response. After splenectomy for hypersplenism and weekly phlebotomies, she showed progressive improvement in function, mental status, weight, and seizure control. The hypothesis advanced is that the underlying pathophysiology of the separate diseases contributed to the formation of the brain stones through mechanisms of defective iron transport and free radical production.
Intravenous phenytoin is an effective anticonvulsant in the kindling model
Authors: McNamara James O. , Rigsbee Lewis C. , Butler Linda S. , Shin Cheolsu 
Published: January 2005

AbstractWe reexamined the efficacy of the clinically effective anticonvulsant drug phenytoin in the kindling model. We investigated the effects of varying doses of intravenous phenytoin on serum concentrations and on several indexes of stimulation‐evoked kindled seizures. Intravenous phenytoin produced a dose‐dependent increase in serum phenytoin concentration and powerfully suppressed both limbic and clonic motor seizures. Although focal afterdischarge threshold was elevated to some extent, the most profound effect of phenytoin was limitation of seizure propagation. Variable and low serum concentrations of intraperitoneal or oral phenytoin may explain previous findings that phenytoin is only partly effective or ineffective against kindled seizures. Together with previous results with other drugs, the excellent correlation among drugs effective against human and kindled seizures strengthens the validity of this model. We suggest that the efficacy of experimental anticonvulsant drugs be established in the kindling model before initiation of clinical trials for partial and secondarily generalized seizures.
Intraventricular infusion of dopamine in Parkinson's disease
Authors: Horne M. K. , Butler E. G. , Gilligan B. S. , Wodak J. , Stark R. J. , Brazenor G. A. 
Published: January 2005

AbstractA patient with serve end‐stage Parkinson's disease and troublesome fluctuations in motor function was treated with a long‐term intraventricular infusion of dopamine. There was modest improvement in speech and mentation and there was smoother control of motor symptoms that was superior to that achieved by conventional oral medications.
Creutzfeldt‐Jakob disease patients with congophilic kuru plaques have the missense variant prion protein common to Gerstmann‐Sträussler syndrome
Authors: Doh‐Ura Katsumi , Tateishi Jun , Kitamoto Tetsuyuki , Sasaki Hiroyuki , Sakaki Yoshiyuki 
Published: January 2005

AbstractCongophilic kuru plaques, one of the pathological hallmarks in kuru and Gerstmann‐Sträussler syndrome, are sometimes present in patients with Creutzfeldt‐Jakob disease (CJD). The congophilic kuru plaques are composed partly of a host‐encoded prion protein, and a missense variant prion protein with the codon 102 proline‐to‐leucine change (Leu102) is commonly present in patients with Gerstmann‐Sträussler syndrome. To investigate the relationship between this syndrome and CJD with congophilic kuru plaques, we made a sequence analysis of the prion protein gene from patients with CJD, with or without congophilic kuru plaques. We found no alterations other than the Leu102 change, common to Gerstmann‐Sträussler syndrome, in one of the prion proteins alleles of the patient with congophilic kuru plaques. In the prion protein genotype analysis of other patients with CJD, the Leu102 allele was revealed to be carried heterozygously by 6 of 7 patients who had CJD with congophilic kuru plaques, yet no patient with CJD without congophilic kuru plaques had this allele. Interestingly, the Leu102 allele was also carried by some unaffected relatives of 3 patients with CJD with congophilic kuru plaques but with no apparent familial occurrence of a similar neurological disorder. Our findings show that CJD with congophilic kuru plaques should be categorized as belonging to Gerstmann‐Sträussler syndrome, not CJD, and also suggest that the variant prion protein with Leu102 is closely related to the amyloidgenesis seen in subjects with congophilic kuru plaques.
Hyperfractionated radiotherapy for children with brainstem gliomas: A pilot study using 7,200 cGy
Authors: Packer Roger J. , Allen Jeffrey C. , Goldwein Joel L. , Newall Joseph , Zimmerman Robert A. , Priest John , Tomita Tadanori , Mandelbaum David E. , Cohen Bruce H. , Finlay Jonathan L. , Sutton Leslie N. , D'Angio Giulio 
Published: January 2005

AbstractBrainstem gliomas, constituting approximately 10% of all childhood central nervous system tumors, remain the most resistant of all brain tumors to therapy. A subgroup of high‐risk patients with tumors that diffusely involve the brainstem or that microscopically demonstrate foci of anaplasia on biopsy specimens rarely survive after treatment. Conventional doses of radiotherapy result in temporary clinical improvement in the majority of these high‐risk patients; however, few if any remain alive 18 months after treatment. Hyperfractionated radiotherapy, with delivery of larger numbers of smaller fractions of radiotherapy, is a possible way to increase tumor control without increasing neurological toxicity. In 1985, a multiinstitutional phase I/phase II trial, using 100 cGy of radiation therapy twice daily to a total dose of 7,200 cGy, was undertaken for patients with high‐risk brainstem gliomas. At the time of writing, 24 (69%) had developed progressive disease and 11 remained in continuous progression‐free remission. Actuarial progression‐free survival at 20 months is approximately 30% Twenty‐three of 31 evaluable patients had an objective radiographic response to therapy. In comparison to both historical control patients and patients treated in a previous trial using 6,480 cGy of hyperfractionated radiation therapy, there was a statistically significant improvement in progression‐free survival rate for patients treated with 7,200 cGy of hyperfractionated radiation therapy (p < 0.01). To date no patient has died as a result of treatment. Six patients developed transient neurological deterioration or cystic intralesional changes, as demonstrated on magnetic resonance imaging, within 6 weeks of the completion of radiotherapy. Postmortem examination performed in 7 patients did not disclose significant radiation necrosis. The results of this study suggest that further investigations into the potential benefits of hyperfractionated radiation therapy for children with brainstem gliomas are warranted.
Metabolic causes of myoglobinuria
Authors: Tonin Paola , Lewis Paulette , Servidei Serenella , Dimauro Salvatore 
Published: January 2005

AbstractTo evaluate the proportion of cases of myoglobinuria that can be ascribed to specific metabolic defects, we have studied eight enzymes–phosphorylase, phosphorylase kinase, phosphofructokinase (PFK), phosphoglycerate kinase (PGK), phosphoglycerate mutase (PGAM), lactate dehydrogenase (LDH), carnitine palmitoyltransferase (CPT), and myoadenylate deaminase (MAD)–in muscle biospy specimens from 77 consecutive patients with myoglobinuria (documented in 44, suspected in 33). Enzyme defects were found in 36 patients: CPT deficiency in 17, phosphorylase deficiency in 10, phosphorylase kinase deficiency in 4, MAD deficiency in 3, PGK deficiency in 1, and a combined defect of CPT and MAD in 1. Exercise was the main precipitating factor, both in patients with and in those without detectable enzymopathies. Thirty patients had specific enzymopathies without myoglobinuria: 14 had phosphorylase deficiency, 9 had MAD deficiency, 3 had phosphorylase kinase deficiency, 3 had PFK deficiency, and 1 had PGAM deficiency. Systematic biochemical evaluation of muscle biopsy specimens revealed specific enzymopathies in about half of the patients with idiopathic myoglobinuria. The rest may have blocks of metabolic pathways not yet studied routinely, such as beta oxidation, or genetic defects of the sarcolemma, such as Becker's muscular dystrophy.
Degeneration of pyramidal projection neurons in Huntington's disease cortex
Authors: Cudkowicz Merit , Kowall Neil W. 
Published: January 2005

AbstractWe examined the distribution of neuropeptide Y immunoreactive local circuit neurons and nonphosphorylated neurofilament (SMI 32) immunoreactive pyramidal projection neurons in superior frontal cortex of patients with Huntington's disease and age‐matched control subjects to determine the histological counterpart of increased neuropeptide Y and decreased glutamate concentrations previously found in the cortex of patients with Huntington's disease. We found no difference between the relative density of neuropeptide Y neurons in Huntingon's disease and control brains in regions where the relative density of SMI 32 immunoreactive neurons was significantly reduced. Animal studies show that cortical local circuit neurons are resistant to N‐methyl‐D‐aspartate‐type excitotoxins such as quinolinic acid. Relative sparing of neuropeptide Y neurons in cerebral cortex with Huntington's disease may, therefore, be a result of excessive N‐methyl‐D‐asparate receptor activation.