Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149958 (complex partial seizures)
 2,563 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of successful radiosurgical treatment of lesional epilepsy of mesial temporal origin. A patient presented with a 2-year history of medically intractable complex partial seizures associated with a mesial temporal angioma. Interictal scalp EEG and MEG showed focal epileptiform activity around the lesion. 99mTc-HMPAO-SPECT and 18F-FDG-PET demonstrated depressed blood flow and glucose metabolism in the corresponding temporal lobe. The patient underwent gamma knife radiosurgery for the causative lesion with a low marginal dose of 18 Gy. After treatment, the partial attack ceased without shrinkage of the lesion or peri-lesional parenchymal radiation injury.
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PMID:Successful radiosurgical treatment of lesional epilepsy of mesial temporal origin. 1140 11

Primary malignant melanoma of the leptomeninges is a rare and aggressive tumor in children and accounts for less than 1% of all pediatric malignancies. Usually its symptoms include raised intracraneal pressure resulting from hydrocephalus secondary to tumoral obliteration of basal cisterns, but the passage of time from the initial symptomatology to diagnosis is frequently delayed. A 7-year-old male with primary leptomeningeal melanoma is reported. At the beginning, he presented ataxia and dysarthria followed by symptoms of raised intracranial pressure, complex partial seizures, progressive loss of consciousness, and coma. Cerebrospinal fluid analysis demonstrated raised opening pressure, normal glucose, and increased protein concentration, but malignant melanoma cells were not found. Magnetic resonance imaging scans depicted bright signals in the subarachnoid spaces on T(1) images and gadolinium-enhanced focal lesions. Cerebral biopsy was proposed, but it was not authorized. Definitive diagnosis was thus made by pathologic postmortem examination.
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PMID:Primary leptomeningeal melanoma in a child. 1151 18

Epilepsy is one of the most prevalent neurologic disorders and affects approximately 1% of the population. Most complex seizures arise from the temporal lobes and the condition of 20-30% of these patients is refractory to medication. Many can be rendered seizure free with surgery. Epilepsy surgery requires accurate identification of the site and extent of the epileptogenic area responsible for seizures. EEG is accepted as a gold standard, however only 50% of the patients are safely diagnosed. The need for invasive monitoring with possible hemorrhage or infection has been greatly reduced by the introduction of new technologies such as PET, SPECT and MRI in the clinical practice. MRI demonstrate morphologic changes in approximately 80% of patients with epilepsy. However, structural lesions may not always correlate with clinical, EEG and pathologic localization of epileptogenic foci. Seizures are associated with pronounced changes in regional cerebral blood flow. The real power of SPECT lies in the opportunity of ictal examinations, with a sensitivity ranging from 90 to 97%. Interictal PET studies using 18F-FDG measure regional glucose metabolism, have been investigated for their value as non-invasive focus-localizing techniques. These studies have sensitivity ranging from 80 to 85%. The benzodiazepine binding site that are associated with and modulate the activity of GABA receptors have been imaged by SPECT (using 123I-iomazenil) and PET (using 11C-Flumazenil). Combined measurements of benzodiazepine binding sites and perfusion/metabolism provide a more accurate visualization of epileptogenic site than perfusion or metabolism measurements alone. Functional imaging modalities (PET and SPECT) are highly important in the presurgical evaluation of patients with medically refractory complex partial seizures.
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PMID:[Functional imaging (PET and SPECT) in epilepsy]. 1176 33

Erwinia pyrifoliae is a novel bacterial pathogen, which causes Asian pear blight and is related to Erwinia amylovora, the causative agent of fire blight. E. pyrifoliae produces exopolysaccharide (EPS) related to amylovoran in its sugar composition and sugar linkages. This was shown by degradation of the EPS with a viral depolymerase, and by methylation analysis and ESI/MS. The structure of the repeating units was confirmed by (1)H-NMR spectra. The EPS of E. pyrifoliae carried side chains, which were mainly terminated by acetyl and pyruvyl residues as found previously for amylovoran. On the other hand, a second side chain with glucose found for up to 65% of the repeating units of amylovoran was completely absent. The nucleotide sequences of five genes of the cps cluster of E. pyrifoliae encoding proteins for EPS synthesis were characterized and displayed a high homology with the corresponding ams genes. Similar functions of the gene products are assumed. As for ams mutants of E. amylovora, a cpsB mutant of E. pyrifoliae did not synthesize EPS and did not produce ooze on slices of immature pears or symptoms on pear seedlings. The cps mutant was complemented for EPS synthesis and virulence on pear slices with a gene cluster of E. amylovora that included amsB.
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PMID:Genetics of biosynthesis and structure of the capsular exopolysaccharide from the Asian pear pathogen Erwinia pyrifoliae. 1248 Sep 5

In Escherichia coli, capsular colanic acid polysaccharide synthesis is regulated through the multistep RcsC-->YojN-->RcsB phosphorelay. By monitoring a hallmarked cps::lacZ reporter gene, we first searched for physiological stimuli that propagate the Rcs signaling system. The expression of cps::lacZ was activated when cells were grown at a low temperature (20 degrees C) in the presence of glucose as a carbon source and in the presence of a relatively high concentration of external zinc (1 mM ZnCl(2)). In this Rcs signaling system, the rcsF gene product (a putative outer membrane-located lipoprotein) was also an essential signaling component. Based on the defined signaling pathway and physiological stimuli for the Rcs signaling system, we conducted genome-wide analyses with microarrays to clarify the Rcs transcriptome (i.e., Rcs regulon). Thirty-two genes were identified as putative Rcs regulon members; these genes included 15 new genes in addition to 17 of the previously described cps genes. Using a set of 37 two-component system mutants, we performed alternative genome-wide analyses. The results showed that the propagation of the zinc-responsive Rcs signaling system was largely dependent on another two-component system, PhoQ/P. Considering the fact that the PhoQ/P signaling system responds to external magnesium, we obtained evidence which supports the view that there is a signaling network that connects the Rcs system with the PhoQ/P system, which coordinately regulates extracellular polysaccharide synthesis in response to the external concentrations of divalent cations.
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PMID:Genome-wide analyses revealing a signaling network of the RcsC-YojN-RcsB phosphorelay system in Escherichia coli. 1312 44

A mutational block in the early stages of the glycolytic pathway facilitates the degradation of the ptsG mRNA encoding the major glucose transporter IICBGlc in Escherichia coli. The degradation is RNase E dependent and is correlated with the accumulation of either glucose-6-P or fructose-6-P (Kimata et al., 2001, EMBO J 20: 3587-3595; Morita et al., 2003, J Biol Chem 278: 15608-15614). In this paper, we investigate additional physiological effects resulting from the accumulation of glucose-6-P caused by a mutation in pgi encoding phosphoglucose isomerase, focusing on changes in gene expression. The addition of glucose to the pgi strain caused significant growth inhibition, in particular in the mlc background. Cell growth then gradually resumed as the level of IICBGlc decreased. We found that the transcription of the cps operon, encoding a series of proteins responsible for the synthesis of colanic acid, was markedly but transiently induced under this metabolic stress. Both genetic and biochemical studies revealed that the metabolic stress induces cps transcription by activating the RcsC/YojN/RcsB signal transduction system. Overexpression of glucose-6-P dehydrogenase eliminated both growth inhibition and cps induction by reducing the glucose-6-P level. Mutations in genes responsible for the synthesis of glucose-1-P and/or dTDP-glucose eliminated the activation of the Rcs system by the metabolic stress. Taken together, we conclude that an increased synthesis of dTDP-glucose activates the Rcs phosphorelay system, presumably by affecting the synthesis of oligosaccharides for enterobacterial common antigen and O-antigen.
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PMID:Metabolic block at early stages of the glycolytic pathway activates the Rcs phosphorelay system via increased synthesis of dTDP-glucose in Escherichia coli. 1476 84

BACKGROUND: The high fat, low carbohydrate ketogenic diet (KD) was developed as an alternative to fasting for seizure management. While the mechanisms by which fasting and the KD inhibit seizures remain speculative, alterations in brain energy metabolism are likely involved. We previously showed that caloric restriction (CR) inhibits seizure susceptibility by reducing blood glucose in the epileptic EL mouse, a natural model for human multifactorial idiopathic epilepsy. In this study, we compared the antiepileptic and anticonvulsant efficacy of the KD with that of CR in adult EL mice with active epilepsy. EL mice that experienced at least 15 recurrent complex partial seizures were fed either a standard diet unrestricted (SD-UR) or restricted (SD-R), and either a KD unrestricted (KD-UR) or restricted (KD-R). All mice were fasted for 14 hrs prior to diet initiation. A new experimental design was used where each mouse in the diet-restricted groups served as its own control to achieve a 20-23% body weight reduction. Seizure susceptibility, body weights, and the levels of plasma glucose and beta-hydroxybutyrate were measured once/week over a nine-week treatment period. RESULTS: Body weights and blood glucose levels remained high over the testing period in the SD-UR and the KD-UR groups, but were significantly (p < 0.001) reduced in the SD-R and KD-R groups. Plasma beta-hydroxybutyrate levels were significantly (p < 0.001) increased in the SD-R and KD-R groups compared to their respective UR groups. Seizure susceptibility remained high in both UR-fed groups throughout the study, but was significantly reduced after three weeks in both R-fed groups. CONCLUSIONS: The results indicate that seizure susceptibility in EL mice is dependent on plasma glucose levels and that seizure control is more associated with the amount than with the origin of dietary calories. Also, CR underlies the antiepileptic and anticonvulsant action of the KD in EL mice. A transition from glucose to ketone bodies for energy is predicted to manage EL epileptic seizures through multiple integrated changes of inhibitory and excitatory neural systems.
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PMID:Management of multifactorial idiopathic epilepsy in EL mice with caloric restriction and the ketogenic diet: role of glucose and ketone bodies. 1550 33

Alginate-immobilized Trametes versicolor decolorized Amaranth at similar rates in repeated batch culture when the dye was present in either (i) modified Kirk's medium containing 0.22 gl(-1) ammonium tartrate, (ii) the same buffer, thiamine, trace elements and glucose concentrations as in the modified Kirk's medium, or (iii) glucose alone at either 1, 5 or 10 gl(-1). With glucose alone (0.5 gl(-1)), Amaranth, Reactive Black 5, Reactive Blue 19 and Direct Black 22 had first-order decoloration rate constants of 0.56, 0.76, 0.52, and 0.15 h(-1), respectively. Mixtures of these dyes were also completely decolorized. After four successive decolorations, beads were kept in storage solutions for 48 d at 6 degrees C. CaCl2 (1g l(-1)) was the best storage solution as the beads were easier to handle and had the fastest decoloration rates after storage. Decoloration rates were faster with lower viscosity (less than 2000 cps) alginates and with softer beads which had a lower resistance to compression. Fungal colonization of the beads resulted in higher biomass concentrations with a corresponding higher decoloration rate but the beads became larger, had a lower resistance to compression and a higher percentage of bead breakage in a stirred tank reactor. Biomass, recovered from beads in which there was no growth, could be dispersed while the biomass from colonized beads formed a hollow, spherical shell due to growth on and near the bead surface and no growth in the bead interior. If alginate-immobilized T. versicolor is to be used in a stirred tank reactor, a high biomass loading during the immobilization phase and no fungal growth in the beads is recommended to have high decoloration rates and low bead breakage.
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PMID:Decoloration of textile dyes by alginate-immobilized Trametes versicolor. 1587 90

A 65-year-old male with an old cerebral contusion in the frontal lobes had suffered from status complex partial seizures. Ictal positron emission tomography with an F-18 fluorodeoxyglucose ([18F]FDG-PET) scan revealed hypermetabolism in the frontal, temporal and parietal lobes and the contralateral cerebellar hemisphere. The patient underwent a next-day PET scan with the 15O-labeled gas inhalation technique, which showed mild hyperperfusion and oxygen hypermetabolism in these areas. An interictal [18F]FDG-PET scan 17 days after the initial epilepsy demonstrated glucose hypometabolism of the frontal, temporal and parietal lobes and the contralateral cerebellar hemisphere. Increased glucose metabolism on the interictal PET scan and decreased glucose metabolism on an interictal PET scan in the epileptogenic supratentorial zones and the contralateral cerebellar hemisphere are interesting observations for understanding the pathophysiology in long-standing partial seizures.
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PMID:Crossed cerebellar glucose hypermetabolism demonstrated using PET in symptomatic epilepsy--case report. 1598 77

Insulinoma is a rare disease presenting with episodic neuroglycopenic and/or adrenergic symptoms. We describe the case of a 36 year-old female that had been in treatment for complex partial seizures during 4 years without improvement. She presented many crises with marking hypoglycemia. Cranium tomography and electroencephalogram were normal. A 72-hour fast test showed hypoglycemic symptoms with raised insulin and C-peptide. The insulinoma localization was possible during exploratory laparatomy; image methods did not reveal the tumor. Histological findings confirmed an insulinoma. We conclude that blood glucose level should be requested during the investigation of convulsive and behavioral disorders since an insulinoma can present like them.
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PMID:[Insulinoma presenting as seizure: case report]. 1617 25


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