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Biomedical subjects

F Wendling

Publications and source records attributed to F Wendling.

At least 19 recordsLinked to original sources

Epileptic fast activity can be explained by a model of impaired GABAergic dendritic inhibition.

This paper focuses on high-frequency (gamma band) EEG activity, the most characteristic electrophysiological pattern in focal seizures of human epilepsy. It starts with recent hypotheses about: (i) the behaviour of inhibitory interneurons in hippocampal or neocortical networks in the generation of gamma frequency oscillations; (ii) the nonuniform alteration of GABAergic inhibition in experimental epilepsy (reduced dendritic inhibition and increased somatic inhibition); and (iii) the possible depression of GABA(A,fast) circuit activity by GABA(A,slow) inhibitory postsynaptic currents. In particular, these hypotheses are introduced in a new computational macroscopic model of EEG activity that includes a physiologically relevant fast inhibitory feedback loop. Results show that strikingly realistic activity is produced by the model when compared to real EEG signals recorded with intracerebral electrodes. They show that, in the model, the transition from interictal to fast ictal activity is explained by the impairment of dendritic inhibition.

Action Potentials↗

[The presurgical evaluation of epilepsies].

In this article, we present an overview of the principles, practices and procedures of the presurgical evaluation of the epilepsies in use in our center and in the majority of French teams. Surgery for epilepsy is offered to patients presenting with severe epilepsy with partial seizures. Its aim is to stop the seizures, or to significantly reduce their frequency. To do that, the epileptogenic zone should theoretically be removed and/or the propagation pathways of the seizures should be cut. Discussion of these indications inevitably includes prior assessment of the functional sequels (sensory, motor, cognitive or behavioral) which surgery is liable cause. The presurgical evaluation involves a multidisciplinary approach involving epileptologists, neurophysiologists, neuroradiologists, neuropsychologists and neurosurgeons and is carried out in two phases. The phase I is based on non-invasive investigations, including functional and structural neuroimaging, neuropsychological assessment, source localization of interictal spike and video-EEG recordings of seizures. The phase II is often required and is aimed to precisely define the anatomical localization of the epileptogenic zone and the relationships with a structural lesion. This invasive phase is mainly based on stereoelectroencephalography (SEEG). Finally, the surgical procedure must be adapted according to the distribution and dynamics of the anatomical and functional abnormalities which individually define each case of epilepsy.

Age Factors↗

Role of p21(Cip1/Waf1) in cell-cycle exit of endomitotic megakaryocytes.

The cyclin-dependent kinase inhibitor p21(Waf-1/Cip-1) is expressed at high level during megakaryocyte differentiation, but its precise function remains unknown. In this study, it is confirmed that p21 was expressed at a high level in hypoploid (2N and 4N) and polyploid (at least 8N) human megakaryocytes derived from CD34(+) cells. A high expression of p27(Kip1), p16, cyclin E, and cyclin D3 was also found in both populations associated with a hypophosphorylated form of retinoblastoma protein, suggesting that the majority of hypoploid and polyploid megakaryocytes are G(1)-arrested cells. As human megakaryocytes grown in vitro present a defect in their polyploidization, the study switched to the murine model. The modal ploidy of megakaryocytes derived from lineage-negative cells was 32N, and an elevated expression of p21 was found in high-ploidy megakaryocytes. In addition, p21 and p27 were coexpressed in the majority of mature polyploid megakaryocytes. The p21 was detected by immunofluorescence in megakaryocytes derived from p53(-/-) mice, demonstrating a p53-independent regulation during megakaryocyte differentiation. Megakaryocytopoiesis of p21(-/-) mice was subsequently studied. No marked abnormality in the ploidy of primary or cultured megakaryocytes was detected. Overexpression of p21 in p21(-/-) or normal murine megakaryocytes and in human megakaryocytes showed in all these cases a marked inhibition in megakaryocyte polyploidization. In conclusion, while a reciprocal relation is observed between p21 levels in megakaryocytes and the cycling state of the cells, p21 is not essential for the determination of the ploidy profile in normal megakaryocytes in vivo. However, high levels of its expression in cultured megakaryocytes arrest the endomitotic cell cycle.

Animals↗

Germ-line deletion of p53 reveals a multistage tumor progression in spi-1/PU.1 transgenic proerythroblasts.

Activation of the spi-1/PU.1 proto-oncogene and loss of p53 function are genetic alterations associated with the emergence of Friend malignant erythroleukemic cells. To address the role of p53 during erythroleukemogenesis, spi-1 transgenic mice (spi-1-Tg) which develop erythroleukemia were bred with p53-deficient mice. Three classes of spi-1 transgenic mice differing in their p53 functional status (p53(+/+), p53(+/-) and p53(-/-)) were generated. These mice developed a unique pattern of erythroleukemia. In wild-type p53 spi-1-Tg mice, none of the primary erythroleukemic spleen cells displayed autonomous growth in vitro and in vivo. In contrast, in p53(+/-) spi-1-Tg mice, erythroleukemic cells gave rise to growth factor-independent cell lines and generated tumors in vivo. Malignancy was associated with loss of the wild-type p53 allele. The p53(-/-) spi-1-Tg mice developed erythroleukemia with a total incidence and a reduced latency compared to the two other genotypes. Unexpectedly, 50% of p53(-/-) spi-1-Tg erythroleukemic spleens generated cell lines that were strictly dependent upon erythropoietin (Epo) for proliferation, whereas the remainder proliferated independently of cytokines. Moreover, only 70% of these spleen cells were tumorigenic. These findings indicate that p53 germ-line deletion did not confer malignancy to spi-1-transgenic proerythroblasts. Moreover Epo independence and tumorigenicity appear as separable phenotypic characteristics revealing that the spi-1-Tg proerythroblasts progress towards malignancy through multiple oncogenic events.

Animals↗

[Identification of epileptogenic networks from modeling and nonlinear analysis of SEEG signals].

This work is focused on the study of the organization of the epileptogenic zone (E.Z.) in humans based on the analysis of stereo-electroencephalographic (SEEG) signals with signal processing methods, and more especially those dedicated to the estimation of signal interdependencies. In order to evaluate quantities provided by these methods and in order to relate them to the notion of functional coupling between cerebral structures, we developed a neurophysiologically relevant model able to generate EEG signals from organized networks of neural populations. We showed that the model can produce realistic multichannel epileptiform signals (when compared to real SEEG signals) under certain conditions (excitation/inhibition ratio within populations, uni/bi-directional coupling between populations). In this paper, the model framework is used to evaluate the performances of nonlinear regression analysis as a method to characterize couplings between cerebral structures from the SEEG signals they produce. Two quantities, a nonlinear correlation coefficient and a direction index, respectively related to coupling parameters in the model (degree/direction) are presented. These two quantities are measured on real SEEG signals recorded in patients suffering from temporal lobe epilepsy and candidate to surgical treatment. Results show that the characterization of functional couplings leads to the identification of networks referred to as 'epileptogenic networks', which might be responsible for the triggering of seizures. These results also corroborate our previous results on the classification of temporal lobe epilepsies, showing that there exist recurrent seizure patterns that can be classified on the basis of interactions between medial and lateral neocortical structures.

Algorithms↗

Interpretation of interdependencies in epileptic signals using a macroscopic physiological model of the EEG.

This paper presents a neurophysiologically relevant model in which vectorial epileptiform electroencephalographic (EEG) signals are produced from multiple coupled neural populations. This model is used to evaluate the performances of non-linear regression analysis as a method to characterize couplings between neural populations from EEG signals they produce. Two quantities, estimated on generated signals, namely the non-linear correlation coefficient and the direction index, are related to the degree and direction of coupling parameters of the model. Their statistical behavior is first studied on a set of signals simulated for relevant configurations of the model. They are then measured on real stereoelectroencephalographic (SEEG) signals. Results obtained in three patients suffering from temporal lobe epilepsy (TLE) show that abnormal functional couplings between cerebral structures, that establish during seizures, can be interpreted in terms of causality. Perspectives are oriented to the identification of epileptogenic networks in TLE.

Algorithms↗

Neural networks involving the medial temporal structures in temporal lobe epilepsy.

OBJECTIVES: In a previous study using the averaged coherence technique to study interactions between medial/limbic and lateral/neocortical regions, we observed that epileptogenic networks in temporal lobe epilepsy seizures (TLES) could be divided into 4 subtypes, i.e. medial (M), medial-lateral (ML), lateral-medial (LM), and lateral (L). In the ML and LM subtypes, medial structures and the anterior temporal neocortex are co-activated at the onset of seizures. However, using this approach, we were unable to determine the direction of coupling and may have overlooked non-linear variations in interdependency. The purpose of the present study using non-linear regression for analysis of stereoelectroencephalographic (SEEG) signal pairs was to measure the degree and direction of coupling between medial and neocortical areas during TLES in patients with the M, ML, and LM subtypes. METHODS: Eighteen patients with drug-resistant TLEs who underwent SEEG recording were studied. We used a non-linear correlation method as a measure of the degree and the direction of coupling on SEEG signal pairs. Patients with pure lateral TLEs were not studied. We analyzed the functional coupling between 3 regions of the temporal lobe: the anterior temporal neocortex, the amygdala, and the anterior hippocampus. A physiological model of EEG generation was used to validate the non-linear quantification method and assess its applicability to real SEEG signals. RESULTS: Results are first based on a physiological model of EEG data in which both degree and direction of coupling are explicitly represented, thus allowing construction of the neural systems inside which causality relationships are controlled and generation of multichannel EEG signals from these systems. These signals provide an objective way of studying the performance of non-linear regression analysis on real signals. In medial networks (10 patients), the ictal discharge is limited to the medial limbic structures and may propagate secondarily to the cortex. Quantified results demonstrated no significant coupling between medial and lateral structures at the beginning of the seizures. Conversely, almost constant unidirectional or bidirectional coupling was observed between hippocampus and amygdala. In medial-lateral (5 patients) and lateral-medial (3 patients) networks, the initial ictal discharge includes both limbic and neocortical regions. A rapid "tonic" discharge is observed over the temporal neocortex at the onset of seizure. Quantitative analysis showed an initial increase in the non-linear correlation coefficient between neocortex and medial structures. Quantification of the coupling direction demonstrated influence of medial over lateral structures (medial-lateral) or of the lateral neocortex over medial structures (lateral-medial). CONCLUSIONS: These results confirm the existence of several generic and organized networks involving the medial structures during TLE seizures.

Amygdala↗

Rapid generation of a tetracycline-inducible BCR-ABL defective retrovirus using a single autoregulatory retroviral cassette.

The development of chronic myelogenous leukemia (CML) models in mice using an inducible BCR-ABL gene has been hampered by the requirement of sequential expression of tTA (Tet repressor-VP16 fusion protein) and Tet-OP sequences in the same cells after separate transfection. This double transfection strategy is time consuming as it requires screening of many hundreds of individual clones and cannot be applied to primary hematopoietic cells. To generate a tetracycline-inducible BCR-ABL retrovirus, we have subcloned BCR-ABL p210 cDNA in the SIN-Retro-TET vector, which allows regulated expression of a gene of interest in a single autoregulatory cassette, containing both tTA and Tet OP sequences. Retroviral particles were obtained by transfecting the SIN-BCR-ABL p210 construct into the 293 cells and by VSVG pseudotyping. To determine the functionality of the retrovirus, the IL-3-dependent murine Ba/F3 cell line was retrovirally transduced and clones were grown in the absence of both IL-3 (to select for transformed cells) and a tetracycline analog, doxycycline (to induce BCR-ABL expression). Using this technique, polyclonal Ba/F3 cells and several growth factor-independent Ba/F3 clones expressing BCR-ABL were obtained within 2-3 weeks. A single dose of doxycycline added to the medium (1 microg/ml), induced in different clones, a reduction of BCR-ABL protein levels by 60-90% at 24 h, leading to cell death in the absence of IL-3. In several individual clones, BCR-ABL expression was further reduced to become almost undetectable at 48 h. The doxycycline-regulated BCR-ABL expression was stable, as many clones maintained in culture for >8 months showed a persistent inhibitory response to doxycycline addition in the medium. In in vivo experiments, subcutaneous injection of 2 x 10(6) Ba/F3-SIN p210 cells in nude mice induced visible tumors in 2 weeks and all established tumors completely regressed upon addition of doxycycline in the drinking water (200 microg/ml). To determine the functionality of the inducible BCR-ABL retrovirus in vivo, primary Lin- bone marrow cells were transduced with SIN-p210 and transplanted in lethally irradiated mice. All transplanted mice had successful hematopoietic reconstitution and BCR-ABL integration was found in the peripheral blood of seven out of 14 mice available for long-term analysis (>6 months). However, despite evidence of retrovirus-mediated gene transfer, there was no evidence of leukemia, due either to low viral titers or to the relative inefficiency of the minimal CMV promoter in primary hematopoietic cells. Thus, these results demonstrate for the first time, to our knowledge, the feasibility to generate an inducible BCR-ABL retrovirus in a single step, in the context of an immortalized cell line. Our data suggest that with further improvements of the retrovirus-mediated gene transfer technology, it might be possible to generate inducible leukemia models in mice by the use of single retroviral constructs.

Animals↗

Modeling EEG signals and interpreting measures of relationship during temporal-lobe seizures: an approach to the study of epileptogenic networks.

This work is focused on the study of the epileptogenic zone organization (EZ) in humans, based on the analysis of stereoelectroencephalographic (SEEG) signals with signal processing methods, and more specially those dedicated to the estimation of signal interdependencies. In order to evaluate quantities provided by these methods and in order to relate them to the notion of functional coupling between cerebral structures, we developed a neurophysiologically relevant model able to generate EEG signals from organized networks of neural populations. We showed [2, 3] that the model can produce realistic multichannel epileptiform signals (when compared to real SEEG signals) under certain conditions (excitation/inhibition ratio within populations, uni/bi-directional coupling between populations). In this paper, the model framework is used to evaluate the performance of nonlinear regression analysis as a method to characterize couplings between cerebral structures from SEEG signals they produce. Two quantities, a nonlinear correlation coefficient and a direction index, respectively related to coupling parameters in the model (degree/direction) are presented. These two quantities are measured on real SEEG signals recorded in patients suffering from temporal lobe epilepsy and candidate for surgical treatment. Results show that the characterization of functional couplings leads to the identification of networks referred to as "epileptogenic networks" and that might be responsible for the triggering of seizures. These results also corroborate our previous results on the classification of temporal lobe epilepsies [4, 5] showing that a recurrent seizure pattern exists that can be classified on the basis of interactions between medial and lateral neocortical structures. From the identified networks, it is also possible to describe "propagation networks" with a different organization is different and which play a major role in the clinical expression of seizures.

Journal Article↗

Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis.

Idiopathic myelofibrosis (MF) is a myeloproliferative syndrome characterized by an increase in bone marrow collagen. Megakaryocytes (Mks), which store growth factors in their alpha granules, are known to be involved in the pathogenesis of MF. Previously, mice given bone marrow grafts infected with a retrovirus carrying murine thrombopoietin (TPO) complementary DNA developed a disease resembling human idiopathic MF. In this study, we used this murine model (TPO mice) to determine whether release of alpha granules is responsible for fibroblast activation and development of fibrosis. The intracellular trafficking of several alpha-granule proteins (von Willebrand factor, fibrinogen, and transforming growth factor beta (TGF beta), which are stored in the granule matrix; and alpha(IIb)beta(3) integrin and P-selectin (CD62p), which are located in the alpha-granule membrane) was studied with immune electron microscopy in bone marrow Mks from TPO mice. P-selectin immunolabeling increased consistently and was occasionally found lining the demarcation membrane system. Evidence of extensive emperipolesis was also found in TPO mouse Mks, involving almost exclusively neutrophil and eosinophil polymorphonuclear (PMN) cells with altered morphologic features. In parallel, the host Mks had myeloperoxidase-positive granules scattered in their cytoplasm, associated with marked ultrastructural cytoplasmic alterations and ruptured alpha-granule membranes. Similar observations were made in bone marrow biopsy specimens from 12 patients with idiopathic MF; indeed, there was an increased rate of emperipolesis involving mostly PMN cells, abnormal P-selectin expression, and mutual subcellular PMN and Mk alterations. This study indicates that in idiopathic MF, abnormal P-selectin distribution in Mks induces selective sequestration of PMN cells. This results in a release of alpha-granular proteins and growth factors, which in turn induces fibroblast activation and fibrosis deposition. (Blood. 2000;96:1342-1347)

Animals↗

Existence of a differentiation blockage at the stage of a megakaryocyte precursor in the thrombocytopenia and absent radii (TAR) syndrome.

The thrombocytopenia and absent radii (TAR) syndrome is a rare disease associating bilateral radial agenesis and congenital thrombocytopenia. Here, we investigated in vitro megakaryocyte (MK) differentiation and expression of c-mpl in 6 patients. Using blood or marrow CD34(+) cells, the colony-forming unit (CFU)-MK number was markedly reduced. CD34(+) cells were also cultured in liquid medium in the presence of a combination of 3 cytokines (stem cell factor, interleukin-3, and interleukin-6) or megakaryocyte growth and development factor (PEG-rHuMGDF) with or without SCF. In the presence of PEG-rHuMGDF, the majority of mature megakaryocytes (CD41 high, CD42 high) underwent apoptosis. This phenomenon was also observed in cultures stimulated by three cytokines. However, this last combination of cytokines allowed a more complete terminal MK differentiation. Surprisingly, a homogeneous population of CD34(-)CD41(+)CD42(-) cells accumulated during the cultures. This population was unable to differentiate along the myeloid pathways. This result suggests that a fraction of MK cells is unable to differentiate in the TAR syndrome. We subsequently investigated whether this could be related to an abnormality in c-mpl. No mutation or rearrangement in the c-mpl gene was found by Southern blots or by sequencing of the c-mpl coding region and its promoter in any of the patients. Using Western blot analysis, a decreased level of Mpl was found in patient platelets. A decreased level of c-mpl messenger RNA in TAR platelets was also detected with a lower c-mpl-P to c-mpl-K ratio in comparison to adult platelets. Altogether, these results demonstrate that the thrombocytopenia of the TAR syndrome is associated with a dysmegakaryocytopoiesis characterized by cells blocked at an early stage of differentiation. (Blood. 2000;95:1633-1641)

Adolescent↗

Relevance of nonlinear lumped-parameter models in the analysis of depth-EEG epileptic signals.

In the field of epilepsy, the analysis of stereoelectroencephalographic (SEEG, intra-cerebral recording) signals with signal processing methods can help to better identify the epileptogenic zone, the area of the brain responsible for triggering seizures, and to better understand its organization. In order to evaluate these methods and to physiologically interpret the results they provide, we developed a model able to produce EEG signals from "organized" networks of neural populations. Starting from a neurophysiologically relevant model initially proposed by Lopes Da Silva et al. [Lopes da Silva FH, Hoek A, Smith H, Zetterberg LH (1974) Kybernetic 15: 27-37] and recently re-designed by Jansen et al. [Jansen BH, Zouridakis G, Brandt ME (1993) Biol Cybern 68: 275 283] the present study demonstrates that this model can be extended to generate spontaneous EEG signals from multiple coupled neural populations. Model parameters related to excitation, inhibition and coupling are then altered to produce epileptiform EEG signals. Results show that the qualitative behavior of the model is realistic; simulated signals resemble those recorded from different brain structures for both interictal and ictal activities. Possible exploitation of simulations in signal processing is illustrated through one example; statistical couplings between both simulated signals and real SEEG signals are estimated using nonlinear regression. Results are compared and show that, through the model, real SEEG signals can be interpreted with the aid of signal processing methods.

Cybernetics↗

Three-dimensional computer morphometry of the maxilla and face in infants with Pierre Robin sequence--a comparative study.

OBJECTIVE: To analyze the morphology of the maxillary crest in infants with Pierre Robin sequence using an anthropometric coordinate system and to compare the data with those of healthy infants. SETTING: The study was performed at a craniofacial center servicing a large geographic area. PARTICIPANTS: The study involved eight infants aged 1-28 days (average, 7 days) with an established diagnosis of Pierre Robin sequence and six healthy infants aged 1-43 days (average, 22 days). MAIN OUTCOME MEASURES: Physical models of the maxilla and face obtained by alginate replication were analyzed by computer morphometry yielding the three-dimensional topology of the maxillary crest. RESULTS: The maxillary crest of children with Pierre Robin sequence shows an increased inclination relative to the transverse plane (30 +/- 3.9 degrees) as compared with that of healthy infants (20 +/- 2.9 degrees). The maxillary crest of the patients is shortened in the sagittal direction by comparison with healthy controls. CONCLUSIONS: The increased inclination of the maxilla in infants with Pierre Robin sequence may aggravate the retroposition of the mandible and may thus be a pathogenetic factor contributing to the severe respiratory problems.

Alginates↗

High-level expression of Mpl in platelets and megakaryocytes is independent of thrombopoietin.

Thrombopoietin (TPO) is a hematopoietic growth factor that regulates megakaryocytopoiesis and platelet production through binding to its receptor, Mpl, encoded by the c-mpl proto-oncogene. Circulating levels of TPO are regulated by receptor-mediated uptake and degradation. To better understand this mode of TPO regulation, we examined whether expression of Mpl was regulated by its ligand. Using RNase protection analysis, we found no differences in the levels of c-mpl transcripts in megakaryocytes (MKs) produced in vitro either in the presence or absence of TPO and in platelets (PLTs) obtained from mice hyperstimulated in vivo by ectopic secretion of TPO. Similarly, Western blot analysis of MKs produced in the presence or absence of TPO showed no difference in Mpl levels. Levels of Mpl, GpIIb, or P-selectin were virtually identical in platelet lysates obtained from normal, TPO knockout and mildly TPO-stimulated mice. In contrast, the expression of Mpl was significantly reduced in PLTs from severely thrombocythemic mice. These results show that TPO does not have a major effect on the transcription or translation of Mpl. However, they do suggest that an excess of circulating TPO can lead to the disappearance of Mpl from PLTs via catabolism.

Animals↗

Autonomous megakaryocyte growth in essential thrombocythemia and idiopathic myelofibrosis is not related to a c-mpl mutation or to an autocrine stimulation by Mpl-L.

Essential thrombocythemia (ET) and idiopathic myelofibrosis (PMF) are two myeloproliferative diseases characterized by a marked megakaryocytic (MK) involvement. The pathogenesis of these two diseases is unknown. Recently it has been shown that overexpression of Mpl-ligand (Mpl-L) in mice induces thrombocytosis and myelofibrosis. In this study, we investigated whether Mpl-L was responsible for the pathogenesis of ET and PMF. Using in vitro cultures of blood or marrow CD34(+) cells, we investigated whether MK growth was abnormal in these two diseases. Spontaneous MK growth involving only a fraction (20%) of the MK progenitors, as compared with growth in the presence of pegylated recombinant human megakaryocyte growth and development factor (PEG-rhuMGDF), was found in both diseases (21ET and 14PMF) using serum-free semisolid and liquid cultures, including cultures at one cell per well. We first searched for a c-mpl mutation/deletion by sequencing the entire coding region of the gene by polymerase chain reaction (PCR) in nine ET patients and five PMF patients, but no mutation was found. We subsequently investigated whether an autocrine stimulation by Mpl-L could explain the autonomous MK growth. Addition of different preparations of soluble Mpl receptor (sMpl) containing a Fc domain of IgG1 (sMpl-Fc) markedly inhibited MK spontaneous growth in both ET and PMF patients. This effect was specific for sMpl because a control soluble receptor (s4-1BB-Fc) had no inhibitory effect and an sMpl devoid of the Fc fragment had the same inhibitory efficacy as the sMpl-Fc. This inhibition was reversed by addition of PEG-rhuMGDF or a combination of cytokines. The sMpl-Fc markedly altered the entry into cell cycle of the CD34(+) cells and increased the apoptosis that occurs in most patient CD34(+) cells in the absence of exogenous cytokine, suggesting an autocrine stimulation. In contrast, a neutralizing antibody against Mpl-L did not alter the spontaneous MK growth, whereas it totally abolished the effects of 10 ng/mL PEG-rhuMGDF on patient or normal CD34(+) cells. Mpl-L transcripts were detected at a very low level in the patient CD34(+)cells and MK and only when a highly sensitive fluorescent PCR technique was used. By quantitative reverse-transcription (RT)-PCR, the number of Mpl-L transcripts per actin transcripts was lower than detected in human Mpl-L-dependent cell lines, suggesting that this synthesis of Mpl-L was not biologically significant. In favor of this hypothesis, the Mpl-L protein was not detected in culture supernatants using either an enzyme-linked immunosorbent assay (ELISA) or a biological (Ba/F3hu c-mpl) assay, except in one PMF patient. Investigation of Mpl-L signaling showed an absence of constitutive activation of STATs in spontaneously growing patient MKs. Addition of PEG-rhuMGDF to these MKs activated STATs 3 and 5. This result further suggests that spontaneous growth is neither related to a stimulation by Mpl-L nor to a c-mpl mutation. In conclusion, our results show that Mpl-L or Mpl are not directly implicated in the abnormal proliferation of MK cells from ET and PMF. The mechanisms by which the sMpl mediates a growth inhibition will require further experiments.

Adult↗

Seizures of temporal lobe epilepsy: identification of subtypes by coherence analysis using stereo-electro-encephalography.

OBJECTIVES: Two subtypes of temporal lobe epilepsy (TLE) according to the structures initially involved during seizures are currently recognized: medial TLE (MTLE) and lateral (or neocortical) TLE (LTLE). A few reports have suggested that the classification of TLE subtypes might be larger according to variations in the interactions between medial structures and the neocortex. In this study, we analyzed these interactions using coherence analysis of stereo-encephalographic (SEEG) signals during spontaneous seizures. METHODS: Twenty-seven patients with drug-resistant TLE, diagnosed from ictal SEEG recordings obtained during pre-surgical evaluation, were studied. Orthogonally implanted depth electrodes with multiple leads according to Talairach's method were used to sample medial and neocortical structures. Coherence analysis of ictal discharges was performed between two SEEG bipolar signals from adjacent leads located either in medial structures (amygdala and hippocampus) or in neocortical regions of the temporal lobe. A new algorithm, which was designed to reduce the bias inherent in coherence estimation, was used to compute the coherence. RESULTS: We were able to classify TLE seizures (TLES) into 4 distinct categories: (1) 'medial' TLES, characterized by medial onset with later involvement of the neocortex in the form of a 'phasic' discharge. High ictal coherence values were observed between medial structures; (2) 'medial-lateral' TLES which started in medial structures with a fast low-voltage discharge (FLVD) which rapidly affects the neocortex (< or = 3 s). High coherence values were observed between medial and lateral structures; (3) 'lateral-medial' TLES, which are different from medial-lateral TLES in that the FLVD starts in the lateral neocortex and involves the amygdala and/or hippocampus almost immediately after; (4) 'lateral' TLES: characterized by a neocortical onset, a delayed involvement of medial structures (when present), and high coherence values between neocortical structures. CONCLUSIONS: These results demonstrate the existence of numerous interactions between medial limbic structures and the neocortex during TLE seizures. Such findings could have implications for surgical strategies and the prognosis of epilepsy surgery, particularly when limited resection is indicated.

Adolescent↗

Time-frequency matching of warped depth-EEG seizure observations.

A methodology of comparing depth-EEG seizure recordings is presented. The approach is based on an extension of Wagner and Fischer's algorithm to N x 2-dimensional sets, allowing a confrontation of nonequal duration observations characterized by their time-frequency distributions. It proceeds by time and frequency warping on the first observation to match the second, under cost constraints. Preliminary results show that relevant signatures can be extracted from recordings.

Algorithms↗