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F Wendling

Publications and source records attributed to F Wendling.

At least 19 recordsLinked to original sources

Automatic lateralization of temporal lobe epilepsy based on scalp EEG.

OBJECTIVE: The objective of this work is the determination of the lateralization of the epileptic seizure onset zone using the scalp EEG signal processing. METHODS: A comprehensive method based on the evaluation of the evolution of the correlation coefficients computed between bipolar channels (longitudinal montage) was applied to 43 patients (87 seizures). The correlation coefficients are estimated by a nonlinear regression analysis. The methodology that leads to the lateralization is based on several processing steps: segmentation, seizure onset determination and then lateralization. RESULTS: Results show that the mean level of the nonlinear correlation values computed between EEG channels at the seizure onset time is significantly higher on the side of the beginning of a seizure. CONCLUSIONS: The side of the seizure onset was determined for about 80-90% of the seizures studied with a satisfactory high reproducibility level. SIGNIFICANCE: Comparison of nonlinear correlation coefficients between both sides of the brain leads to the determination of the side of seizure onset.

Adolescent↗

Realistic modeling of entorhinal cortex field potentials and interpretation of epileptic activity in the guinea pig isolated brain preparation.

Mechanisms underlying epileptic activities recorded from entorhinal cortex (EC) were studied through a computational model based on review of cytoarchitectonic and neurobiological data about this structure. The purpose of this study is to describe and use this model to interpret epileptiform discharge patterns recorded in an experimental model of ictogenesis (guinea pig isolated brain perfused with bicuculline). A macroscopic modeling approach representing synaptic interactions between cells subpopulations in the EC was chosen for its adequacy to mimic field potentials reflecting overall dynamics rising from interconnected cells populations. Therefore intrinsic properties of neurons were not included in the modeling design. Model parameters were adjusted from an identification procedure based on quantitative comparison between real and simulated signals. For both EC deep and superficial layers, results show that the model generates very realistic signals regarding temporal dynamics, spectral features, and cross-correlation values. These simulations allowed us to infer information about the evolution of synaptic transmission between principal cell and interneuronal populations and about connectivity between deep and superficial layers during the transition from background to ictal activity. In the model, this transition was obtained for increased excitation in deep versus superficial layers. Transitions between epileptiform activities [interictal spikes, fast onset activity (25 Hz), ictal bursting activity] were explained by changes of parameters mainly related to GABAergic interactions. Notably, the model predicted an important role of GABAa,fast- and GABAb-receptor-mediated inhibition in the generation of ictal fast onset and burst activities, respectively. These findings are discussed with respect to experimental data.

Action Potentials↗

A method to identify reproducible subsets of co-activated structures during interictal spikes. Application to intracerebral EEG in temporal lobe epilepsy.

OBJECTIVE: We present a novel quantitative method to statistically analyze the distribution of multichannel intracerebral interictal spikes (multi-IIS) in stereoelectroencephalographic (SEEG) recordings. The method automatically extracts groups of brain structures conjointly and frequently involved in the generation of interictal activity. These groups are referred to as 'subsets of co-activated structures' (SCAS). We applied the method to long duration interictal recordings in patients with mesial temporal lobe epilepsy (MTLE) and analyzed the reproducibility of subsets of structures involved in the generation of multi-IIS for each patient and among patients. METHODS: Fifteen patients underwent long-term intracerebral EEG recording (SEEG technique) using depth electrodes. A 1 h period of continuous interictal EEG recording was selected for each patient with precautions regarding the time after anesthesia pre-SEEG, the temporal distance with respect to seizures, the vigilance state of the patient, and the anti-epileptic drug withdrawal. A research of SCAS was conducted on each recording using the developed method that includes 3 steps: (i) automatic detection of monochannel intracerebral interictal spikes (mono-IIS), (ii) formation of multi-IIS using a temporal sliding window, and (iii) extraction of SCAS. In the third step, statistical tests are used to evaluate the frequency of multi-IIS as well as their significance (with respect to the 'random distribution of mono-IIS' case). RESULTS: In each patient, several thousands of multi-IIS (mean+/-SD, 3322+/-2190) were formed and several SCAS (mean+/-SD, 3.80+/-1.47) were automatically extracted. Results show that reproducible subsets of brain structures are involved in the generation of interictal activity. Although SCAS were found to be variable from one patient to another, some invariant information was pointed up. In all patients, multi-IIS distribute over two distinct groups of structures: mesial structures (15/15) and lateral structures (7/15). Moreover, two particular structures, the internal temporal pole and the temporo-basal cortex, may be conjointly involved with either the first or the second group. Finally, some extracted SCAS seem to match well-defined anatomo-functional circuits of the temporal lobe. CONCLUSIONS AND SIGNIFICANCE: During interictal activity in MTLE, similar subsets of temporal lobe structures are involved in the generation of spikes. This paper brings statistical evidence for the existence of these subsets and presents a method to automatically extract them from SEEG recordings. Interictal activity is spatially organized in the temporal lobe and preferentially involves two functional systems of the temporal lobe (either mesial or lateral).

Adolescent↗

[Spatio-temporal dynamics of neuronal networks in partial epilepsy].

INTRODUCTION: The anatomo-functional organization of partial drug-resistant epilepsies is the subject of much current research aiming at better understanding these pathologies and improving their treatment. The work carried out by our team on the study of intracerebral recording falls within this category of research. The objectives are to identify the neural networks involved in the generation of paroxysmal activity and to understand their spatio-temporal dynamics, in order to be able in the long term to propose targeted therapeutic approaches likely to "control" these networks. STATE OF ART: The traditional concept of epileptic "focus" must nowadays be replaced by a more complex model taking into account potential interactions within the neural networks involved in the seizure. Indeed, during partial seizures, involved cerebral structures are the site of characteristic oscillations which may be synchronized or on the contrary transiently desynchronized. These epileptic rhythms may disturb the physiological rhythms underlying normal cognitive processes; these cognitive processes may thus be impaired in partial epilepsy, even those remote from the site of origin of the discharge. In this article we describe a model of organization of human partial seizures, through characterization of the relationships ("synchrony") between intracerebral signals recorded in the involved structures. We propose that seizures are generated in an initial network of highly epileptogenic brain structures (epileptogenic zone network, EZN) whose activity is synchronized; this activity is then transiently desynchronized with the appearance of fast oscillations. During a second ictal phase, other cortical and subcortical structures are the seat of slower rhythmic modifications that are synchronized (propagation network, PN). The emergence of a particular clinical semiology in the course of the seizure depends on these phenomena which can in certain cases "mimic" a normal cerebral process or on the contrary provoke a major rupture in normal cerebral functioning. CONCLUSIONS: These studies contribute to improvement in our knowledge of the neural networks involved in partial epilepsies. In the future, this type of research may contribute to the development of specific treatments that target certain pathophysiological mechanisms involved in seizure generation.

Brain↗

Epileptic fast intracerebral EEG activity: evidence for spatial decorrelation at seizure onset.

Low-voltage rapid discharges (or fast EEG ictal activity) constitute a characteristic electrophysiological pattern in focal seizures of human epilepsy. They are characterized by a decrease of signal voltage with a marked increase of signal frequency (typically beyond 25 Hz). They have long been observed in stereoelectroencephalographic (SEEG) signals recorded with intra-cerebral electrodes, generally occurring at seizure onset and simultaneously involving distinct brain regions. Spectral properties of rapid ictal discharges as well as spatial correlations measured between SEEG signals generated from distant sites before, during and after these discharges were studied. Cross-correlation estimates within typical EEG sub-bands and statistical tests performed in 10 patients suffering from partial epilepsy (frontal, temporal or fronto-temporal) reveal that SEEG signals are significantly de-correlated during the discharge period compared with periods that precede and follow this discharge. These results can be interpreted as a functional decoupling of distant brain sites at seizure onset followed by an abnormally high re-coupling when the seizure develops. They lead to the concept of 'disruption' that is complementary of that of 'activation' (revealed by significantly high correlations between signals recorded during seizures), both giving insights into our understanding of pathophysiological processes involved in human partial epilepsies as well as in the interpretation of clinical semiology.

Adolescent↗

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↗