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Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) and T cell-mediated autoimmune processes are assumed to be involved in its pathogenesis. Recently, accumulating evidence has indicated that commensal bacteria interact with the host immune system and that the alteration of commensal bacteria composition, termed dysbiosis, is associated with various autoimmune diseases including CNS autoimmune diseases. The effect of gut microbiota on disease has been initially shown in experimental autoimmune encephalomyelitis (EAE), animal model of MS. Recent analysis of microbiota revealed dysbiosis in patients with MS including the reduction of short chain fatty acid (SCFA). Administration of SCFA ameliorates disease severity of EAE in association with the induction of regulatory T cells. Moreover metabolites of microbiota such as SCFA and tryptophan have been shown to influence glial functions in CNS. In this review, we introduce recent findings regarding the interaction between gut microbiota and MS both in EAE and MS.

To enrich the three-dimensional experimental details of vortex structures in rotating Rayleigh–Benard convection, we established a technique visualizing three-dimensional vortex structures using scanning planar particle image velocimetry. Experiments were performed at fixed Rayleigh number, $$\hbox {Ra} = 1.0 \times 10^7$$ and different Taylor numbers from $$\hbox {Ta} = 6.0 \times 10^6$$ to $$1.0 \times 10^8$$ , corresponding to convective Rossby numbers from $$0.1 \le \hbox {Ro} \le 0.5$$ at which gradual transition between vortical plumes and convective Taylor columns regime is observed. Stream function distributions calculated from horizontal velocity vector fields visualize the vortex structure formed in the regimes. As quantitative information extracted from the visualized structures, distances between vortices recognized on the distributions show a good agreement with that evaluated by a theory. With the accumulated planar stream function distributions and vertical velocity component calculated from the horizontal velocity vectors, the three-dimensional representations of vortices indicate that quasi-two-dimensional columnar vortices straighten in the vertical direction with increasing Ta.

Background Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of various autoantibodies. This disease causes disabling neuropsychiatric symptoms even in the absence of apparent inflammation in the central nervous system (CNS), but the mechanisms involved remain unknown. Innate immune-mediated inflammation has attracted attention as a pathogenic mechanism in neuropsychiatric diseases. Methods We investigated the CNS of lupus-prone mice focusing on innate immunity. Three strains of lupus-prone mice, FcγRIIB−/−Yaa, an F1 hybrid of NZB and NZW (NZB/NZW) mice, and MRL/Faslpr (MRL/lpr) mice were used to analyze CNS immunopathology. Results Flow cytometry analysis demonstrated the numbers of brain CD45+ cells were increased compared with controls in lupus-prone mice. Upregulation of MHC class I and PDCA1 was observed in microglia and CD11b+ myeloid cells of lupus-prone mice, indicating they were activated in response to interferons (IFN). Microglial gene expression analysis of FcγRIIB−/−Yaa mice revealed the upregulation of IFN-responsive genes and inflammation-related genes including Axl, Clec7a, and Itgax, which were previously reported in neurodegenerative conditions and primed conditions. Upregulated chemokine gene expressions including Ccl5 and Cxcl10 were concurrent with increased numbers of T cells and monocytes, especially Ly6Clo monocytes in the CNS. Upregulation of Axl, Clec7a, Itgax, Ccl5, and Cxcl10 was also observed in NZB/NZW mice, indicating common lupus pathology. The primed status of microglia in FcγRIIB−/−Yaa mice was also demonstrated by morphological changes such as enlarged cell bodies with hypertrophic processes, and hyperreactivity to lipopolysaccharide. Immunohistochemistry of FcγRIIB−/−Yaa mice indicated reactive responses of astrocytes and vascular endothelium. Behavioral studies of FcγRIIB−/−Yaa mice revealed depressive-like behavior and heat hyperalgesia in the forced swim test and the tail-flick test, respectively. Conclusions Our data indicated that microglia in lupus exhibit a unique primed phenotype characterized by the upregulated expressions of neurodegeneration-related genes and IFN-responsive genes. Interaction with peripheral cells and brain resident cells was presumed to orchestrate neuroinflammation. Targeting innate immune cells, such as microglia and monocytes, may be a promising therapeutic approach for neuropsychiatric SLE.

Background The association of gut microbiota and diseases of the central nervous system (CNS), including multiple sclerosis (MS), has attracted much attention. Although a previous analysis of MS gut microbiota revealed a reduction in species producing short-chain fatty acids (SCFAs), the influence of these metabolites on demyelination and remyelination, the critical factors of MS pathogenesis, remains unclear. Methods To investigate the relationship between demyelination and gut microbiota, we administered a mixture of non-absorbing antibiotics or SCFAs to mice with cuprizone-induced demyelination and evaluated demyelination and the accumulation of microglia. To analyze the direct effect of SCFAs on demyelination or remyelination, we induced demyelination in an organotypic cerebellar slice culture using lysolecithin and analyzed the demyelination and maturation of oligodendrocyte precursor cells with or without SCFA treatment. Results The oral administration of antibiotics significantly enhanced cuprizone-induced demyelination. The oral administration of butyrate significantly ameliorated demyelination, even though the accumulation of microglia into demyelinated lesions was not affected. Furthermore, we showed that butyrate treatment significantly suppressed lysolecithin-induced demyelination and enhanced remyelination in an organotypic slice culture in the presence or absence of microglia, suggesting that butyrate may affect oligodendrocytes directly. Butyrate treatment facilitated the differentiation of immature oligodendrocytes. Conclusions We revealed that treatment with butyrate suppressed demyelination and enhanced remyelination in an organotypic slice culture in association with facilitating oligodendrocyte differentiation. Our findings shed light on a novel mechanism of interaction between the metabolites of gut microbiota and the CNS and may provide a strategy to control demyelination and remyelination in MS.

In laboratory experiments, horizontal translational motion of columnar vortices formed in rotating Rayleigh–Bénard convection was investigated. Two types of measurements, vertical velocity fields and horizontal temperature fields, were conducted with water as the test fluid. Using particle image velocimetry, the vertical velocity fields determined the parameter range at which the quasi-two-dimensional columnar vortices emerged. Locally, the duration characteristics of the columns, evaluated with their vertical coherence, indicate the minimum time scale of translational motion of the vortices in the horizontal plane. Vortex tracking of the horizontal temperature fields over long observation periods (${>}10^{3}~\text{s}$) was conducted using encapsulated thermochromic liquid crystal visualization. Two cylindrical vessels with different radii showed the emergence of the centrifugal effect in $O({>}10^{2}~\text{s})$ despite the small Froude number ($Fr). Further, in the horizontal plane the columnar vortices behaved in a random-walk-like diffusive motion. The statistically calculated mean-squared displacements indicated anomalous diffusive motion of the columns; displacement increasing with time as $t^{\unicode[STIX]{x1D6FE}}$ with $\unicode[STIX]{x1D6FE}\neq 1$. We discuss the causes of this anomaly in both the instantaneous and long-term statistical data gathered from experimental observations over different time scales. The enclosure effect from the repulsion of up-welling and down-welling vortices ensures that vortices diffuse only little, resulting in a sub-diffusive (decelerated) motion $\unicode[STIX]{x1D6FE} in $O(10^{1}~\text{s})$. With this weak centrifugal contribution, the translational motion of the columns slowly accelerates in the radial direction and thereby yields a super-diffusive (accelerated) motion $\unicode[STIX]{x1D6FE}>1$ in $O(10^{2}~\text{s})$.

We propose a seeding particle-based color-to-depth calibration methodology for three-dimensional color particle tracking velocimetry (3D color PTV) using a single camera and volumetric rainbow gradient illumination. The use of sheet-color illumination from a liquid crystal display projector enables in situ calibration, namely the color-to-depth relationships of particles seeded in a fluid are determined without inserting any calibration equipment or taking a different optical setup. That is, in this methodology, the calibration and application can be performed using the same optical configuration, and only the digital illumination patterns need to be changed. Adopting this calibration allows evaluating actual color-to-depth relationships of the particles in measurements. The calibration is conducted regarding the relationship between spatially distributed particle colors and their depth coordinates by support of an artificial neural network. By combining conventional PTV with the depth estimated by the color, particle trajectories in 3D real space can be reconstructed from the calibration. The performance of the proposed method was evaluated using a rotating flow in a cylindrical tank by comparing its results with the flow fields measured by conventional particle image velocimetry. Good accordance in the comparison at the highly 3D flow suggests the applicability of the present methodology for various flow configurations.

As an extension of single-pixel particle image velocimetry (SP-PIV), a novel method to determine both the out-of-plane and in-plane velocity fields with single-pixel resolution is presented. With this approach, called dual-plane SP-PIV, mean velocity vector fields with three components (3C) can be measured with single-pixel resolution using a single-color camera and two differently colored laser sheets. The method computes the ensemble correlation across two planes illuminated simultaneously by the two laser sheets. The method is demonstrated via an experiment in which a rotating flow is induced by an impeller. The method is validated by comparing its results with those of conventional PIV. The results show good accordance in terms of the magnitudes and spatial distributions of the out-of-plane velocities and provide a new approach to 3C velocity field measurements with high spatial resolution.

Background Mucosal‐associated invariant T (MAIT) cells have been associated with inflammation in several autoimmune diseases. However, their relation to ischemic stroke remains unclear. This study attempted to elucidate the role of MAIT cells in acute ischemic stroke in mice. Methods and Results We used MR1 knockout C57BL/6 (MR1 −/− ) mice and wild‐type littermates (MR1 +/+ ). After performing a transient middle cerebral artery occlusion (tMCAO), we evaluated the association with inflammation and prognosis in the acute cerebral ischemia. Furthermore, we analyzed the tMCAO C57BL/6 mice administered with the suppressive MR1 ligand and the vehicle control. We also evaluated the infiltration of MAIT cells into the ischemic brain by flow cytometry. Results showed a reduction of infarct volume and an improvement of neurological impairment in MR1 −/− mice (n=8). There was a reduction in the number of infiltrating microglia/macrophages (n=3–5) and in their activation (n=5) in the peri‐infarct area of MR1 −/− mice. The cytokine levels of interleukin‐6 and interleukin‐17 at 24 hours after tMCAO (n=3–5), and for interleukin‐17 at 72 hours after tMCAO (n=5), were lower in the MR1 −/− mice. The administration of the suppressive MR1 ligand reduced the infarct volume and improved functional impairment (n=5). Flow cytometric analysis demonstrated there was a reduction of MAIT cells infiltrating into the ischemic brain at 24 hours after tMCAO (n=17). Conclusions Our results showed that MAIT cells play an important role in neuroinflammation after focal cerebral ischemia and the use of MAIT cell regulation has a potential role as a novel neuroprotectant for the treatment of acute ischemic stroke.

Transient behaviours during spin-up in rotating Rayleigh–Benard convection (RBC) with imposed rotation were quantitatively investigated in laboratory experiments. Horizontal and vertical velocity fields were measured by particle image velocimetry with water as the test fluid. Varying the aspect ratio, Rayleigh number and Taylor number, a total of twenty parameters were systematically explored. Toroidal and spiral rolls were formed when the flow reached the rigid-body rotation state, and creation of these structures propagated from the rim towards the internal regions together with the development of the spin-up. Alternate alignments of rolls with opposite meridional circulation transported azimuthal momentum in the rigid-body rotation, and a meandering velocity profile in the radial direction, induced Kelvin–Helmholtz (KH) instability generating azimuthally aligned vortices. The vortices progressively decreased in horizontal dimensions with the wall-to-centre propagation of the vortex formation, but the vortical structures remain larger than the columnar vortices formed in the equilibrium state of a rotating RBC. At the intermediate radius of the fluid layer, the wall-to-centre propagation of the roll formation was overtaken by that of the KH vortex formation. Farther into the interior region, thermal plumes forming columnar vortices were generated as separations of the thermal boundary layers, and the system reached an equilibrium state of rotating RBC dominated by columnar vortices. Use of a fluid vessel with a moderate aspect ratio clarified these transitions to occur in a stepwise fashion, and a spin-up time scale unique in the rotating RBC was found to be from a few to 10 times the Ekman time scale.

We report autopsy results of a female patient who was confirmed pathologically as having corticobasal degeneration (CBD). This patient presented with progressive gait disturbance at the age of 66 years, and subsequently showed parkinsonism with a right-sided predominance and dementia. She was clinically diagnosed as having possible corticobasal syndrome without palatal myoclonus throughout the disease course. An autopsy at 72 years of age revealed that neuronal loss with gliosis was severe in the substantia nigra and the portion from hippocampal cornu ammonis (CA1) region to the parahippocampal gyrus, and mild-to-moderate in the basal ganglia, thalamus, red nucleus, dentate nucleus, and cerebral cortices, predominantly in the frontal lobe. Myelin pallor was observed in the pyramidal tract of the brainstem and central tegmental tract. Neurodegenerative or axonal degenerative findings were observed predominantly on the left side, except for the dentate nucleus, which was more affected on the right side. The inferior olivary nucleus exhibited hypertrophic degeneration predominantly on the left side. The topography of neurodegeneration was likely to correspond to the dentate nucleus and inferior olivary nucleus. Phosphorylated tau-immunoreactive pretangles, neurofibrillary tangles, coiled bodies, and threads were diffusely observed in the whole brain. The distribution of tau deposits was prominent in the deeper affected lesions of the dentate nucleus and inferior olivary nucleus. Inferior olivary hypertrophy is unusual in patients with CBD. It is highly possible that the neurodegeneration of the inferior olivary nucleus followed that of the dentate nucleus in our patient. Moreover, these results indicate not only the severity of neurodegenerative changes, but also that of tau deposition that could be related to the topography of the projections of the dentato-olivary pathway. Tau propagation and subsequent neurodegeneration along the fiber connections may have occurred. Our results support the possibility that progression of CBD lesions can be mediated by tau propagation.

Abstract We introduce a complex image processing scheme for the simultaneous application of liquid crystal thermometry (LCT), in addition to the previously established method in Anders et al. (Exp Fluids 60(4):68, 2019. 10.1007/s00348-019-2703-8) for particle tracking velocimetry and particle image velocimetry. This scheme was developed for an experimental study on the double-diffusive convection in an aqueous ammonium chloride solution NH4Cl(aq) during crystallization. The use of thermochromic liquid crystals (TLC) enables to visualize the flow and temperature field simultaneously. We present a color interpolation method that enhances the accuracy of the LCT by yielding RGB images only representative of the TLC’s coloration. An artificial neural network (ANN) which processes RGB triplets and spatial color dependencies transforms these images into temperature fields. The combination of the ANN system and a corresponding calibration procedure enhances the accuracy and measurable temperature range of the LCT compared to state-of-the-art procedures. By using the here established measurement scheme, quantitative global studies of the mutual influence between solidification and convection are enabled and exemplary results are presented. Graphic abstract

Multiple sclerosis (MS) is an inflammatory disease of the central nervous system (CNS) and T cell-mediated autoimmune processes are assumed to be involved in its pathogenesis. Recently, accumulating evidence has indicated that commensal bacteria interact with the host immune system and that the alteration of commensal bacteria composition, termed dysbiosis, is associated with various autoimmune diseases including CNS autoimmune diseases. In this review, we introduce recent findings regarding the association between gut microbiota and MS and related diseases and microbiota function in an animal model of MS.

We established a vortex detection method using instantaneous temperature fields that were visualized using thermochromic liquid crystals (TLCs) to investigate behaviors of vortical structures in a rotating Rayleigh–Benard convection. Experimental testing was performed at a fixed Rayleigh number $$Ra = 1.0 \times 10^7$$ and different Taylor numbers from $$Ta = 1.0 \times 10^6$$ to $$1.0 \times 10^8$$ in water containing encapsulated TLCs. Vortices were recognized as undulations that appear in the horizontal temperature fields, thus making vortex detection with high spatial resolution possible, and this enabled quantitative investigation of the dynamics of vortical structures. Standard template matching was used to detect individual vortices on visualized temperature fields, and two-dimensional curved surface fitting was adopted to remove erroneous detections and to evaluate shapes of local temperature fields corresponding to vortical structures. Additionally, vortex tracking clearly showed geometric advection pattern of vortical structures.

Coupling structures of buoyancy induced thermal convection in two immiscible layers with and without weak background rotation were investigated in laboratory experiments using a glycerol solution and a silicon oil as the test fluids. A cylindrical vessel was employed to yield three-dimensional (3D) development of the two-layer convection, and the influence of background rotation on convective motion was investigated to imitate the rotation of the Earth. Using color image processing, velocity fields at two horizontal cross-sections, one in each layer, of the vessel were measured simultaneously by stereoscopic particle image velocimetry to identify the interactions on interface between the layers. The temporal evolution of the velocity fields showed that thermal convection, first developed in the lower layer subsequently progresses ahead of the upper layer both with and without rotation. The rotation suppressed developments of convection in both of the two layers in cases of small thermal forces. The coupling structures of the 3D convection cells were evaluated from the perspectives of cross-correlations and the dominance ratio, representing the accumulation of the local couplings, by using simultaneously measured velocity information. The coupling structures were found to be well characterized by the dominance ratio, as various local couplings were coexisting in the 3D domain. The global coupling structures were in the thermal coupling, and those were weakened by the rotation.

The effect of background stable temperature stratification in the developing stage of horizontal convection is studied by conducting laboratory experiments. By imposing horizontally differential heating at the top of a layer of low-temperature water, both vertical and horizontal temperature differences are explicitly defined. In developing horizontal convection of the present study, the flow structures are driven only by the baroclinic torque produced by the horizontal temperature difference, and braked by the restoring force of stable temperature stratification. We thus defined a nondimensional stratification parameter, which represents the balance of the braking force and the baroclinic torque, in addition to the Rayleigh number. Various features of the flow structures, maximum velocity, stream function, roll thickness, circulation of the roll, total kinetic energy, and Reynolds number, which are quantified via particle-tracking velocimetry, are summarized in spaces of the two nondimensional parameters. In the developing horizontal convection, the quantified flow features are well organized by power laws of the nondimensional parameters. The finite domain of the fluid container augments the effect of the apparent braking force, and the bulk quantities of the roll structures are suppressed by the stable temperature stratification. These results are the evidences for the significance of the nondimensional stratification parameter in the developing horizontal convection, unlike thermally equilibrated horizontal convection conventionally considering destabilizing thermal buoyancy as the primary driving force.

Summary Background The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) showed that thymectomy combined with prednisone was superior to prednisone alone in improving clinical status as measured by the Quantitative Myasthenia Gravis (QMG) score in patients with generalised non-thymomatous myasthenia gravis at 3 years. We investigated the long-term effects of thymectomy up to 5 years on clinical status, medication requirements, and adverse events. Methods We did a rater-blinded 2-year extension study at 36 centres in 15 countries for all patients who completed the randomised controlled MGTX and were willing to participate. MGTX patients were aged 18 to 65 years at enrolment, had generalised non-thymomatous myasthenia gravis of less than 5 years' duration, had acetylcholine receptor antibody titres of 1·00 nmol/L or higher (or concentrations of 0·50–0·99 nmol/L if diagnosis was confirmed by positive edrophonium or abnormal repetitive nerve stimulation, or abnormal single fibre electromyography), had Myasthenia Gravis Foundation of America Clinical Classification Class II–IV disease, and were on optimal anticholinesterase therapy with or without oral corticosteroids. In MGTX, patients were randomly assigned (1:1) to either thymectomy plus prednisone or prednisone alone. All patients in both groups received oral prednisone at doses titrated up to 100 mg on alternate days until they achieved minimal manifestation status. The primary endpoints of the extension phase were the time-weighted means of the QMG score and alternate-day prednisone dose from month 0 to month 60. Analyses were by intention to treat. The trial is registered with ClinicalTrials.gov, number NCT00294658. It is closed to new participants, with follow-up completed. Findings Of the 111 patients who completed the 3-year MGTX, 68 (61%) entered the extension study between Sept 1, 2009, and Aug 26, 2015 (33 in the prednisone alone group and 35 in the prednisone plus thymectomy group). 50 (74%) patients completed the 60-month assessment, 24 in the prednisone alone group and 26 in the prednisone plus thymectomy group. At 5 years, patients in the thymectomy plus prednisone group had significantly lower time-weighted mean QMG scores (5·47 [SD 3·87] vs 9·34 [5·08]; p=0·0007) and mean alternate-day prednisone doses (24 mg [SD 21] vs 48 mg [29]; p=0·0002) than did those in the prednisone alone group. 14 (42%) of 33 patients in the prednisone group, and 12 (34%) of 35 in the thymectomy plus prednisone group, had at least one adverse event by month 60. No treatment-related deaths were reported during the extension phase. Interpretation At 5 years, thymectomy plus prednisone continues to confer benefits in patients with generalised non-thymomatous myasthenia gravis compared with prednisone alone. Although caution is appropriate when generalising our findings because of the small sample size of our study, they nevertheless provide further support for the benefits of thymectomy in patients with generalised non-thymomatous myasthenia gravis. Funding National Institutes of Health, National Institute of Neurological Disorders and Stroke.

Objective Peripheral helper T (TPH) cells are a recently identified Th cell subset that promotes B cell differentiation and antibody production in inflamed tissues. This study investigated circulating TPH cells to determine their involvement in systemic lupus erythematosus (SLE). Methods Peripheral blood mononuclear cells collected from SLE patients and healthy individuals were analysed. TPH cells were identified as CD3+CD4+CD45RA-CXCR5- cells with a high expression of PD-1. The frequency, activation status and subsets of TPH cells were evaluated by flow cytometry. The production of IL-21 was assessed by intracellular staining and the association of TPH cells with disease activity and B cell populations was determined. Results Circulating TPH cells, identified as CD3+CD4+CD45RA-PD-1highCXCR5- cells were increased in the peripheral blood of SLE patients compared with controls. Circulating TPH cells produced similar amounts of IL-21 compared with follicular Th cells. The expansion and activation of TPH cells were correlated with SLE disease activity. Activated TPH cells, particularly Th1-type TPH cells, were associated with the promotion of B cell differentiation in SLE patients. Conclusion The association of TPH cells with disease activity suggests the involvement of extrafollicular T-B cell interactions in the pathogenesis of SLE. TPH cells promote autoantibody production in aberrant lymphoid organs and therefore might be a novel therapeutic target in autoantibody-producing disorders.

A novel masking technique for particle image velocimetry (PIV)-analysis of multiphase flows is presented. With this new approach, the velocity-field of an unmasked particle fraction (e.g., PIV-tracers) can be determined without the influence of a second (masked) particle fraction (e.g., bubbles or solid particles). Starting from a series of segmented grayscale images in which different particle fractions are determined for each frame, different masking techniques are compared. The problems caused by state of the art masking in case of dynamic masks (individual masks for each frame) are discussed. As a solution, the novel spectral random masking algorithm is presented which replaces masked regions in the images by randomized intensity patterns in order to hide them from subsequent PIV-analysis. The advantages over conventional masking techniques are demonstrated for a model experiment of crystallizing ammonium-chloride solution.

Background Microglia, as well as other tissue-resident macrophages, arise from yolk sac progenitors. Thus, it is likely that the central nervous system environment is critical for the acquisition of a distinct microglial phenotype. Several microRNAs that are enriched in the brain play crucial roles in brain development and may also play a role in the differentiation of microglia. Methods To track the differentiation of hematopoietic cells into microglia, lineage-negative bone marrow cells were co-cultured with astrocytes in the absence or presence of microRNAs or their inhibitors. Microglia-like cells were identified as small, round cells that were immunopositive for CD11b, Iba1, CX3CR1, and triggering receptor expressed on myeloid cells (TREM)-2. Results Five microRNAs (miR-101a, miR-139-3p, miR-214*, miR-218, and miR-1186) were identified as modifiers of the differentiation of bone marrow-derived microglia-like cells. Among them, miR-101a facilitated the differentiation of bone marrow cells into microglia-like cells most potently. Small, round cells expressing CD11b, Iba1, CX3CR1, and TREM-2 were predominant in cells treated by miR-101a. miR-101a was abundantly expressed in non-microglial brain cells. Transfection of miR-101a into microglia significantly increased the production of IL-6 in response to LPS. Finally, miR-101a downregulated the expression of MAPK phosphatase-1. Conclusions miR-101a, which is enriched in the brain, promotes the differentiation of bone marrow cells into microglia-like cells. Electronic supplementary material The online version of this article (doi:10.1186/s12974-017-0884-8) contains supplementary material, which is available to authorized users.

We report a patient with GNE myopathy with a homozygous mutation (c.1505-4G>A) in GNE gene. The patient recognized progressive weakness of extremities at age 60. Neurological examination at age 65 revealed severe weakness and atrophy in the tibialis anterior muscles and distal predominant moderate weakness in the extremities. Muscle biopsy performed at age 65 showed myopathic changes with rimmed vacuoles, and the noteworthy finding was non-caseating epithelioid cell granuloma formation surrounded by numerous inflammatory cells. Granuloma formation has never been reported in patients with GNE myopathy. We presume that aggregation of abnormal proteins and autophagy dysregulation in the myocytes of GNE myopathy could induce granuloma formation.

金沢大学医薬保健研究域医学系, Microglia are believed to be the only resident immune cells in the CNS, originating from hematopoietic-derived myeloid cells and invading the CNS during development. However, the detailed mechanisms of differentiation and transformation of microglial cells are not fully understood. Here, we demonstrate that murine microglial cells show two morphological forms in vitro, namely, small round cells expressing CD11b, Iba1, triggering receptor expressing on myeloid cells-2 (TREM2), and weakly expressing major histocompatibility complex class II and large flat cells expressing only CD11b and Iba1. Moreover, lineage-negative bone marrow (LN) cells cultured with primary mixed glial culture cells could differentiate into only the small round microglia-like cells, despite the absence of CCR2 and Gr-1 expression. Addition of macrophage colony stimulating factor (M-CSF) to LN cell culture allowed the proliferation and expression of TREM2 in LN cells, and the addition of neutralizing anti-M-CSF antibodies suppressed the proliferation of LN cells despite the expression of TREM2. When LN cells were cultured with M-CSF, the number of small round cells in the culture was considerably low, indicating that the small round morphology of the immature cells is not maintained in the presence of only M-CSF. On the other hand, when LN cells were grown in the presence of astrocytes, the small round cells were maintained at a concentration of approximately 30% of the total population. Therefore, cell-cell contact with glial cells, especially astrocytes, may be necessary to maintain the small round shape of the immature cells expressing TREM2. © Federation of European Neuroscience Societies and Blackwell Publishing Ltd.

SUMMARY Improving the speed of fluorescent X-ray CT imag-ing using synchrotron radiation has been studied from thepoints of view of the imaging system and the processingalgorithm. Regarding the imaging system, high-speed im-aging at 2 seconds per point with a dead time of about 6%has been realized by incorporating an electronic systemwhich operates stably in the high count rate region. Inaddition, the amounts of computation and memory requiredhave been reduced to about 1/100 of the levels realized upto now by improving the efficiency of the reconstructionalgorithm. The quantitative properties of this system havebeen confirmed by tests with a physical phantom and theiodine distribution has been estimated in in vitro humanthyroid images. These results indicate the implementabilityof in vivo fluorescent X-ray CT instrumentation. ' 2002Scripta Technica, Syst Comp Jpn, 33(2): 1Œ11, 2002; DOI10.1002/scj.1101Key words: Fluorescent X-ray; CT (computed to-mography); synchrotron radiation; image reconstruction.

金沢大学附属病院神経内科, We report two cases of superficial siderosis (SS) of the central nervous system (CNS), which is caused by chronic haemorrhaging into the subarachnoid space with haemosiderin deposition in the superficial portion of the CNS. Patient 1 had fluid collection in the spinal canal, which was reported as the source of the chronic bleeding. Patient 2 was bleeding from thickened dura at the level of the sacral vertebrae. Both of the patients had xanthochromic cerebrospinal fluid. We surgically repaired the sources of bleeding. Subsequently the cerebrospinal fluid (CSF) cleared and their symptoms were not aggravated for about 1 year. We measured several CSF markers of SS before and after surgery. Total tau protein (CSF-t-tau), phosphorylated tau protein (CSF-p-tau), iron (CSF-iron) and ferritin (CSF-ferritin) in the CSF were highly elevated at diagnosis. After surgery, the levels of CSF-t-tau and CSF-p-tau were markedly reduced while CSF-iron and CSF-ferritin had not decreased. It is suggested that CSF-t-tau and CSF-p-tau reflected the neural damage in SS and were useful to evaluate the effectiveness of SS therapies. © 2009 Elsevier B.V. All rights reserved.

Autoradiography is a useful imaging technique to understand biological functions using tracers including radio isotopes (RI's). However, it is not easy to describe the distribution of different kinds of tracers simultaneously by conventional autoradiography using X-ray film or Imaging plate. Each tracer describes each corresponding biological function. Therefore, if we can simultaneously estimate distribution of different kinds of tracer materials, the multispectral autoradiography must be a quite powerful tool to better understand physiological mechanisms of organs. So we are developing a system using a solid state detector (SSD) with high energy- resolution. Here, we introduce an imaging technique with a coded aperture to get spatial and spectral information more efficiently. In this paper, the imaging principle is described, and its validity and fundamental property are discussed by both simulation and phantom experiments with RI's such as 201Tl, 99mTc, 67Ga, and 123I.© (2000) COPYRIGHT SPIE--The International Society for Optical Engineering. Downloading of the abstract is permitted for personal use only.