Spatial patterns of the pathological changes in neuronal intermediate filament inclusion disease (NIFID):an α-internexin immunohistochemical study

Neuronal intermediate filament inclusion disease (NIFID) is characterized by α-internexin positive neuronal cytoplasmic inclusions (NCI), swollen achromatic neurons (SN), neuronal loss, and gliosis. This study tested: 1) whether the spatial patterns of the lesions was topographically organized in areas of the frontal and temporal lobe and 2) whether a spatial relationship exists between the NCI and SN. The NCI were distributed in regular clusters and in a quarter of these areas, the clusters were 400-800 μm in diameter approximating to the size of the cells of origin of the cortico-cortical pathways. Variations in the density of the NCI were positively correlated with the SN. Hence, cortical degeneration in NIFID appears to be topographically organized and may affect the cortico-cortical projections, the clusters of NCI and SN developing within the same vertical columns of cells. © 2007 Springer-Verlag.

Laminar degeneration of the frontal and temporal cortex in neuronal intermediate filament inclusion disease (NIFID): a study using a-internexin immunohistochemistry

Objective: To determine the laminar distribution of the pathological changes in the frontal and temporal lobe in neuronal intermediate filament inclusion disease (NIFID). Method: The distribution of the alpha-intenexin-positive neuronal cytoplasmic inclusions (NCI), surviving neurons, swollen achromatic neurons (SN) and glial cell nuclei was studied across the cortex in gyri of the frontal and temporal lobe in 10 cases of NIFID. Results: The distribution of the NCI was highly variable within different gyri, a peak in the upper cortex, a bimodal distribution with peaks of density in the upper and lower laminae, or no significant variation in density across the cortex. The surviving neurons were either bimodally distributed or exhibited no significant change in density across the cortex. The SN and glial cell nuclei were most abundant in the lower cortical laminae. In half of the gyri, variations in density of the NCI across the cortex were positively correlated with the SN. In some gyri, the surviving neurons were positively correlated with the SN and negatively correlated with the glial cell nuclei. In addition, the SN and glial cell nuclei were positively correlated in over half the gyri studied. Conclusion: The data suggest that frontal and temporal lobe degeneration in NIFID characterized by NCI, SN, neuronal loss and gliosis extends across the cortical laminae with considerable variation between cases and gyri. alpha-internexin-positive neurons in the upper laminae appear to be particularly vulnerable. The gliosis appears to be largely correlated with the appearance of SN and with neuronal loss and not related to the NCI.

Neuropathological changes in ten cases of neuronal intermediate filament inclusion disease (NIFID):A study using α-internexin immunohistochemistry and principal components analysis (PCA)

Ten cases of neuronal intermediate filament inclusion disease (NIFID) were studied quantitatively. The α-internexin positive neurofilament inclusions (NI) were most abundant in the motor cortex and CA sectors of the hippocampus. The densities of the NI and the swollen achromatic neurons (SN) were similar in laminae II/III and V/VI but glial cell density was greater in V/VI. The density of the NI was positively correlated with the SN and the glial cells. Principal components analysis (PCA) suggested that PC1 was associated with variation in neuronal loss in the frontal/temporal lobes and PC2 with neuronal loss in the frontal lobe and NI density in the parahippocampal gyrus. The data suggest: 1) frontal and temporal lobe degeneration in NIFID is associated with the widespread formation of NI and SN, 2) NI and SN affect cortical laminae II/III and V/VI, 3) the NI and SN affect closely related neuronal populations, and 4) variations in neuronal loss and in the density of NI were the most important sources of pathological heterogeneity. © Springer-Verlag 2005.

Topography of α-internexin-positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease

Abnormal neuronal intermediate filament (IF) inclusions immunopositive for the type IV IF α-internexin have been identified as the pathological hallmark of neuronal intermediate filament inclusion disease (NIFID). We studied the topography of these inclusions in the frontal and temporal lobe in 68 areas from 10 cases of NIFID. In the cerebral cortex, CA sectors of the hippocampus, and dentate gyrus granule cell layer, the inclusions were distributed mainly in regularly distributed clusters, 50-800 μm in diameter. In seven cortical areas, there was a more complex pattern in which the clusters of inclusions were aggregated into larger superclusters. In 11 cortical areas, the size of the clusters approximated to those of the cells of origin of the cortico-cortical pathways but in the majority of the remaining areas, cluster size was smaller than 400 μm. The topography of the lesions suggests that there is degeneration of the cortico-cortical projections in NIFID with the formation of α-internexin-positive aggregates within vertical columns of cells. Initially, only a subset of cells within a vertical column develops inclusions but as the disease progresses, the whole of the column becomes affected. The corticostriate projection appears to have little effect on the cortical topography of the inclusions. © 2006 EFNS.

Spatial patterns of the pathological changes in the temporal lobe of patients with neuronal intermediate filament inclusion disease

Neuronal intermediate filament inclusion disease (NIFID) is a new neurodegenerative disease characterized histologically by the presence of neuronal cytoplasmic inclusions (NI) immunopositive for intermediate filament proteins, neuronal loss, swollen achromatic neurons (SN), and gliosis. We studied the spatial patterns of these pathological changes parallel to the pia mater in gyri of the temporal lobe in four cases of NIFID. Both the NI and SN occurred in clusters that were regularly distributed parallel to the pia mater, the cluster sizes of the SN being significantly greater than those of the NI. In a significant proportion of areas studied, there was a spatial correlation between the clusters of NI and those of the SN and with the density of the surviving neurons. In addition, the clusters of surviving neurons were negatively correlated (out of phase) with the clusters of glial cell nuclei. The pattern of clustering of these histological features suggests that there is degeneration of the cortico-cortical projections in NIFID leading to the formation of NI and SN within the same vertical columns of cells. The glial cell reaction may be a response to the loss of neurons rather than to the appearance of the NI or SN.

Spatial correlations between the neuronal inclusions, swollen achromatic neurons, and glial cells in neuronal intermediate filament inclusion disease (NIFID)

Neuronal intermediate filament (IF) inclusion disease (NIFID) is characterized by neuronal loss, neuronal cytoplasmic IF-positive inclusions (NI), swollen neurons (SN), and a glial cell reaction. We studied the spatial correlations between the clusters of NI, SN, and glial cells in four gyri of the temporal lobe (superior temporal gyrus, inferior temporal gyrus, lateral occipitotemporal gyrus, and parahippocampal gyrus) in four cases of NIFID. The densities of histological features (per 50x250 μ sample field) were as follows: NI (mean = 0.41, range 0.28-0.68), SN (mean = 1.41, range 0.47-2.65), glial cell nuclei (mean = 5.21, range 3.63-8.17). The NI and the SN were positively correlated in half of the brain regions examined, the correlations being present at the smallest field size (50x250 μm). The NI were also positively or negatively correlated with the glial cell nuclei in different areas, the negative correlations being present at the smallest field size. Glial cell nuclei were positively or negatively correlated with the SN in different brain areas, mainly at the larger field sizes (400x250 and 800x250 μm). The spatial correlation between the clusters of NI and SN in the cortex suggests their development within the same columns of cells. At first, the glial cell reaction is also confined to these columns but later becomes more generally distributed across the cortex. © Springer-Verlag 2004.

Intermediate pyrolysis:a sustainable biomass-to-energy concept-biothermal valorisation of biomass (BtVB) process

Wastewater treatment coupled with energy crop cultivation provides an attractive source of cheap feedstock. This study reviews an advanced, closed-loop bioenergy conversion process [biothermal valorisation of biomass (BtVB)], in which pyroformer is coupled to a gasifier. BtVB process was developed at European Bioenergy Research Institute (EBRI), Aston University, UK and demonstrates an improved method for thermal conversion of ash-rich biomass.

The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease

Neuronal intermediate filament inclusion disease (NIFID), a rare form of frontotemporal lobar degeneration (FTLD), is characterized neuropathologically by focal atrophy of the frontal and temporal lobes, neuronal loss, gliosis, and neuronal cytoplasmic inclusions (NCI) containing epitopes of ubiquitin and neuronal intermediate filament proteins. Recently, the 'fused in sarcoma' (FUS) protein (encoded by the FUS gene) has been shown to be a component of the inclusions of familial amyotrophic lateral sclerosis with FUS mutation, NIFID, basophilic inclusion body disease, and atypical FTLD with ubiquitin-immunoreactive inclusions (aFTLD-U). To further characterize FUS proteinopathy in NIFID, and to determine whether the pathology revealed by FUS immunohistochemistry (IHC) is more extensive than a-internexin, we have undertaken a quantitative assessment of ten clinically and neuropathologically well-characterized cases using FUS IHC. The densities of NCI were greatest in the dentate gyrus (DG) and in sectors CA1/2 of the hippocampus. Anti-FUS antibodies also labeled glial inclusions (GI), neuronal intranuclear inclusions (NII), and dystrophic neurites (DN). Vacuolation was extensive across upper and lower cortical layers. Significantly greater densities of abnormally enlarged neurons and glial cell nuclei were present in the lower compared with the upper cortical laminae. FUS IHC revealed significantly greater numbers of NCI in all brain regions especially the DG. Our data suggest: (1) significant densities of FUS-immunoreactive NCI in NIFID especially in the DG and CA1/2; (2) infrequent FUS-immunoreactive GI, NII, and DN; (3) widely distributed vacuolation across the cortex, and (4) significantly more NCI revealed by FUS than a-internexin IHC.

Spatial patterns of FUS-immunoreactive neuronal cytoplasmic inclusions (NCI) in neuronal intermediate filament inclusion disease (NIFID)

Neuronal intermediate filament inclusion disease (NIFID), a rare form of frontotemporal lobar degeneration (FTLD), is characterized neuropathologically by focal atrophy of the frontal and temporal lobes, neuronal loss, gliosis, and neuronal cytoplasmic inclusions (NCI) containing epitopes of ubiquitin and neuronal intermediate filament (IF) proteins. Recently, the 'fused in sarcoma' (FUS) protein (encoded by the FUS gene) has been shown to be a component of the inclusions of NIFID. To further characterize FUS proteinopathy in NIFID, we studied the spatial patterns of the FUS-immunoreactive NCI in frontal and temporal cortex of 10 cases. In the cerebral cortex, sectors CA1/2 of the hippocampus, and the dentate gyrus (DG), the FUS-immunoreactive NCI were frequently clustered and the clusters were regularly distributed parallel to the tissue boundary. In a proportion of cortical gyri, cluster size of the NCI approximated to those of the columns of cells was associated with the cortico-cortical projections. There were no significant differences in the frequency of different types of spatial patterns with disease duration or disease stage. Clusters of NCI in the upper and lower cortex were significantly larger using FUS compared with phosphorylated, neurofilament heavy polypeptide (NEFH) or a-internexin (INA) immunohistochemistry (IHC). We concluded: (1) FUS-immunoreactive NCI exhibit similar spatial patterns to analogous inclusions in the tauopathies and synucleinopathies, (2) clusters of FUS-immunoreactive NCI are larger than those revealed by NEFH or ???, and (3) the spatial patterns of the FUS-immunoreactive NCI suggest the degeneration of the cortico-cortical projections in NIFID.

Atomic structure of the two intermediate phase glasses SiSe4 and GeSe4

The microscopic origin of the intermediate phase in two prototypical covalently bonded AxB1-x network glass forming systems, where A=Ge or Si, B=Se, and 0=x=1, was investigated by combining neutron diffraction with first-principles molecular-dynamics methods. Specifically, the structure of glassy GeSe4 and SiSe4 was examined, and the calculated total structure factor and total pair-correlation function for both materials are in good agreement with experiment. The structure of both glasses differs markedly from a simple model comprising undefective AB4 corner-sharing tetrahedra in which all A atoms are linked by B2 dimers. Instead, edge-sharing tetrahedra occur and the twofold coordinated Se atoms form three distinct structural motifs, namely, Se-Se2, Se-SeGe (or Se-SeSi), and Se-Ge2 (or Se-Si2). This identifies several of the conformations that are responsible for the structural variability in GexSe1-x and SixSe1-x glasses, a quantity that is linked to the finite width of the intermediate phase window.

Characterisation of waste derived intermediate pyrolysis oils for use as diesel engine fuels

This paper studies the characteristics of intermediate pyrolysis oils derived from sewage sludge and de-inking sludge (a paper industry residue), with a view to their use as fuels in a diesel engine. The feedstocks were dried and pelletised, then pyrolysed in the Pyroformer intermediate pyrolysis system. The organic fraction of the oils was separated from the aqueous phase and characterised. This included elemental and compositional analysis, heating value, cetane index, density, viscosity, surface tension, flash point, total acid number, lubricity, copper corrosion, water, carbon residue and ash content. Most of these results are compared with commercial diesel and biodiesel. Both pyrolysis oils have high carbon and hydrogen contents and their higher heating values compare well with biodiesel. The water content of the pyrolysis oils is reasonable and the flash point is found to be high. Both pyrolysis oils have good lubricity, but show some corrosiveness. Cetane index is reduced, which may influence ignition. Also viscosity is increased, which may influence atomisation quality. Carbon residue and ash content are both high, indicating potential deposition problems. Compared with de-inking sludge pyrolysis oil (DSPO), sewage sludge pyrolysis oil (SSPO) has a higher heating value, but higher corrosiveness and viscosity. The conclusions are that both intermediate pyrolysis oils will be able to provide sufficient heat when used in diesel engine; however poor combustion and carbon deposition may be encountered. Blending of these pyrolysis oils with diesel or biodiesel could overcome these problems and is recommended for further investigation.