BACKGROUND Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory disease. OBJECTIVE To describe the disease ...course of CLIPPERS. DESIGN A nationwide study was implemented to collect clinical, magnetic resonance imaging, cerebrospinal fluid, and brain biopsy specimen characteristics of patients with CLIPPERS. SETTING Academic research. PATIENTS Twelve patients with CLIPPERS. MAIN OUTCOME MEASURES The therapeutic management of CLIPPERS was evaluated. RESULTS Among 12 patients, 42 relapses were analyzed. Relapses lasted a mean duration of 2.5 months, manifested frequent cerebellar ataxia and diplopia, and were associated with a mean Expanded Disability Status Scale (EDSS) score of 4. Besides typical findings of CLIPPERS, magnetic resonance imaging showed brainstem mass effect in 5 patients, extensive myelitis in 3 patients, and closed ring enhancement in 1 patient. Inconstant oligoclonal bands were found on cerebrospinal fluid investigation in 4 patients, with an increased T-cell ratio of CD4 to CD8. Among 7 available brain biopsy specimens, staining was positive for perivascular CD4 T lymphocytes in 5 samples. Thirty-eight of 42 relapses were treated with pulse corticosteroid therapy, which led to improvement, with a mean residual EDSS score of 1.9 (range, 0-7). In 1 patient with untreated relapses, scores on the EDSS progressively increased to a score of 10 at death. Among 5 patients without long-term corticosteroid therapy, the mean annualized relapse rate was 0.5 (range, 0.25-2.8). Among 7 patients taking oral corticosteroids, no relapses occurred in those whose daily dose was 20 mg or higher. No progressive course of CLIPPERS was observed. Four patients with a final EDSS score of 4 or higher had experienced previous severe relapses (EDSS score, ≥5) and brainstem and spinal cord atrophy. CONCLUSIONS CLIPPERS is a relapsing-remitting disorder without progressive forms. Long-term disability is correlated with the severity of previous relapses. Further studies are needed to confirm that prolonged corticosteroid therapy prevents further relapses.
The retrograde transport and transynaptic transfer properties of the nontoxic tetanus toxin C-fragment (TTC) can be used to visualize specific neural pathways or to deliver biomolecules in the ...central nervous system (CNS). Here we tested different delivery techniques to explore the potential use of a new GFP-TTC fusion construct for use as a genetic tracer
in vivo. Plasmids encoding GFP-TTC were targeted to brain regions using intracerebral grafted transfected cells or adenoviral transduction. Transport was monitored using GFP fluorescence. We show that following GFP-TTC synthesis in grafted transfected cells, the TTC fragment alone, with no signal peptide, is necessary and sufficient to provide secretion and uptake of the fusion protein into neighboring neurons around the injection site. Using an adenoviral vector to express the fusion protein into brain neurons, we show that transduced neurons can deliver the fusion protein specifically into connected neurons, demonstrating that synaptic transfer in the CNS can be visualized with GFP-TTC.
Abstract The Second International Workshop on Human Endogenous Retroviruses and Disease, Washington DC, March 13–14 2017 brought together international basic and clinical scientists investigating the ...involvement of human endogenous retroviruses (HERVs) in complex human diseases.
Growth and form are the two main traits used for genetic improvement of maritime pine (Pinus pinaster Ait.) in the southwest of France. In this paper, wood density is studied to answer two main ...questions: Is there a general trend for density variability throughout tree development and has selection indirectly reduced wood density variability over breeding populations, owing to genetic unfavourable correlation with growth? Wood density and its components were studied in three polycross tests, each representative of one of the successive breeding populations. Wood density was measured with an X-ray densitometer in approximately 50 families per test with >1900 trees. A preliminary study showed that bark-to-pith ring indexing allows for a better estimation of genetic effects than does pith-to-bark indexing. Genetic variability of wood density appears to be highly dependent on the year considered and no general pattern can be detected over time. Whereas the variability of selected traits is known to have decreased over breeding populations, no significant change was found for variability of wood density.
Celotno besedilo
Dostopno za:
BF, DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Pilocytic astrocytomas (PA) and hemangioblastomas (HB) can present the same morphological characteristics on conventional MRI sequences, most usually in the form of a cerebellar cystic mass with a ...mural nodule that strongly enhances on post-contrast T1 images. We discuss here the value of perfusion MRI in the differentiation of these two tumors, the diagnoses of which have already been histopathologically established.
Eleven patients with PA and eight with HB underwent first-pass perfusion MRI. The maximum relative cerebral blood volume (rCBV(max)), defined as the ratio between the CBV(max) in tumor tissue and the CBV in healthy, contralateral white matter, is considered to be indicative of the type of tumor.
The difference between the rCBV(max) of PA (rCBV(max)=1.19+/-0.71, range 0.6-3.27) compared with that of HB (rCBV(max)=9.37+/-2.37, range 5.38-13) was significant (P<0.001). The first-pass curve crossed the baseline, corresponding to vascular permeability problems in both PA and HB.
The first-pass method of perfusion MRI is a quick and useful way to differentiate between PA and HB.
Volume and stem straightness were the main selection criteria for the first two generations of the French maritime pine (Pinus pinaster Ait.) breeding programme. In this article, we investigate the ...consequences of this selection on wood quality. Wood density, as a predictor of wood quality, is studied both in the breeding populations and in commercial varieties. Phenotypic and genetic correlations between wood density and growth traits are investigated in successive breeding populations with three genetic field experiments of respectively 30, 29 and 12 years old. Correlation estimates were either slightly negative or non-significantly different from zero depending on the test considered. Consequently, a low impact of growth selection on wood quality should be expected in improved seed sources. However, we observed a significant wood density decrease in two improved varieties as compared to unimproved seed sources at age 15. In addition to this first effect on wood density, growth improvement is also expected to reduce the rotation age and thus increase the proportion of juvenile wood, which is known as having a lower density than mature wood. This change was studied and quantified using a growth model. Finally, a wood density decrease reaching up to 6% was predicted in the improved varieties compared to unimproved material, when both the observed decrease in wood density and the predicted increase in juvenile wood proportion were taken into account. Implications for the breeding programme were considered.
The fine‐scale spatial genetic structure of eight tropical tree species (Chrysophyllum sanguinolentum, Carapa procera, Dicorynia guianensis, Eperua grandiflora, Moronobea coccinea, Symphonia ...globulifera, Virola michelii, Vouacapoua americana) was studied in populations that were part of a silvicultural trial in French Guiana. The species analysed have different spatial distribution, sexual system, pollen and seed dispersal agents, flowering phenology and environmental demands. The spatial position of trees and a RAPD data set for each species were combined using a multivariate genetic distance method to estimate spatial genetic structure. A significant spatial genetic structure was found for four of the eight species. In contrast to most observations in temperate forests, where spatial structure is not usually detected at distances greater than 50 m, significant genetic structure was found at distances up to 300 m. The relationships between spatial genetic structure and life history characteristics are discussed.
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