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Network anatomy in logopenic variant of primary progressive aphasia

The logopenic variant of primary progressive aphasia (lvPPA) is a neurodegenerative syndrome characterized linguistically by gradual loss of repetition and naming skills resulting from left posterior temporal and inferior parietal atrophy. Here, we sought to identify which specific cortical loci are initially targeted by the disease (epicenters) and investigate whether atrophy spreads through predetermined networks. First, we used cross-sectional structural MRI data from individuals with lvPPA to define putative disease epicenters using a surface-based approach paired with an anatomically fine-grained parcellation of the cortical surface (i.e., HCP-MMP1.0 atlas). Second, we combined cross-sectional functional MRI data from healthy controls and longitudinal structural MRI data from individuals with lvPPA to derive the epicenter-seeded resting-state networks most relevant to lvPPA symptomatology and ascertain whether functional connectivity in these networks predicts longitudinal atrophy spread in lvPPA. Our results show that two partially distinct brain networks anchored to the left anterior angular and posterior superior temporal gyri epicenters were preferentially associated with sentence repetition and naming skills in lvPPA. Critically, the strength of connectivity within these two networks in the neurologically-intact brain significantly predicted longitudinal atrophy progression in lvPPA. Taken together, our findings indicate that atrophy progression in lvPPA, starting from inferior parietal and temporoparietal junction regions, predominantly follows at least two partially nonoverlapping pathways, which may influence the heterogeneity in clinical presentation and prognosis

Cutaneous lymphoma international consortium study of outcome in advanced stages of mycosis fungoides and Sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model

Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers. PATIENTS AND METHODS: Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS). RESULTS: Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%). CONCLUSION: To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients

La maladie de Kimura (étude rétrospective multicentrique de 25 cas et relation avec la maladie associée aux IgG4)

La maladie de Kimura (MK) est une maladie lymphoproliférative d'étiologie inconnue, décrite principalement en Asie. Les données dans les pays occidentaux sont rares, surtout en ce qui concerne les modalités thérapeutiques. Une relation avec la maladie associée aux IgG4 (IgG4-RD) a été évoquée. Nous avons analysé les données cliniques, thérapeutiques et histopathologiques chez 25 patients français avec une MK.Les patients étaient majoritairement des hommes (84%), d un âge médian de 42 ans (16-89), avec un suivi médian de 3,5 ans (0,7-18). Des nodules cutanés étaient présents chez 88% des patients. Une atteinte des ganglions lymphatiques, des glandes lacrymales ou salivaires et du rein était rapportée respectivement dans 45%, 24% et 12% des cas. On observait de nombreuses similitudes clinico-pathologiques avec les 27 cas publiés d atteinte cutanée d IgG4-RD. L hybridation in situ par sonde EBER était négative dans 17/18 cas (94%). En cas de lésion résécable, l exérèse chirurgicale était le traitement de première intention avec 100% de réponse complète et 60% de rechutes. Dans les cas inopérables, à lésions multiples, ou avec atteinte extra-cutanée, la corticothérapie générale permettait une rémission complète ou partielle dans 100% des cas avec 50% de rechute. Le thalidomide, la ciclosporine, ou l'interféron-alpha étaient de bonnes alternatives aux corticoïdes généraux avec un taux de réponse de 100%, mais avec respectivement 100%, 20% et 50% de rechutes.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF

Lipofilling: A New Therapeutic Option for the Treatment of Lupus Panniculitis-Induced Atrophy

Lupus panniculitis is a rare manifestation of cutaneous lupus erythematosus, which may lead to major aesthetic sequelae with a severe impact on patients’ quality of life. We report 2 cases supporting the short- and long-term efficacy and safety of lipofilling in the treatment of lupus panniculitis-induced atrophy. These observations pave the way for prospective, larger-scale studies in patients with scarring lupus panniculitis, provided that the autoimmune pathogenic process is in complete, stable remission