Malignant catatonia due to anti-NMDA-receptor encephalitis in a 17-year-old girl: case report

Anti-NMDA-Receptor encephalitis is a severe form of encephalitis that was recently identified in the context of acute neuropsychiatric presentation. Here, we describe the case of a 17-year-old girl referred for an acute mania with psychotic features and a clinical picture deteriorated to a catatonic state. Positive diagnosis of anti-NMDA-receptor encephalitis suggested specific treatment. She improved after plasma exchange and immunosuppressive therapy. Post-cognitive sequelae (memory impairment) disappeared within 2-year follow-up and intensive cognitive rehabilitation

Hybrid PET/MRI co-segmentation based on joint fuzzy connectedness and graph cut

International audienceBACKGROUND AND OBJECTIVE:Tumor segmentation from hybrid PET/MRI scans may be highly beneficial in radiotherapy treatment planning. Indeed, it gives for both modalities the suitable information that could make the delineation of tumors more accurate than using each one apart. We aim in this work to propose a co-segmentation method that deals with several challenges, notably the lack of one-to-one correspondence between tumors of the two modalities and the boundaries' smoothing.METHODS:The proposed method is designed to surpass these limits, we propose a segmentation method based on the GCsummax technique. The method takes the advantage of Iterative Relative Fuzzy Connectedness (IRFC) on seeds initialization, and the standard min-cut/max-flow technique for the boundary smoothing. Seed initialization was accurately performed thanks to high uptake regions on PET. Besides, a visibility weighting scheme was adapted to achieve the task of co-segmentation using the IRFC algorithm. Then, given the co-segmented regions, we introduce a morphological-based technique that provides object seeds to standard Graph Cut (GC) allowing it to avoid the shrinking problem. Finally, for each modality, the segmentation task is formulated as an energy minimization problem which is resolved by a min-cut/max-flow technique.RESULTS:The overlap ratio (denoted DSC) between our segmentation results and the ground-truth for PET images is 92.63  ±  1.03, while the DSC for MRI images is 90.61  ±  3.70.CONCLUSIONS:The proposed method was tested on different types of diseases and it outperformed the state-of-the-art methods. We show its superiority in terms of assymetric relation between PET and MRI and tumors heterogeneity

Efficacy of the MEK inhibitor cobimetinib for wild-type BRAF Erdheim-Chester disease.

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Quantitative simultaneous (99m)Tc-ECD/123I-FP-CIT SPECT in Parkinson's disease and multiple system atrophy.

PURPOSE: The purpose of this study was to investigate the feasibility and utility of dual-isotope SPECT for differential diagnosis of idiopathic Parkinson's disease (IPD) and multiple system atrophy (MSA). METHODS: Simultaneous (99m)Tc-ECD/123I-FP-CIT studies were performed in nine normal controls, five IPD patients, and five MSA patients. Projections were corrected for scatter, cross-talk, and high-energy penetration, and iteratively reconstructed while correcting for patient-specific attenuation and variable collimator response. Perfusion and dopamine transporter (DAT) function were assessed using voxel-based statistical parametric mapping (SPM2) and volume of interest quantitation. DAT binding potential (BP) and asymmetry index (AI) were estimated in the putamen and caudate nucleus. RESULTS: Striatal BP was lower in IPD (55%) and MSA (23%) compared to normal controls (p<0.01) , and in IPD compared to MSA (p<0.05). AI was greater for IPD than for MSA and controls in both the caudate nucleus and the putamen (p<0.05). There was significantly decreased perfusion in the left and right nucleus lentiformis in MSA compared to IPD and controls (p<0.05). CONCLUSION: Dual-isotope studies are both feasible in and promising for the diagnosis of parkinsonian syndromes

Carte 15. Salle Hillaire (partie Nord)

International audienceThis first volume of Monographie de la grotte Chauvet-Pont d’Arc  presents a reasoned inventory of the information contained in the cave in the form of an atlas. While this type of publication may seem unusual, the dimensions of the cave, the morphology of the chambers and galleries that compart-mentalize its space, as well as the inscription of artworks in its underground landscape, are all invitations to reflect upon, study and structure our knowledge of the cave using a cartographic approach. The progressive presentation of the Atlas de la grotte Chauvet-Pont d’Arc enables us to address, in the most neutral manner possible, the complexity and factual richness, often emphasized, of this underground sanctuary.The proposed itinerary, close to that of the current path of the walkways from the entrance to the end of the cavity, invites us to discover the cave via twenty maps extracted from the integrated map of the cave floors. Moving through the cave and into its smallest and most intimate spaces, each person can shape their reading according to their interests. The visit to each of the twenty zones is organized according to the same narrative structure. The first page, accompa-nied by a panoramic photograph, presents the main charac-teristics of the zone and the themes that will be addressed. Depending on the sectors, the archaeology, paleontology, geology, speleogenesis or underground landscape is empha-sized. The second and third pages are positioned opposite each other and are designed to interact. The second page presents the integrated map reproduced at the 1:100 scale. Below it, a thumbnail representation enables the reader to identify the location of the map within the cave. A small map at the 1:300 scale presents the cave floor in 3D and shows the location, feature and artwork designations, the permanent and temporary paths installed for observations, as well as the camera angles and the position of the longitudinal profile of the zone. A synoptic figure shows the relative representation of each of the research domains in the map zone. The pages that follow provide an initial synthesis of the observations and research in progress. These aspects will be presented in detail in the succeeding volumes of the Atlas de la grotte Chauvet-Pont d’Arc

Lung Involvement in Destombes-Rosai-Dorfman Disease: Clinical and Radiological Features and Response to the MEK Inhibitor Cobimetinib

International audienceBackground: Destombes-Rosai-Dorfman disease (RDD) is a rare multisystemic histiocytosis. Pulmonary involvement during RDD has been poorly described. The goal of this study was to examine the clinical presentations, radiological features, and outcomes of 15 patients with RDD and lung involvement. Methods: The cases of RDD with lung involvement were extracted from the French National Histiocytosis registry. Efficacy of the MEK inhibitor cobimetinib in treating lung disease was evaluated with an 18fluorodeoxyglucose PET scanner and chest CT scans. Results: Fifteen patients (six women; median age, 40 years at RDD diagnosis) were included. All patients had evidence of systemic disease with extrapulmonary localizations of the disease (lymphadenopathy [n = 12], skin [n = 9], bones [n = 6], retroperitoneal involvement [n = 3], sinuses [n = 3], parotid gland [n = 2], submandibular gland [n = 1], and breast [n = 1]). Presenting symptoms were dominated by dyspnea and dry cough in seven patients. Restrictive physiology was observed in two of five patients. BAL showed lymphocytosis in one of five cases. Eight patients received corticosteroids, all but one with variable immunosuppressive or immunomodulatory therapies. Two patients received cobimetinib for severe lung disease, with dramatic pulmonary metabolic and tumoral responses. Two patients died during follow-up: one of hemoptysis, and the other of an unrelated cerebral tumor. Conclusions: Pulmonary involvement in RDD is rare, proteiform, and sometimes severe. The MEK inhibitor cobimetinib can lead to dramatic responses

Low regional diversity of late cave bears mitochondrial DNA at the time of Chauvet Aurignacian paintings

The Chauvet-Pont d\u27Arc and Deux-Ouvertures caves, located along the Ardèche River (France), contain abundant remains of the extinct cave bear (Ursus spelaeus). Because they also display a variety of Palaeolithic anthropogenic evidences, such as the earliest charcoal drawings recorded to date (Chauvet-Pont d\u27Arc), and delicate engravings (Deux-Ouvertures), they offer the opportunity of studying the interaction between animals and human beings during a key period for Pleistocene species extinctions. We characterized cave bear specimens from these two sites by radiocarbon dating, stable isotopes, and mitochondrial DNA analysis. In Chauvet-Pont d\u27Arc, we obtained radiocarbon ages that ranged between 29,000 and 37,300 years before present (BP). The Deux-Ouvertures cave bear specimens clustered to the bottom of this time frame, returning radiocarbon ages of 27,440–30,220 years BP. Cave bear nitrogen isotope values were all compatible with a vegetarian diet. Mitochondrial DNA analysis, carried out on a highly variable domain of the control region, evidenced only two cave bear haplotypes, including a new haplotype, and a common one which largely predominated. We detected both haplotypes in Chauvet-Pont d\u27Arc, but only recorded the predominant one in the Deux-Ouvertures Cave. Our data put forward the surprising observation that cave bears inhabited Ardèche over a short period of time, from about 37,000 to 27,400 years BP. They were notably present during the first (Aurignacian) phase of human intrusions in Chauvet-Pont d\u27Arc, 30,000–32,000 years BP. This points to the possible competition for cave sites, presumably on a seasonal scale considering the cave bear habit for hibernation. During this time period, the small number of haplotypes is at variance with the extensive genetic diversity reported elsewhere for much more ancient specimens

Marked efficacy of vemurafenib in suprasellar Erdheim-Chester disease.

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