Peripheral and central nervous system involvement in a patient with primary Sjögren's syndrome: a case report.

BACKGROUND: Primary Sjögren's syndrome is the second most common rheumatological disorder after rheumatoid arthritis. It typically presents as xerophthalmia and xerostomia in postmenopausal women. Involvement of the central nervous system has been recognized, although its pathogenesis and characteristics are poorly understood. Central nervous system complications are a diagnostic challenge and emphasize the need for systematic screening of patients with new peripheral and central neurological symptoms. CASE REPORT: We report a case of a 58-year-old Swiss woman presenting with rapidly progressive sensorimotor distal polyneuropathy together with new-onset generalized seizures. Initial magnetic resonance imaging (MRI) of the brain performed after the first seizure showed multiple, bihemispheric, confluent white matter hyperintensities with contrast enhancement. Follow-up imaging 3 days after the initial magnetic resonance imaging demonstrated a fulminant disease progression associated with the serious clinical deterioration of the patient. In light of the results of a minor salivary gland biopsy, autoantibody testing, nerve conduction studies, and cranial magnetic resonance imaging, primary Sjögren's syndrome with cryoglobulinemia type II was diagnosed. Response to plasmapheresis and subsequent administration of cyclophosphamide was favorable. CONCLUSION: Even though exocrinopathy is the hallmark of Sjögren's syndrome, systemic symptoms are observed in one-third of patients. There is an urgent need to better characterize the mechanisms underlying different disease phenotypes and to perform randomized controlled trials in order to provide tailored and evidence-based treatment for primary Sjögren's syndrome.

Radiosurgery of vestibular schwannoma: prognostic factors for hearing outcome using 3D-constructive interference in steady state (3D-CISS).

PURPOSE: Stereotactic radiosurgery (SRS) is an effective treatment for vestibular schwannoma (VS). Three-dimensional (3D) constructive interference in steady state (CISS) is the preferred magnetic resonance imaging (MRI) sequence for evaluating signal changes in the inner ear endolymph. Previous studies demonstrated a correlation between pretreatment cochlear signal intensity in 3D-CISS and posttherapeutic hearing outcomes. The purpose of our study was to compare 3D-CISS sequences before and after primary SRS of unilateral VSs to evaluate the effect of radiosurgery on the 3D-CISS signal intensities of cochlea and sacculus/utriculus. METHODS: We retrospectively reviewed 47 patients with unilateral VS treated with SRS. The neuroradiological MRI datasets were analysed to evaluate the signal intensity of the inner ear structure, tumour size, Koos grade, tumour volume, and infiltration of the cochlear aperture before therapy and at follow-up. The differences in these signal intensities before SRS and at follow-up were correlated with clinical symptoms, cochlear radiation dose, tumour volume and infiltration of the cochlear aperture. RESULTS: No differences were found between signal intensities in cochlea and utriculus/sacculus before and after SRS and no correlation with clinical symptoms, cochlear radiation dose, tumour volume, Koos grade or infiltration of the cochlear aperture (all p > 0.05). CONCLUSION: Our study supports the theory of a complex interaction causing alteration of the endolymph protein concentration and not a direct dependency on the SRS. Use of modern dosing schemes will have a positive impact on clinical outcome with preservation of hearing in patients with VS.

Diverging lesion and connectivity patterns influence early and late swallowing recovery after hemispheric stroke.

Knowledge about the recovery of oral intake after hemispheric stroke is important to guide therapeutic decisions, including the administration of enteral tube feeding and the choice of the appropriate feeding route. They aimed to determine the localization and connectivity of lesions in impaired recovery versus recovered swallowing after initially dysphagic stroke. Sixty-two acute ischemic hemispheric stroke patients with impaired oral intake were included in a prospective observational cohort study. Voxel-based lesion-symptom mapping and probabilistic tractography were used to determine the association of lesion location and connectivity with impaired recovery of oral intake ≥7 days (indication for early tube feeding) and ≥4 weeks (indication for percutaneous endoscopic gastrostomy feeding) after stroke. Two distinct patterns influencing recovery of swallowing were recognized. Firstly, impaired recovery of oral intake after ≥7 days was significantly associated with lesions of the superior corona radiata (65% of statistical map, P < 0.05). The affected fibers were connected with the thalamus, primary motor, and supplemental motor areas and the basal ganglia. Secondly, impaired recovery of oral intake after ≥4 weeks significantly correlated with lesions of the anterior insula (54% of statistical map, P < 0.05), which was connected to adjacent operculo-insular areas of deglutition. These findings indicate that early swallowing recovery is influenced by white matter lesions disrupting thalamic and corticobulbar projection fibers. Late recovery is determined by specific cortical lesions affecting association fibers. This knowledge may help clinicians to identify patients at risk of prolonged swallowing problems that would benefit from enteral tube feeding. Hum Brain Mapp 38:2165-2176, 2017. © 2017 Wiley Periodicals, Inc.

Characterization of Enhancing MS Lesions by Dynamic Texture Parameter Analysis of Dynamic Susceptibility Perfusion Imaging.

PURPOSE: The purpose of this study was to investigate statistical differences with MR perfusion imaging features that reflect the dynamics of Gadolinium-uptake in MS lesions using dynamic texture parameter analysis (DTPA). METHODS: We investigated 51 MS lesions (25 enhancing, 26 nonenhancing lesions) of 12 patients. Enhancing lesions (n = 25) were prestratified into enhancing lesions with increased permeability (EL+; n = 11) and enhancing lesions with subtle permeability (EL-; n = 14). Histogram-based feature maps were computed from the raw DSC-image time series and the corresponding texture parameters were analyzed during the inflow, outflow, and reperfusion time intervals. RESULTS: Significant differences (p < 0.05) were found between EL+ and EL- and between EL+ and nonenhancing inactive lesions (NEL). Main effects between EL+ versus EL- and EL+ versus NEL were observed during reperfusion (mainly in mean and standard deviation (SD): EL+ versus EL- and EL+ versus NEL), while EL- and NEL differed only in their SD during outflow. CONCLUSION: DTPA allows grading enhancing MS lesions according to their perfusion characteristics. Texture parameters of EL- were similar to NEL, while EL+ differed significantly from EL- and NEL. Dynamic texture analysis may thus be further investigated as noninvasive endogenous marker of lesion formation and restoration.

Effect of Decompressive Craniectomy on Perihematomal Edema in Patients with Intracerebral Hemorrhage.

BACKGROUND: Perihematomal edema contributes to secondary brain injury in the course of intracerebral hemorrhage. The effect of decompressive surgery on perihematomal edema after intracerebral hemorrhage is unknown. This study analyzed the course of PHE in patients who were or were not treated with decompressive craniectomy. METHODS: More than 100 computed tomography images from our published cohort of 25 patients were evaluated retrospectively at two university hospitals in Switzerland. Computed tomography scans covered the time from admission until day 100. Eleven patients were treated by decompressive craniectomy and 14 were treated conservatively. Absolute edema and hematoma volumes were assessed using 3-dimensional volumetric measurements. Relative edema volumes were calculated based on maximal hematoma volume. RESULTS: Absolute perihematomal edema increased from 42.9 ml to 125.6 ml (192.8%) after 21 days in the decompressive craniectomy group, versus 50.4 ml to 67.2 ml (33.3%) in the control group (Δ at day 21 = 58.4 ml, p = 0.031). Peak edema developed on days 25 and 35 in patients with decompressive craniectomy and controls respectively, and it took about 60 days for the edema to decline to baseline in both groups. Eight patients (73%) in the decompressive craniectomy group and 6 patients (43%) in the control group had a good outcome (modified Rankin Scale score 0 to 4) at 6 months (P = 0.23). CONCLUSIONS: Decompressive craniectomy is associated with a significant increase in perihematomal edema compared to patients who have been treated conservatively. Perihematomal edema itself lasts about 60 days if it is not treated, but decompressive craniectomy ameliorates the mass effect exerted by the intracerebral hemorrhage plus the perihematomal edema, as reflected by the reduced midline shift.

A novel method for analyzing DSCE-images with an application to tumor grading.

OBJECTIVES: (a) The development of a novel analysis method, named Dynamic pixel intensity Histogram Analysis (DHA) allowing for pixel intensity-histogram-model-parameter fitting of arbitrary-shaped regions defined in dynamic-susceptibility-contrast-enhanced (DSCE) difference MR-image time-series, and (b) its prospective application and evaluation for glioma grading. MATERIALS AND METHODS: For each difference-image, pixel intensity histograms of arbitrary-shaped ROIs were computed and fitted using the Levenberg-Marquardt algorithm. Time-dependent histogram center-position- and width-parameters are computed during bolus-passage. The method was applied to 25 patients with low and high grade gliomas. RESULTS: During bolus outflow-time, histogram-center-position-parameter and histogram-width-parameter reach highest significance levels and discriminate gliomas of different grades. The histogram center-position-parameter discriminated grade-II from grade-III, grade-II from grade-IV but not grade-III from grade-IV. The observed histogram width-parameters discriminated grade-II from grade-III (P < 0.00022), grade-II from grade-IV (P <8.3 10), and grade-III from grade-IV (P < 0.00063). CONCLUSIONS: DHA is a easy-to-use method for glioma grading; the histogram width parameter is best indicator for histologic grade.

ChEBI: a database and ontology for chemical entities of biological interest.

Chemical Entities of Biological Interest (ChEBI) is a freely available dictionary of molecular entities focused on 'small' chemical compounds. The molecular entities in question are either natural products or synthetic products used to intervene in the processes of living organisms. Genome-encoded macromolecules (nucleic acids, proteins and peptides derived from proteins by cleavage) are not as a rule included in ChEBI. In addition to molecular entities, ChEBI contains groups (parts of molecular entities) and classes of entities. ChEBI includes an ontological classification, whereby the relationships between molecular entities or classes of entities and their parents and/or children are specified. ChEBI is available online at http://www.ebi.ac.uk/chebi/