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PurposeAutosomal dominant lateral temporal epilepsy (ADLTE) is a genetic focal epilepsy syndrome characterized by focal seizures with dominant auditory symptomatology. We present a case report of an 18-year-old patient with acute onset of seizures associated with epilepsy. Based on the clinical course of the disease and the results of the investigation, the diagnosis of ADLTE with a proven mutation in the RELN gene, which is considered causative, was subsequently confirmed. The aim of this study was to use 3 Tesla (3 T) magnetic resonance imaging (MRI) and advanced neuroimaging methods in a patient with a confirmed diagnosis of ADTLE.Methods3 T MRI brain scan and advanced neuroimaging methods were used in the standard protocols to analyzse voxel-based MRI, cortical thickness, and functional connectivity.ResultsMorphometric MRI analysis (blurred grey-white matter junctions, voxel-based morphometry, and cortical thickness analysis) did not provide any informative results. The functional connectivity analysis revealed higher local synchrony in the patient in the left temporal (middle temporal gyrus), left frontal (supplementary motor area, superior frontal gyrus), and left parietal (gyrus angularis, gyrus supramarginalis) regions and the cingulate (middle cingulate gyrus) as compared to healthy controls.ConclusionsEvidence of multiple areas of functional connectivity supports the theory of epileptogenic networks in ADTLE. Further studies are needed to elucidate this theory.

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BackgroundLaryngeal mask UNIQUE® (LMAU) is supraglottic airway device with good clinical performance and low failure rate. Little is known about the ideal position of the LMAU on the magnetic resonance imaging (MRI) and whether radiological malposition can be associated with clinical performance (audible leak) in children. The primary aim of the study was to evaluate incidence of the radiologic malposition of the LMAU according to size. The secondary outcome was the clinical performance and associated complications (1st attempt success rate, audible leak) in LMAUs in correct position vs. radiologically misplaced LMAUs.MethodsIn prospective observational study, all paediatric patients undergoing MRI of the brain under general anaesthesia with the LMAU were included (1.9.2016–16.5.2017). The radiologically correct position: LMAU in hypopharynx, proximal cuff opposite to the C1 or C2 and distance A (proximal cuff end and aditus laryngis) ≤ distance B (distal cuff end and aditus laryngis). Malposition A: LMAU outside the hypopharynx. Malposition B: proximal cuff outside C1-C2. Malposition C: distance A ≥ distance B. We measured distances on the MRI image. Malposition incidence between LMAU sizes and first attempt success rate in trainees and consultant groups was compared using Fisher exact test, difference in incidence of malpositions using McNemar test and difference in leakage according to radiological position using two-sample binomial test.ResultsOverall 202 paediatric patients were included. The incidence of radiologically defined malposition was 26.2% (n = 53). Laryngeal mask was successfully inserted on the 1st attempt in 91.1% (n = 184) cases. Audible leak was detected in 3.5% (n = 7) patients. The radiologically defined malposition was present in 42.9% (n = 3) cases with audible leak. The rate of associated complications was 1.5% (n = 3): laryngospasm, desaturation, cough. In 4.0% (n = 8) the LMAU was soiled from blood.Higher incidence of radiological malposition was in LMAU 1.0, 1.5 and LMAU 3, 4 compared to LMAU 2 or LMAU 2.5 (p < 0.001).ConclusionMalposition was not associated with impaired clinical performance (audible leak, complications) of the LMAU or the need for alternative airway management.Trial registrationClinicaltrials.gov  (NCT02940652) Registered 18 October 18 2016.