Objective: Our primary goal was to measure the accuracy of fully automated absence seizure detection, using a wearable electroencephalographic (EEG) device.As a secondary goal, we also tested the feasibility of automated behavioral testing triggered by the automated detection. Methods:We conducted a phase 3 clinical trial (NCT04615442), with a prospective, multicenter, blinded study design. The input was the one-channel EEG recorded with dry electrodes embedded into a wearable headband device connected to a smartphone. The seizure detection algorithm was developed using artificial intelligence (convolutional neural networks). During the study, the predefined algorithm, with predefined cutoff value, analyzed the EEG in real time. The gold standard was derived from expert evaluation of simultaneously recorded fullarray video-EEGs. In addition, we evaluated the patients' responsiveness to the automated alarms on the smartphone, and we compared it with the behavioral changes observed in the clinical video-EEGs. Results:We recorded 102 consecutive patients (57 female, median age = 10 years) on suspicion of absence seizures. We recorded 364 absence seizures in 39 patients. Device deficiency was 4.67%, with a total recording time of 309 h. Average sensitivity per patient was 78.83% (95% confidence interval [CI] = 69.56%-88.11%), and median sensitivity was 92.90% (interquartile range [IQR] = 66.7%-100%). The average false detection rate was .53/h (95% CI = .32-.74). Most patients (n = 66, 64.71%) did not have any false alarms. The median F1 score per patient was .823 (IQR = .57-1). For the total recording duration, F1 score was .74.We assessed the feasibility of automated behavioral testing in 36 seizures; it correctly documented nonresponsiveness in 30 absence seizures, and responsiveness in six electrographic seizures.

Objectives -Since 2005 a New Wilson Index (NWI) ≥ 11 is used as a predictor of death without transplantation in fulminant Wilson's disease (WD). Plasma exchange is advocated as a new treatment modality.Methods -We present a patient with fulminant WD treated with plasma exchange. All published cases applying plasma exchange for fulminant WD were reviewed systematically.Results -A 14-year-old girl presented with hemolysis and fulminant liver failure. She had no encephalopathy; NWI was 14. As a bridge to transplantation plasma exchange was started immediately. Complete remission was achieved with plasma exchange and later chelation therapy with D-penicillamine. She is now at 3-year transplant free survival. Literature review identified 37 patients presenting with fulminant WD and NWI ≥ 11 who were treated with plasma exchange. Seventeen of these patients (i.e. 46%) recovered without transplantation.Conclusions -Multiple case reports and case series demonstrate transplant free survival after plasma exchange and subsequent chelation therapy, despite a NWI ≥ 11. Plasma exchange affects the clinical course and is a therapeutic option in children and young adults presenting with fulminant WD.

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Background During ambulatory follow-up of patients with cerebral palsy (CP) systematic radiographic screening is required firstly to evaluate hip migration and development in the prevention of hip dislocation and secondly to analyse lower limb alignment and leg length. The Migration Percentage (MP) is a radiographic measurement used to describe the extent of femoral head lateralisation on conventional supine pelvic radiographs. Our goal was to assess the comparability of the MP measured on low radiation dose EOS® standing full-leg radiographs with that of conventional supine pelvic radiographs. Methods Patients presenting with CP were prospectively selected from our outpatient follow-up consultation at our institutions CP reference centre and underwent conventional supine pelvic and EOS® standing full-leg radiographs the same day for diagnostic and screening reasons. Results Out of 28 prospectively selected patients we included 21 (42 hips), of which 10 were female, with a mean age of 9.25 years and GMFCS levels of I, II and III. Seven out of 28 patients were excluded due to insufficient quality of radiographic images. The absolute differences in MP measured on both conventional supine pelvic and EOS® standing full-leg radiographs ranged between − 8 and 6% with an absolute mean difference of 0% (SD ±3.5) and were not statistically significant ( p = 0.99). A Bland-Altman plot showed acceptable agreement between both measurements without proportional bias. Conclusion There is no statistical significant difference between the Migration Percentage measured on conventional supine pelvic radiographs and EOS® standing full-leg radiographs in ambulant patients. These images use lower radiation doses and contain more radiographic information. Trial registration Approved by the Medical Research Ethics committee of the University Hospitals Leuven ( MP001492 ).