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Objective: Objective evaluation of the efficacy of tongue lip adhesion (TLA) in the management of Robin sequence (RS)., Study Design: Retrospective cohort study., Setting: Tertiary referral hospital., Patients, Participants: The craniofacial database of Amsterdam UMC, Vrije Universiteit Amsterdam was searched to identify infants with RS who underwent tong lip adhesion (TLA). Forty-one RS infants who underwent TLA from 1993 to 2016 were identified., Interventions: TLA., Main Outcome Measure: The outcome measures were pre- and postoperative polysomnography results, nutritional status, weight gain, age at operation, hospital stay length, extubation time after TLA, and complications., Results: Forty-one RS patients were included who had TLA at an average age of 26.6 days. In 16 cases a pre- and postoperative polysomnography was performed. In 13 of these cases (81.3%) improvement was observed, in 2 (12.5%) the results were inconclusive, and in 1 (6.3%) no improvement was seen. Patients were extubated after a mean of 2.2 days.The mean hospital stay was 40.2 days. Reintervention was needed in 7 patients because of a wound dehiscence. The mean age of TLA release was 9.7 months. At discharge, 9 (22%) children still needed total nutritional support for persistent feeding difficulties. The average growth from birth to adhesion release was 4.6 kg., Conclusion: This cohort demonstrates that TLA is a successful procedure in children with RS in terms of respiratory, feeding, and growth outcome. Only minor complications were seen in our cohort.

Purpose: The aim of the present study was to evaluate the efficacy of an oral formulation of Δ9-tetrahydrocannabinol (ECP002A) in patients with progressive multiple sclerosis (MS)., Methods: This accelerated proof-of-concept study consisted of 2 phases: a crossover challenge (dose-finding) phase and a 4-week, parallel, randomized, placebo-controlled treatment phase. Twenty-four patients with progressive MS and moderate spasticity were enrolled. During the treatment phase, biomarkers for efficacy and secondary pharmacodynamic effects were measured at baseline and after 2 and 4 weeks of treatment. Serum samples were collected to determine pharmacokinetic properties and perform population modeling. Safety and tolerability profiles were assessed based on adverse events and safety measurements., Findings: Pain was significantly reduced when measured directly after administration of ECP002A in the clinic but not when measured in a daily diary. A similar pattern was observed in subjective muscle spasticity. Other clinical outcomes were not significantly different between active treatment and placebo. Cognitive testing indicated that there was no decline in cognition after 2 or 4 weeks of treatment attributable to ECP002A compared with placebo. Implications This study specifically underlines the added value of thorough investigation of pharmacokinetic and pharmacodynamic associations in the target population. Despite the complex interplay of psychoactive effects and analgesia, the current oral formulation of Δ9-tetrahydrocannabinol may play a role in the treatment of spasticity and pain associated with MS because it was well tolerated and had a stable pharmacokinetic profile., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To investigate the presence of EEG abnormalities in frontotemporal lobar degeneration (FTLD) in comparison with Alzheimer's disease (AD) and non-demented individuals with subjective memory complaints (SMC), using an elaborated visual EEG rating scale; furthermore, to investigate whether assessment of resting-state functional connectivity of the EEG is superior to visual evaluation in distinguishing between FTLD, AD and non-demented controls., Methods: EEGs of 15 patients with FTLD, 20 with AD and 23 individuals with SMC were visually compared using the Grand Total EEG (GTE) score. The synchronization likelihood (SL) as a measure of functional connectivity between different EEG channels was calculated for the 0.5-4Hz, 4-8Hz, 8-10Hz, 10-13Hz, 13-30Hz and 30-45Hz frequency bands. Patients had mild to moderate dementia., Results: In AD, as expected, the GTE revealed significant differences from FTLD and SMC, indicating more EEG slowing and loss of reactivity. Patients with FTLD, however, could not be discriminated from individuals with SMC by the GTE score. Analysis of resting-state functional connectivity showed decreased SL in AD compared to both FTLD and SMC in the lower and higher alpha frequency band and decreased SL in AD compared to SMC in the beta frequency band, whereas no differences between FTLD and AD or SMC were found., Conclusions: In patients with mild to moderate FTLD both the visually rated EEG and EEG measures of resting-state functional connectivity are normal., Significance: Although widespread neuronal degeneration takes place in frontotemporal lobar degeneration, this is not reflected in the EEG during the mild to moderate stages of the disease. An abnormal EEG in a mildly demented subject favours a diagnosis of AD.