European Patent Organisation

Background and ObjectivesFew studies have investigated sleep features in people with alternating hemiplegia of childhood (AHC). In this study, we present a bespoke survey of individuals with AHC to characterize sleep disturbances in this condition.MethodsA cross-sectional survey was disseminated through the UK and Italian AHC family associations, addressing their registered families with at least 1 child or adult with a confirmed diagnosis of AHC by their national reference centers and who had consented to be contacted by email. The entire questionnaire implemented in the online surveys was organized into 3 main sections and included the Pittsburgh Sleep Quality Index (PSQI) scale. This scale ranges from 0 to 21, with higher scores indicating poorer sleep quality. Internal consistency of the questionnaire was assessed using Cronbach alpha statistic.ResultsForty-eight of 54 families with AHC (88.89%) filled out at least 1 item of the AHC-Sleep Questionnaire, and 36 of 54 (66.67%) completed the whole questionnaire. Eighteen of 44 people (40.91%) from our cohort reported having a problem with sleep, which was persistent for 10 of 44 (22.7%). Twenty-five of 44 people (56.82%) reported multiple arousals during a typical night, and for 9 of 25 (36%), these could be very prolonged. For 16 of 40 (40%) and 19 of 40 (47.5%), plegic spells resolved or improved, respectively, during sleep, whereas 5 of 40 (12.5%) reported no effect of sleep on these spells. The mean global PSQI score in 40 people was 5.40 (SD ± 4.69). Fifteen of 40 individuals (37.5%) had a global PSQI score ≥5. PSQI scores were significantly higher in those individuals who completed the questionnaire during summer (n = 6) compared with those who completed them during winter (n = 33) (p = 0.011, Mann-Whitney test). We found a strong correlation between AHC paroxysmal spell burden and PSQI score (Spearman r = 0.39, p < 0.01). The survey demonstrated a high degree of internal consistency, with a Cronbach alpha of 0.88 (95% CI 0.83–0.91).DiscussionWe dissected sleep issues and reported sleep disruption, including elevated global PSQI scores, in a significant proportion of individuals with AHC. The main implication of this study is the importance of conducting comprehensive and regular assessments of sleep in both children and adults with AHC.

The pricing of medical devices, both internationally and locally, is inherently complex. It is influenced by diverse regulatory frameworks, varying market access and reimbursement options, and differing willingness to pay for both clinical and economic benefits. Complexity is further increased by a mix of international and local market players, diverse customer landscapes, varied sales channel structures, and distinct purchasing modes. To help navigate these challenges, this chapter introduces a dedicated pricing framework, paying special attention to the critical issue that every medical device manufacturer faces: how to set and manage prices for a single product portfolio sold internationally in highly diverse markets. Insights are provided on strategic questions as well as on existing practices, processes, and instruments.

Topological insulators (TIs) have drawn considerable attention for next generation high sensitivity, high efficiency devices thanks to their unique metallic surface conductivity via their topologically-protected surface states (TSSs). In this study, we utilized near-field nanoimaging and nanoscale spectroscopy in the Terahertz (THz) frequency range to explore the optical response of the surface layer from Bi2Te3 TI flakes. In all cases, we observe a higher near-field optical response from the TI flakes compared to a Si substrate. This contrast increases for more surface sensitive probing, which indicates the presence of a conductive surface state. In addition, we have performed temperature dependent THz nanoscopy and demonstrate a significantly enhanced near-field contrast for the TI flake at a sample temperature of 8.9 K compared to the Si substrate. This temperature-dependent behavior provides vital insight into the underlying mechanisms behind the observed conductive surface state in TI materials.

During its term in power from 2015 to 2023, the PiS broke with the principles of liberal democracy and the separation of powers and distorted the rule of law in Poland. Restoring the rule of law was the most important goal of the current government under Donald Tusk. Its record is sobering. The PiS has left behind a minefield that is almost impossible to defuse. The illiberal order is proving more resilient than expected. After the 2025 presidential elections, in which PiS candidate Karol Nawrocki emerged as the winner, the situation will not become any easier. Poland offers a lesson for Europe that shows how difficult it is to overcome the legal, institutional, and normative devastation of illiberal rule.