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In CLN3 disease, impairments in motor function are frequently reported to have later onset compared to visual and cognitive decline, but upper limb motor function has yet to be explored in this population. In a cohort of 22 individuals with CLN3, we used a novel application of multiple measures to (1) characterize motor function, particularly of the upper limbs, in activities of daily living (ADLs), and (2) explore associations between motor function and age as well as visual ability, disease severity, and cognitive function, as evaluated by the Unified Batten Disease Rating Scale (UBDRS), a validated CLN3 disease measure. ADLs that required coordination, speed, and fine motor control were particularly challenging for children with CLN3 based on item-level performance across direct assessments (Jebsen-Taylor Hand Function Test [JTHFT] and MyoSet Tools) and caregiver reports (Pediatric Evaluation of Disability Inventory-Computer Adaptive Testing [PEDI-CAT] and Patient-Reported Outcomes Measurement Information System [PROMIS] Pediatric Upper Extremity). Poorer visual ability, disease severity, and cognitive function were associated with worse performance on these measures, whereas age had limited impact. These findings support the need for children with CLN3 to receive skilled clinical evaluation and treatment tailored to their individual needs, particularly in the context of ADLs, as their symptom profile progresses., (© 2021 Wiley Periodicals LLC.)

We present an optical setup capable of mirroring an arbitrary, potentially time-varying, polarization state of an ultrashort laser pulse. The incident beam is split up in two and the polarization of one beam is mirrored by reflection off a mirror in normal incidence. Afterwards, both beams are recombined in time and space such that two collinear ultrashort laser pulses with mutually mirrored polarization, i.e., laser-pulse enantiomers, leave the setup. We employ the Jones formalism to describe the function of the setup and analyze the influence of alignment errors before describing the experimental implementation and alignment protocol. Since no wave plates are utilized, broadband pulses in a large wavelength range can be processed. In particular, we show that the setup outperforms broadband achromatic wave plates. Furthermore, since the two beams travel separately through the optical system they can be blocked independently. This opens the possibility for circular dichroism, ellipsometry, and anisotropy spectroscopy with shot-to-shot chopping and detection schemes as well as chiral coherent control applications.

We theoretically investigate the photon-echo spectroscopy of coupled electron-nuclear quantum dynamics. Two situations are treated. In the first case, the Born-Oppenheimer (adiabatic) approximation holds. It is then possible to interpret the two-dimensional (2D) spectra in terms of vibrational motion taking place in different electronic states. In particular, pure vibrational coherences which are related to oscillations in the time-dependent third-order polarization can be identified. This concept fails in the second case, where strong non-adiabatic coupling leads to the breakdown of the Born-Oppenheimer-approximation. Then, the 2D-spectra reveal a complicated vibronic structure and vibrational coherences cannot be disentangled from the electronic motion.

Smith-Magenis syndrome (SMS), the result of an interstitial deletion within chromosome 17p11.2, is a disorder that may include minor dysmorphic features, brachydactyly, short stature, hypotonia, speech delays, cognitive deficits, signs of peripheral neuropathy, scoliosis, and neurobehavioral problems including sleep disturbances and maladaptive repetitive and self-injurious behaviors. Physical and occupational therapists provide services for children who have the syndrome, whose genetic disorder is frequently not identified or diagnosed before 1 year of age. A comprehensive physical and occupational therapy evaluation was completed in nonidentical twins with one having SMS, using the Sensory Profile; Brief Assessment of Motor Function (BAMF); Peabody Developmental Motor Scales, Second Edition (PDMS-2); and Pediatric Evaluation of Disability Inventory (PEDI). This provides a framework for conducting assessments to enhance early detection and interdisciplinary management with this specialized population.

Lidocaine, Mexiletine, Procainamide, and Phenytoin were administered intravenously to anaesthesized rabbits. BERA alterations showed two different patterns. If the intoxication dose was exceeded, amplitude depression, threshold elevation, desynchronization, and severe cumulative prolongation of all latencies and interpeak latencies appeared. Below this dose Lidocaine and Mexiletine induced a single, reversible, dose related, cumulative prolongation of all latencies and interpeak latencies. Procainamide induced counterrelated shiftings of interpeak latencies I-III and III-V, whereas Phenytoin showed no influence. One should, therefore, take into account these effects when BERA is used clinically, since otherwise serious errors can occur. On the other hand, there are diagnostic and therapeutic aspects for the tinnitus patient.