Assessment of disease impact through health-related quality of life measurement in primary progressive aphasia.

Introduction: Measurements of health-related quality of life (HRQoL) are important for capturing disease impact beyond physical health and relative to other diseases but have rarely been assessed in primary progressive aphasia (PPA).

Methods: HRQoL was characterized overall, by sex and subtype in PPA ( = 118) using the Health Utilities Index-2/3 (HUI2/3). Multiple linear regression assessed associations between HRQoL and language severity.

Fourier-transform spectroscopy and global deperturbation treatment of the A1Σu+ and b3Πu states of K2 in the entire bound energy range.

Rotationally resolved Fourier-transform spectra of laser-induced fluorescence A1Σu+∼b3Πu→X1Σg+ of K2 molecules were recorded and analyzed, yielding 4053 term values of the spin-orbit (SO) coupled A ∼ b complex of the 39K2 isotopologue with ∼0.01 cm-1 accuracy. Their compilation with 1739 term values from previously published sources allowed them to cover the energy range [9955, 17 436] cm-1 from the bottom of the lower-lying b3Πu state up to the vicinity of the atomic asymptote 4s2S12 + 4p2P12, with a rotational quantum number J ∈ [0, 149].

A Novel Variant (Lys694del) Presenting With Corticobasal Syndrome in a Family With FTD-ALS Spectrum Diseases: Case Report.

Several variants of the TANK-Binding Kinase 1 () gene have been associated with frontotemporal dementia - amyotrophic lateral sclerosis (FTD-ALS) spectrum diseases. Corticobasal syndrome (CBS) is characterized by asymmetric limb rigidity, dystonia or myoclonus, in association with speech or limb apraxia, cortical sensory deficit, and/or alien limb. It can result from a variety of underlying pathologies and although typically sporadic, it has been occasionally associated with and variants.

Regulating Set-β's Subcellular Localization Toggles Its Function between Inhibiting and Promoting Axon Growth and Regeneration.

The failure of the CNS neurons to regenerate axons after injury or stroke is a major clinical problem. Transcriptional regulators like Set-β are well positioned to regulate intrinsic axon regeneration capacity, which declines developmentally in maturing CNS neurons. Set-β also functions at cellular membranes and its subcellular localization is disrupted in Alzheimer's disease, but many of its biological mechanisms have not been explored in neurons.