Citations

AbstractIntroductionBehavior/Comportment/Personality (BEHAV) and Language (LANG) domains were added to the Clinical Dementia Rating (CDR®) for improving evaluation of patients with frontotemporal lobar degeneration (FTLD) (CDR® plus NACC FTLD).MethodsWe analyzed the CDR® plus NACC FTLD among participants from the baseline visit of the Advancing Research and Treatment for Frontotemporal Lobar Degeneration/Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects Consortium.ResultsThe CDR® plus NACC FTLD was able to detect early symptoms in the mildly impaired participants who were rated as CDR® sum of boxes (CDR®‐SB) = 0. The CDR®‐SB was not sensitive, particularly in participants with mild nonfluent/agrammatic primary progressive aphasia. Participants with familial and sporadic behavioral variant FTD exhibited similar CDR® plus NACC FTLD profiles except that language impairment was more frequent in participants with mild sporadic behavioral variant FTD. Adding the BEHAV and/or LANG domains to the CDR®‐SB significantly enhanced discriminatory power in differentiating among the FTLD spectrum disorders.DiscussionThe BEHAV and LANG domains enable the CDR® plus NACC FTLD to capture early symptomatology of FTLD.

Abstract Background The arrangement of the facial soft tissue layers is different with respect to the line of ligaments: medially oblique and laterally in parallel. Aims This split‐face study was designed to investigate the effects on midfacial volumization if the same medial vs lateral injection points are targeted in various sequences. Methods Twelve patients (3 males, 9 females; 46.67 years ± 4.5) were included in this interventional study. On the right side of the face, lateral injection points were performed first, whereas on the left side, medial injection points were executed first. The infraorbital hollowness score, the upper cheek fullness score, the global aesthetic improvement scale, and the injected volume were assessed. Results No side differences were observed after the intervention with P = 1.00 for all scores. When the lateral injection points were performed first, the volume injected into the medially located injection points (0.46 ± 0.26 cc vs 0.73 ± 0.31 cc [ P = .037]), into the lateral injection points (0.79 ± 0.40 cc vs 1.15 ± 0.28 cc [ P = .017]), and overall (1.26 ± 0.64 cc vs 1.88 ± 0.57 cc [ P = .02]) was significantly reduced. Conclusion The results of the present study emphasize the importance of respecting the layered arrangement of the facial soft tissues when performing minimally invasive soft tissue filler injections. Targeting injection points lateral to the line of ligaments first reduces the volume needed to symmetrically and aesthetically appealing manner and volumizes the infraorbital and upper cheek regions.

Abstract NKAP and HDAC3 are critical for T cell maturation. NKAP and HDAC3 physically associate, and a point mutation in NKAP, NKAP(Y352A), abrogates this interaction. To evaluate the significance of NKAP and HDAC3 association in T cell maturation, transgenic mice were engineered for cre-mediated endogenous NKAP gene deletion coupled to induction of NKAP(Y352A) or a wild type (WT) control transgene, NKAP(WT), in double positive thymocytes or regulatory T cells (Tregs). T cell maturation was normal in mice with endogenous NKAP deletion coupled to NKAP(WT) induction. However, severe defects occurred in T cell and Treg maturation and in iNKT cell development when NKAP(Y352A) was induced, recapitulating NKAP deficiency. Conventional T cells expressing NKAP(Y352A) failed to enter the long-term T cell pool, did not produce cytokines, and remained complement susceptible, whereas Tregs expressing NKAP(Y352A) were eliminated as recent thymic emigrants leading to lethal autoimmunity. Overall, these results demonstrate the significance of NKAP–HDAC3 association in T cells.

Abstract Introduction The aim of this study was to investigate the rates of lobar atrophy in the asymptomatic microtubule‐associated protein tau ( MAPT ) mutation carriers. Methods MAPT mutation carriers (n = 14; 10 asymptomatic, 4 converters from asymptomatic to symptomatic) and noncarriers (n = 13) underwent structural magnetic resonance imaging and were followed annually with a median of 9.2 years. Longitudinal changes in lobar atrophy were analyzed using the tensor‐based morphometry with symmetric normalization algorithm. Results The rate of temporal lobe atrophy in asymptomatic MAPT mutation carriers was faster than that in noncarriers. Although the greatest rate of atrophy was observed in the temporal lobe in converters, they also had increased atrophy rates in the frontal and parietal lobes compared to noncarriers. Discussion Accelerated decline in temporal lobe volume occurs in asymptomatic MAPT mutation carriers followed by the frontal and parietal lobe in those who have become symptomatic. The findings have implications for monitoring the progression of neurodegeneration during clinical trials in asymptomatic MAPT mutation carriers.