BACKGROUND Patients with inflammatory bowel disease (IBD) have intestinal inflammation and are treated with immune-modulating medications. In the face of the coronavirus disease-19 pandemic, we do not know whether patients with IBD will be more susceptible to infection or disease. We hypothesized that the viral entry molecules angiotensin I converting enzyme 2 (ACE2) and transmembrane serine protease 2 (TMPRSS2) are expressed in the intestine. We further hypothesized that their expression could be affected by inflammation or medication usage. METHODS We examined the expression of Ace2 and Tmprss2 by quantitative polymerase chain reacion in animal models of IBD. Publicly available data from organoids and mucosal biopsies from patients with IBD were examined for expression of ACE2 and TMPRSS2. We conducted RNA sequencing for CD11b-enriched cells and peripheral and lamina propria T-cells from well-annotated patient samples. RESULTS The molecules ACE2 and TMPRSS2 were abundantly expressed in the ileum and colon and had high expression in intestinal epithelial cells. In animal models, inflammation led to downregulation of epithelial Ace2. Expression of ACE2 and TMPRSS2 was not increased in samples from patients with compared with those of control patients. In CD11b-enriched cells but not T-cells, the level of expression of ACE2 and TMPRSS2 in the mucosa was comparable to other functional mucosal genes and was not affected by inflammation. Anti-tumor necrosis factor drugs, vedolizumab, ustekinumab, and steroids were linked to significantly lower expression of ACE2 in CD11b-enriched cells. CONCLUSIONS The viral entry molecules ACE2 and TMPRSS2 are expressed in the ileum and colon. Patients with IBD do not have higher expression during inflammation; medical therapy is associated with lower levels of ACE2. These data provide reassurance for patients with IBD. © 2020 Crohn’s & Colitis Foundation. Published by Oxford University Press. All rights reserved. For permissions, please e-mail journals.permissions@oup.com.BACKGROUND AND OBJECTIVES While older adults’ social participation has attracted sustained attention, the realities faced by seniors with impairments in this area are still understudied. Social representations are formed from socially constructed opinions regarding a given object and have four functions (knowledge, identity, orientation, justification). The purpose of this article is to document the social representations that exist within seniors’ organizations regarding participation by older people with impairments. RESEARCH DESIGN AND METHODS In this mixed-methods study, an exploratory sequential design was applied. First, a qualitative phase involved meetings with five focus groups to explore how participants would manage difficult situations in which the inclusion of an older person with impairments must be addressed. Second, a quantitative phase consisted of a questionnaire administered to 86 respondents to examine the components of social representations about the participation of older people with impairments. RESULTS Results show that, although research participants acknowledge that all seniors have the right to participate, this right confronts a collective identity infused with aging model that demands a youthful, „un-disabled” appearance and activities. In terms of the orientation function, making participation a reality is seen as relying on the efforts of people with impairments, but when there is a failure of inclusiveness, the reasons offered to justify it concern the resources available, rather than possible prejudices. DISCUSSION AND IMPLICATIONS Interpretation of the results leads to recommendations for both implementing an inclusion philosophy and practices in seniors’ organizations and revisiting the view of impairment in old age as otherness. © The Author(s) 2020. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail journals.permissions@oup.com.STUDY OBJECTIVES Sleep deprivation and circadian disruptions impair brain function and cognitive performance, but few studies have examined the effect of sleep inconsistency. Here, we investigated how inconsistent sleep duration and sleep timing between weekends (WE) and weekdays (WD) correlated with changes in behavior and brain function during task and at rest in 56 (30 female) healthy human participants. METHODS WE-WD differences in sleep duration and sleep midpoint were calculated using 1-week actigraphy data. All participants underwent 3 Tesla blood-oxygen-level-dependent functional Magnetic Resonance Imaging (fMRI) to measure brain activity during a visual attention task (VAT) and in resting-state condition. RESULTS We found that WE-WD inconsistency of sleep duration and sleep midpoint were uncorrelated with each other (r = .08, p = .58) and influenced behavior and brain function differently. Our healthy participants showed relatively small WE-WD differences (WE-WD 0.59 hours). Longer WE sleep duration (relative to WD sleep duration) was associated with better attentional performance (3-ball β = .30, t = 2.35, p = .023; 4-ball β = .30, t = 2.21, p = .032) and greater deactivation of the default mode network (DMN) during VAT (p less then .05, cluster-corrected) and greater resting-state functional connectivity (RSFC) between anterior DMN and occipital cortex (p less then .01, cluster-corrected). In contrast, later WE sleep timing (relative to WD sleep timing) (WE-WD 1.11 hours) was associated with worse performance (4-ball β = -.33, t = -2.42, p = .020) and with lower occipital activation during VAT and with lower RSFC within the DMN. CONCLUSIONS Our results document the importance of consistent sleep timing for brain function in particular of the DMN and provide evidence of the benefits of WE catch-up sleep in healthy adults. Published by Oxford University Press on behalf of Sleep Research Society (SRS) 2020.BACKGROUND The pathological features of Parkinson disease (PD) include motor deficits, glial cell activation, and neuroinflammation. The neurotoxin, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), has an oxidation product, 1-methyl-4-phenylpyridinium ion (MPP+). This study aimed to investigate the effects of 2-aminoquinoline on motor deficits in a mouse model of MPTP-induced PD and cultured mouse astrocytes treated with MPP+, to determine the effects on astrocyte proliferation and apoptosis. MATERIAL AND METHODS Motor deficits in the mouse model of MPTP-induced PD were investigated using the climbing time, suspension time, and swim time tests. Cultured mouse astrocytes were treated with MPP+, and mice with MPTP-induced PD were treated with increasing doses of 2-aminoquinoline. The MTT assay was used to measure astrocyte viability. Astrocyte apoptosis was assessed by confocal fluorescence microscopy using Annexin‑V and fluorescein isothiocyanate (FITC) staining. Western blot measured the levels of Bax, p‑JNK, Bcl‑2, and caspase‑3. RESULTS In the mouse model of MPTP-induced PD, motor deficit tests showed that 2-aminoquinoline reduced the impaired motor function during the climbing time, the suspension time, and the swim time tests in a dose-dependent manner. Pre-treatment with 2-aminoquinoline significantly reduced the proliferation and apoptosis of astrocytes induced by MPP+ in vitro, in a dose-dependent manner (P less then 0.05). The levels of p‑JNK and cleaved caspase‑3 levels were significantly reduced in astrocytes treated with MPP+ following pre-treatment with 2-aminoquinoline, which also reversed the increase in the Bax/Bcl‑2 ratio. CONCLUSIONS In the mouse model of MPTP-induced PD, 2-aminoquinoline reduced motor deficiencies, inhibited MPP+ activated astrocyte apoptosis, and regulated the Bax/Bcl-2 ratio by targeting p-JNK.Carey-Fineman-Ziter syndrome is a congenital myopathy associated with mutations in the MYMK gene. It is clinically defined by the combination of hypotonia, Moebius-Robin sequence, facial anomalies and motor delay. Historically it was considered a brainstem dysgenesis syndrome. We provide detailed information of a Spanish boy with compound heterozygous variants in MYMK gene. A muscle biopsy performed as a toddler only disclosed minimal changes, but muscle MRI showed severe fatty infiltration of gluteus muscles and to a lesser extent in adductors magnus, sartorius and soleus muscles. Clinical course is fairly static, but the identification of new well characterized genetic cases will help to delineate the complete phenotype.BACKGROUND Infantile hereditary proximal spinal muscular atrophy (SMA) type 1 is characterized by onset in the first 6 months of life and severe and progressive muscle weakness. Dysphagia is a common complication but has not been studied in detail. OBJECTIVE To study feeding and swallowing problems in infants with SMA type 1, and to explore the relation between these problems and functional motor scores. METHODS We prospectively included 16 infants with SMA type 1 between September 2016 and October 2018. Eleven infants received palliative care and five infants best supportive care in combination with nusinersen. We compiled and used an observation list with feeding related issues and observed feeding sessions during inpatient and outpatient visits. The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) was used as a measure of motor function. RESULTS All infants in the palliative care group (median onset of disease 14 days (range 1-56); median inclusion in the study 52 datype 1 after the start of nusinersen. Improvement of motor function does not imply similar gains in bulbar function.BACKGROUND The impact of nusinersen therapy on outcomes in adults with Spinal Muscular Atrophy (SMA) remains uncertain. OBJECTIVE To demonstrate whether nusinersen therapy, at currently prescribed doses, can stabilize or improve motor function in adults with SMA using existing outcome measures. METHODS A single-center prospective cohort study of 6 adults with SMA type 3, with inclusion/exclusion criteria intended to optimize the ability to demonstrate change using established outcome measures. Primary outcomes were the Hammersmith Functional Motor Scale-Expanded (HFMSE) and the Revised Upper Limb Measure (RULM). Secondary outcomes were the PedsQL Fatigue scale, the SMA Functional Rating Scale (SMAFRS), and the 6-minute and 10-meter walk tests (6 MWT and 10 MWT). Estimates of change in HFMSE and RULM mean scores across visits were calculated using a linear mixed effects model. Change from baseline was used for other outcome measures. RESULTS HFMSE and RULM scores over 12 months were stable or improved in all participants, with a mean increase of 2 points in each. Other measures showed high intra-individual variability. Adverse events related to the primary diagnosis, including injury and infection, significantly impacted the ability to reliably perform walk tests in the four ambulatory participants. CONCLUSIONS HFMSE and RULM show potential as responsive outcome measures of motor function in ambulatory and non-ambulatory adults with SMA type 3. A time-dependent accrual of benefit of nusinersen on motor function was apparent in this cohort. More sensitive alternative measures of quality of life, fatigue, exercise tolerance, stability and ADLs are clearly needed for adults with SMA.