scholarly journals The Influence of Hearing Impairment on Mental Age in Down Syndrome: Preliminary Results

2021 ◽  
Vol 9 ◽  
Author(s):  
Amanda Saksida ◽  
Davide Brotto ◽  
Giulia Pizzamiglio ◽  
Elena Bianco ◽  
Sara Bressan ◽  
...  

The increased life expectancy for patients with Down Syndrome (DS) has elicited the need to improve their quality of life by enhancing functional outcomes and identifying the factors that contribute to their long-term cognitive decline. Although the majority of individuals with DS have issues with hearing impairment (HI) since early childhood, to our knowledge no study has investigated whether HI represents a potential modulator of cognitive decline over time. The present explorative cohort study, albeit very preliminary due to the limited cohort (17 children), highlights the significant relation of a significant HI not only with receptive language abilities, but also with mental age in young patients with DS. Additional studies are required to confirm the link between HI and mental age and to assess the impact of audiological treatment on the enhancement of functional outcomes and of cognitive decline in individuals with DS.

2017 ◽  
Vol 45 (4) ◽  
pp. 476-484 ◽  
Author(s):  
A. M. Deane ◽  
C. L. Hodgson ◽  
P. Young ◽  
L. Little ◽  
V. Singh ◽  
...  

The capacity to measure the impact of an intervention on long-term functional outcomes might be improved if research methodology reflected our clinical approach, which is to individualise goals of care to what is achievable for each patient. The objective of this multicentre inception cohort study was to evaluate the feasibility of rapidly and accurately categorising patients, who were eligible for simulated enrolment into a clinical trial, into unique categories based on premorbid function. Once a patient met eligibility criteria a rapid ‘baseline assessment’ was conducted to categorise patients into one of eight specified groups. A subsequent ‘gold standard’ assessment was made by an independent blinded assessor once patients had recovered sufficiently to allow such an assessment to occur. Accuracy was predefined as agreement in >80% of assessments. One hundred and twenty-two patients received a baseline assessment and 104 (85%) were categorised to a unique category. One hundred and six patients survived to have a gold standard assessment performed, with 100 (94%) assigned to a unique category. Ninety-two patients had both a baseline and gold standard assessment, and these agreed in 65 (71%) patients. It was not feasible to rapidly and accurately categorise patients according to premorbid function.


1996 ◽  
Vol 39 (3) ◽  
pp. 655-660 ◽  
Author(s):  
Julia L. Evans

A performance-based model was employed to investigate the impact of discourse demands on the pattern of morphosyntactic deficits exhibited by children with Specific Language Impairments (SLI). The pattern of grammatical errors varied with respect to discourse demands for children with good receptive language abilities but remained stable and independent of changes in discourse demands for children with both expressive and receptive deficits. These findings suggest distinct deficit profiles for subgroups of children with SLI differing in receptive language abilities that are not evident when syntactic skills are investigated outside the context of ongoing spontaneous discourse.


Author(s):  
Sangjun Yoo ◽  
Min Chul Cho ◽  
Min Soo Choo ◽  
Sung Yong Cho ◽  
Hyeon Jeong ◽  
...  

Introduction: We assessed the effects of preoperative bladder compliance on the long-term functional outcomes, especially focused on postoperative storage symptom changes, after laser prostatectomy. Materials and Methods: From January 2008 to March 2014, 1608 men who underwent laser prostatectomy, including holmium laser enucleation or photo-vaporization of the prostate, were included in the analysis. We divided patients into 3 groups according to bladder compliance on a baseline urodynamic study: < 12.5; 12.5–25.0; ≥25 mL/cm H2O. A multivariable analysis was performed to determine the impact of bladder compliance on long-term functional outcomes after laser prostatectomy. Results: Bladder compliance was less than 12.5 ml/cm H2O in 50 (3.1%), 12.5-25 ml/cm H2O in 232 (14.4%) patients. As bladder compliance decreased, the baseline International Prostate Symptom (I-PSS) total score and storage sub-score were increased; the voiding sub-score remain unchanged. At postoperative 36 months, improvements in the I-PSS total score and storage sub-score were significantly higher in < 12.5 mL/cm H2O group compared to other groups, although those were equivalent at postoperative 1 and 12 months. On the multivariable analysis, decreased bladder compliance < 12.5 mL/cm H2O was significantly associated with superior improvement in storage sub-score at postoperative 36 months, although it was not associated with voiding sub-score. Conclusion: In patients with preoperative bladder compliance < 12.5 mL/cm H2O, storage symptoms could be further improved at 36 months after laser prostatectomy compared to others. Thus, laser prostatectomy could be a considerable treatment option for patients with severely decreased bladder compliance


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 9580-9580
Author(s):  
Bjoern Loeppenberg ◽  
Christian von Bodman ◽  
Marko Brock ◽  
Joachim Noldus ◽  
Jueri Palisaar

9580 Background: Patients who underwent open retropubic radical prostatectomy (ORRP) for prostate-cancer (PCA) have excellent long-term survival. Besides oncologic safety, recovery of continence and erectile function are highly important, as adverse functional outcomes may have a detrimental effect on health-related quality of life (HRQOL). We report the long-term HRQOL of PCA survivors after ORRP using standardized tools. Methods: Men treated between August 2003 and December 2007 with ORRP for localized PCA at a single academic hospital received validated questionnaires (International consultation on incontinence questionnaire (ICIQ), International index of erectile function (IIEF-5), Erection hardness score (EHS), EORTC QLQ-C30) to assess functional outcomes and HRQOL. Results were correlated with the global-health score (GHS) of the EORTC QLQ-C30 to assess the impact of ORRP on HRQOL. Results: In the study period 1936 men underwent ORRP of whom 1156 (59.7%) received a nerve-sparing (NS) procedure. Questionnaire return-rate was 59% (n=1141) comprising the final study cohort. Median follow-up (FU) was 62 months. Mean age at surgery and FU was 63.7±6.2 and 69.2±6.2 years, respectively. Biochemical recurrence (BCR) occurred in 17.5% (n=200/1141) and 2% (n=40/1936) deceased. Mean GHS in the study population was 71.5±20.8. In the ICIQ 28% (n=320) scored 0 indicating complete continence and 9.9% (n=113) scored ≥11 indicating severe incontinence. The corresponding GHS was 78.1±19.5 and 55.4±21.8, respectively. 68.5% (n=782) of patients used no pads and 17.9% (n=204) ≥2 pads. Corresponding GHS scores were 74.9±19.8 and 58.9±20.7. Using the IIEF-5 in men who received NS, 24.1% (n=154) had no erectile dysfunction versus 50% (n=318) using the EHS. Corresponding GHS scores were 82.2±16.3 and 74.7±19.8, respectively. Patients with BCR had a GHS of 66.8±21.8 versus 72.5±20.5 for patients without. Men who achieved the Trifecta and Pentafecta criteria had a GHS of 83.1±15.1 and 83.3±15, respectively. Conclusions: Incontinence severely impacts the HRQOL of long-term survivors after ORRP while erectile dysfunction and BCR have a lesser effect. Every effort should be undertaken to maintain functional integrity.


Hypertension ◽  
2020 ◽  
Vol 76 (Suppl_1) ◽  
Author(s):  
Michael E Ernst ◽  
Joanne Ryan ◽  
Enayet K Chowdhury ◽  
Anne M Murray ◽  
Robyn L Woods ◽  
...  

Greater blood pressure variability (BPV) in midlife increases the risk of dementia, but the impact of BPV in cognitively intact older adults is unknown. We examined the risk of incident dementia and cognitive decline associated with long-term, visit-to-visit BPV in participants of the ASPirin in Reducing Events in the Elderly (ASPREE) study, a randomized primary prevention trial of daily low-dose aspirin in community-dwelling adults in Australia and the US aged 70 and older (65 if US minority), who were free of dementia or evidence of cognitive impairment at enrollment. The mean of three BPs using an automated cuff was recorded at baseline and annually; participants also underwent baseline and biennial standardized assessments of global cognition, delayed episodic memory, verbal fluency, processing speed and attention. Cognitive decline was pre-specified as a >1.5 standard deviation (SD) decline in score from baseline on any of the cognitive tests, while incident dementia was a pre-specified secondary endpoint of ASPREE which was adjudicated using DSM-IV criteria. BPV was estimated using within-individual SD of mean systolic BP across baseline and the first two annual visits, and participants with cognitive decline or incident dementia during this period were excluded from the analysis to avoid immortal time bias. After adjustment for key covariates, Cox proportional hazards regression revealed increased risks for dementia and cognitive decline during follow-up for individuals in the highest SD tertile of BPV (Table). Our findings suggest that high BPV in older ages should be considered a potential therapeutic target to preserve cognitive function.


2021 ◽  
Vol 11 (5) ◽  
pp. 551
Author(s):  
Yingying Yang ◽  
Zachary M. Himmelberger ◽  
Trent Robinson ◽  
Megan Davis ◽  
Frances Conners ◽  
...  

Although memory functions in people with Down Syndrome (DS) have been studied extensively, how well people with DS remember things about everyday life is not well understood. In the current study, 31 adolescents/young adults with DS and 26 with intellectual disabilities (ID) of mixed etiology (not DS) participated. They completed an everyday memory questionnaire about personal facts and recent events (e.g., school name, breakfast). They also completed a standard laboratory task of verbal long-term memory (LTM) where they recalled a list of unrelated words over trials. Results did not indicate impaired everyday memory, but impaired verbal LTM, in people with DS relative to people with mixed ID. Furthermore, the laboratory verbal LTM task predicted everyday memory for both groups after taking into account mental age equivalent. Our research showed both an independence and a connection between everyday memory and the standard laboratory memory task and has important research and clinical implications.


2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 474-474
Author(s):  
Alison Huang ◽  
George Rebok ◽  
Bonnielin Swenor ◽  
Jayant Pinto ◽  
Linda Waite ◽  
...  

Abstract Hearing and vision impairment have been independently linked to accelerated cognitive decline in older adults, however there is limited evidence on the effect of dual sensory impairment (DSI) (both hearing and vision impairment) on cognition. Additionally, the impact of social isolation and loneliness, both correlates of DSI and independent risk factors for cognitive decline, on the DSI-cognition relationship has yet to be studied. Using data from the National Social Life, Health, and Aging Project (N=3,091), multivariable linear regression models were used to describe the cross-sectional relationship between self-reported functional sensory impairment (none, hearing only, vision only, DSI) and cognitive function, measured by the survey adapted Montreal Cognitive Assessment. We also included an interaction term in the model to investigate whether cognition is worse among older adults with sensory impairment who also are socially isolated or lonely. Participants in this sample are between 62-91 years with 15% reporting hearing impairment, 11% reporting vision impairment, and 7% reporting DSI. DSI was associated with significantly lower global cognitive function compared to no sensory impairment (-0.31 standard deviations (SD), 95% CI:-0.44 to-0.18), hearing impairment alone (-0.29 SD, 95% CI: -0.44 to -0.15), and vision impairment alone (-0.22 SD, 95% CI: -0.39 to -0.06). Furthermore, cognitive function was significantly worse among older adults with both DSI and smaller social networks (p-interaction &lt;0.05). No differences in the DSI-cognition relationship were observed by level of loneliness. These findings add to the limited research on the relationship between DSI, social isolation and loneliness, and cognition.


2021 ◽  
Author(s):  
Veronique Brault ◽  
Thu Lan Nguyen ◽  
Javier Flores-Gutierrez ◽  
Marie-Christine Birling ◽  
Valerie Lalanne ◽  
...  

Perturbation of the excitation/inhibition (E/I) balance leads to neurodevelopmental diseases including to autism spectrum disorders, intellectual disability, and epilepsy. Mutation in the DYRK1A gene located on human chromosome 21 (Hsa21) leads to an intellectual disability syndrome associated with microcephaly, epilepsy, and autistic troubles (MRD7). Overexpression of DYRK1A, on the other hand, has been linked with learning and memory defects observed in people with Down syndrome (DS). Dyrk1a is expressed in both glutamatergic and GABAergic neurons, but its impact on each neuronal population has not yet been elucidated. Here we investigated the impact of Dyrk1a gene copy number variation in glutamatergic neurons using a conditional knockout allele of Dyrk1a crossed with the Tg(Camk2-Cre)4Gsc transgenic mouse. We explored this genetic modification in homozygotes, heterozygotes and combined with the Dp(16Lipi-Zbtb21)1Yey trisomic mouse model to unravel the consequence of Dyrk1a dosage from 0 to 3, to understand its role in normal physiology, and in MRD7 and DS. Overall, Dyrk1a dosage in glutamatergic neurons did not impact locomotor activity, working memory or epileptic susceptibility, but revealed that Dyrk1a is involved in long-term explicit memory. Molecular analyses pointed at a deregulation of transcriptional activity through immediate early genes and a role of DYRK1A at the glutamatergic post-synapse by deregulating and interacting with key post-synaptic proteins implicated in mechanism leading to long-term enhanced synaptic plasticity. Altogether, our work gives important information to understand the action of DYRK1A inhibitors and have a better therapeutic approach.


Author(s):  
Michael E. Ernst ◽  
Joanne Ryan ◽  
Enayet K. Chowdhury ◽  
Karen L. Margolis ◽  
Lawrence J. Beilin ◽  
...  

Background Blood pressure variability (BPV) in midlife increases risk of late‐life dementia, but the impact of BPV on the cognition of adults who have already reached older ages free of major cognitive deficits is unknown. We examined the risk of incident dementia and cognitive decline associated with long‐term, visit‐to‐visit BPV in a post hoc analysis of the ASPREE (Aspirin in Reducing Events in the Elderly) trial. Methods and Results ASPREE participants (N=19 114) were free of dementia and significant cognitive impairment at enrollment. Measurement of BP and administration of a standardized cognitive battery evaluating global cognition, delayed episodic memory, verbal fluency, and processing speed and attention occurred at baseline and follow‐up visits. Time‐to‐event analysis using Cox proportional hazards regression models were used to calculate hazard ratios (HR) and corresponding 95% CI for incident dementia and cognitive decline, according to tertile of SD of systolic BPV. Individuals in the highest BPV tertile compared with the lowest had an increased risk of incident dementia and cognitive decline, independent of average BP and use of antihypertensive drugs. There was evidence that sex modified the association with incident dementia (interaction P =0.02), with increased risk in men (HR, 1.68; 95% CI, 1.19–2.39) but not women (HR, 1.01; 95% CI, 0.72–1.42). For cognitive decline, similar increased risks were observed for men and women (interaction P =0.15; men: HR, 1.36; 95% CI, 1.16–1.59; women: HR, 1.14; 95% CI, 0.98–1.32). Conclusions High BPV in older adults without major cognitive impairment, particularly men, is associated with increased risks of dementia and cognitive decline. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01038583; isrctn.com . Identifier: ISRCTN83772183.


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