Citations

Abstract Introduction Introduction: Rapid Eye Movement (REM) Sleep Behavior Disorder (RBD) is characterized by a lack of muscle atonia during REM sleep with dream enactment. RBD is regarded as a prodromal synucleinopathy as a high proportion of patients eventually phenoconvert to Parkinson’s Disease and related synucleinopathies, suggesting RBD may be an early non-motor symptom of disease. Accordingly, patients with RBD are ideally situated to test potential therapeutic interventions to prevent phenoconversion to synucleinopathy. However, RBD itself, and associated patient registries, are rare. The North American Prodromal Synucleinopathy Consortium (NAPS) establishes a multisite registry of RBD patients with standardized neurological, neuropsychiatric, and neuropsychological assessments and biomarker collection. The present work reports baseline characteristics of this RBD patient database at its current state. Methods Methods: Participants >18 years of age with overnight polysomnogram-confirmed RBD by ICSD-3 criteria who did not meet criteria for the diagnosis of PD, dementia, MSA, or narcolepsy were enrolled from 10 sites across North America (8/2018 to 4/2021). Data collection included family and personal history of RBD and related symptoms, as well as standardized assessments related to cognitive, motor, sensory and autonomic function. Additionally, all subjects have contributed blood, and a subset of subjects have contributed cerebrospinal fluid samples to the National Centralized Repository for Alzheimer's Disease and Related Dementias for future analysis. Results Results: A total of n=251 participants were enrolled. Outcomes are reported based on sex (n=202 male, n=49 female). Data were further examined based on participants’ history of antidepressant use (n=142 with, n=103 without) and based on participants’ extent of synucleinopathy burden (n=70 defined as isolated RBD, n=181 defined as RBD+ [i.e., exhibiting ≥1 abnormality]). Any observed sex differences among the data did not persist after correction for antidepressant use. Conclusion Conclusions: This prospective, cross-sectional data on history, demographic, cognitive, motor, sensory, and autonomic function in n=251 participants with RBD highlight the lack of sex differences and the high preponderance of concomitant neurological abnormalities with RBD, and provide a valuable registry for future longitudinal studies and neuroprotective clinical trials. Support (If Any) NIH NIA R34 AG056639 (YJ, BB)

AbstractBackground and ObjectiveIntensive interdisciplinary pain treatment (IIPT) for youth with high impact chronic pain has been found to be effective in improving child symptoms and functioning. However, it remains unclear how these interventions impact the parents' own well‐being, as well as cognitions and behaviours which are known to influence child pain and functioning. Thus, the current study sought to determine the effect of IIPT on parent mental health, cognitions and behaviours in parents of youth attending IIPT with their child.Databases and Data TreatmentA search of the electronic databases CINAHL, Ovid EBM Reviews, Embase, Medline, APA PsychINFO, Scopus and web of Science was conducted. Studies were included if they comprised at least 10 parents of patients aged 9–22 with nonmalignant chronic pain attending an IIPT and were written in English.ResultsA random‐effects model was used to pool the standardized mean change (SMC) across seven prepost studies. At discharge, IIPT participation was associated with small to moderate improvements in direct parental outcomes (general mental health, anxiety, depression and parenting stress), a moderate improvement in pain catastrophizing and large improvements in psychological flexibility and parenting behaviours. Most improvements were maintained at follow‐up. The risk of bias of all studies was rated as serious and the certainty of the evidence as low, suggesting limited confidence in the estimates.ConclusionsAlthough parents appear to benefit from attending an IIPT with their child, RCTs are needed to substantiate the effect of these interventions on important aspects of parent well‐being and functioning.

Background: Describe the incidence and associated outcomes of gastrointestinal (GI) manifestations of acute coronavirus disease 2019 (COVID-19) and multisystem inflammatory syndrome in hospitalized children (MIS-C). Methods: Retrospective review of the Viral Infection and Respiratory Illness Universal Study registry, a prospective observational, multicenter international cohort study of hospitalized children with acute COVID-19 or MIS-C from March 2020 to November 2020. The primary outcome measure was critical COVID-19 illness. Multivariable models were performed to assess for associations of GI involvement with the primary composite outcome in the entire cohort and a subpopulation of patients with MIS-C. Secondary outcomes included prolonged hospital length of stay defined as being >75th percentile and mortality. Results: Of the 789 patients, GI involvement was present in 500 (63.3%). Critical illness occurred in 392 (49.6%), and 18 (2.3%) died. Those with GI involvement were older (median age of 8 yr), and 18.2% had an underlying GI comorbidity. GI symptoms and liver derangements were more common among patients with MIS-C. In the adjusted multivariable models, acute COVID-19 was no associated with the primary or secondary outcomes. Similarly, despite the preponderance of GI involvement in patients with MIS-C, it was also not associated with the primary or secondary outcomes. Conclusions: GI involvement is common in hospitalized children with acute COVID-19 and MIS-C. GI involvement is not associated with critical illness, hospital length of stay or mortality in acute COVID-19 or MIS-C.

Abstract Multiple clinical trials have assessed de-escalation strategies from combined modality therapy (CMT) to chemotherapy-alone for the treatment of early-stage classical Hodgkin lymphoma (cHL), confirming similar outcomes. The application of these data to the real-world is limited, however. We conducted a retrospective, multicenter cohort study comparing CMT vs chemotherapy-alone in patients with early-stage cHL (stage IA-IIB) treated between January 2010 and December 2020. Positron emission tomography (PET) scans after chemotherapy cycle 2 (PET2) were independently reviewed by a nuclear radiologist (Deauville score ≥4, positive; ≤3, negative). Patient outcomes were compared by using an intention-to-treat analysis. Among 125 patients (CMT, n = 63; chemotherapy-alone, n = 62) with a median follow-up of 59.8 months (95% CI, 48.6-71.0), no differences in overall survival were observed (5-year overall survival, CMT 98.0% vs chemotherapy-alone 95.1%; log-rank test, P = .38). However, there was reduced progression-free survival (PFS) with chemotherapy-alone among all patients (2-year PFS, CMT 95.1% vs chemotherapy-alone 75.3%; log-rank test, P = .005) and in those with bulky (n = 43; log-rank test, P < .001), unfavorable (n = 81; log-rank test, P = .002), or PET2-positive (n = 15; log-rank test, P = .02) disease. No significant differences in PFS were seen for patients with non-bulky (log-rank test, P = .35), favorable (log-rank test, P = .62), or PET2-negative (log-rank test, P = .19) disease. Based on our real-world experience, CMT seems beneficial for patients with early-stage cHL, especially those with PET2-positive and unfavorable disease. Chemotherapy-alone regimens can lead to comparable outcomes for patients with favorable, non-bulky, or PET2-negative disease. We conclude that although results seen in clinical trials are replicated in certain patient subgroups, other subgroups not fitting trial criteria do poorly when radiotherapy is excluded.

Purpose: To identify risk factors for the development of new-onset, postoperative diplopia following orbital decompression surgery based on patient demographics, clinical exam characteristics, radiographic parameters, and surgical techniques. Methods: We conducted a multi-center retrospective chart review of patients who underwent orbital decompression for thyroid eye disease (TED). Patient demographics, including age, gender, smoking history, preoperative exophthalmometry, clinical activity score (CAS), use of peribulbar and/or systemic steroids, and type of orbital decompression were reviewed. Postoperative diplopia was determined at a minimum of 3 months postoperatively and before any further surgeries. Cross-sectional area ratios of each extraocular muscle to orbit and total fat to orbit were calculated from coronal imaging in a standard fashion. All measurements were carried out using PACS imaging software. Multivariable logistic regression modeling was performed using Stata 14.2 (StataCorp, College Station, TX). Results: A total of 331 patients without preoperative diplopia were identified. At 3 months postoperatively, 249 patients had no diplopia whereas 82 patients developed diplopia. The average postoperative follow-up was 22 months (range 3–156) months. Significant preoperative clinical risk factors for postoperative diplopia included older age at surgery, proptosis, use of peribulbar or systemic steroids, elevated clinical activity score, and presence of preoperative compressive optic neuropathy. Imaging findings of enlarged cross-sectional areas of each rectus muscle to the overall orbital area also conferred a significant risk of postoperative diplopia. Regarding surgical factors, postoperative diplopia was more common among those undergoing medial wall decompression, bilateral orbital surgery, and balanced decompression, whereas endoscopic medial wall decompression was found to be relatively protective. Conclusions: This study identifies risk factors associated with the development of diplopia following orbital decompression using multivariable data. This study demonstrates that several characteristics including age, clinical activity score, the cross-sectional muscle to orbit ratios, in addition to the type of orbital decompression surgery, are predictive factors for the development of new-onset postoperative diplopia.

Purpose: While there has been mounting research centered on the diagnosis of childhood apraxia of speech (CAS), little has focused on differentiating CAS from pediatric dysarthria. Because CAS and dysarthria share overlapping speech symptoms and some children have both motor speech disorders, differential diagnosis can be challenging. There is a need for clinical tools that facilitate assessment of both CAS and dysarthria symptoms in children. The goals of this tutorial are to (a) determine confidence levels of clinicians in differentially diagnosing dysarthria and CAS and (b) provide a systematic procedure for differentiating CAS and pediatric dysarthria in children. Method: Evidence related to differential diagnosis of CAS and dysarthria is reviewed. Next, a web-based survey of 359 pediatric speech-language pathologists is used to determine clinical confidence levels in diagnosing CAS and dysarthria. Finally, a checklist of pediatric auditory–perceptual motor speech features is presented along with a procedure to identify CAS and dysarthria in children with suspected motor speech impairments. Case studies illustrate application of this protocol, and treatment implications for complex cases are discussed. Results: The majority (60%) of clinician respondents reported low or no confidence in diagnosing dysarthria in children, and 40% reported they tend not to make this diagnosis as a result. Going forward, clinicians can use the feature checklist and protocol in this tutorial to support the differential diagnosis of CAS and dysarthria in clinical practice. Conclusions: Incorporating this diagnostic protocol into clinical practice should help increase confidence and accuracy in diagnosing motor speech disorders in children. Future research should test the sensitivity and specificity of this protocol in a large sample of children with varying speech sound disorders. Graduate programs and continuing education trainings should provide opportunities to practice rating speech features for children with dysarthria and CAS. Supplemental Material: https://doi.org/10.23641/asha.19709146

Abstract ObjectivePrevious studies suggested that hypernatremia or hyperosmolarity may have protective effects in lung injury. We hypothesized that hypernatremia and/or hyperosmolarity would prevent ARDS in those at risk of lung injury and associated with favorable outcomes in ARDS patients.SubjectsAll admissions at medical, surgical, and multidisciplinary intensive care units in Mayo Clinic, Rochester from the year of 2009 to 2019.DesignRetrospective cohort study. The occurrence of ARDS was identified using a validated computerized search strategy. The association between serum sodium/osmolarity and the occurrence of ARDS was analyzed using a multivariable logistic regression model. The relationship between serum sodium/osmolarity and outcomes of ARDS was analyzed using linear and logistic regression models.ResultsAmong 50,498 patients, the serum sodium level on admission did not have a significant association with the occurrence of ARDS, with an adjusted odds ratio of 0.95 [95% CI (0.86, 1.05)]. There was no significant association between calculated serum osmolarity and the occurrence of ARDS, with an adjusted odds ratio of 1.03 [95% CI (1.00, 1.07)]. 1560 patients developed ARDS during the ICU stay. Their serum sodium level did not have a significant association with their outcomes [adjusted estimated difference in 28-day ICU-free days, 0.08 days, 95% CI (-0.85, 1.02), adjusted estimated difference in 28-day ventilator-free days, -0.02, 95% CI (-0.62, 0.58), adjusted odds ratio of 28-day mortality 1.02, 95%CI (0.84, 1.24)]. There was no significant association between their calculated serum osmolarity and their outcomes [adjusted estimated difference in 28-day ICU-free days, -0.26, 95% CI, (-0.63, 0.12); adjusted estimated difference in 28-day ventilator-free days, -0.05, 95% CI (-0.28, 0.19); adjusted odds ratio of 28-day mortality, 1.07, 95% CI (0.99, 1.15)].ConclusionsAdmission serum sodium or serum osmolarity were not associated with the occurrence or outcomes of ARDS in ICU.