Citations

AbstractIntroductionCaregivers of patients with frontotemporal lobar degeneration (FTLD) spectrum disorders experience tremendous burden, which has been associated with the neuropsychiatric and behavioral features of the disorders.MethodsIn a sample of 558 participants with FTLD spectrum disorders, we performed multiple‐variable regressions to identify the behavioral features that were most strongly associated with caregiver burden, as measured by the Zarit Burden Interview, at each stage of disease.ResultsApathy and disinhibition, as rated by both clinicians and caregivers, as well as clinician‐rated psychosis, showed the strongest associations with caregiver burden, a pattern that was consistent when participants were separated cross‐sectionally by disease stage. In addition, behavioral features appeared to contribute most to caregiver burden in patients with early dementia.DiscussionCaregivers should be provided with early education on the management of the behavioral features of FTLD spectrum disorders. Interventions targeting apathy, disinhibition, and psychosis may be most useful to reduce caregiver burden.

Objective We aimed to measure provider perspectives on the acceptability, appropriateness, and feasibility of teleneonatology in neonatal intensive care units (NICUs) and community hospitals. Study Design Providers from five academic tertiary NICUs and 27 community hospitals were surveyed using validated implementation measures to assess the acceptability, appropriateness, and feasibility of teleneonatology. For each of the 12 statements, scale values ranged from 1 to 5 (1 = strongly disagree; 5 = strongly agree), with higher scores indicating greater positive perceptions. Survey results were summarized, and differences across respondents assessed using generalized linear models. Results The survey response rate was 56% (203/365). Respondents found teleneonatology to be acceptable, appropriate, and feasible. The percent of respondents who agreed with each of the twelve statements ranged from 88.6 to 99.0%, with mean scores of 4.4 to 4.7 and median scores of 4.0 to 5.0. There was no difference in the acceptability, appropriateness, and feasibility of teleneonatology when analyzed by professional role, years of experience in neonatal care, or years of teleneonatology experience. Respondents from Level I well newborn nurseries had greater positive perceptions of teleneonatology than those from Level II special care nurseries. Conclusion Providers in tertiary NICUs and community hospitals perceive teleneonatology to be highly acceptable, appropriate, and feasible for their practices. The wide acceptance by providers of all roles and levels of experience likely demonstrates a broad receptiveness to telemedicine as a tool to deliver neonatal care, particularly in rural communities where specialists are unavailable. Key Points

Abstract At present, no research criteria exist for the diagnosis of prodromal behavioural variant frontotemporal dementia (bvFTD), though early detection is of high research importance. Thus, we sought to develop and validate a proposed set of research criteria for prodromal bvFTD, termed ‘mild behavioural and/or cognitive impairment in bvFTD’ (MBCI-FTD). Participants included 72 participants deemed to have prodromal bvFTD; this comprised 55 carriers of a pathogenic mutation known to cause frontotemporal lobar degeneration, and 17 individuals with autopsy-confirmed frontotemporal lobar degeneration. All had mild behavioural and/or cognitive changes, as judged by an evaluating clinician. Based on extensive clinical workup, the prodromal bvFTD group was divided into a Development Group (n = 22) and a Validation Group (n = 50). The Development Group was selected to be the subset of the prodromal bvFTD group for whom we had the strongest longitudinal evidence of conversion to bvFTD, and was used to develop the MBCI-FTD criteria. The Validation Group was the remainder of the prodromal bvFTD group and was used as a separate sample on which to validate the criteria. Familial non-carriers were included as healthy controls (n = 165). The frequencies of behavioural and neuropsychiatric features, neuropsychological deficits, and social cognitive dysfunction in the prodromal bvFTD Development Group and healthy controls were assessed. Based on sensitivity and specificity analyses, seven core features were identified: apathy without moderate-severe dysphoria, behavioural disinhibition, irritability/agitation, reduced empathy/sympathy, repetitive behaviours (simple and/or complex), joviality/gregariousness, and appetite changes/hyperorality. Supportive features include a neuropsychological profile of impaired executive function or naming with intact orientation and visuospatial skills, reduced insight for cognitive or behavioural changes, and poor social cognition. Three core features or two core features plus one supportive feature are required for the diagnosis of possible MBCI-FTD; probable MBCI-FTD requires imaging or biomarker evidence, or a pathogenic genetic mutation. The proposed MBCI-FTD criteria correctly classified 95% of the prodromal bvFTD Development Group, and 74% of the prodromal bvFTD Validation Group, with a false positive rate of <10% in healthy controls. Finally, the MBCI-FTD criteria were tested on a cohort of individuals with prodromal Alzheimer’s disease, and the false positive rate of diagnosis was 11–16%. Future research will need to refine the sensitivity and specificity of these criteria, and incorporate emerging biomarker evidence.

Objective We aimed to measure provider perspectives on the acceptability, appropriateness, and feasibility of teleneonatology in neonatal intensive care units (NICUs) and community hospitals. Study Design Providers from five academic tertiary NICUs and 27 community hospitals were surveyed using validated implementation measures to assess the acceptability, appropriateness, and feasibility of teleneonatology. For each of the 12 statements, scale values ranged from 1 to 5 (1 = strongly disagree; 5 = strongly agree), with higher scores indicating greater positive perceptions. Survey results were summarized, and differences across respondents assessed using generalized linear models. Results The survey response rate was 56% (203/365). Respondents found teleneonatology to be acceptable, appropriate, and feasible. The percent of respondents who agreed with each of the twelve statements ranged from 88.6 to 99.0%, with mean scores of 4.4 to 4.7 and median scores of 4.0 to 5.0. There was no difference in the acceptability, appropriateness, and feasibility of teleneonatology when analyzed by professional role, years of experience in neonatal care, or years of teleneonatology experience. Respondents from Level I well newborn nurseries had greater positive perceptions of teleneonatology than those from Level II special care nurseries. Conclusion Providers in tertiary NICUs and community hospitals perceive teleneonatology to be highly acceptable, appropriate, and feasible for their practices. The wide acceptance by providers of all roles and levels of experience likely demonstrates a broad receptiveness to telemedicine as a tool to deliver neonatal care, particularly in rural communities where specialists are unavailable. Key Points

ObjectiveTo evaluate patients who become symptomatic from superior semicircular canal dehiscence (SSCD) following head trauma.Study DesignCase series assessing patients presenting with SSCD after a trauma.MethodsA case series was completed assessing patients presenting with SSCD after trauma. Data from three academic medical centers were evaluated, including the following: imaging, videonystagmography (VNG)/vestibular evoked myogenic potential (VEMP) testing, audiometric assessment, and surgical repair. Outcome measures included the following: 1) Description of audio‐vestibular symptoms, 2) mean pre‐ and post‐operative pure tone average (PTA), word recognition score (WRS), and air bone gap (ABG).ResultsA total of 14 patients were included; 86% were male. Approximately 43% were found to have bilateral SSCD on imaging, with 57% of patients pursuing surgical management. The most common presenting symptoms included pulsatile tinnitus (93%), autophony (79%), and hearing loss (64%). Approximately 36% of patients underwent VNG/VEMP testing, with 83.3% of those demonstrating abnormal results. The mean audiometric findings on the symptomatic side included an air‐conduction PTA of 38.0 dB, bone‐conduction PTA of 24.3 dB, WRS of 81%, and ABG of 17.9 dB. Among patients who underwent surgery (57%), there was no significant change in the air‐conduction PTA, bone‐conduction PTA, or WRS (P > .05). However, there was an improvement in the ABG (preoperative = 22.8 dB versus postoperative = 9.7 dB; P = .005).ConclusionHead trauma may be a potentiating event for SSCD syndrome. This study advances the hypothesis that these patients likely have underlying radiographic SSCD prior to their trauma, and a traumatic event increases in intra‐vestibular or intracranial pressures, unmasking SSCD syndrome.Level of Evidence4 Laryngoscope, 131:E2810–E2818, 2021

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status and/or POPH diagnosis were associated with treatment and health‐care utilization. We performed a cross‐sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n = 344) and POPH (n = 57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6% vs. 34.9%, p = 0.02) and more likely to be unemployed (54.7% vs. 30.5%, p < 0.001) and have an annual household income below poverty level (45.7% vs. 19.0%, p < 0.001). Patients with POPH had similar functional class, quality of life, 6‐min walk distance, and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4% vs. 62.2%, p = 0.03) and endothelin receptor antagonists (28.6% vs. 55.1%, p < 0.001) at enrollment with similar treatment at follow‐up. Patients with POPH had more emergency department visits (1.7 ± 2.1 vs. 0.9 ± 1.2, p = 0.009) and hospitalizations in the six months preceding enrollment (1.5 ± 2.1 vs. 0.8 ± 1.1, p = 0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of emergency department visits. Compared to IPAH, patients with POPH have lower socioeconomic status, are less likely to receive initial combination therapy and endothelin receptor antagonists but have similar treatment at follow‐up, and have increased health‐care utilization.