Racial Inequities in Palliative Referral for Children with High-Intensity Neurologic Impairment.

OBJECTIVE: To evaluate whether racial and socioeconomic inequities in pediatric palliative care utilization extend to children with high-intensity neurologic impairment (HI-NI), which is a chronic neurological diagnosis resulting in substantial functional morbidity and mortality. STUDY DESIGN: We conducted a retrospective study of patients with HI-NI who received primary care services at a tertiary care center from 2014 through 2019. HI-NI diagnoses that warranted a palliative care referral were identified by consensus of a multidisciplinary team. The outcome was referral to palliative care. The primary exposure was race, categorized as Black or non-Black to represent the impact of anti-Black racism. Additional exposures included ethnicity (Hispanic/non-Hispanic) and insurance status (Medicaid/non-Medicaid). Descriptive statistics, bivariate analyses, and multivariable logistic regression models were performed to assess associations between exposures and palliative care referral. RESULTS: A total of 801 patients with HI-NI were included; 7.5% received a palliative referral. There were no differences in gestational age, sex, or ethnicity between patients who received a referral and those who did not. In multivariable analysis, adjusting for ethnicity, sex, gestational age, and presence of complex chronic conditions, Black children (aOR 0.47, 95% CI 0.26, 0.84) and children with Medicaid insurance (aOR 0.40, 95% CI 0.23, 0.70) each had significantly lower odds of palliative referral compared with their non-Black and non-Medicaid-insured peers, respectively. CONCLUSIONS: We identified inequities in pediatric palliative care referral among children with HI-NI by race and insurance status. Future work is needed to develop interventions, with families, aimed at promoting more equitable, antiracist systems of palliative care.

Participation-focused practices in paediatric rehabilitation for children with neurodisability in New Zealand: An observational study using MAPi audit tool.

BACKGROUND: Participation outcomes and family-centred services are now widely agreed standards of care in paediatric rehabilitation. Evidence suggests that adoption of these practices into usual care has been slow internationally, and no studies of participation-focused or family-centred practices in New Zealand have been undertaken to date. The aims of this study were to identify the extent that goal setting in paediatric rehabilitation services is participation focused and family centred and to profile clinicians' beliefs about these foci. METHODS: Observational study including a retrospective case note audit and questionnaire. Case notes ('charts') were audited for participation-focused goal-related practice. Subgroup analysis of audit data by service, profession and child ethnicity were completed using weighted mean differences. Clinicians whose case notes were audited completed a 42-item questionnaire on factors related to practice behaviour including text responses with each item. Numerical and text data were analysed descriptively. RESULTS: Five services were recruited (N = 46 clinicians) with case notes for 220 children audited. Auditors extracted 368 potentially participation-related goals (Mdn = 2 goals per child) with no goals extracted for 43 (20%) of case notes. Subsequent coding of extracted goals identified 61% reflected participation (225/368). Subgroup analysis revealed significant differences between services (p = 0.006) and professions (p < 0.001). Clinicians' reported valuing participation outcomes and family involvement and largely felt they did target participation. Skill gaps, absence of self-monitoring strategies and unsupportive professional and work cultures were cited as key challenges. CONCLUSIONS: Although valued by clinicians, participation-focused practice is unusual care in paediatric rehabilitation in New Zealand. Substantial gains in the quality of care for children with disabilities could be afforded through training in collaborative goal setting to target participation outcomes.

Towards diversity in genomics: The emergence of neurogenomics in Africa?

There is a high burden of mental and neurological disorders in Africa. Nevertheless, there appears to be an under-representation of African ancestry populations in large-scale genomic studies. Here, we evaluated the extent of under-representation of Africans in neurogenomic studies in the GWAS Catalog. We found 569 neurogenomic studies, of which 88.9% were exclusively focused on people with European ancestry and the remaining 11.1% having African ancestry cases included. In terms of population, only 1.2% of the total populations involved in these 569 GWAS studies were of African descent. Further, most of the individuals in the African ancestry category were identified to be African-Americans/Afro-Caribbeans, highlighting the huge under-representation of homogenous African populations in large-scale neurogenomic studies. Efforts geared at establishing strong collaborative ties with European/American researchers, maintaining freely accessible biobanks and establishing comprehensive African genome data repositories to track African genome variations are critical for propelling neurogenomics/precision medicine in Africa.

Performance Stability and Interrater Reliability of Culturally Adapted 10-Meter Walking Test for Danes with Neurological Disorders.

BACKGROUND: The 10-meter Walking Test (10MWT) is often used to assess people with, e.g., stroke, but often using different procedures. The aims of this study were to translate the 10MWT into Danish, to determine the number of trials needed to achieve performance stability, and to examine the interrater reliability and agreement of the 10MWT in people with neurological disorders. METHODS: Translation followed international recommendations, and evaluated in a consecutive sample of 50 people with a neurological disorder. All participants performed 5 timed 10MWT trials (usual speed) with 20-seconds rest intervals between trials, supervised by a physical therapist. A second session was conducted with another physical therapist, separated with a mean (SD) of 2.7 (0.9) hours. The order of raters was randomized and they were blinded to each other's ratings. Repeated measures ANOVA determined performance stability, while ICC1.1, standard error of measurement (SEM), and minimal detectable change (MDC95) determined reproducibility. RESULTS: Participant's improved their 10MWT scores significantly between the first and second trial only. The faster of the 2 trials took a mean of 11.95 (5.40) seconds, and significantly (P < 0.001) faster than the slowest; mean of 12.80 (6.13) seconds. The intraclass correlation coefficient (ICC; 95% confidence interval), SEM, and MDC, based on the fastest of 2 trials, were 0.97 (0.95-0.98), 0.06 m/s, and 0.17 m/s, respectively, and with no systematic between rater's bias. CONCLUSIONS: We suggest that the faster of 2 timed trials be recorded for the 10MWT in people with neurological disorders, as we found excellent interrater reliability and low measurement error using this score.

Bullous pemphigoid and neurological disorders among a Jewish population.

A retrospective case control study included data regarding 65 Jewish bullous pemphigoid (BP) patients diagnosed in a tertiary medical center and a control group consisting of 182 matched Jewish patients. The study indicates that Jewish patients with BP suffer from a higher prevalence of neurological diseases compared to patients with no BP, similarly to previous reports showing high prevalence of neurological diseases among BP patients from different ethnic groups.

[Acute effects of particulate matter pollution on daily emergency ambulance dispatches due to neurological diseases in one haze polluted city of the North China].

OBJECTIVE: To access the relationship between ambient particulate matter( PM_(2. 5)and PM_(10)) concentration and daily emergency ambulance dispatches due to neurological diseases in one city of the North China. METHODS: Semi-parametric generalized additive model was used to analyze the relationship between particulate matter( PM_(2. 5)and PM_(10)) concentration and daily emergency ambulance dispatches due to neurological diseases from January 1, 2013 to December 31, 2015 after adjustment for time trends, weather conditions and "days of the week". RESULTS: An 10 µg/m~3 increase in the lag 03 day concentration of PM_(2. 5)corresponded to increase of 0. 75%( 95% CI0. 34%-1. 17%) in daily emergency ambulance dispatches due to neurological diseases, and an 0. 35%( 95% CI 0. 05%-0. 66%) increase correspondence for PM_(10) in the lag04. CONCLUSION: Outdoor particulate matter( PM_(2. 5)and PM_(10)) concentration is significantly associated with increased risk of emergency ambulance dispatches due to neurological diseases in the North China.

Reliability and validity of the Chinese version of the Neurological Disorders Depression Inventory for Epilepsy (C-NDDI-E).

OBJECTIVE: The aim of this study was to evaluate the clinical reliability and validity of the Chinese version of the Neurological Disorders Depression Inventory for Epilepsy (C-NDDI-E). METHODS: A total of 248 Chinese patients with epilepsy underwent psychometric tests, including the Chinese version of the Mini International Neuropsychiatric Interview (C-MINI), the Chinese version of the Beck Depression Inventory - II (C-BDI-II), and the C-NDDI-E. RESULTS: None of the patients had difficulties understanding or completing the C-NDDI-E. Cronbach's α coefficient was 0.824. At a cutoff score of ≥14, the C-NDDI-E had a sensitivity of 0.854, a specificity of 0.899, a positive predictive value of 0.625, and a negative predictive value of 0.969. The scores for the C-NDDI-E were positively correlated with those for the C-BDI-II (P<0.001). CONCLUSION: The C-NDDI-E is a reliable and valid screening tool for the detection of major depression in Chinese patients with epilepsy.

Validation of the Chinese version of the Neurological Disorders Depression Inventory for Epilepsy (C-NDDI-E) in West China.

OBJECTIVE: We aimed to validate the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) for Chinese people with epilepsy (PWE). METHODS: The NDDI-E was translated into a Chinese version. A consecutive cohort of PWE from West China Hospital was recruited to test the reliability and validity of the Chinese version of the NDDI-E (C-NDDI-E). Each patient underwent the Mini International Neuropsychiatric Interview (MINI) and C-NDDI-E. RESULTS: A total of 202 PWE completed the psychiatric evaluation. The C-NDDI-E was easily comprehended and quickly completed by all participants. Fifty-four patients (26.7%) had current major depressive disorder (MDD) according to the MINI criteria. The Cronbach's α coefficient for the C-NDDI-E was 0.825. Receiver operating characteristic analyses showed an area under the curve of 0.936 (95% CI=0.904-0.968). At a cutoff score of >12, the C-NDDI-E had a sensitivity of 0.926, a specificity of 0.804, a positive predictive value of 0.633, and a negative predictive value of 0.967. CONCLUSION: The C-NDDI-E is a valuable instrument for screening MDD in Chinese PWE.

Addressing ethnic disparities in neurological research in the United Kingdom: An example from the prospective multicentre COVID-19 Clinical Neuroscience Study.

BACKGROUND: Minority ethnic groups have often been underrepresented in research, posing a problem in relation to external validity and extrapolation of findings. Here, we aimed to assess recruitment and retainment strategies in a large observational study assessing neurological complications following SARS-CoV-2 infection. METHODS: Participants were recruited following confirmed infection with SARS-CoV-2 and hospitalisation. Self-reported ethnicity was recorded alongside other demographic data to identify potential barriers to recruitment. RESULTS: 807 participants were recruited to COVID-CNS, and ethnicity data were available for 93.2%. We identified a proportionate representation of self-reported ethnicity categories, and distribution of broad ethnicity categories mirrored individual centres' catchment areas. White ethnicity within individual centres ranged between 44.5% and 89.1%, with highest percentage of participants with non-White ethnicity in London-based centres. Examples are provided how to reach potentially underrepresented minority ethnic groups. CONCLUSIONS: Recruitment barriers in relation to potentially underrepresented ethnic groups may be overcome with strategies identified here.

Joubert syndrome 2 (JBTS2) in Ashkenazi Jews is associated with a TMEM216 mutation.

Patients with Joubert syndrome 2 (JBTS2) suffer from a neurological disease manifested by psychomotor retardation, hypotonia, ataxia, nystagmus, and oculomotor apraxia and variably associated with dysmorphism, as well as retinal and renal involvement. Brain MRI results show cerebellar vermis hypoplasia and additional anomalies of the fourth ventricle, corpus callosum, and occipital cortex. The disease has previously been mapped to the centromeric region of chromosome 11. Using homozygosity mapping in 13 patients from eight Ashkenazi Jewish families, we identified a homozygous mutation, R12L, in the TMEM216 gene, in all affected individuals. Thirty individuals heterozygous for the mutation were detected among 2766 anonymous Ashkenazi Jews, indicating a carrier rate of 1:92. Given the small size of the TMEM216 gene relative to other JBTS genes, its sequence analysis is warranted in all JBTS patients, especially those who suffer from associated anomalies.

Patients with xeroderma pigmentosum complementation groups C, E and V do not have abnormal sunburn reactions.

BACKGROUND: Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder of DNA repair. It is divided into eight complementation groups: XP-A to XP-G (classical XP) and XP variant (XP-V). Severe and prolonged sunburn reactions on minimal sun exposure have been considered a cardinal feature of classical XP. However, it has recently become clear that not all patients have abnormal sunburn reactions. OBJECTIVES: To examine sunburn reactions in a cohort of patients with XP and correlate this to the complementation group. METHODS: Sixty patients with XP attending the U.K. National XP Service from 2010 to 2012 were studied. Their history of burning after minimal sun exposure was assessed using a newly developed sunburn severity score. The age at which the first skin cancer was histologically diagnosed in each patient, and the presence of any neurological abnormality, was also recorded. RESULTS: Sunburn severity scores were abnormally high in patients with XP-A, XP-D, XP-F and XP-G compared with non-XP controls. There was no significant difference in sunburn score of patients with XP-C, XP-E and XP-V compared with controls (P > 0·05). Patients with XP-C, XP-E and XP-V were more likely to have skin cancer diagnosed at an earlier age than those with severe sunburn on minimal sun exposure. In addition, patients with XP with severe sunburn had an increased frequency of neurological abnormalities. CONCLUSIONS: Not all patients with XP have a history of severe and prolonged sunburn on minimal sun exposure. The normal sunburn response of patients with XP-C, XP-E and XP-V may relate to the preservation of transcription-coupled DNA repair in these groups. Those with a history of severe sunburn on minimal sun exposure developed their first skin cancer at an older age compared with patients with XP-C, XP-E and XP-V, but they had an increased frequency of neurological abnormalities. Physicians need to be aware that about half of all patients with XP will present without a history of abnormal sunburn.

The Lives Lost to Inequities: Avertable Deaths From Neurologic Diseases in the Past Decade.

BACKGROUND AND OBJECTIVES: Mortality rates for neurologic diseases are increasing in the United States, with large disparities across geographical areas and populations. Racial and ethnic populations, notably the non-Hispanic (NH) Black population, experience higher mortality rates for many causes of death, but the magnitude of the disparities for neurologic diseases is unclear. The objectives of this study were to calculate mortality rates for neurologic diseases by race and ethnicity and-to place this disparity in perspective-to estimate how many US deaths would have been averted in the past decade if the NH Black population experienced the same mortality rates as other groups. METHODS: Mortality rates for deaths attributed to neurologic diseases, as defined by the International Classification of Diseases, were calculated for 2010 to 2019 using death and population data obtained from the Centers for Disease Control and Prevention and the US Census Bureau. Avertable deaths were calculated by indirect standardization: For each calendar year of the decade, age-specific death rates of NH White persons in 10 age groups were multiplied by the NH Black population in each age group. A secondary analysis used Hispanic and NH Asian populations as the reference groups. RESULTS: In 2013, overall age-adjusted mortality rates for neurologic diseases began increasing, with the NH Black population experiencing higher rates than NH White, NH American Indian and Alaska Native, Hispanic, and NH Asian populations (in decreasing order). Other populations with higher mortality rates for neurologic diseases included older adults, the male population, and adults older than 25 years without a high school diploma. The gap in mortality rates for neurologic diseases between the NH Black and NH White populations widened from 4.2 individuals per 100,000 in 2011 to 7.0 per 100,000 in 2019. Over 2010 to 2019, had the NH Black population experienced the neurologic mortality rates of NH White, Hispanic, or NH Asian populations, 29,986, 88,407, or 117,519 deaths, respectively, would have been averted. DISCUSSION: Death rates for neurologic diseases are increasing. Disproportionately higher neurologic mortality rates in the NH Black population are responsible for a large number of excess deaths, making research and policy efforts to address the systemic causes increasingly urgent.

A proposition against using the terms "Hispanic" and "Latino" in research on HIV-associated neurocognitive disorders.

In the United States, the term "Hispanic" has been used to refer to a person or groups of persons who originate from Spanish-speaking countries. However, this term fails to account for variables such as nationality, ethnicity, race, and cultural origin as well as the extent of assimilation to a new culture. In addition, factors such as the individual's generation, specific migratory status, years of education in each country, fluency, and day-to-day language usage contribute to variance in neuropsychological testing outcomes, which are sensitive to these factors. We have noted that the usage of the terms "Hispanic" and "Latino" is problematic in HIV-associated neurocognitive disorder (HAND) research; therefore, we propose grouping individuals by nationality or by the Spanish-speaking culture to which they belong. The rationale for not using these terms is based upon the sociodemographic findings among Spanish speakers infected with HIV and how these terms inadequately describe the rich heterogeneity of this population.

[Stories of foreigners]. / Storie di stranieri.

Immigrant foreigners who attend a public neurology clinic live in our country for a long time and they usually have a fair degree of integration into the cultural fabric. They do not seem to have pathologies or social problems different from those of the Italian users of the same service. However, many of them carry personal and collective stories of deprivation, struggle and suffering that can not be considered common. Some cases are briefly told.

Prevalence and Risk Factors Associated with Self-Rated Morbidities Among South Asian Migrant Gas Station Workers in Kuwait.

Gas station workers (GSWs) are at elevated risk of morbidities associated with exposure to petrochemical substances. This cross-sectional study assessed the prevalence and examined demographics and occupation-related risk factors associated with self-rated ill-health among GSWs in Kuwait. Structured questionnaire was used to record self-rated ill-health from 460 selected GSWs of South-Asian origin. Prevalence of self-rated ill-health conditions was computed. Random-intercept multivariable logistic regression model was used to identify the demographics and/or work-place related potential risk factors associated with one or more morbidities. Prevalent morbidities were eye(s) complaint (45.2%), asthma/shortness of breath (7.6%), frequent sneezing and allergic rhinitis (44.3%), recurrent headache (48.3%), dizziness (9.8%), tremors (42%), and eczema/skin complaint (44.1%). Furthermore, GSWs were significantly more likely to report one or more ill-health conditions (vs. none), if they were overweight/obese (aOR 1.8; 95% CI 1.1-2.9), married (aOR 2.6; 95% CI 1.5-4.5) or on current job for more than one year (aOR 6.4; 95% CI 2.9-15.4). In conclusion, GSWs suffer from ailments of multiple organ systems and need education and facilitation for self-protection.

Ultrasound biomicroscopy image patterns in normal upper eyelid and congenital ptosis in the Indian population.

PURPOSE: To study the features of upper eyelid in healthy individual and different types of congenital ptosis in the Indian population using ultrasound biomicroscopy (UBM). METHODS: This was a prospective observational study at a tertiary care center. Eyelid structure of healthy individuals with no eyelid abnormalities (n = 19); simple congenital ptosis (n = 33) cases; Marcus Gunn jaw-winking ptosis (MGJWP, n = 7) cases, and blepharophimosis-ptosis-epicanthus inversus syndrome (BPES, n = 20) cases were studied on a vertical UBM scan using 50-MHz probe. Lid-thickness, tarsal-thickness, orbicularis oculi and levator-Muller-orbital septum-conjunctival (LMSC) complex were measured in primary gaze. Comparison was made between four groups and results were statistically analyzed using ANOVA test. In normal individuals, LMSC measurements were repeated in down-gaze imaging. RESULTS: Skin with subcutaneous tissue, LMSC complex and pre-aponeurotic fat-pad appeared echodense while orbicularis oculi and tarsus appeared echolucent. In primary gaze, mean thickness (± standard deviation) of the eyelid, tarsus, orbicularis oculi and LMSC, respectively, were: 1.612 ± 0.205, 0.907 ± 0.098, 0.336 ± 0.083, and 0.785 ± 0.135 mm in normal individual. LMSC showed 46.64% increase in thickness on down-gaze. The mean eyelid thickness and LMSC were thicker in MGJWP and BPES as compared to normal. In different types of congenital ptosis cases, various patterns of UBM imaging were observed. CONCLUSION: UBM allows noninvasive imaging of eyelid structures with good anatomical correspondence in normal eyelids and study the structural alterations of eyelids in different types of congenital ptosis. UBM can be used to highlight the anatomical difference in normal eyelids that may help modify the surgery for better cosmetic outcomes. Furthermore, it has the potential to be used in preoperative evaluation and operative planning in certain types of acquired ptosis, which needs to be evaluated.

Neuro-Behçet's disease in Caucasians: a study of 22 patients.

Several analyses of the neurological features of Behçet's disease (BD) have concluded that there are significant racial differences in its clinical expression. Other series, however, failed to elicit such differences. We aimed to describe in this retrospective survey the frequency, nature and relationship to systemic disease of the neurological features in a cohort of BD patients of Caucasian origin. We searched hospital records from nine District General or Regional Centre hospitals in south-west Great Britain and identified 22 individuals of Caucasian ethnic origin with neuro-BD, with a mean of 10 years follow-up per patient - the largest 'western' case series with the longest period of follow-up reported. We found that presentation with neurological features was commoner in our patients (23%) than Middle Eastern series (3-10%). Seizures (27%) were likewise commoner (0-5%), as was optic neuritis (9% compared with 1-2%). Two patients developed movement disorders (chorea and parkinsonism), which have only been rarely reported. Of further clinical significance, we noted that non-neuropsychiatric features: oral ulceration, intraocular inflammation and skin lesions - were virtually always present or exacerbated during neurological complications. Ethnicity--or conceivably environment--may play a significant role in the manifestation of neurological BD.

Barriers and Strategies for Recruitment of Racial and Ethnic Minorities: Perspectives from Neurological Clinical Research Coordinators.

INTRODUCTION: Randomized controlled trials (RCTs) are the gold standard within evidence-based research. Low participant accrual rates, especially of underrepresented groups (e.g., racial-ethnic minorities), may jeopardize clinical studies' viability and strength of findings. Research has begun to unweave clinical trial mechanics, including the roles of clinical research coordinators, to improve trial participation rates. METHODS: Two semi-structured focus groups were conducted with a purposive sample of 29 clinical research coordinators (CRCs) at consecutive international stroke conferences in 2013 and 2014 to gain in-depth understanding of coordinator-level barriers to racial-ethnic minority recruitment and retention into neurological trials. Coded transcripts were used to create themes to define concepts, identify associations, summarize findings, and posit explanations. RESULTS: Barriers related to translation, literacy, family composition, and severity of medical diagnosis were identified. Potential strategies included a focus on developing personal relationships with patients, community and patient education, centralized clinical trial administrative systems, and competency focused training and education for CRCs. CONCLUSION: Patient level barriers to clinical trial recruitment are well documented. Less is known about barriers facing CRCs. Further identification of how and when barriers manifest and the effectiveness of strategies to improve CRCs recruitment efforts is warranted.