Neurology. Clinical practice最新文献

Background and objectives: Limited English proficiency (LEP) impairs health access-including outpatient specialty care-and quality care, i.e., inappropriate use of diagnostic tests. At least in some cases, studies have suggested that clinicians may substitute testing for time-consuming clinical evaluation involving medical interpreters. This study (1) examines disparities in receipt of diagnostic testing among patients with LEP and neurologic illness, in both the ambulatory and emergency department (ED) settings, including (2) whether better patient-provider communication is associated with reduced testing disparities and (3) how testing disparities vary according to insurance.

Methods: We analyzed nationally representative data from the 2003-2018 Medical Expenditure Panel Survey and identified adults with neurologic illness using diagnostic codes. To assess the association between LEP status and diagnostic testing (CT/MRI, laboratory tests, and any diagnostic tests), we estimated logistic regression models that included year-fixed effects. We constructed separate models for ambulatory and ED settings, including models with a patient-provider communication measure to see how that influenced the LEP-diagnostic testing association. Finally, we conducted stratified analyses by sources of health insurance.

Results: LEP status was associated with greater receipt of laboratory tests (OR = 1.46, p < 0.05) but less CT/MRI in the ambulatory setting (0.86, p < 0.05), patterns that persisted in analyses stratified by insurance status. Factoring in patient-provider communication attenuated but did not eliminate these disparities, with attenuation most notable in rates of CT/MRI. We found fewer testing disparities for patients with LEP in the ED than in ambulatory settings.

Discussion: In this nationwide study of patients with neurologic illness, we observed both greater and less use of diagnostic tests for patients with LEP and neurologic illness. The greater use of laboratory tests may reflect the overuse of easily obtainable tests for patients with LEP. Conversely, the less use of CT/MRI may be due to time and transportation challenges in scheduling follow-up visits, alongside other barriers to patient follow-up. The population of patients with LEP is growing, making it critical to study not only disparities in their care but also nuances and determinants of these disparities beyond patient-provider communication and across clinical settings.

Background and objectives: Neurosarcoidosis poses a diagnostic and management challenge due to its rarity, phenotypic variability, and lack of randomized controlled studies to guide treatment selection. Recommendations for management based on expert opinion are useful in clinical practice and provide a framework for designing prospective studies.

Methods: In this Delphi survey study, specialists with experience in managing patients with neurosarcoidosis were invited to anonymously complete 2 surveys about key elements of evaluation, diagnosis, treatment, monitoring, and long-term management of neurosarcoidosis. Expert consensus recommendations were adopted if >80% threshold of agreement was reached.

Results: Of the 41 invited expert clinicians across the United States, 32 (78%) participated in the study. All round 1 respondents self-identified as neuroimmunologists (except for 1 pulmonologist). Consensus was reached regarding the need to consider neurosarcoidosis phenotype and severity to guide the choice of initial immunosuppression in both the acute (relapse) and maintenance phases. Experts endorsed the use of TNF-α inhibitors as first-line agents in selected phenotypes with poor prognosis. Neuroimaging was recommended to complement clinical surveillance for treatment response.

Discussion: There was agreement on several key issues, most importantly on the need to consider neurosarcoidosis phenotype and severity when deciding initial treatment. No consensus was achieved on the dosing and duration of specific immunosuppressants, nor regarding the management of the peripheral nervous system manifestation of neurosarcoidosis. These topics warrant further investigation.

Background and objectives: Early presentation and acute treatment for patients presenting with ischemic stroke are associated with improved outcomes. The onset of the COVID-19 pandemic was associated with a large decrease in patients presenting with ischemic stroke, but it is unknown whether these changes persisted.

Methods: This study analyzed emergency department (ED) stroke presentations (n = 158,060) to all nonfederal hospitals in the 50 states and Washington, D.C., from 2019 through 2021 using administrative claims data of traditional fee-for-service Medicare enrollees aged 66 years or older. Patients presenting with stroke were identified using the ICD-10 CM (I63.X). We examined the number of beneficiaries presenting with ischemic stroke to the ED, both overall and by demographic categories (race, age, sex, region, Medicaid eligibility, comorbidity status), admission rates conditional on presentation, use of neurovascular interventions, thirty-day mortality, intensive care unit and mechanical ventilation use, length of stay, and discharge destination.

Results: With the onset of the pandemic in March 2020, there was a drop of 32.1% in ED stroke presentations compared with March 2019 levels, and by December 2021, the rate remained 17.7% lower than baseline levels in December 2019. Relative to the prepandemic period, there were decreases in the proportions of those dually eligible for Medicaid (-0.8%, p < 0.0001) or Black (-0.8%, p < 0.0001), as well as those with atrial fibrillation (-1.1%, p < 0.0001), hypertension (-0.7%, p < 0.0001), and chronic obstructive pulmonary disease (-1.8%, p < 0.0001). Admitted patients were more often discharged to home as opposed to postacute care settings (+3.5%, p < 0.0001). The percentage of patients receiving intravenous thrombolysis changed minimally while those receiving intracranial mechanical thrombectomy (+17.8%, p < 0.0001) and carotid interventions (+6.9%, p < 0.0001) increased from baseline throughout the pandemic. Adjusted thirty-day mortality or referral to hospice increased (+1.81%, p < 0.0001) with larger increases seen among Black beneficiaries and those dually eligible for Medicaid.

Discussion: After an initial sharp decline, stroke presentations remained substantially lower than at baseline through the end of 2021, especially among racial minority and those dually eligible for Medicaid. The observed increased mortality rates for those presenting with stroke may have resulted from later time of presentation after the onset of symptoms or preferential presentation of more vs less severe strokes.

Background and objectives: Multiple sclerosis (MS) affects more than 1 million people in the United States, including reproductive-age women. There has been a paucity of prospective, pregnancy registries based on MS disease rather than medication exposures. A prospective MS pregnancy registry (PREG-MS) was established in 2017 as a prospective, single-cohort, real-world MS pregnancy registry in New England States of the United States, with goals to evaluate (1) course of MS and disease-modifying therapies (DMT) use during conception attempts and in the peripartum period, (2) pregnancy outcomes in women with MS (WwMS), and (3) longer-term developmental outcomes in offspring of WwMS.

Methods: Between 2017 and 2020, PREG-MS recruited from 11 preselected academic and community MS centers and followed WwMS and their children from conception attempts and any pregnancy trimester, up to 3 years of postpartum. Comprehensive neurologic, obstetric, and pediatric development information was collected through telephone interviews and medical records.

Results: One hundred forty-six patients were enrolled between 2017 and 2020; there were 122 pregnancies from 135 participants, and 105 infants were born on study. 24.6% pregnancies were unplanned; 14.1% had an infertility diagnosis. Assisted reproductive technologies were used by 12.6%. 54% of pregnancies were designated as "high-risk", and ∼40% had peripartum obstetrical complications with 17% adverse pregnancy outcomes. Mean baseline Expanded Disability Status Scale was 1.09 ± 0.84. ∼85% were treated with DMTs up to the time of conception. 19.7% had 1 or more relapses within prepregnancy year, correlating with increased duration of conception attempts (p < 0.0001). 12% had intrapartum, and 24.5% had postpartum relapses. Any fertility treatments predicted intrapartum relapses independent of DMT status (OR 5.18, 95% CI 1.58-17.02, p = 0.007). 33.6% were exposed to DMTs in pregnancy. Intrapartum relapses (p = 0.008) and high-risk pregnancy (p = 0.036) were associated with postpartum exacerbations.

Discussion: Our real-world, prospective, nondisabled MS pregnancy cohort had a sizable proportion of participants with clinical disease activity in the prepartum and intrapartum period, despite high-DMT utilization prepartum. A greater-than-expected number of participants were considered to have high-risk pregnancies and reported peripartum complications. The use of any fertility treatments was independently predictive of intrapartum relapses, supporting hormonal-immune interactions as disease modulators in MS. Larger prospective, longitudinal registries are needed to confirm our findings.

Trial registration information: Clinical trial registration number: NCT03368157.

Background: Huntington disease (HD) is a genetic neurodegenerative disorder. Given the focus on motor manifestations, nonmotor symptoms are frequently underappreciated in clinical evaluations, despite frequently contributing to primary functional impairment.

Recent findings: A diagnosis of motor-onset as the definition of manifest symptoms misrepresents the complex nature of HD presentation. Despite recent attempt to integrate nonmotor diagnostic criteria, practical guidelines are necessary to inform clinical diagnosis. We propose an HD diagnostic framework and staging system that prioritizes genetic testing, integrates motor and nonmotor symptom considerations in the determination of clinical disease onset and severity, and acknowledges the secondary role of clinically indicated diagnostic assessments, incorporating the broad symptom profiles observed in clinical practice.

Implications for practice: The proposed diagnostic criteria more accurately reflect the presentation of HD and provide greater opportunities for health care professionals to provide appropriate clinical care guidelines for adults with gene-expanded HD.

Background and objectives: Available disease-modifying therapies (DMTs) for multiple sclerosis (MS) are rapidly expanding; although escalation approaches aim to balance safety and efficacy, emerging evidence suggests superior outcomes for people with MS who are exposed to early high-efficacy therapies. We aimed to explore practice differences in prevailing management strategies for relapsing-remitting MS.

Methods: We used a worldwide electronic survey launched by the Practice Current section of Neurology® Clinical Practice. Questions pertained to a case of a 37-year-old woman presenting with optic neuritis. Respondents were asked to indicate their initial investigations, relapse management strategy, choice of disease-modifying therapy, and plan for follow-up imaging (contrast/noncontrast). Survey responses were stratified by key demographic variables along with 95% confidence intervals (95% CIs).

Results: We received 153 responses from 42 countries; 32.3% responders identified as MS specialists. There was a strong preference for intravenous delivery of high-dose corticosteroids (87.7%, 95% CI 80.7-92.5), and most of the responders (61.3%, 95% CI 52.6-69.4) indicated they would treat a nondisabling (mild sensory) MS relapse. When asked to select a single initial DMT, 56.6% (95% CI 47.6-65.1) selected a high-efficacy therapy (67.5% MS specialists vs 53.7% non-MS specialists). The most selected agents overall were fingolimod (14.7%), natalizumab (15.5%), and dimethyl fumarate (20.9%). Two-thirds of respondents indicated they would request contrast-enhanced surveillance MRI.

Discussion: Although there is a slight preference for initiating high-efficacy DMT at the time of initial MS diagnosis, opinions regarding the most appropriate treatment paradigm remain divided.

Background and objectives: Tourette syndrome (TS) is defined by multiple motor tics and one or more phonic tics with a symptom duration of >1 year. Coprophenomena are uncommon tics characterized by obscene sounds, words, or gestures. Youth with TS commonly have psychiatric co-occurring conditions such as attention-deficit hyperactivity disorder or obsessive-compulsive disorder and have reported lower scores on measures of individual and family functioning than youth without TS. This study aimed to determine associations among co-occurring condition symptoms, tic severity, and function in youth with TS and coprophenomena compared with those with TS without coprophenomena.

Methods: Data were collected through a multicenter, cross-sectional study. Youth with TS were recruited from 2 referral centers, and data were collected from youth and their parents or caregivers. Tic severity was assessed using the Yale Global Tic Severity Scale, and individual function was measured with the Children's Global Assessment Scale. Family impact was measured using the Family Impact Module in domains of parent health-related quality of life (HRQOL), family functioning, and total family impact. We compared individual and family function in youth with TS with coprophenomena (TS+copro) and without coprophenomena (TS-copro). Wilcoxon rank-sum tests were used to compare scores on individual function and family function measures.

Results: Of 169 participants, 17 (10.1%) reported coprophenomena. Participants with TS and coprophenomena had higher tic severity scores than those without coprophenomena (TS+copro mean = 36.9, TS-copro = 20.8). Youth with coprophenomena had lower scores for global function (TS+copro median = 51, TS-copro = 60), family functioning (TS+copro = 43.8, TS-copro = 59.4), parent HRQOL (TS+copro = 57, TS-copro = 72), and total family QOL (TS+copro = 50.7, TS-copro = 65.3).

Discussion: Youth with TS and coprophenomena had lower individual function, family function, and parent HRQOL than youth without coprophenomena. Coprophenomena presence may indicate that youth have a more severe phenotype of TS, and youth with copropheneomena may benefit from additional caregiver or family supports.

Purpose of the review: The aim of this narrative review was to explore the interplay between functional movement disorders (FMDs) and deep brain stimulation (DBS).

Recent findings: Patients with unrecognized FMD who are referred for DBS usually present with functional dystonia. By contrast, patients who present with FMD after DBS are mostly presenting with functional tremor, in keeping with non-DBS FMD cohorts. Comorbid presentation of FMD in established DBS indications makes the decision to opt for surgery challenging. Many contributing factors can play a role in the development of FMD, including the trauma caused by awake neurosurgery and/or extensive DBS programming.

Summary: FMDs in the context of DBS are often overlooked and should be diagnosed promptly because they determine surgical outcome. The approach to DBS candidates with comorbid FMD and the risk factors of FMD after DBS should be further explored.

Background and objectives: Despite increasing interest in post-intensive care unit (ICU) clinical care and management, there have been limited descriptions focused on the post-neurologic (neuro)-ICU population. Here, we describe the design of a post-neuro-ICU Neurorecovery Clinic (NRC) and present data collected regarding the clinic's population, referrals, visits, and clinician satisfaction.

Methods: This is a single-institution experience with a NRC designed to provide an infrastructure for post-ICU care to patients recovering from acute neurologic disorders or systemic conditions with neurologic sequelae. The clinic offers 2 visit types with different frequencies: a weekly visit and a monthly multidisciplinary visit. This study assessed clinical utilization and clinician perspectives regarding the clinic. Data on clinic referrals, no-show frequency, visit types, and diagnoses for both weekly and monthly visits were collected. A survey was conducted to assess clinician satisfaction and perspectives. Qualitative thematic analysis was performed to identify major themes among survey free responses.

Results: In a 2-year period, 225 patients were referred from the Massachusetts General Hospital neuro-ICU to the NRC. Of those, 105 (47%) were seen in clinic for at least one visit. The most common reasons for loss to follow-up were no shows (38%) and noncontracted insurance (21%). Twenty percent of visits were in-person (the rest were by telehealth). Forty-eight percent were new patients compared with return visits. The most common diagnoses were other (36%), ischemic stroke (26%), and traumatic brain injury (17%). An additional monthly multidisciplinary clinic has seen 14 patients with one no show. Clinicians found their experience in the NRC valuable. Identified benefits included interdisciplinary collaboration, being a more well-rounded and better clinician, improving effectiveness in managing post-ICU problems, and influencing ICU prognosis. Clinicians' greatest challenge was navigating resource limitations for patients.

Discussion: A postneuro-ICU NRC is a feasible model of care delivery for patients after severe acute neurologic disorders. Patients with a broad variety of diagnoses were seen in a 2-year period. Providers valued their clinic time and experiences. Future studies should evaluate whether this model of care improves patients' postneuro-ICU outcomes.

Background and objectives: There are few specialists that serve adults with neurodevelopmental disabilities (NDD), and most adults with NDD receive care from providers without specialty training in NDD. Care for this population is highly variable, and patient and caregiver priorities in this age group are not well known. We aimed to explore individual and caregiver values around adult neurodevelopmental care.

Methods: In this qualitative study, a qualitative analyst conducted 22 semistructured virtual interviews from September 2021 to February 2022 with randomly selected adults with NDD and/or their caregivers. Each individual with NDD had at least one appointment in the adult NDD clinic, which started in October 2020. Interviews were recorded and professionally transcribed. An inductive codebook was developed and reconciled through an iterative process; transcripts were coded in Atlas.ti with 20% double-coding. Major themes were developed through team discussion.

Results: Most interviewees were caregivers of patients with NDD (12); 9 interviews were with patient/caregiver dyads; 1 interview was with a patient alone. Three main themes emerged from the interviews. (1) Value in providers who are curious, engaged, and knowledgeable about NDD-related conditions, which individuals and caregivers referred to as "Unicorn Providers." (2) Value in a connected and coordinated web of care. (3) Value in comfortable and adaptable clinic spaces. They value clinical environments that foster patient success during visits.

Discussion: The need for adult neurodevelopmental care is growing as more individuals with NDD are living into adulthood. Better understanding of patient and caregiver values can help shape this emerging field to meet the needs of this unique, often overlooked and underserved, population.