Neurology. Clinical practice最新文献

Background and objectives: Diagnostic criteria for anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated diseases (MOGADs) were published in 2023, but debate regarding optimal therapeutic strategies for pediatric MOGAD exists. The aim of this study was to evaluate treatment approaches and preferred diagnostic investigations for pediatric MOGAD among neurologists.

Methods: A survey questionnaire focused on pediatric MOGAD treatment was launched through the Practice Current Section of Neurology® Clinical Practice in April-October 2024. Responses from neurologists were solicited through advertisements on American Academy of Neurology (AAN) social media platforms, on the AAN website, and in print editions of Neurology®, as well as through QR codes shared at professional neurologic meetings. The questionnaire included 12 questions evaluating clinical decision making after a first and second neuroinflammatory episode, in a child testing positive for MOG-IgG antibody. Demographic questions were included. Responses were evaluated using descriptive statistics. A comparative analysis was conducted between those who self-identified as neuroimmunologists (NIs) and those who did not.

Results: A total of 346 neurologists completed the survey (52.3% of general neurologists, 32.1% of NIs, and 15.6% in other neurology fields). Of all respondents, 90.5% chose to send serum MOG-IgG antibody after the first event (59.7% serum, 36.4% CSF + serum). For acute treatment, 84.1% chose to give a 3-5-day course of high-dose IV steroids. Approaches to steroid tapering varied, with 33.0% choosing a 2-4-week taper, 27.2% choosing a 7-12-week taper, and 21% not offering a steroid taper. 56.6% of non-NIs chose to initiate maintenance therapy after the first episode while only 18.9% of NIs chose to do so. After the second episode, 98.3% of all respondents recommended starting maintenance therapy, with rituximab (RTX) (37.1%) being the most frequently chosen agent, followed by monthly IV immunoglobulin (IVIG) (25.6%) and azathioprine (17.1%). NIs selected monthly IVIG (50%) over RTX (27.3%). The duration of treatment in relapsing cases varied: 42.9% elected to maintain treatment for 2 years or less and 35.3% for more than 2 years, and 21.8% chose to continue treatment indefinitely.

Discussion: The survey demonstrated substantial variability in management decisions related to MOGAD among neurologists, reflecting current gaps in knowledge about therapies for MOGAD. Future efforts are needed to improve the uptake of knowledge and ensure that current guidelines are effectively translated into clinical practice.

Background and objectives: Data on how percutaneous endoscopic gastrostomy (PEG) utilization has changed over time in patients with acute ischemic stroke (AIS) in the United States are sparse. This study evaluates 17-year trends in PEG utilization in AIS hospitalizations in the United States.

Methods: The 2006-2022 National Inpatient Sample was used to conduct a serial cross-sectional study. International Classification of Diseases codes were used to identify primary AIS hospitalizations with and without PEG. We used joinpoint regression to compute the annualized percentage change (APC) in PEG usage over time and used multivariable regression to evaluate the association of IV thrombolysis (IV-tPA), mechanical thrombectomy (MT), and other hospitalization factors with odds of PEG use.

Results: Of 8,079,538 primary AIS admissions over the study period, the overall PEG prevalence was 3.9% but usage in the subset of AIS admissions undergoing MT was 11.0%. PEG utilization increased with age in both sexes (18-39-year-olds: men 2.7%; women 2.5%; 80 years or older: men 4.7% and women 4.6%). After multivariable adjustment, increasing Elixhauser comorbidity scores (OR 1.31, 95% CI 1.30-1.32) and MT utilization (OR 1.61, 95% CI 1.54-1.68) were associated with higher odds of PEG use while IV-tPA was associated with lower odds of utilization (OR 0.94, 95% CI 0.91-0.97) when compared with no treatment. PEG use declined by 2.9% annually across the study period (average APC 2.9%, 95% CI -3.2 to -2.5%), but the rate of decline was fastest in the period 2014-2018 (APC -7.4%, 95% CI -8.6 to -4.9), followed by 2006-2014 (APC -1.9%, 95% CI -2.5 to -0.9). Utilization did not change significantly in the period 2018-2022. The average time from admission to PEG placement was 9.3 days, and this increased progressively over time (p trend <0.001).

Discussion: PEG use in patients with AIS declined over the past decade. This decline is likely due to multifactorial reasons that warrant further studies, but changing clinical practice toward allowing for some more time for patients with AIS to recover from poststroke dysphagia may be one of the potential contributory factors.

Background and objectives: Stereoelectroencephalography (SEEG) is increasingly used worldwide for epilepsy presurgical evaluation. A data-driven view of SEEG practice evolution is lacking for any world region. We aimed to perform a nationwide survey of SEEG use in Australian adult epilepsy centers. We predicted that the proportion of nonlesional MRI cases, number of electrodes, and radiofrequency thermocoagulation (RF-TC) use would increase over time.

Methods: All epilepsy centers in Australia undertaking SEEG in patients older than 16 years at any time were invited to participate in a nationwide survey. Data were retrospectively and/or prospectively collected on patient demographics and SEEG exploration. Descriptive statistics were used.

Results: A total of 281 SEEG explorations were performed in 8 centers from 2012 until 2023, 278 of which (99%) are reported here. A definite epileptogenic MRI lesion was present in 30.6% with no overall significant growth in the proportion of nonlesional cases (χ² [1, N = 278] = 0.067, p = 0.79), although this varied per center. Patients identifying as Indigenous Australians comprised 1.6% of this national SEEG survey vs an estimated national indigenous population of 3.8%. The median number of electrodes increased from 8 in 2012 to 15 in 2023. A total of 174 patients (62.6%) were offered resective surgery after SEEG. Surgery was performed with 1-year follow-up in 114, of whom 72.8% were seizure-free at the last follow-up. The presence/absence of an MRI lesion was not associated with postoperative seizure-free outcomes (χ² [1, N = 114] = 0.06, p = 0.80). RF-TC use differed between centers and evolved, with a crossover to the majority use of RF-TC occurring 5 years after regulatory approval.

Discussion: This comprehensive nationwide and regional survey of SEEG practice comprises full ascertainment of all centers and allows analysis of changes in practice over time. Whole cohort results show a high proportion of nonlesional MRI cases achieving satisfactory surgical outcomes and evolution to increased RF-TC use. Indigenous access to SEEG seems to be lower than expected, consistent with broader health care access issues for this population group. Nationwide collaboration on SEEG data provides a basis for analysis of practice at the individual center and state and national levels to optimize health care planning and sharing of expertise.

Purpose of review: While chronic subdural hematoma (cSDH) has been considered a neurosurgical disease, conventional management with surgical evacuation has been associated with high rates of disease recurrence and long-term patient morbidity and mortality. In this narrative review, we summarize the current knowledge regarding the epidemiology, pathophysiology, and treatment modalities for cSDH with a particular focus on middle meningeal artery embolization (MMAE) and other novel treatment modalities.

Recent findings: A growing body of literature has suggested that inflammation and angiogenesis may play a central role in cSDH pathophysiology, and major advances have been made on nonsurgical treatment modalities for cSDH such as MMAE and antiangiogenic agents. Furthermore, recent studies, including several large randomized controlled trials, have confirmed that MMAE is generally an effective treatment for promoting cSDH resorption and reducing recurrence rates.

Summary: Chronic SDH is a common neurovascular disease that is expected to increase in incidence because of global population aging and widespread use of antithrombotic medications. The current pathophysiologic understanding suggests that cSDHs may form because of a positive feedback loop of inflammation, angiogenesis, and persistent exudation of blood into the subdural space. Surgical management is the standard treatment for relieving acute neurologic deficits; however, rates of cSDH recurrence are high and risks of perioperative morbidity and mortality are substantial. Conservative medical management options for cSDH are limited. MMAE is a novel treatment with high-quality data from multiple randomized trials suggesting efficacy regarding preventing cSDH recurrence and promoting hematoma resorption.

Background and objectives: The field of neurology lacks neurocritical care (NCC) quality measures because of the complexity of neurologic disorders and challenges in data collection, hindering efforts to assess care quality and improve patient outcomes. This study examined the feasibility, usability, and scientific acceptability of 3 new facility-level neurology electronic clinical quality measures (eCQMs): (1) adult patients with generalized convulsive status epilepticus (GCSE) treated with benzodiazepine within 20 minutes of arrival to the emergency department, (2) dexamethasone administration before or within an hour of the first dose of antibiotics in patients with acute bacterial meningitis (BM), and (3) immunomodulatory therapy [plasma exchange or IV immunoglobulin] for myasthenic crisis (MG).

Methods: We evaluated the feasibility, usability, face validity, and reliability of the 3 eCQMs across 3 sites in the United States using a combination of qualitative and quantitative methodologies. The feasibility of reporting was evaluated by the percentage of data elements that do not require manual patient record review. Face validity was determined through semistructured clinician interviews and web survey, and usability was assessed through clinician interviews. Signal-to-noise reliability was calculated based on the testing data obtained from 3 hospitals.

Results: The total number of denominator-eligible patients in the sample was 86 (GCSE), 88 (BM), and 193 (MG). The mean measure rates were as follows: GCSE, 12.3% (11.3%-13.3%); BM, 23.3% (10.8%-35.7%); and MG, 64.2% (46.2%-81.4%). All 3 eCQMs had high face validity (GCSE: 90%, BM: 90%, MG: 94%). The measures had high reliability: GCSE, 0.890 (0.816-0.963); BM, 0.817 (0.669-0.964); and MG, 0.958 (0.948-0.969). We found high feasibility for MG; however, BM and GCSE had feasibility concerns for several key data elements. Clinicians supported the measures' potential to improve care but expressed several usability concerns.

Discussion: The eCQMs showed potential for being used for quality improvement. However, significant barriers to feasibility include inconsistent recording of diagnosis and procedure codes in electronic health records and underestimation of denominator and/or numerator cases, which hinder reporting in Centers for Medicare & Medicaid Services quality improvement programs. Continued refinement of the eCQM specifications is required before they can be implemented to enhance their impact on NCC quality.

Backgrounds and objectives: Managing multiple sclerosis (MS) during pregnancy and postpartum is a therapeutic challenge. We aim to describe therapy regimens, clinical and MRI disease activity, and serum neurofilament light chain (sNfL) levels in all pregnancies observed between 2012 and 2023 among participants of the Swiss MS cohort.

Methods: We assessed the treatment strategies during pregnancy and 1 year postpartum in all included pregnancies. We compared pregnancies continuously exposed to high-efficacy disease-modifying therapies (cHET: anti-CD20 monoclonal antibodies (aCD20) and natalizumab [NTZ]) with those where other therapeutic regimens (OTRs) were used. Disease activity was assessed by occurrence of relapses or new or enlarging T2w lesions (neT2Ls), as well as sNfL Z scores. We estimated odds ratios adjusted for age, disease duration, Expanded Disability Status Scale (EDSS) score, and previous relapse rate using generalized estimating equation (GEE) models for the relapse endpoint and using Firth logistic regression for MRI activity. sNfL Z scores were compared between treatment categories at sampling using GEE models.

Results: We analyzed 123 pregnancies in 93 women (median age [interquartile range {IQR}] 32.2 years [29.3, 35.7]; EDSS score [IQR] 1.5 [1.0, 2.0]). The last disease-modifying therapy (DMT) before birth was NTZ in 29 (23.5%) and aCD20 in 25 (20.3%) pregnancies; of those, 3 and 24 were exposed until birth, respectively (cHET). Fingolimod was the last treatment before birth in 25 pregnancies (20.3%), stopped in all before or after confirmation of pregnancy. Other DMTs were used in 39 pregnancies (31.7%); 5 remained untreated. Compared with cHET, patients with pregnancies under OTRs had higher proportions of relapses (34.4% vs 13.0%; n = 113, OR 4.52, 95% CI [1.35-15.11], p = 0.0142) and neT2Ls (40.9% vs 3.8%; n = 91, OR 9.15, 95% CI [2.14-85.21], p = 0.0013). sNfL Z scores during pregnancy and postpartum were higher in patients untreated at sampling compared with patients under high-efficacy DMT (HET) (+0.43 Z score units, 95% CI [0.05-0.81], p = 0.0255). No serious adverse events were observed.

Discussion: Treatment strategies for pregnant patients with MS were heterogeneous, and continuous exposure to HET showed superior efficacy against acute disease activity compared with other DMTs or no treatment. Further studies are needed to confirm these results and assess maternal and fetal longer term outcomes.

Background and objectives: The literature on severe West Nile virus (WNV) neuroinvasive disease (WNND) in patients treated with anti-CD20 therapies is limited. We systematically characterize cases of WNND in the tertiary academic UCHealth system.

Methods: A retrospective cohort (January 2016 to January 2024) of patients with a validated diagnosis of WNND and anti-CD20 medication use was identified with electronic medical record query followed by individual chart review.

Results: We identified 25 patients; multiple sclerosis was the most common indication for anti-CD20 therapy in 13 patients (52%). Twenty-one patients (84%) presented with meningoencephalitis. CSF WNV IgM was positive in 5 of 21 patients (24%) who were tested while 13 of 14 tested patients (93%) had positive reverse-transcription PCR (RT-PCR) findings in the CSF. MRI demonstrated anomalies associated with WNND in 12 of 23 patients (52%) with available imaging. Intensive care unit admission was required in 8 patients (32%), and 12 patients (48%) were treated with intravenous immunoglobulin. Worsening of ≥1 point from pre-WNV baseline modified Rankin Scale (mRS) score to the 90-day postdischarge mRS score was seen in 18 patients (75%). Two patients (8%) died by 90 days.

Discussion: WNND leads to disability accrual in patients on B cell-depleting anti-CD20 therapies. Utilization of RT-PCR is important in optimizing diagnosis in this patient population because of limited sensitivity of the WNV-IgM testing commonly used in the general population.

[This corrects the article DOI: 10.1212/CPJ.0000000000200463.].

Background and objectives: The aim of this study was to explore practice patterns in managing mild cognitive impairment (MCI). The investigation and management of MCI is considered important because it offers the opportunity to potentially stave off conversion to dementia. However, there are few data on current practices/approaches in this area, especially worldwide; such data can help identify potential disparities and anticipate adoption of new therapies.

Methods: We performed a worldwide electronic survey of neurology practitioners through the Practice Current section of Neurology® Clinical Practice with clinical and practice-related questions in November 2019-August 2021 and repeated it in May-October 2023 after the FDA's approval of aducanumab and lecanemab but before the approval of donanemab. Clinical questions addressed access to and utilization of diagnostic investigations, pharmacologic and nonpharmacologic management of MCI, and (in 2023) attitudes toward novel anti-amyloid agents. Responses were compared using the Fisher exact test and multivariable logistic regression adjusted for region, regional income, year of survey response, years in practice, and number of cognitive patients seen annually.

Results: We received 1,257 responses from 95 countries, including 237 cognitive subspecialists and 464 respondents from low-/middle-income countries. On multivariable analysis, cognitive subspecialists were more likely than other practitioners to investigate MCI with a lumbar puncture (aOR 1.90, 95% CI 1.32-2.73), luorodeoxyglucose-PET (FDG-PET) (aOR 1.45, 95% CI 1.00-2.10) and to offer pharmacotherapy if investigations suggested neurodegeneration (aOR 1.92, 95% CI 1.29-2.85). Regionally, respondents from Europe, Latin America, and Asia were more likely than those from the United States/Canada to order FDG-PET (e.g., Europe: aOR 2.38, 95% CI 1.29-4.39) and amyloid PET (Europe: aOR 3.30, 95% CI 1.85-5.87), controlling for reported access to these tests. Pharmacologic and nonpharmacologic approaches were comparable between cognitive subspecialists and other respondents. Despite concerns about safety (77.1% expressed being somewhat or very concerned), attitudes toward prescribing new anti-amyloid agents were similar among all respondents, reflecting a generally favorable attitude (e.g., 62% would prescribe anti-amyloid therapy if it was available).

Discussion: Our results highlight practice differences among cognitive subspecialists and other practitioners worldwide in the management of MCI. Attitudes toward anti-amyloid therapy indicate cautious optimism, with concerns about side effects but a general interest to prescribe.