Adult patients with IIH were identified from the 2016–2020 National Inpatient Sample databases, and surgical procedures and hospital characteristics were recorded. Temporal trends of procedure numbers for VSS, CSF shunts, and optic nerve sheath fenestrations (ONSFs) were assessed and compared.
A total of 46,065 (95% CI 44,710–47,420) patients with IIH were identified, of whom 7,535 patients (95% CI 6,982–8,088) received surgical IIH treatments. VSS procedures increased 80% (150 [95% CI 55–245] to 270 [95% CI 162–378] per year, p < 0.001). Concurrently, the number of CSF shunts decreased by 19% (1,365 [95% CI 1,126–1,604] to 1,105 [95% CI 900–1,310] per year, p < 0.001), and ONSF procedures decreased by 54% (65 [95% CI 20–110] to 30 [95% CI 6–54] per year, p < 0.001).
Practice patterns for surgical IIH treatment in the United States are rapidly evolving, and VSS is becoming increasingly common. These findings highlight the urgency of randomized controlled trials to investigate the comparative effectiveness and safety of VSS, CSF shunts, ONSF, and standard medical treatments.
Patients with ischemic stroke and intracerebral hemorrhage from the population-based Brain Attack Surveillance in Corpus Christi project (2009–2018) were compared by language preference in 90-day neurologic, functional, and cognitive outcomes using weighted Tobit regression. Models were adjusted for demographics, initial NIH Stroke Scale (NIHSS), medical history, stroke characteristics, and insurance status.
Of 1,096 stroke patients, 926 were English-speaking and 170 were Spanish-only–speaking. Spanish speakers were older (p < 0.01), received less education (p < 0.01), had higher initial NIHSS values (p = 0.02), had higher prevalence of atrial fibrillation (p < 0.01), and had lower prevalence of smoking (p = 0.01) than English speakers. In fully adjusted models, Spanish-only speakers had worse neurologic outcome (NIHSS, range 0–44 [higher worse], mean difference: 1.93, p < 0.01) but no difference in functional outcome measured by activities of daily living/instrumental activities of daily living or cognitive outcome compared with English speakers.
This population-based study found worse neurologic but similar functional and cognitive stroke outcomes among Spanish-only–speaking MA patients compared with English-speaking MA patients.
Patients with various epilepsies were evaluated with simultaneous EEGs during their response to visual stimuli in a single-flash test, a car-driving video game, and a realistic driving simulator. Reaction times (RTs) and missed reactions or crashes (miss/crash) during normal EEG and IEDs were measured. IEDs, as considered in this study, were a series of epileptiform potentials (>1 potential) and were classified as generalized typical, generalized atypical, or focal. RT and miss/crash in relation to IED type, duration, and test type were analyzed. RT prolongation, miss/crash probability, and odds ratio (OR) of miss/crash due to IEDs were calculated.
Generalized typical IEDs prolonged RT by 164 ms, compared with generalized atypical IEDs (77.0 ms) and focal IEDs (48.0 ms) (p < 0.01). Generalized typical IEDs had a session miss/crash probability of 14.7% compared with a zero median for focal and generalized atypical IEDs (p < 0.01). Long repetitive bursts of focal IEDs lasting >2 seconds had a 2.6% miss/crash probabilityIED. Cumulated miss/crash probability could be predicted from RT prolongation: 90.3 ms yielded a 20% miss/crash probability. All tests were nonsuperior to each other in detecting miss/crash probabilitiesIED (zero median for all 3 tests) or RT prolongations (flash test: 56.4 ms, car-driving video game: 75.5 ms, simulator 86.6 ms). IEDs increased the OR of miss/crash in the simulator by 4.9-fold compared with normal EEG. A table of expected RT prolongations and miss/crash probabilities for IEDs of a given type and duration was created.
IED-associated miss/crash probability and RT prolongation were comparably well detected by all tests. Long focal IED bursts carry a low risk, while generalized typical IEDs are the primary cause of miss/crash. We propose a cumulative 20% miss/crash risk at an RT prolongation of 90.3 ms as a clinically relevant IED effect. The IED-associated OR in the simulator approximates the effects of sleepiness or low blood alcohol level while driving on real roads. A decision aid for fitness-to-drive evaluation was created by providing the expected RT prolongations and misses/crashes when IEDs of a certain type and duration are detected in routine EEG.
We performed a cross-sectional retrospective study using standardized questionnaires for clinicians and caregivers of individuals with STXBP1-related disorders capturing medical histories, genetic findings, and developmental outcomes. Motor and language function were assessed using Gross Motor Function Classification System (GMFCS) scores and a speech impairment score and were compared within and across clinically defined subgroups.
We collected data of 71 individuals with STXBP1-related disorders, including 44 previously unreported individuals. Median age at inclusion was 5.3 years (interquartile range 3.5–9.3) with the oldest individual aged 43.8 years. Epilepsy was absent in 18/71 (25%) of individuals. The range of developmental outcomes was broad, including 2 individuals presenting with close to age-appropriate motor development. Twenty-nine of 61 individuals (48%) were able to walk unassisted, and 24/69 (35%) were able to speak single words. Individuals without epilepsy presented with a similar onset and spectrum of phenotypic features but had lower GMFCS scores (median 3 vs 4, p < 0.01) than individuals with epilepsy. Individuals with epileptic spasms were less likely to walk unassisted than individuals with other seizure types (6% vs 58%, p < 0.01). Individuals with early epilepsy onset had higher speech impairment scores (p = 0.02) than individuals with later epilepsy onset.
We expand the spectrum of STXBP1-related disorders and provide clinical features and developmental trajectories in individuals with and without a history of epilepsy. Individuals with epilepsy, in particular epileptic spasms, and neonatal or early-onset presented with less favorable motor and language functional outcomes compared with individuals without epilepsy. These findings identify children at risk for severe disease and can serve as comparator for future interventional studies in STXBP1-related disorders.
The Neuro-COVID Italy study was a multicenter, observational, cohort study with ambispective recruitment and prospective follow-up. Consecutive hospitalized patients presenting new neurologic disorders associated with COVID-19 infection (neuro-COVID), independently from respiratory severity, were systematically screened and actively recruited by neurology specialists in 38 centers in Italy and the Republic of San Marino. The primary outcomes were incidence of neuro-COVID cases during the first 70 weeks of the pandemic (March 2020–June 2021) and long-term functional outcome at 6 months, categorized as full recovery, mild symptoms, disabling symptoms, or death.
Among 52,759 hospitalized patients with COVID-19, 1,865 patients presenting 2,881 new neurologic disorders associated with COVID-19 infection (neuro-COVID) were recruited. The incidence of neuro-COVID cases significantly declined over time, comparing the first 3 pandemic waves (8.4%, 95% CI 7.9–8.9; 5.0%, 95% CI 4.7–5.3; 3.3%, 95% CI 3.0–3.6, respectively; p = 0.027). The most frequent neurologic disorders were acute encephalopathy (25.2%), hyposmia-hypogeusia (20.2%), acute ischemic stroke (18.4%), and cognitive impairment (13.7%). The onset of neurologic disorders was more common in the prodromic phase (44.3%) or during the acute respiratory illness (40.9%), except for cognitive impairment whose onset prevailed during recovery (48.4%). A good functional outcome was achieved by most patients with neuro-COVID (64.6%) during follow-up (median 6.7 months), and the proportion of good outcome increased throughout the study period (r = 0.29, 95% CI 0.05–0.50; p = 0.019). Mild residual symptoms were frequently reported (28.1%) while disabling symptoms were common only in stroke survivors (47.6%).
Incidence of COVID-associated neurologic disorders decreased during the prevaccination phase of the pandemic. Long-term functional outcome was favorable in most neuro-COVID disorders, although mild symptoms commonly lasted more than 6 months after infection.
In this prospective cohort study, we included FDRs, aged 20–70 years, of patients with UIA without a family history of aSAH who visited the Neurology outpatient clinic in 1 of 3 participating tertiary referral centers in the Netherlands. FDRs were screened for UIA with magnetic resonance angiography between 2017 and 2021. We determined UIA prevalence and developed a prediction model for UIA risk at screening using multivariable logistic regression. QoL was evaluated with questionnaires 6 times during the first year after screening and assessed with a linear mixed-effects model.
We detected 24 UIAs in 23 of 461 screened FDRs, resulting in a 5.0% prevalence (95% CI 3.2–7.4). The median aneurysm size was 3 mm (interquartile range [IQR] 2–4 mm), and the median 5-year rupture risk assessed with the PHASES score was 0.7% (IQR 0.4%–0.9%). All UIAs received follow-up imaging, and none were treated preventively. After a median follow-up of 24 months (IQR 13–38 months), no UIA had changed. Predicted UIA risk at screening ranged between 2.3% and 14.7% with the highest risk in FDRs who smoke and have excessive alcohol consumption (c-statistic: 0.76; 95% CI 0.65–0.88). At all survey moments, health-related QoL and emotional functioning were comparable with those in a reference group from the general population. One FDR with a positive screening result expressed regret about screening.
Based on the current data, we do not advise screening FDRs of patients with UIA because all identified UIAs had a low rupture risk. We observed no negative effect of screening on QoL. A longer follow-up should determine the risk of aneurysm growth requiring preventive treatment.
Participants (N = 940) were mean age 71 years (SD = 9) at incident stroke, 51% female, and 38% Black. Using adjusted multinomial logistic regression, the risk of having a more severe stroke (reference NIHSS ≤5) was higher among embolic stroke vs thrombotic stroke patients, with a step-wise increase for embolic stroke patients when moving from mild (odds ratio [OR] 1.95, 95% CI 1.14–3.35) to very severe strokes (OR 4.95, 95% CI 2.34–10.48). After adjusting for atrial fibrillation, there was still a higher risk of having a worse NIHSS among embolic vs thrombotic strokes but with attenuation of effect (very severe stroke OR 3.91, 95% CI 1.76–8.67). Sex modified the association between stroke subtype and severity (embolic vs thrombotic stroke, p interaction = 0.03, per severity category, females OR 2.38, 95% CI 1.55–3.66; males OR 1.75, 95% CI 1.09–2.82). The risk of death (median follow-up 5 years, interquartile range 1–12) was also increased for embolic vs thrombotic stroke patients (hazard ratio 1.66, 95% CI 1.41–1.97).
Embolic stroke was associated with greater stroke severity at the time of the event and a higher risk of death vs thrombotic stroke, even after careful adjustment for patient-level differences.
We searched the PubMed, Embase, Cochrane Library, and Web of Science databases to obtain articles related to mechanical thrombectomy for large ischemic core from inception until February 10, 2023. The primary outcome was independent ambulation (modified Rankin Scale [mRS] 0–3). Effect sizes were computed as risk ratio (RR) with random-effect or fixed-effect models. The quality of articles was evaluated through the Cochrane risk assessment tool and the Newcastle-Ottawa Scale. This study was registered in PROSPERO (CRD42023396232).
A total of 5,395 articles were obtained through the search and articles that did not meet the inclusion criteria were excluded by review of the title, abstract, and full text. Finally, 3 RCTs and 10 cohort studies met the inclusion criteria. The RCT analysis showed that EVT improved the 90-day functional outcomes of patients with large ischemic core with high-quality evidence, including independent ambulation (mRS 0–3: RR 1.78, 95% CI 1.28–2.48, p < 0.001) and functional independence (mRS 0–2: RR 2.59, 95% CI 1.89–3.57, p < 0.001), but without significantly increasing the risk of symptomatic intracranial hemorrhage (sICH: RR 1.83, 95% CI 0.95–3.55, p = 0.07) or early mortality (RR 0.95, 95% CI 0.78–1.16, p = 0.61). Analysis of the cohort studies showed that EVT improved functional outcomes of patients without an increase in the incidence in sICH.
This systematic review and meta-analysis indicates that in patients with LVO stroke with a large ischemic core, EVT was associated with improved functional outcomes over MM without increasing sICH risk. The results of ongoing RCTs may provide further insight in this patient population.
Data of mild noncardioembolic stroke patients with admission NIH Stroke Scale (NIHSS) score ≤3 who received IVT or early DAPT in the period 2018–2021 were extracted from a nationwide, prospective stroke unit registry. Study endpoints included symptomatic intracerebral hemorrhage (sICH), early neurologic deterioration ≥4 NIHSS points (END), and 3-month functional outcome by modified Rankin scale (mRS).
A total of 1,195 mild stroke patients treated with IVT and 2,625 patients treated with DAPT were included. IVT patients were younger (68.1 vs 70.8 years), had less hypertension (72.8% vs 83.5%), diabetes (19% vs 28.8%), and a history of myocardial infarction (7.6% vs 9.2%), and slightly higher admission NIHSS scores (median 2 vs median 1) when compared with DAPT patients. After propensity score matching and multivariable adjustment, IVT was associated with sICH (4 [1.2%] vs 0) and END (adjusted odds ratio [aOR] 2.8, 95% CI 1.1–7.5), and there was no difference in mRS 0–1 at 3 months (aOR 1.3, 95% CI 0.7–2.6).
This analysis from a prospective nationwide stroke unit network indicates that IVT is not superior to DAPT in the setting of mild noncardioembolic stroke and may eventually be associated with harm. Further research focusing on acute therapy of mild stroke is highly warranted.
This study provides Class III evidence that IVT is not superior to DAPT in patients with acute mild (NIHSS score ≤3) noncardioembolic stroke. The study lacks the statistical precision to exclude clinically important superiority of either therapy.
Adults in acute coma after cardiac arrest were included in a retrospective database involving 7 hospitals. The combination of 3 quantitative EEG features (burst suppression ratio [BSup], spike frequency [SpF], and Shannon entropy [En]) was used to define 5 distinct neurophysiology states: epileptiform high entropy (EHE: SpF ≥4 per minute and En ≥5); epileptiform low entropy (ELE: SpF ≥4 per minute and <5 En); nonepileptiform high entropy (NEHE: SpF <4 per minute and ≥5 En); nonepileptiform low entropy (NELE: SpF <4 per minute and <5 En), and burst suppression (BSup ≥50% and SpF <4 per minute). State transitions were measured at consecutive 6-hour blocks between 6 and 84 hours after return of spontaneous circulation. Good neurologic outcome was defined as best cerebral performance category 1–2 at 3–6 months.
One thousand thirty-eight individuals were included (50,224 hours of EEG), and 373 (36%) had good outcome. Individuals with EHE state had a 29% rate of good outcome, while those with ELE had 11%. Transitions out of an EHE or BSup state to an NEHE state were associated with good outcome (45% and 20%, respectively). No individuals with ELE state lasting >15 hours had good recovery.
Transition to high entropy states is associated with an increased likelihood of good outcome despite preceding epileptiform or burst suppression states. High entropy may reflect mechanisms of resilience to hypoxic-ischemic brain injury.
The observed longitudinal changes seen in football, and especially concussed athletes, could reveal diminished myelination, altered axonal calibers, or depressed pruning processes leading to a static, nondecreasing axonal dispersion. This prospective longitudinal study demonstrates divergent tract-specific trajectories of brain microstructure, possibly reflecting a concussive and repeated subconcussive impact-related alteration of white matter development in football athletes.
Approximately 10 years after publication of the baseline characteristics of the 126 initial patients of the French Late-Onset Pompe Disease registry, we provide here an update of the clinical and biological features of patients included in this registry.
We describe 210 patients followed at 31 hospital-based French neuromuscular or metabolic centers. The median age at inclusion was 48.67 ± 14.91 years. The first symptom was progressive lower limb muscle weakness, either isolated (50%) or associated with respiratory symptoms (18%), at a median age of 38 ± 14.9 years. At inclusion, 64% of the patients were able to walk independently and 14% needed a wheelchair. Positive associations were found between motor function measure, manual motor test, and 6-minute walk test (6MWT) results, and these parameters were inversely associated with the time taken to sit up from a lying position at inclusion. Seventy-two patients had been followed for at least 10 years in the registry. Thirty-three patients remained untreated a median of 12 years after symptom onset. The standard ERT dose was administered for 177 patients.
This update confirms previous findings for the adult population included in the French Pompe disease registry, but with a lower clinical severity at inclusion, suggesting that this rare disease is now diagnosed earlier; thanks to greater awareness among physicians. The 6MWT remains an important method for assessing motor performance and walking ability. The French Pompe disease registry provides an exhaustive, nationwide overview of Pompe disease and can be used to assess individual and global responses to future treatments.