Neurology最新文献

Background and objectives: Costs for neurologic medications have increased considerably in recent years. Since 2014, more than 30 neurologic medications have been approved by the US Food and Drug Administration (FDA) for neurologic conditions. This study aims to characterize recent trends in annual costs and aggregate spending from 2012 to 2021 for new-to-market (NTM) medications for 9 neurologic conditions.

Methods: We used the Merative MarketScan commercial and Medicare supplemental databases to observe patients seen by a neurologist with neurologic diseases with newly FDA-approved medications from 2014 to 2021: amyotrophic lateral sclerosis (ALS), transthyretin amyloidosis (ATTR), Duchenne muscular dystrophy (DMD), Huntington disease (HD), myasthenia gravis (MG), migraine, orthostatic hypotension (OH), tardive dyskinesia (TD), and spinal muscular atrophy (SMA). Patients were included if they had ≥1 disease-related prescription medication fill from 2012 to 2021. NTM (medications approved from 2014 to 2021) and older evidence-based guideline-supported medications were observed annually. Outcomes examined were annual and aggregate out-of-pocket (OOP) and total medication costs.

Results: We identified 2,687 unique individuals with ALS, 38 with ATTR, 69 with DMD, 884 with HD, 9,984 with MG, 441,099 with migraine, 4,723 with OH, 1,266 with TD, and 17 with SMA. The youngest population was DMD (mean = 25 years [SD = 7]), and the oldest was TD (mean = 66 years [SD = 14]). For DMD, the population was 99% male and for migraine, the population was 84% female, and the other conditions had more relatively even sex divides. Collectively, migraine medications had the largest increase in aggregate costs (1993%) and had a substantial increase in OOP costs on average by 234% ($86-$288). Eculizumab for MG was an extreme outlier, with OOP costs increasing by 4,099% ($413-$17,359) and aggregate OOP costs by 7,005% ($5,375-$381,894). OOP costs of edaravone ($304-$5,707) and deutetrabenazine ($670-$7,170) sharply increased by 1,775% and 971%, respectively.

Discussion: NTM medications for neurologic conditions have substantial and increasing individual and societal costs, which was not observed for older generic medications. These data suggest a need for policies to limit the financial burden of NTM medications on patients with neurologic conditions.

Headache accompanied by eosinophilia in blood or CSF presents a complex clinical challenge. We present a case of a 26-year-old woman with headache, peripheral eosinophilia with meningeal enhancement, and intracranial vasculopathy on imaging. This case illustrates a systematic approach to a patient with meningitis, peripheral eosinophilia, and cerebral vasculitis, which includes comprehensive clinical history taking and appropriate investigative workup to differentiate between infectious and noninfectious causes and timely tissue sampling to conclusively determine the causative agent.

Background and objectives: Hypertension is an important modifiable risk factor of Alzheimer disease (AD), but previous studies reported heterogeneous associations of late-life blood pressure (BP) with brain amyloid and tau pathologies. We investigated how the associations of BP with brain amyloid and tau vary by APOE ε4 carriership, age, cerebrovascular burden, and cognitive status.

Methods: We performed analyses among participants with postmortem neuropathology measurements (2005-June 2022) from the National Alzheimer's Coordinating Center. The average systolic BP (SBP) of the first 3 annual visits was the primary exposure, and baseline hypertension status was the secondary exposure. Brain AD pathologies were assessed using Thal and Braak staging. Potential modifiers included APOE ε4 carriership, age, stroke history, and cognitive status. Multinomial logistic regressions with interaction terms were used to test effect modification, adjusting for age, sex, APOE ε4 carriership, education, antihypertensive medication use, and years to death.

Results: Among 2,094 participants (baseline age: 75 ± 9.5 years; 51.4% women), the association of higher SBP with higher amyloid and tau burdens varied by stroke history and cognitive status while the effect modification by age or APOE ε4 carriership was less consistent. More pronounced associations of SBP with higher amyloid and tau burdens were observed in those with dementia (vs without dementia) and those with a history of stroke (vs without stroke) (All p interaction<0.05). The odds ratios (ORs) per 10-mm Hg increase in SBP in the stroke vs nonstroke subgroup were 1.58 (95% CI 1.04-2.41) vs 1.14 (1.03-1.27) for amyloid and 1.54 (1.00-2.36) vs 1.04 (0.96-1.12) for tau. When comparing dementia with cognitively normal subgroups, ORs were 1.39 (1.17-1.64) vs 1.12 (0.96-1.31) for amyloid and 1.24 (1.08-1.42) vs 0.98 (0.85-1.14) for tau. Similar findings were observed for baseline hypertension status.

Discussion: Preexisting cerebrovascular burden and cognitive status might interact with elevated SBP in their association with higher brain amyloid and tau, which could help identify high-risk subgroups for BP management and AD prevention. These heterogeneous association patterns need to be confirmed in longitudinal studies with in vivo AD pathology assessments.

Background and objectives: Cerebral palsy (CP) is the most prevalent physical disability in children and is often accompanied by other neurodevelopmental disorders (NDDs) such as attention deficit hyperactivity disorder (ADHD). Both conditions are influenced by genetic and environmental factors and significantly affect daily functioning. This study aims to estimate the prevalence of ADHD in school-aged children with CP from a large, population-based registry and explore associated factors including sex, material and social deprivation, epilepsy, prematurity, CP subtype, and motor functioning.

Methods: This cross-sectional study linked a population-based registry (the Registre de la paralysie cérébrale du Québec [CP Registry]) and 2 administrative health claims databases (the Régie de l'assurance maladie du Québec [RAMQ] and Maintenance et Exploitation des Données pour l'Étude de la Clientèle Hospitalière). The study included children diagnosed with CP born between 1999 and 2002, tracked through these databases. ADHD diagnosis was identified using International Classification of Diseases codes and specific ADHD medication prescriptions. Odds ratios and 95% confidence intervals were used to explore factors associated with an ADHD diagnosis.

Results: The study comprised 302 children with CP and 6,040 controls matched by age, sex, and region. The prevalence of ADHD in the CP cohort was significantly higher (38%) compared with the control group (12%). Univariate analysis showed that odds of ADHD in the CP cohort were higher in male children (OR 1.63, 95% CI 1.02-2.62) and individuals with no epilepsy diagnosis (OR 1.70, 95% CI 1.02-2.87), a spastic hemiplegic CP subtype (OR 1.87, 95% CI 1.10-3.20), and less severe motor impairment (OR 2.48, 95% CI 1.37-4.65). In the multivariate analysis, odds of ADHD were only higher in those with less severe motor impairment (OR 2.02, 95% CI 1.07-3.94).

Discussion: ADHD is significantly more prevalent among children with CP compared with their peers, aligning with previous literature that suggests a neurodevelopmental overlap. The study highlights the importance of considering NDDs in CP management, particularly ADHD, which may contribute to the challenges faced by these children. Future research is needed to explore the neurobiological links between CP and ADHD and the impact of NDDs on health outcomes in this population.

Background and objectives: Overweight and obesity in pregnant women are a growing problem contributing to increased risks of obstetric and perinatal complications. However, the impact of maternal overweight and obesity on the risk of perinatal stroke in the infant remains unexplored. We aimed to evaluate the association between maternal early pregnancy body mass index (BMI) and risk of perinatal ischemic stroke.

Methods: This nationwide cohort study includes singleton births in Sweden at ≥22 + 0 weeks without major congenital malformations, between January 1, 1998, and December 31, 2019, with a follow-up time of up to 28 days after birth. Data were obtained by individual record linkages of nationwide Swedish registers. Exposure was maternal BMI in early pregnancy. The outcome, perinatal ischemic stroke, was defined as a diagnosis of ischemic stroke at ≤28 days of age in the Medical Birth Register, the National Patient Register, or the Swedish Neonatal Quality Register. Multivariable Poisson log-linear regressions and spline regression were used to estimate adjusted rate ratios (aRRs) and 95% CIs.

Results: Among the 2,140,852 births, 415 infants (192 girls) were diagnosed with perinatal ischemic stroke. Rates of perinatal ischemic stroke increased from 19/100,000 in infants to normal-weight women (BMI 18.5 < 25 kg/m²) to 22/100,000 among infants to mothers with overweight (BMI 25 < 30 kg/m²), to 35/100,000 among infants to women with obesity class II (BMI 30 < 35 kg/m²), and to 40/100,000 among infants to women with obesity class III (BMI ≥35 kg/m²). The adjusted rate ratio of perinatal ischemic stroke increased almost linearly with increasing maternal BMI. When estimating risk per BMI class, aRRs of perinatal ischemic stroke were 1.16 (95% CI 0.91-1.46) for overweight, 1.82 (95% CI 1.34-2.44) for obesity class I, and 1.96 (95% CI 1.27-2.91) for obesity classes II-III, compared with infants of mothers with normal weight.

Discussion: The risk of perinatal ischemic stroke increased with increasing maternal BMI in a dose-response manner. The findings support maternal obesity as a potential risk factor of perinatal ischemic stroke. A limitation of this study was that although the perinatal ischemic stroke diagnosis has high predictive value in Swedish registers, we cannot rule out that cases might be underdetected.