Neurology最新文献

Macroglossia can be seen in multiple conditions, but its evaluation becomes more challenging when approached as an isolated presenting symptom. This is a case of a 65-year-old patient with isolated progressive tongue hypertrophy of unclear etiology for 5 years. We navigate the causes of macroglossia and discuss the clinical and diagnostic procedures that helped us narrow the differential diagnoses for our patient. We emphasize searching for evidence of more systemic involvement and the use of appropriate genetic testing to change the course of the disease and avoid therapeutic delay.

Objectives: In aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), disability accrual is mostly attributed to relapses. This study aimed to assess the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in AQP4-IgG NMOSD.

Methods: This was a retrospective cohort study of patients with AQP4-IgG NMOSD enrolled in the MSBase international data registry. Patients required a minimum of 3 recorded Expanded Disability Status Scale (EDSS) scores: baseline, event, and a 6-month confirmation score. Presence and absence of relapses between the baseline and event EDSS scores determined RAW and PIRA, respectively. Descriptive statistics were used to present the results.

Results: A total of 181 patients followed for a median of 4.5 years (Q1 1.7, Q3 7.8) were included. Most patients were female (88.4%), and the median age at disease onset was 38.1 years. Overall, 4 patients (2.2%) developed 5 incidences of PIRA and 13 patients developed RAW (7.2%).

Discussion: This multicenter study highlights that PIRA is very rare in AQP4-IgG NMOSD. Limitations of this study include the sole focus of overall EDSS to measure disability, lack of requirement for a second EDSS score to confirm baseline EDSS, and the absence of magnetic resonance imaging information for all patients.

Objectives: The objective of this study was to quantify trends (2008-2019) in stroke outcomes by race-ethnicity.

Methods: Patients with ischemic stroke from a population-based study were interviewed at 90 days to assess outcomes. Linear regression with multiple imputation and inverse probability weighting was used to model trends.

Results: The median age was 66 years (n = 1,449); 61% were Mexican American (MA). QOL remained stable with no race-ethnic difference in trends (p for time*race-ethnicity interaction = 0.81). Neurologic outcomes improved for MA (p < 0.01) but not non-Hispanic White (NHW) persons with stroke (p = 0.23) with no race-ethnic difference in trends (p for interaction = 0.23). For functional outcomes, trends were stable and then improved in MA persons with stroke (p for interaction = 0.01), whereas trends were stable in NHW persons with stroke (p = 0.52). For cognitive outcomes, there was little change in NHW persons with stroke (p = 0.50); in MA persons with stroke, there was improvement followed by decline and then improvement (p = 0.03). No race-ethnic differences in trends in functional (p for interaction = 0.51) or cognitive (p for interaction = 0.21) outcomes were noted.

Discussion: Outcome improvements were noted in MA but not NHW persons with stroke; race-ethnic differences were not present in 2019. Understanding factors contributing to favorable trends in MA persons may be informative for improving outcomes in all persons.

Age is an important risk factor of stroke, cognitive decline, and dementia. Senescent endothelial cells (ECs) accumulate with advancing age through exposure to cellular stress, such as that exerted by hypertension and diabetes. These senescent ECs have altered characteristics, such as altered tight junction proteins, use of a more indiscriminate transcellular transport system, increased inflammation, and increased immune cell interactions. ECs are the main component of the blood-brain barrier (BBB), separating the brain from systemic circulation. As senescent ECs accumulate in the BBB, their altered functioning results in the disruption of the barrier. They have inadequate barrier-forming properties, disrupted extracellular matrix, and increased transcytosis, resulting in an overly permeable barrier. This disruption of the BBB can have important effects in stroke and cognitive impairment, as presented in this review. Besides increasing the permeability of the BBB, senescent ECs can also impair angiogenesis and vascular remodeling, which in ischemic stroke may increase risk of hemorrhagic transformation and worsen outcomes. Senescent ECs may also contribute to microvascular dysfunction, with disruption of cerebral perfusion and autoregulation. These may contribute to vascular cognitive impairment along with increased permeability. With an aging population, there is growing interest in targeting senescence. Several ongoing trials have been evaluating whether senolytics can slow aging, improve vascular health, and reduce the risk of stroke and cognitive decline.

Functional neurologic disorder is common and a significant cause of disability and stress in neurologic patients. The nature of this disorder has been unclear. Originally called hysteria, the disorder interested Charcot who postulated that a functional lesion, la lésion dynamique, was responsible. Recent studies of functional neurologic disorders now allow us to understand what la lésion dynamique is and identifies these disorders without ambiguity as arising from the brain. Functional neurologic disorders are best understood as a multifactorial process with a biopsychosocial model. There can be a genetic predisposition. Commonly there is early life trauma that leads to a developmental abnormality of the amygdala, including loss of inhibition. This abnormality can be considered a predisposing factor. When stressed, the amygdala becomes hyperactive, driving the limbic system to cause widespread network dysfunction in the brain. This dysfunction can improve, correlating with clinical improvement. Network dysfunction is becoming recognized as an important pathologic process in neurology and psychiatry, as real as any other pathology. We should be able to make progress in helping patients with functional neurologic disorders with this understanding of la lésion dynamique.