Search Results (6)

Multiple sclerosis (MS) is a chronic autoimmune-mediated neurodegenerative disease that affects young adults. The diagnosis of MS currently based on the McDonald criteria, which based on four core principles: the presence of a symptomatic demyelinating syndrome, an objective neurologic finding, the dissemination in space (DIS), and the dissemination in time (DIT). In addition, the diagnosis of MS relies on the exclusion of any alternative diagnosis. This may implicate the absence of systemic non-neurological symptoms and signs, such as rheumatological, cutaneous, or ophthalmological findings. Nevertheless, the non-neurological symptoms are commonly observed in patients with MS either at the onset of MS, which therefore can delay the diagnosis and the incrementation of a disease-modifying therapy, or during the course of the disease progression. The purpose of our review is to highlight non-neurological symptoms of MS that frequently go undiagnosed or mistakenly linked to other conditions, aiming for the more accurate and earlier diagnosis of MS. Full article

Narcolepsy is a chronic central disorder of hypersomnolence most frequently arising during childhood/adolescence. This review article examined the literature concerning the etiology, prevalence, clinical course, and treatment of children with type 1 narcolepsy (NT1). Core symptoms of pediatric NT1 include excessive daytime sleepiness (EDS) and cataplexy, together with disrupted night sleep, sleep paralysis, and hypnagogic and hypnopompic hallucinations that can also occur. This disease frequently presents several comorbidities, such as obesity and precocious puberty, conditions ranging from psychological distress to psychiatric disorders, and cognitive aspects that further worsen the clinical picture. NT1 impairs the quality of life of children, thus calling for an early diagnosis and adequate treatment. To date, pharmacological treatments have been registered for childhood NT1 and can improve symptoms. Non-pharmacological approaches are also essential to improve patients’ well-being, ranging from behavioral treatments (e.g., planned napping) to psychosocial interventions (e.g., school programs). Multidisciplinary treatment management and early diagnosis are key factors in order to allow for adequate quality of life and development in children with NT1. Full article

This article deals with recommendations on the management of symptoms of MS and on the provision of vaccinations in patients receiving disease-modifying therapies (DMTs). Symptoms of MS, such as fatigue, depression, urinary symptoms, spasticity, impairment of gait, and sexual dysfunction, are common in this population. Recognizing and addressing these symptoms is key to maintaining the quality of life of people with MS. Vaccination status should be reviewed and updated prior to initiation of DMTs. In general, vaccination should be avoided for variable periods after the initiation of some DMTs. Live attenuated vaccines are contraindicated and should be considered on a case-by-case basis. These consensus recommendations will present the best practices for vaccination in Saudi Arabia before, during, and after the COVID-19 pandemic. The recommendations will be updated periodically and as needed as new evidence becomes available. Full article

Female sex has been shown to be associated with an unfavorable outcome after ischemic stroke. In this nationwide analysis, we evaluate a large dataset of patients suffering from acute ischemic stroke to elucidate the factors associated with an increased risk of mortality after stroke in women. We analyzed a nationwide dataset from the German Federal Bureau of Statistics including 1,577,884 (761,537 female sex, 48.3%) in-hospital cases admitted between 1 January 2014 and 31 December 2019 with a primary diagnosis of acute ischemic cerebral infarction. Patients were analyzed regarding morbidity, treatments and in-hospital mortality. A multiple logistic regression analysis was performed, adjusted by patients’ risk profile including age, to evaluate the association of sex and in-hospital mortality. According to the median, women were older than men (79 years vs. 73 years). The multiple logistic regression analysis however revealed female sex remained an independent factor for an increased in-hospital mortality (odds ratio [OR] 1.12; 95% confidence interval [CI] 1.11–1.14; p < 0.001). Women had a higher prevalence of relevant risk factors, namely arterial hypertension (77.0% vs. 74.7%), arterial fibrillation (33.3% vs. 25.6%), chronic heart failure (12.3% vs. 9.7%), chronic kidney disease (15.6% vs. 12.9%) and dementia (6.6% vs. 4.1%), but were less affected with respect to other relevant co-morbidities such as cerebrovascular disease (11.7% vs. 15.1%), coronary heart disease (11.7% vs. 18.8%), diabetes mellitus (26.4% vs. 29.6%), dyslipidemia (38.1% vs. 42.0%), ischemic heart disease (12.3% vs. 19.3%) and previous coronary artery bypass grafting (1.1% vs. 3.2%). Overall, therapeutic interventions were performed less frequently in women such as carotid endarterectomy (1.1% vs. 2.3%), carotid stent (0.7% vs. 1.4%), as well as hematoma drainage (0.1% vs. 0.2%), and renal replacement therapy (0.4% vs. 0.6%). Conclusions: Our nationwide analysis revealed a higher mortality rate after stroke in women. Nevertheless, women had fewer in-hospital complications and were also less likely to experience the severe effects of some important co-morbidities. The dataset, however, showed that women received surgical or interventional carotid treatments after stroke less often. It is important for research on sex disparities in stroke to keep these treatment frequency differences in mind. Full article

Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by the degeneration of cortical and spinal motor neurons. With no effective treatment available to date, patients face progressive paralysis and eventually succumb to the disease due to respiratory failure within only a few years. Recent research has revealed the multifaceted nature of the mechanisms and cell types involved in motor neuron degeneration, thereby opening up new therapeutic avenues. Intriguingly, two key features present in both ALS patients and rodent models of the disease are cortical hyperexcitability and hyperconnectivity, the mechanisms of which are still not fully understood. We here recapitulate current findings arguing for cell autonomous and non-cell autonomous mechanisms causing cortical excitation and inhibition imbalance, which is involved in the degeneration of motor neurons in ALS. Moreover, we will highlight recent evidence that strongly indicates a cardinal role for the motor cortex as a main driver and source of the disease, thus arguing for a corticofugal trajectory of the pathology. Full article

Background: An early treatment start with disease modifying therapies (DMT) and long-term adherence is crucial in the treatment of people with multiple sclerosis (PwMS) to prevent future disability. Objectives: To gain information on the diagnostic process, decision making, treatment start and adherence with regard to DMT as well as satisfaction in PwMS in Switzerland to optimize management of PwMS. Methods: A survey was conducted between June 2017 and March 2018 in six hospital-based MS centres and eight private practices in Switzerland. PwMS according to the 2010 McDonald criteria, aged 18–60 years, having a clinical isolated syndrome, relapsing remitting MS, or secondary progressive MS were eligible. The survey contained 40 questions, covering participants’ background and circumstances, treatment decisions, therapy start, treatment adherence, and satisfaction (EKNZ Req-2016-00701). Results: 212 questionnaires were returned for analysis. Of these, 125 (59.0%) were answered by patients treated by practice-based neurologists and 85 (40.1%) by patients treated in hospitals. That PwMS were satisfied overall with current medical care, that they were free of relapses and disease progression, and that they were able to live independently were the main goals of patients. Satisfaction was reflected by an early therapy start and a high adherence to DMT in our cohort. The treating neurologist played a major role in this regard. Furthermore, a satisfactory first diagnostic consultation (FDC) was crucial for successful long-term patient care positively influencing an early treatment start, longer duration of the initial therapy, as well as adherence to treatments and general satisfaction. Conclusion: The treating neurologist and especially a satisfactory FDC play a major role for the successful long-term treatment of PwMS. Detailed information on various aspects of the disease and time with the treating neurologist seems to be of major importance. Full article