JNS.jpgThe June issue of the Journal of the Neurological Sciences Vol 461 is now available online.


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Issue highlights

gr1.sml Research Article

Selection of disease modifying therapies in multiple sclerosis based on patient's age and disease activity: Data from a nationwide registry

Piedrabuena et al.

Published online: May 16, 2024

Knowledge of the safety and efficacy of disease-modifying therapies (DMTs) in older patients with Multiple Sclerosis (pwMS) is limited due to their exclusion from clinical trials. Our purpose is to evaluate the choice of DMTs in pwMS older than 50 years old in a real-world setting in a cross-sectional study of pwMS from the Argentine MS and NMOSD Registry.

The study shows the preference of LET in older patients regardless of disease activity. However it does not demonstrate a difference in disability in older patients based on low vs high efficacy DMTs used, probably due to the design of the study. Further longitudinal studies are warranted to address this issue.

gr1.sml Research Article

Sex differences in outcomes of carotid artery stenting

Uchida et al.

Published online: May 22, 2024

Existing evidence is inconclusive on whether women after carotid artery stenting (CAS) experience worse outcomes than men.

The outcomes of women and men were compared using the data from nationwide retrospective registry between 2015 and 2019. The primary outcome was the incidence of ischemic stroke and all-cause death. Secondary outcomes included the incidence of ischemic stroke, all-cause death, acute occlusion, and acute myocardial infarction. Functional outcomes were the achieving an mRS score of 0–1 and 0–2. Outcomes were assessed at 30 days after CAS.

This study suggests that there was no significant sex differences in the incidence of ischemic stroke and all-cause death at 30 days. However, women have higher rate of all-cause death and poorer functional outcomes at 30 days than men.

ga1.sml Research Article

Effect of lipid-lowering therapy on carotid plaque burden in older adults

Iankov et al.

Published online: April 30, 2024

Little is known about the benefits of lipid-lowering medications in those age ≥ 75 years. We assessed the effect of lipid-lowering medications on progression to severe atherosclerosis in patients age > 75.

Data was retrospectively obtained from the Stroke Prevention & Atherosclerosis Research Centre, Canada. Atherosclerosis burden was measured as carotid total plaque area (TPA), a powerful predictor of cardiovascular risk. Survival time free of severe atherosclerosis (SFSA) was defined as the period when TPA remained <1.19 cm2. Kaplan–Meier, multiple Cox proportional hazard and hierarchical mixed-effect models were used to determine the effects of lipid-lowering medications on progression to severe atherosclerosis.

In total 1404 cases (mean age 81 ± 4 years; women 52%) were included.

Lipid-lowering therapy is effective in controlling the burden of atherosclerosis among older adults with and without vascular disease. The measurement of plaque burden can guide selection and follow-up of those who may benefit from treatment.

gr1.sml Review Article

Cerebral cavernous malformations – An overview on genetics, clinical aspects and therapeutic strategies

Dulamea et al.

Published online: May 11, 2024

Cerebral cavernous malformations (CCMs) are abnormally packed blood vessels lined with endothelial cells, that do not exhibit intervening tight junctions, lack muscular and elastic layers and are usually surrounded by hemosiderin and gliosis. CCMs may be sporadic or familial autosomal dominant (FCCMs) caused by loss of function mutations in CCM1 (KRIT1), CCM2 (MGC4607), and CCM3 (PDCD10) genes.

In the FCCMs, patients have multiple CCMs, different family members are affected, and developmental venous anomalies are absent. CCMs may be asymptomatic or may manifest with focal neurological deficits with or without associated hemorrhage andseizures.

Recent studies identify a digenic "triple-hit" mechanism involving the aquisition of three distinct genetic mutations that culminate in phosphatidylinositol-3-kinase (PIK3CA) gain of function, as the basis for rapidly growing and clinically symptomatic CCMs. The pathophysiology of CCMs involves signaling aberrations in the neurovascular unit, including proliferative dysangiogenesis, blood-brain barrier hyperpermeability, inflammation and immune mediated processes, anticoagulant vascular domain, and gut microbiome-driven mechanisms.

Clinical trials are investigating potential therapies, magnetic resonance imaging and plasma biomarkers for hemorrhage and CCMs-related epilepsy, as well as different techniques of neuronavigation and neurosonology to guide surgery in order to minimize post-operatory morbidity and mortality.

This review addresses the recent data about the natural history, genetics, neuroimaging and therapeutic approaches for CCMs.