Neurology Alert – December 1, 2020
December 1, 2020
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Big Data Look at Optic Neuritis
In this population-based study of 11 million people in the United Kingdom, the incidence of optic neuritis was 3.7/100,000 person years, and was stable over the time period 1995-2019. The 10-year risk of developing multiple sclerosis in this population was 28.2%.
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Sporadic Creutzfeldt-Jakob Disease: A Genome-Wide Association Study
Sporadic Creutzfeldt-Jacob disease (sCJD), the most common form of human prion disease, is characterized as a rapidly fatal neurodegenerative process caused by propagation of a transmissible misfolded prion protein gene (PRNP). However, selective PRNP mutations only account for a small subset of sCJD cases, leading to interest in discovering additional genetic risk factors. Through a two-stage study design using genome-wide association studies, the authors have identified two novel risk loci, STX6 and GAL3ST1, which encode for proteins involved in cellular trafficking of prions and sphingolipid metabolism, respectively. These findings provide insights into sCJD pathogenesis and are an avenue for further research.
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Multiple Sclerosis and Vascular Disease
This postmortem study of multiple sclerosis (MS) patients evaluated the presence and pathological significance of extracranial systemic and cerebral small vessel disease in patients with MS compared to healthy controls. MS patients had less systemic vascular disease and more small vessel disease in the brain compared to controls.
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Disease-Modifying Therapy and Long-Term Disability in Multiple Sclerosis
In a multicenter, observational, retrospective “real world” analysis of a large cohort of relapsing multiple sclerosis (MS) patients, the authors found that being on disease-modifying therapy decreased the risk of long-term disability progression in both pediatric and adult MS patients.
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Nutritional Interventions in Prodromal Alzheimer’s Disease: The 36-Month LipiDiDiet Multinutrient Clinical Trial
In a randomized, double-blind, placebo-controlled trial of Fortasyn Connect (Souvenaid), a nutraceutical drink, patients with prodromal Alzheimer’s disease demonstrated, over a 36-month period, a slower decline in cognitive functions compared to the control group.