Basic CSF parameters and MRZ reaction help in differentiating MOG antibody-associated autoimmune disease versus multiple sclerosis.

Background: Myelin oligodendrocyte glycoprotein antibody-associated autoimmune disease (MOGAD) is a rare monophasic or relapsing inflammatory demyelinating disease of the central nervous system (CNS) and can mimic multiple sclerosis (MS). The variable availability of live cell-based MOG-antibody assays and difficulties in interpreting low-positive antibody titers can complicate diagnosis. Literature on cerebrospinal fluid (CSF) profiles in MOGAD versus MS, one of the most common differential diagnoses, is scarce. We here analyzed the value of basic CSF parameters to i) distinguish different clinical MOGAD manifestations and ii) differentiate MOGAD from MS. Methods: This is retrospective, single-center analysis of clinical and laboratory data of 30 adult MOGAD patients and 189 adult patients with relapsing-remitting multiple sclerosis. Basic CSF parameters included CSF white cell count (WCC) and differentiation, CSF/serum albumin ratio (QAlb), intrathecal production of immunoglobulins, CSF-restricted oligoclonal bands (OCB) and MRZ reaction, defined as intrathecal production of IgG reactive against at least 2 of the 3 viruses measles (M), rubella (R) and varicella zoster virus (Z). Results: MOGAD patients with myelitis were more likely to have a pleocytosis, a QAlb elevation and a higher WCC than those with optic neuritis, and, after review and combined analysis of our and published cases, they also showed a higher frequency of intrathecal IgM synthesis. Compared to MS, MOGAD patients had significantly more frequently neutrophils in CSF and WCC>30/µl, QAlb>10×10-3, as well as higher mean QAlb values, but significantly less frequently CSF plasma cells and CSF-restricted OCB. A positive MRZ reaction was present in 35.4% of MS patients but absent in all MOGAD patients. Despite these associations, the only CSF parameters with relevant positive likelihood ratios (PLR) indicating MOGAD were QAlb>10×10-3 (PLR 12.60) and absence of CSF-restricted OCB (PLR 14.32), whereas the only relevant negative likelihood ratio (NLR) was absence of positive MRZ reaction (NLR 0.00). Conclusion: Basic CSF parameters vary considerably in different clinical phenotypes of MOGAD, but QAlb>10×10-3 and absence of CSF-restricted OCB are highly useful to differentiate MOGAD from MS. A positive MRZ reaction is confirmed as the strongest CSF rule-out parameter in MOGAD and could be useful to complement the recently proposed diagnostic criteria.

Myosin Modulators: The New Era of Medical Therapy for Systolic Heart Failure and Hypertrophic Cardiomyopathy.

A new uprising pharmacological treatment for systolic heart failure and hypertrophic cardiomyopathy demonstrates very promising results the last years. Myosin modulators have already been tested in numerous studies. Myosin inhibitor (mavacamten) and myosin stimulator, (omecamtiv mecarbil) exhibit their effect by improving clinical outcomes, as well as reducing morbidity and mortality. More studies are however required for further evaluation and particularly effect on sarcomeric level. Side effects of both pharmacological agents have been described and should always be close monitored. Cardiopulmonary exercise test has a pivotal role by means of assessing treatment efficacy.

Causes, Diagnosis, Risk Stratification and Treatment of Bicuspid Aortic Valve Disease: An Updated Review.

The most common congenital heart disease is the bicuspid aortic valve. Understanding the pathophysiology and the altered hemodynamics is a key component for the diagnosis, risk stratification and treatment. Among others, aortic valve stenosis is the most common complication. Treatment strategies vary depending on the severity of the disease, particularly the dilation of the aorta playing a major role. Together with valve replacement, transcatheter aortic valve implantation is now considered as an alternative option with good results. With this review we would like to discuss the causes, diagnostic methods, risk stratification and treatment strategies of the bicuspid aortic valve.