First line treatment with subcutaneous efgartigimod in impending myasthenic crisis: a case report.

In acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (gMG), neonatal Fc-receptor (FcRn) inhibition has broadened the therapeutic spectrum. Myasthenic crisis (MC), heralded by an impending myasthenic crisis (iMC), is a critical condition requiring treatments with rapid onset and sustained efficacy. Currently treatments used for iMC, including intravenous immunoglobulins and plasma exchange/immunoadsorption, have limitations, such as delayed onset of action and potential side effects.

Complement inhibition in seropositive generalized myasthenia gravis as rescue therapy in impending and effective treatment in frequently recurring impending myasthenic crisis-a case series.

In seropositive myasthenia gravis (MG), complement inhibition has been shown to be an effective and a fast-acting therapeutic option. Myasthenic crisis (MC), usually preceded by impending MC, is a life-threatening complication requiring highly effective treatments with rapid onset of action. Currently used treatment options of MC are limited, consisting mainly of symptomatic and immune therapies, that is, intravenous immunoglobulins and plasma exchange/immunoadsorption.

Mirror movements in multiple sclerosis -a clinical, electrophysiological, and imaging study.

Background: Mirror movements (MM) are commonly caused by a defect of interhemispheric pathways also affected in multiple sclerosis (MS), particularly the corpus callosum. We investigated the prevalence of MM in MS in relation to functional and morphological callosal fiber integrity by transcranial magnetic stimulation (TMS), magnetic resonance imaging (MRI), as well as fatigue.

Methods: In 21 patients with relapsing-remitting MS and 19 healthy controls, MM were assessed and graded (Woods and Teuber scale: MM 1-4) using a bedside test.

Prospective study validating a multidimensional treatment decision score predicting the 24-month outcome in untreated patients with clinically isolated syndrome and early relapsing-remitting multiple sclerosis, the ProVal-MS study.

Introduction: In Multiple Sclerosis (MS), patients´ characteristics and (bio)markers that reliably predict the individual disease prognosis at disease onset are lacking. Cohort studies allow a close follow-up of MS histories and a thorough phenotyping of patients. Therefore, a multicenter cohort study was initiated to implement a wide spectrum of data and (bio)markers in newly diagnosed patients.

Autologous haematopoietic stem cell transplantation for multiple sclerosis: a position paper and registry outline.

Background: While substantial progress has been made in the development of disease-modifying medications for multiple sclerosis (MS), a high percentage of treated patients still show progression and persistent inflammatory activity. Autologous haematopoietic stem cell transplantation (AHSCT) aims at eliminating a pathogenic immune repertoire through intense short-term immunosuppression that enables subsequent regeneration of a new and healthy immune system to re-establish immune tolerance for a long period of time. A number of mostly open-label, uncontrolled studies conducted over the past 20 years collected about 4000 cases.

Factors associated with depressive mood at the onset of multiple sclerosis - an analysis of 781 patients of the German NationMS cohort.

Background: Depression has a major impact on the disease burden of multiple sclerosis (MS). Analyses of overlapping MS and depression risk factors [smoking, vitamin D (25-OH-VD) and Epstein-Barr virus (EBV) infection] and sex, age, disease characteristics and neuroimaging features associated with depressive symptoms in early MS are scarce.

Objectives: To assess an association of MS risk factors with depressive symptoms within the German NationMS cohort.

Outcomes After Decompressive Surgery for Severe Cerebral Venous Sinus Thrombosis Associated or Not Associated with Vaccine-Induced Immune Thrombosis with Thrombocytopenia: A Multicenter Cohort Study.

Background: Clinical observations indicated that vaccine-induced immune thrombosis with thrombocytopenia (VITT)-associated cerebral venous sinus thrombosis (CVST) often has a space-occupying effect and thus necessitates decompressive surgery (DS). While comparing with non-VITT CVST, this study explored whether VITT-associated CVST exhibits a more fulminant clinical course, different perioperative and intensive care unit management, and worse long-term outcome.

Methods: This multicenter, retrospective cohort study collected patient data from 12 tertiary centers to address priorly formulated hypotheses concerning the clinical course, the perioperative management with related complications, extracerebral complications, and the functional outcome (modified Rankin Scale) in patients with VITT-associated and non-VITT CVST, both with DS.

Retrospective cohort study to devise a treatment decision score predicting adverse 24-month radiological activity in early multiple sclerosis.

Background: Multiple sclerosis (MS) is a chronic neuroinflammatory disease affecting about 2.8 million people worldwide. Disease course after the most common diagnoses of relapsing-remitting multiple sclerosis (RRMS) and clinically isolated syndrome (CIS) is highly variable and cannot be reliably predicted.

Breastfeeding in Mothers with Multiple Sclerosis: The German Experience.

Many female people with multiple sclerosis (pwMS) are in childbearing age; however, only few data exist about the situation of breastfeeding in pwMS. Our study analyzed breastfeeding rate and duration, reasons for weaning, and the impact of disease severity on successful breastfeeding in pwMS. The study included pwMS giving birth within 3 years before study participation.

Humoral COVID-19 vaccine response in patients with NMOSD/MOGAD during anti-IL-6 receptor therapy compared to other immunotherapies.

Background: Data on the humoral vaccine response in patients on anti-interleukin-6 (IL-6) receptor therapy remain scarce.

Objective: The main objective of our study was to investigate the humoral response after vaccination against SARS-CoV-2 in neuromyelitis optica spectrum disorder (NMOSD)/myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) patients treated with anti-IL-6 receptor therapy. Secondarily, we analyzed relapse activity timely associated with vaccination.

Disease-modifying therapies for relapsing/active secondary progressive multiple sclerosis - a review of population-specific evidence from randomized clinical trials.

Although the understanding of secondary progressive multiple sclerosis (SPMS) is evolving, early detection of relapse-independent progression remains difficult. This is further complicated by superimposed relapses and compensatory mechanisms that allow for silent progression. The term relapsing multiple sclerosis (RMS) subsumes relapsing-remitting multiple sclerosis (RRMS) and SPMS with relapses.

Large-scale cross-sectional online survey on patient-neurologist communication, burden of disease assessment and disease monitoring in people with multiple sclerosis.

Background: Management of multiple sclerosis (MS) requires a high level of communication between health care professionals (HCPs) and people with MS (pwMS) including profound investigation and discussion of symptoms to identify therapeutic needs. For treatment decisions, monitoring of disease activity is important, in this respect self-monitoring devices and apps, as well as magnetic resonance imaging are important tools.

Methods: MS Perspectives is a cross-sectional online survey conducted in Germany which was designed to collect data, among others, on the communication between pwMS and HCPs regarding treatment goals, symptom assessment, usage of devices and apps to self-monitor health functions, as well as to identify patients' attitude toward the role of magnetic resonance imaging (MRI).

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Masquerading as Chronic Inflammatory Polyradiculoneuropathy: A Case Report.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare disorder that can mimic chronic inflammatory demyelinating polyradiculopathy (CIDP). In this report, we present a case of a man with a new diagnosis of POEMS syndrome and a clinical picture of CIDP. He had prostate cancer (s/p prostatectomy) with known diffuse bony osteosclerotic lesions and a monoclonal gammopathy of undetermined significance (MGUS).

Clinical Diagnosis of Temporal Arteritis With Seronegative and Negative Biopsy Studies.

Giant cell arteritis (GCA), also known as temporal arteritis is the most common of the systemic vasculitides. It occurs in individuals older than 50 years of age and peaks in incidence in the seventh decade. The gold standard for diagnosis of GCA is temporal artery biopsy (TABx) which will show transmural inflammation, but a negative biopsy does not rule out the disease.

Real-world evidence on siponimod treatment in patients with secondary progressive multiple sclerosis.

Background: Therapeutic options targeting inflammation in multiple sclerosis (MS) have evolved rapidly for relapsing-remitting MS, whereas few therapies are available for progressive forms of MS, in particular secondary progressive MS (SPMS). The approval of siponimod for SPMS has allowed for optimism in the otherwise discouraging therapeutic landscape.

Methods: We conducted a retrospective, multicenter, non-interventional study analyzing the efficacy and safety of siponimod under real-world conditions in 227 SPMS patients.

Association of obesity with disease outcome in multiple sclerosis.

Background: Obesity reportedly increases the risk for developing multiple sclerosis (MS), but little is known about its association with disability accumulation.

Methods: This nationwide longitudinal cohort study included 1066 individuals with newly diagnosed MS from the German National MS cohort. Expanded Disability Status Scale (EDSS) scores, relapse rates, MRI findings and choice of immunotherapy were compared at baseline and at years 2, 4 and 6 between obese (body mass index, BMI ≥30 kg/m) and non-obese (BMI <30 kg/m) patients and correlated with individual BMI values.

Self-assessment of people with relapsing-remitting and progressive multiple sclerosis towards burden of disease, progression, and treatment utilization-Results of a large-scale cross-sectional online survey (MS Perspectives).

Background: Assessment of the disease course by people with multiple sclerosis (pwMS) themselves is important for a better understanding of the complex disease, patient counseling and treatment decisions. This may also facilitate identifying the often-unnoticed transition from relapsing-remitting (RRMS) to secondary progressive multiple sclerosis (SPMS).

Objective: MS Perspectives was designed to collect data on patients' self-assessment of multiple sclerosis (MS) symptoms, relapse-independent progression, and impact on everyday life.

Ovarian Adenocarcinoma With Leptomeningeal Metastases.

Leptomeningeal carcinomatosis (LMC) is an uncommon disease that unfortunately has a rapid deterioration and a very poor prognosis with a devastating outcome. There has been an associated increase in the incidence of the leptomeningeal disease recently. There is a low percentage of LMC, around five percent of patients with metastatic disease.

Long-term management of multiple sclerosis patients treated with cladribine tablets beyond year 4.

Introduction: Oral cladribine is a highly effective pulsed selective immune reconstitution therapy licensed for relapsing multiple sclerosis (RMS) since 2017. A full treatment course comprises two treatment cycles given 1 year apart, followed by two treatment-free years. The management of cladribine-treated patients beyond year 4 needs to be addressed as patients have now passed the initial 4 years since European Medical Agency approval.

Monoclonal and oligoclonal anti-platelet factor 4 antibodies mediate VITT.

Kanack and colleagues analyze anti-platelet factor 4 antibodies from 5 patients with vaccine-induced thrombotic thrombocytopenia (VITT) secondary to COVID-19 adenoviral vaccination and antibodies from patients with spontaneous heparin-induced thrombocytopenia (HIT) and classical HIT. VITT antibodies are monoclonal or oligoclonal, similar to spontaneous HIT, whereas classical HIT antibodies are polyclonal. Heparin inhibits antibody-induced platelet activation in VITT, suggesting that heparin should be considered for the treatment of VITT.

Multiple watershed and cardioembolic strokes in 84-year-old male after cardiac ablation procedure: Case report.

Current guidelines do not include radiologic assessment of the carotid arteries before catheter ablation procedures. There are multiple studies describing the risks of periprocedural cardioembolic strokes during cardiac ablation procedure but none describing the risks of periprocedural watershed strokes due to hypoperfusion during cardiac ablation. It is critically important for neurologists, cardiologists, and all other associated health-care workers to recognize the risks of neurologic complications, such as watershed strokes, before cardiac procedures are performed.

Prolonged-release fampridine for the treatment of myoclonus after cervical myelitis: a case report.

Prolonged-release fampridine (PR-FAM), a potassium channel blocker, is approved for improving walking ability in patients with multiple sclerosis (MS). Beyond this, positive effects on other MS symptoms like fatigue, cognition, and tremor have been described. To our knowledge, a positive effect of PR-FAM on spinal myoclonus has not been described so far.