Interferon-beta-related tumefactive brain lesion in a Caucasian patient with neuromyelitis optica and clinical stabilization with tocilizumab

Background: Neuromyelitis optica (NMO) is a severely disabling inflammatory disorder of the central nervous system and is often misdiagnosed as multiple sclerosis (MS). There is increasing evidence that treatment options shown to be beneficial in MS, including interferon-β (IFN-β), are detrimental in NMO. Case presentation: We here report the first Caucasian patient with aquaporin 4 (AQP4) antibody (NMO-IgG)-seropositive NMO presenting with a tumefactive brain lesion on treatment with IFN-β. Disease started with relapsing optic neuritis and an episode of longitudinally extensive transverse myelitis (LETM) in the absence of any brain MRI lesions or cerebrospinal fluid-restricted oligoclonal bands. After initial misdiagnosis of multiple sclerosis (MS) the patient received subcutaneous IFN-β1b and, subsequently, subcutaneous IFN-β1a therapy for several years. Under this treatment, the patient showed persisting relapse activity and finally presented with a severe episode of subacute aphasia and right-sided hemiparesis due to a large T2 hyperintensive tumefactive lesion of the left brain hemisphere and a smaller T2 lesion on the right side. Despite rituximab therapy two further LETM episodes occurred, resulting in severe neurological deficits. Therapeutic blockade of the interleukin (IL)-6 signalling pathway by tocilizumab was initiated, followed by clinical and radiological stabilization. Conclusion: Our case (i) illustrates the relevance of correctly distinguishing NMO and MS since these disorders differ markedly in their responsiveness to immunomodulatory and -suppressive therapies; (ii) confirms and extends a previous report describing the development of tumefactive brain lesions under IFN-β therapy in two Asian NMO patients; and (iii) suggests tocilizumab as a promising therapeutic alternative in highly active NMO disease courses

Non-epileptic seizures in autonomic dysfunction as the initial symptom of COVID-19

Objective!#!Vestibular evoked myogenic potentials (VEMPs) have been suggested as biomarkers in the differential diagnosis of Menière's disease (MD) and vestibular migraine (VM). The aim of this study was to compare the degree of asymmetry for ocular (o) and cervical (c) VEMPs in large cohorts of patients with MD and VM and to follow up the responses.!##!Study design!#!Retrospective study in an interdisciplinary tertiary center for vertigo and balance disorders.!##!Methods!#!cVEMPs to air-conducted sound and oVEMPs to bone-conducted vibration were recorded in 100 patients with VM and unilateral MD, respectively. Outcome parameters were asymmetry ratios (ARs) of oVEMP n10p15 and cVEMP p13n23 amplitudes, and of the respective latencies (mean ± SD).!##!Results!#!The AR of cVEMP p13n23 amplitudes was significantly higher for MD (0.43 ± 0.34) than for VM (0.26 ± 0.24; adjusted p = 0.0002). MD-but not VM-patients displayed a higher AR for cVEMP than for oVEMP amplitudes (MD 0.43 ± 0.34 versus 0.23 ± 0.22, p < 0.0001; VM 0.26 ± 0.14 versus 0.19 ± 0.15, p = 0.11). Monitoring of VEMPs in single patients indicated stable or fluctuating amplitude ARs in VM, while ARs in MD appeared to increase or remain stable over time. No differences were observed for latency ARs between MD and VM.!##!Conclusions!#!These results are in line with (1) a more common saccular than utricular dysfunction in MD and (2) a more permanent loss of otolith function in MD versus VM. The different patterns of o- and cVEMP responses, in particular their longitudinal assessment, might add to the differential diagnosis between MD and VM

Retinal ganglion cell and inner plexiform layer thinning in clinically isolated syndrome

BACKGROUND: Axonal and neuronal damage are widely accepted as key events in the disease course of multiple sclerosis. However, it has been unclear to date at which stage in disease evolution neurodegeneration begins and whether neuronal damage can occur even in the absence of acute inflammatory attacks. OBJECTIVE: To characterize inner retinal layer changes in patients with clinically isolated syndrome (CIS). METHOD: 45 patients with CIS and age- and sex-matched healthy controls were investigated using spectral domain optical coherence tomography. Patients' eyes were stratified into the following categories according to history of optic neuritis (ON): eyes with clinically-diagnosed ON (CIS-ON), eyes with suspected subclinical ON (CIS-SON) as indicated by a visual evoked potential latency of >115ms and eyes unaffected by ON (CIS-NON). RESULTS: CIS-NON eyes showed significant reduction of ganglion cell- and inner plexiform layer and a topography similar to that of CIS-ON eyes. Seven eyes were characterized as CIS-SON and likewise showed significant retinal layer thinning. The most pronounced thinning was present in CIS-ON eyes. CONCLUSION: Our findings indicate that retinal pathology does occur already in CIS. Intraretinal layer segmentation may be an easily applicable, non-invasive method for early detection of retinal pathology in patients unaffected by ON