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Identifying the transition of relapsing-remitting multiple sclerosis (MS) to the secondary-progressive MS form remains a clinical challenge due to the gradual nature of the transition, superimposed relapses, the heterogeneous course of disease among patients and the absence of validated biomarkers and diagnostic tools. The uncertainty associated with the transition makes clinical care challenging for both patients and physicians. The emergence of new disease-modifying treatments for progressive MS and the increasing emphasis of nonpharmacological strategies mark a new era in the treatment of progressive MS. This article summarizes challenges in diagnosis and management, discusses novel treatment strategies and highlights the importance of establishing a clear diagnosis and instituting an interdisciplinary management plan in the care of patients with progressive MS.

ObjectiveWe describe a case of bilateral sequential optic neuropathies with pachymeningitis and aortitis, with findings that raised suspicion of Erdheim-Chester disease versus IgG-4 related disease.BackgroundErdheim-Chester disease (ECD) is a rare histiocytic neoplasm characterized by tissue infiltration by foamy histiocytes, and chronic, uncontrolled inflammation. IgG4-related disease (IgG4-RD) is an insidiously progressive immune-mediated fibrotic disease typified by tumour-like mass formation in many affected organs. Neurologic manifestations are diverse.Design/MethodsA 58-year-old male was transferred to our centre for acute onset sequential optic neuropathies. His visual acuity was light perception for the right eye and 20/50 in the left eye.ResultsEnhanced MRI of the brain and orbits showed focal pachymeningeal thickening and enhancement in the anterior cranial fossa and over the left frontal lobe with eccentric enhancement of the right optic nerve sheath. CRP was elevated (23 mmol/L to 62 mmol/L); extensive CSF and serum infectious and inflammatory investigations were unrevealing. PET body demonstrated aortitis and CT angiography suggested coronary artery vasculitis. Bone scan showed symmetric involvement of the long bones. Dural biopsy was delayed due to the Covid-19 pandemic and was completed following a protracted steroid course and a 15 mg/kg dose of cyclophosphamide. Pathology showed mixed inflammatory infiltrate and increased expression of IgG4 neutrophils. Clusters of CD68+, CD1a, and S100-negative macrophages were seen in all layers of dura. No BRAF mutation was identified.ConclusionsThis case demonstrates classic imaging findings of ECD including pachymeningitis, symmetric long bone involvement and aortitis. Pathology in ECD may show characteristic foamy histiocytes, that were absent in this case. This case demonstrates the challenge of biopsy interpretation following immunosuppressive and cytotoxic therapy and the difficulty of differentiating ECD from IgG4-RD.

ObjectiveHerein, we present a rare and diagnostically challenging case of neuro-Behcet disease (NBD) manifesting as a psuedotumoral brainstem mass.BackgroundPsuedotumoral-NBD as a neurologic manifestation of Behcet's disease is rare. Imaging is characterized by mass-like lesions that enhance with contrast, are hyperintense with T2-weighted and fluid-attenuated inversion recovery (FLAIR) and show restricted diffusion. The differential includes glial lesions, lymphomas, infectious and granulomatous lesions.Design/MethodsThis is a case study of a 33-year-old male of West-African descent with a history of Behcet's disease.ResultsA 33-year-old male of West-African descent with a history of Behcet's disease, presented two years after his diagnosis with headaches, low-grade fever, genital ulcerations, and horizontal binocular diplopia. Imaging revealed a large right-sided T2/FLAIR hyperintense abnormality in the medulla with a central area of necrosis. Cerebrospinal fluid revealed lymphocytic-predominant pleocytosis with 11·106 cells/L (reference range 0-5), and high levels of interleukin-6. His vasculitis, infectious, paraneoplastic, flow cytometry, and autoimmune panels were negative. He tested positive for hepatitis-B core antigen, and latent tuberculosis. The etiology for his presentation was believed to be parenchymal NBD and he received a 3-day course of intravenous solumedrol (eventually transitioned to prednisone and azathioprine) with significant improvement. Imaging 1-week post-treatment revealed resolution of enhancement, and at 3 months he had near complete lesion resolution.ConclusionsNBD can rarely present with a psuedotumoral presentation, which can cause diagnostic uncertainty. A thorough radiologic/laboratory workup should be conducted to exclude other neurologic diagnoses; however, a high index of suspicion for NBD is required in similar cases and a spectacular response to steroids are invaluable in diagnosis.

BackgroundAt the end of December 2019, a novel respiratory infection, initially reported in China, known as COVID-19 initially reported in China, and later known as COVID-19, led to a global pandemic. Despite many studies reporting respiratory infections as the primary manifestations of this illness, an increasing number of investigations have focused on the central nervous system (CNS) manifestations in COVID-19. In this study, we aimed to evaluate the CNS presentations in COVID-19 patients in an attempt to identify the common CNS features and provide a better overview to tackle this new pandemic.MethodsIn this systematic review and meta-analysis, we searched PubMed, Web of Science, Ovid, Embase, Scopus, and Google Scholar. Included studies were publications that reported the CNS features between January 1st, 2020, to April 20th, 2020. The data of selected studies were screened and extracted independently by four reviewers. Extracted data analyzed by using STATA statistical software. The study protocol registered with PROSPERO (CRD42020184456).ResultsOf 2353 retrieved studies, we selected 64 studies with 11282 patients after screening. Most of the studies were conducted in China (58 studies). The most common CNS symptom of COVID-19 were Headache (8.69%, 95%CI: 6.76%-10.82%), Dizziness (5.94%, 95%CI: 3.66%-8.22%), and Impaired consciousness (1.9%, 95%CI: 1%-2.79%).ConclusionsThe growing number of studies have reported COVID-19, CNS presentations as remarkable manifestations that happen. Hence, understanding the CNS characteristics of COVID-19 can help us for better diagnosis and ultimately prevention of worse outcomes.

This is a case of autoimmune encephalitis with features of faciobrachial dystonic seizures (FBDS) pathognomonic for Leucine Rich Glioma inactivated (LGI)1 antibody encephalitis. This voltage-gated potassium channel complex encephalitis is marked by rapid onset dementia, FBDS and hyponatremia, which is sensitive to management with immunotherapy including steroids, IVIG and other agents. In this case report we review the clinical features, imaging and management of this condition.

Despite updating knowledge and a growing number of medications for multiple sclerosis (MS), no definite treatment is available yet for patients suffering from progressive forms of the disease. Autologous bone marrow derived mesenchymal stem cell (BM-MSC) transplantation is a promising method proposed as a therapy for MS. Although the safety of these cells has been confirmed in hematological, cardiac and inflammatory diseases, its efficacy in MS treatment is still under study. Patients with progressive MS (expanded disability status scale score: 4.0 –6.50) unresponsive to conventional treatments were recruited for this study. Twenty-five patients [f/m: 19/6, mean age: 34.7±7] received a single intrathecal injection of ex-vivo expanded MSCs (mean dose: 29.5×106 cells). We observed their therapeutic response for 12 months. Associated short-term adverse events of injection consisted of transient low-grade fever, nausea /vomiting, weakness in the lower limbs and headache. No major delayed adverse effect was reported. 3 patients left the study for personal reasons. The mean (SD) expanded disability status scale (EDSS) score of 22 patients changed from 6.1 (0.6) to 6.3 (0.4). Clinical course of the disease (measured by EDSS) improved in 4, deteriorated in 6 and had no change in 12 patients. In MRI evaluation, 15 patients showed no change, whereas 6 patients showed new T2 or gadolinium enhanced lesions (1 lost to follow-up). It seems that MSC therapy can improve/stabilize the course of the disease in progressive MS in the first year after injection with no serious adverse effects. Repeating the study with a larger sample size, booster injections and longer follow-up using a controlled study design is advised.

Contexte: La sclerose en plaques (SP) se caracterise par des poussees alternant avec des periodes de stabilite relative. La corticotherapie a haute dose en perfusion intraveineuse rapide pendant trois a cinq jours est le traitement standard actuel des poussees aigues. Cependant, il existe des donnees recentes en faveur de l'utilisation de doses equivalentes de steroides par voie orale. La dose unique la plus elevee de prednisone par voie orale est de 50 mg par comprime, ce qui implique que les patients doivent prendre 25 comprimes par jour. Ceci a souleve des inquietudes quant a la fidelite au traitement par voie orale. Objectifs: Le but de l'etude etait de determiner si les patients atteints de SP sont fideles au traitement lors qu'ils doivent prendre 1250 mg de prednisone par voie orale quotidiennement lors d'une poussee aigue. Methode: Tous les patients chez qui on a pose un diagnostic de poussee aigue entre novembre 2008 et decembre 2009 a la clinique de SP de London en Ontario ont ete identifies de facon prospective. S'ils recevaient la prednisone par voie orale, on leur remettait un questionnaire qu'on leur demandait de remplir et de poster a la clinique de facon anonyme. Resultats: Soixante-huit poussees de SP ont ete diagnostiquees et traitees par corticotherapie chez 66 patients, dont 60 (58) patients qui ont ete traites par la prednisone a 1250 mg. Cinquante-trois (91,4%) des sondages ont ete retournes. Le taux de fidelite au traitement tel que rapporte par les patients etait de 94,3% (50/53) et seulement un patient a rapporte qu'il n'avait pas ete capable de prendre tous les comprimes prescrits parce qu'il ne les tolerait pas. La plupart des sujets (43, soit 86,0%) ont eu au moins un effet secondaire, les plus frequents etant l'insomnie, les changements d'humeur et l'augmentation de l'appetit. Les deux-tiers des sujets (69,8%) ont indique une preference pour le traitement par voie orale lors d'une eventuelle poussee de SP. Conclusion: La prednisone a haute dose (1250 mg) par voie orale est un traitement acceptable chez les patients atteints de SP pour le traitement de poussees aigues et le taux de fidelite au traitement est eleve.

Background Magnetic resonance imaging (MRI) is increasingly important for the early detection of suboptimal responders to disease-modifying therapy for relapsing–remitting multiple sclerosis. Treatment response criteria are becoming more stringent with the use of composite measures, such as no evidence of disease activity (NEDA), which combines clinical and radiological measures, and NEDA-4, which includes the evaluation of brain atrophy. Methods The Canadian MRI Working Group of neurologists and radiologists convened to discuss the use of brain and spinal cord imaging in the assessment of relapsing–remitting multiple sclerosis patients during the treatment course. Results Nine key recommendations were developed based on published sources and expert opinion. Recommendations addressed image acquisition, use of gadolinium, MRI requisitioning by clinicians, and reporting of lesions and brain atrophy by radiologists. Routine MRI follow-ups are recommended beginning at three to six months after treatment initiation, at six to 12 months after the reference scan, and annually thereafter. The interval between scans may be altered according to clinical circumstances. Conclusions The Canadian recommendations update the 2006 Consortium of MS Centers Consensus revised guidelines to assist physicians in their management of MS patients and to aid in treatment decision making.