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Pediatr Neurol 2021 May 19;121:3-10. Epub 2021 May 19.

Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland.

Background: Baló concentric sclerosis is a rare demyelinating disease with characteristic magnetic resonance appearance of multilayered ringlike lesions of demyelination. This disease is extremely rare in children. We present the clinical data, radiological appearance, and development of lesions in eight children. Read More

Rev Neurol 2021 Jun;72(12):433-442

Hospital Universitario de Cruces, Bilbao, España.

Introduction: For more than a decade, after the ECTRIMS Congress, Spain has hosted the Post-ECTRIMS meeting, where neurologists with expertise in multiple sclerosis (MS) meet to review the new developments presented at the ECTRIMS.

Aim: This article, published in two parts, summarises the presentations of the post-ECTRIMS meeting, held online on 16 and 17 October 2020.

Development: This second part highlights the importance of gender and age in understanding the pathology of the disease and optimising its management. Read More

Eur Radiol 2021 Jun 9. Epub 2021 Jun 9.

Department of Diagnostic and Interventional Neuroradiology, School of Medicine, Klinikum rechts der Isar, Technical University of Munich, Ismaninger Str. 22, 81675 Munich, Germany.

Objectives: Multiple sclerosis (MS) is an inflammatory disease frequently involving the spinal cord, which can be assessed by magnetic resonance imaging (MRI). Here, we hypothesize that pre-contrast T1-w imaging does not add diagnostic value to routine spinal MRI for the follow-up of patients with MS.

Methods: 3-T MRI scans including pre- and post-contrast T1-w as well as T2-w images of 265 consecutive patients (mean age: 40 ± 13 years, 169 women) with (suspected) MS were analyzed retrospectively. Read More

BMC Neurol 2021 Jun 9;21(1):219. Epub 2021 Jun 9.

Department of Neurology, West China Hospital, Sichuan University, No. 37 Guo Xue Xiang, 610041, Chengdu, China.

Background: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is an adult-onset rare monogenic microvasculopathy. Its typical neuroimaging features are punctate white matter lesions or pseudotumor alterations. RVCL-S is often under-recognized and misdiagnosed because of its rarity and similar imaging manifestations to multiple sclerosis or brain malignant mass. Read More

PLoS One 2021 8;16(6):e0251338. Epub 2021 Jun 8.

Imaging Sciences, Imaging Institute, Cleveland Clinic, Cleveland, OH, United States of America.

Cognitive impairment is a common symptom in individuals with Multiple Sclerosis (MS), but meaningful, reliable biomarkers relating to cognitive decline have been elusive, making evaluation of the impact of therapeutics on cognitive function difficult. Here, we combine pathway-based MRI measures of structural and functional connectivity to construct a metric of functional decline in MS. The Structural and Functional Connectivity Index (SFCI) is proposed as a simple, z-scored metric of structural and functional connectivity, where changes in the metric have a simple statistical interpretation and may be suitable for use in clinical trials. Read More

J Neuroimaging 2021 Jun 8. Epub 2021 Jun 8.

Computational Radiology Lab, Boston Children's Hospital, Harvard Medical School, Massachusetts, Boston, USA.

Background And Purpose: Functional MRI neurofeedback (fMRI-nf) leverages the brain's ability to self-regulate its own activity. However, self-regulation processes engaged during fMRI-nf are incompletely understood. Here, we used matched feedback in an fMRI-nf experimental protocol to investigate whether brain processes recognize true neurofeedback signals. Read More

Mult Scler 2021 Jun 8:13524585211019650. Epub 2021 Jun 8.

Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.

The mechanisms responsible for the favorable clinical course in multiple sclerosis (MS) remain unclear. In this longitudinal study, we assessed whether magnetic resonance imaging (MRI)-based changes in focal and diffuse brain damage are associated with a long-term favorable MS diseases course. We found that global brain and gray matter (GM) atrophy changes were milder in MS patients with long-standing disease (⩾30 years from onset) and favorable (no/minimal disability) clinical course than in sex-age-matched disable MS patients, independently of lesions accumulation. Read More

J Neurol Sci 2021 Jun 2;427:117528. Epub 2021 Jun 2.

Division of Neurology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

Objective: To evaluate the degree of neuroaxonal injury in Japanese multiple sclerosis (MS) patients using serum neurofilament light (sNfL) and to investigate the relationship of sNFL with the degree of brain volume.

Methods: sNfL levels in 82 consecutive Japanese MS patients in remission were cross-sectionally evaluated using a single molecule array assay. Within this sample, cross-sectional volumetric brain MRI data was evaluated in 80 patients, and longitudinal data was evaluated in 63 patients. Read More

Front Neurol 2021 20;12:675244. Epub 2021 May 20.

UMR1219 Bordeaux Population Health Center (Team VINTAGE), INSERM-University of Bordeaux, Bordeaux, France.

Young adults represent an increasingly large proportion of healthy volunteers in brain imaging research, but descriptions of incidental findings (IFs) in this age group are scarce. We aimed to assess the prevalence and severity of IFs on brain MRIs of healthy young research participants aged 18-35 years, and to describe the protocol implemented to handle them. The study population comprised 1,867 participants aged 22. Read More

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2021 Jun 1. Epub 2021 Jun 1.

AXON Neuroscience R&D Services SE, Bratislava, Slovak Republic.

Background: Neurofilament light chain is a promising biomarker of disease activity and treatment response in relapsing-remitting multiple sclerosis (MS). Its role in progressive MS is less clear.

Aim: The aim of the study was to assess the relationship between plasma neurofilament light chain (pNfL) and disease activity as defined by the concept NEDA-3 (No Evident Disease Activity), and brain volumetry, in a cohort of patients with the progressive disease form (PMS). Read More

J Alzheimers Dis 2021 Jun 1. Epub 2021 Jun 1.

The Framingham Heart Study, Framingham, MA, USA.

Background: Adherence to the Mediterranean-DASH for Neurodegenerative Delay (MIND) diet has previously been associated with cognitive decline and dementia. To our knowledge, no prior study has investigated the association between the MIND diet and measures of brain volume, silent brain infarcts (SBIs), or brain atrophy.

Objective: We evaluated whether adherence to the MIND diet associated with superior cognitive function, larger brain volumes, fewer SBIs, and less cognitive decline in the community-based Framingham Heart Study. Read More

Neuroimage Clin 2021 May 24;31:102705. Epub 2021 May 24.

Buffalo Neuroimaging Analysis Center, Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, Buffalo, NY, USA; IRCCS, Fondazione Don Carlo Gnocchi ONLUS, Milan, Italy. Electronic address:

Background: Although quantitative measures from research-quality MRI provide a means to study multiple sclerosis (MS) pathology in vivo, these metrics are often unavailable in legacy clinical datasets.

Objective: To determine how well an automatically-generated quantitative snapshot of brain pathology, measured only on clinical routine T2-FLAIR MRI, can substitute for more conventional measures on research MRI in terms of capturing multi-factorial disease pathology and providing similar clinical relevance.

Methods: MRI with both research-quality sequences and conventional clinical T2-FLAIR was acquired for 172 MS patients at baseline, and neurologic disability was assessed at baseline and five-years later. Read More

Neurology 2021 Jun 4. Epub 2021 Jun 4.

Department of Anatomy and Neurosciences, MS Center Amsterdam, Amsterdam Neuroscience, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands

Neurology 2021 Jun 4. Epub 2021 Jun 4.

Center for Brain Research, Medical University of Vienna, Vienna, Austria.

In most cases, multiple sclerosis (MS) begins with a relapsing-remitting course followed by insidious disability worsening that is independent from clinically apparent relapses and is termed secondary progressive multiple sclerosis (SMPS). Major differences exist between relapsing-remitting MS (RRMS) and SPMS, especially regarding therapeutic response to treatment. This review provides an overview of the pathology, differentiation, and challenges in the diagnosis and treatment of SPMS. Read More

Hum Brain Mapp 2021 Jun 4. Epub 2021 Jun 4.

Department of Brain and Behavioral Sciences, University of Pavia, Pavia.

Deep gray matter nuclei are the synaptic relays, responsible to route signals between specific brain areas. Dentate nuclei (DNs) represent the main output channel of the cerebellum and yet are often unexplored especially in humans. We developed a multimodal MRI approach to identify DNs topography on the basis of their connectivity as well as their microstructural features. Read More

Cell Mol Neurobiol 2021 Jun 4. Epub 2021 Jun 4.

Department of Biochemistry and Molecular Biology, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.

The blood-brain barrier (BBB) is essential for the homeostasis of the central nervous system (CNS). Functions of the BBB are performed by the neurovascular unit (NVU), which consists of endothelial cells, pericytes, astrocytes, microglia, basement membrane, and neurons. NVU cells interact closely and together are responsible for neurovascular coupling, BBB integrity, and transendothelial fluid transport. Read More

Eur J Neurol 2021 Jun 3. Epub 2021 Jun 3.

Department of Neurology, Otto-von-Guericke University, Magdeburg, Germany.

Introduction: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that causes progressive degeneration of neurons in motor and non-motor brain regions, affecting multiple cognitive domains such as memory. We performed a functional magnetic resonance imaging (fMRI) study to explore working memory function in ALS.

Methods: To contribute to the growing research field that employs structural and functional neuroimaging to investigate the effect of ALS on different working memory components, we explored the localization and intensity of alterations in neural activity using fMRI. Read More

J Neuroimaging 2021 Jun 3. Epub 2021 Jun 3.

Buffalo Neuroimaging Analysis Center, Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, Buffalo, New York, USA.

Background And Purpose: Atrophied T2 lesion volume (LV), reflecting the complete transformation of lesions into cerebrospinal fluid (CSF), has been associated with disease progression in multiple sclerosis (MS). The underlying damage leading to lesion destruction remains poorly understood. The objective of this study was to use diffusion tensor imaging (DTI) to investigate the extent of microstructural tissue damage at baseline in lesions that subsequently transform into CSF. Read More

Front Pharmacol 2021 17;12:621856. Epub 2021 May 17.

Department of Neurosurgery and Brain and Nerve Research Laboratory, The First Affiliated Hospital of Soochow University, Suzhou, China.

The efficacy and safety of fingolimod for relapsing-remitting multiple sclerosis (RRMS) had been well verified in several large randomized controlled trials (RCTs) during the past decade. However, there are fewer systematic comparisons of different doses of fingolimod and whether the dose of 0.5 mg/d is the optimal one still remains to be solved. Read More

Sci Rep 2021 Jun 2;11(1):11586. Epub 2021 Jun 2.

Athinoula A. Martinos Center for Biomedical Imaging, Radiology Department, Massachusetts General Hospital, Harvard Medical School, 149 13th St., Suite 2301, Charlestown, MA, 02129, USA.

Computer-aided detection of brain lesions from volumetric magnetic resonance imaging (MRI) is in demand for fast and automatic diagnosis of neural diseases. The template-matching technique can provide satisfactory outcome for automatic localization of brain lesions; however, finding the optimal template size that maximizes similarity of the template and the lesion remains challenging. This increases the complexity of the algorithm and the requirement for computational resources, while processing large MRI volumes with three-dimensional (3D) templates. Read More

Mult Scler Relat Disord 2021 May 19;53:103031. Epub 2021 May 19.

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York City, United States.

Background: Despite significant interest in diet by the MS community, research on this topic is limited; there are no published studies evaluating associations between diet and neuroimaging in MS.

Methods: We utilized baseline data from the RADIEMS cohort of early MS (diagnosed <5.0 years, n=180). Read More

Cogn Behav Neurol 2021 Jun 2;34(2):79-95. Epub 2021 Jun 2.

REVAL Rehabilitation Research Center, Faculty of Rehabilitation Sciences, Hasselt University, Hasselt, Belgium.

Background: Performing a cognitive task and a motor task simultaneously is an everyday act that can lead to decreased performance on both tasks.

Objective: To provide insight into the neural correlates associated with cognitive-motor dual tasking in individuals with a neurologic disorder.

Method: We searched the PubMed and Web of Science databases for studies that had been published up to January 16th, 2019. Read More

Neuroimage 2021 May 30;238:118225. Epub 2021 May 30.

Wellcome Centre for Integrative Neuroimaging, FMRIB, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DU, United Kingdom; School of Health Sciences, Purdue University, 550 Stadium Mall Drive, West Lafayette, IN 47907, (765) 494-1419, United States; Weldon School of Biomedical Engineering, Purdue University, West Lafayette, IN, United States.

Magnetic Resonance Spectroscopy (MRS) allows for the non-invasive quantification of neurochemicals and has the potential to differentiate between the pathologically distinct diseases, multiple sclerosis (MS) and AQP4Ab-positive neuromyelitis optica spectrum disorder (AQP4Ab-NMOSD). In this study we characterised the metabolite profiles of brain lesions in 11 MS and 4 AQP4Ab-NMOSD patients using an optimised MRS methodology at ultra-high field strength (7T) incorporating correction for T2 water relaxation differences between lesioned and normal tissue. MS metabolite results were in keeping with the existing literature: total N-acetylaspartate (NAA) was lower in lesions compared to normal appearing brain white matter (NAWM) with reciprocal findings for myo-Inositol. Read More

Cureus 2021 Apr 26;13(4):e14690. Epub 2021 Apr 26.

Department of Medicine, Ministry of the National Guard-Health Affairs, Jeddah, SAU.

Combined central and peripheral demyelination (CCPD) is a rare disorder characterized by demyelinating lesions in both the central and peripheral nervous systems. The following case report is of a 29-year-old man who presented with a three-month history of progressive lower and upper limb weakness associated with facial and arm tremor, as well as urinary hesitancy. Brain and spine magnetic resonance imaging showed multiple demyelinating plaques. Read More

Front Neurosci 2021 12;15:665722. Epub 2021 May 12.

Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.

Experimental autoimmune encephalomyelitis (EAE) is a model of multiple sclerosis (MS). EAE reflects important histopathological hallmarks, dissemination, and diversity of the disease, but has only moderate reproducibility of clinical and histopathological features. Focal lesions are less frequently observed in EAE than in MS, and can neither be constrained to specific locations nor timed to occur at a pre-specified moment. Read More

Ann Neurol 2021 May 28. Epub 2021 May 28.

Department of Neurology, School of Medical Sciences, University of Campinas (UNICAMP), Campinas, SP, BRAZIL.

Objective: Protein misfolding plays a central role not only in amyotrophic lateral sclerosis (ALS), but also in other conditions, such as frontemporal dementia (FTD), inclusion body myopathy (hIBM) or Paget's disease of bone. The concept of multisystem proteinopathies (MSP) was created to account for those rare families that segregate at least 2 out of these 4 conditions in the same pedigree. The calcium-dependent phospholipid-binding protein annexin A11 was recently associated to ALS in European pedigrees. Read More

Mult Scler 2021 May 28:13524585211018987. Epub 2021 May 28.

Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK/Oxford University Hospital NHS Foundation Trust, Oxford, UK.

Background: Identifying magnetic resonance imaging (MRI) markers in myelin-oligodendrocytes-glycoprotein antibody-associated disease (MOGAD), neuromyelitis optica spectrum disorder-aquaporin-4 positive (NMOSD-AQP4) and multiple sclerosis (MS) is essential for establishing objective outcome measures.

Objectives: To quantify imaging patterns of central nervous system (CNS) damage in MOGAD during the remission stage, and to compare it with NMOSD-AQP4 and MS.

Methods: 20 MOGAD, 19 NMOSD-AQP4, 18 MS in remission with brain or spinal cord involvement and 18 healthy controls (HC) were recruited. Read More

Mult Scler J Exp Transl Clin 2021 Apr-Jun;7(2):20552173211010843. Epub 2021 May 18.

Mellen Center for Multiple Sclerosis, Neurological Institute, The Cleveland Clinic, Cleveland, OH, USA.

Background: Hardware changes can be an unavoidable confound in imaging trials. Understanding the impact of such changes may play an important role in the analysis of imaging data.

Objective: To characterize the effect of equipment changes in a longitudinal, multi-site multiple sclerosis trial. Read More

Psychol Health Med 2021 May 27:1-8. Epub 2021 May 27.

Neuroimaging Laboratory - IRCCS Centro Neurolesi "Bonino Pulejo", Messina, Italy.

Teriflunomide is a drug with immunosuppressive and selective immunomodulatory action, characterized by anti-inflammatory and antiproliferative properties. Several clinical studies have demonstrated the efficacy and safety of this drug in Multiple Sclerosis, estimating a significant improvement in cognitive performance.The aim of our study is to evaluate the effects of teriflunomide by analysing the correlation between brain atrophy and the general cognitive profile and evaluating long-term changes. Read More

Mult Scler Relat Disord 2021 May 7;52:103015. Epub 2021 May 7.

Mellen Center for Multiple Sclerosis Treatment and Research, Cleveland Clinic, Cleveland, OH, USA. Electronic address:

Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a rare fatal autosomal dominant vasculopathy associated with mutations in the TREX1 gene. Only one de novo case has been reported in the literature. We report the long-term clinical, radiological, and pathological presentation of a patient with a de novo and novel mutation in this gene. Read More