Oligoclonal bands increase the specificity of MRI criteria to predict multiple sclerosis in children with radiologically isolated syndrome

Background: Steps towards the development of diagnostic criteria are needed for children with the radiologically isolated syndrome to identify children at risk of clinical demyelination. Objectives: To evaluate the 2005 and 2016 MAGNIMS magnetic resonance imaging criteria for dissemination in space for multiple sclerosis, both alone and with oligoclonal bands in cerebrospinal fluid added, as predictors of a first clinical event consistent with central nervous system demyelination in children with radiologically isolated syndrome. Methods: We analysed an international historical cohort of 61 children with radiologically isolated syndrome (18 years), defined using the 2010 magnetic resonance imaging dissemination in space criteria (Ped-RIS) who were followed longitudinally (mean 4.2 4.7 years). All index scans also met the 2017 magnetic resonance imaging dissemination in space criteria. Results: Diagnostic indices (95% confidence intervals) for the 2005 dissemination in space criteria, with and without oligoclonal bands, were: sensitivity 66.7% (38.4\u201388.2%) versus 72.7% (49.8\u201389.3%); specificity 83.3% (58.6\u201396.4%) versus 53.9% (37.2\u201369.9%). For the 2016 MAGNIMS dissemination in space criteria diagnostic indices were: sensitivity 76.5% (50.1\u201393.2%) versus 100% (84.6\u2013100%); specificity 72.7% (49.8\u201389.3%) versus 25.6% (13.0\u201342.1%). Conclusions: Oligoclonal bands increased the specificity of magnetic resonance imaging criteria in children with Ped-RIS. Clinicians should consider testing cerebrospinal fluid to improve diagnostic certainty. There is rationale to include cerebrospinal fluid analysis for biomarkers including oligoclonal bands in planned prospective studies to develop optimal diagnostic criteria for radiologically isolated syndrome in children

Posterior fossa involvement in the diagnosis of adult-onset inherited leukoencephalopathies

International audienceAdult-onset genetic leukoencephalopathies are increasingly recognized. They are heterogeneous groups of disorders that commonly have distinct pathologic mechanisms but they share the presence of supratentorial bilateral and symmetric white matter hyperintensities. Although these abnormalities are usually non-specific, some specific MRI findings exist and sometimes help to distinguish these disorders. In this review, our aim is to describe posterior fossa abnormalities seen in the main adult-onset genetic leukoencephalopathies enabling clinicians to perform oriented genetic/metabolic screening

Brain Calcifications in Adult-Onset Genetic Leukoencephalopathies: A Review

International audienceImportance: Adult-onset genetic leukoencephalopathies and leukodystrophies are increasingly recognized as a heterogeneous group of disorders with new diagnostic approaches and potential treatments. In the new era of genomics, the challenging interpretation of individual genetic variations requires an accurate phenotypic description and classification. Clinical and magnetic resonance imaging (MRI)-based approaches have been proposed to improve the diagnostic process of adult-onset leukoencephalopathies. Cerebral calcifications, when associated with white matter hyperintensities, are of major importance in the decision-making process to focus the diagnosis among the diversity of rare causes. Observations: This literature review demonstrated that the morphologic features and topography of the calcifications observed in a careful combined analysis of computed tomographic and MRI scans may help indicate the diagnosis of adult-onset genetic leukoencephalopathies. Vascular genetic leukoencephalopathies are an important cause of leukoencephalopathy with calcifications. Among them, COL4A1-related disorders are frequently associated with spotlike calcifications in the basal ganglia. Adult-onset leukoencephalopathy with axonal spheroids, a probably underestimated disorder, is associated with a specific pattern of calcifications: small, symmetric, sparing the basal ganglia, and a stepping stone appearance in the frontal pericallosal region. Moreover, disorders primarily associated with basal ganglia calcifications, such as primary familial brain calcifications, can be associated with marked leukoencephalopathy. Conclusions and Relevance: The number of identified causes of adult-onset genetic leukoencephalopathies has recently increased. A diagnostic algorithm should take into account the pattern of calcifications to better target the genetic analyses

Low Signals on T2* and SWI Sequences in Patients with MS with Progressive Multifocal Leukoencephalopathy

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Glatiramer acetate-induced hepatitis in a patient with multiple sclerosis

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Optic chiasm and oculomotor nerves involvement in active multiple sclerosis

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Psychological processes associated with insomnia in patients with multiple sclerosis

International audienceStudy Objectives: Despite the high comorbidity of insomnia disorder (ID) with multiple sclerosis (MS), the relevance of psychological processes involved in the maintenance of insomnia is yet to be established in this neurological disorder. This study aimed to ascertain to what extent the suggested emotional, cognitive, and behavioral processes maintaining insomnia are relevant in people with insomnia and MS. Methods: A between-subjects design was used to compare 26 patients with insomnia and MS, with 31 patients with MS only, and with 26 matched neurological disease-free individuals with insomnia. All patients participated in a standardized clinical interview and completed a battery of self-reported measures of cognitive and somatic presleep arousal experienced at bedtime, sleep- or insomnia-related unhelpful beliefs, and sleep-related safety behaviors. All patients with MS underwent a neurological examination. Results: ID comorbid to MS was strongly associated with increased levels of cognitive and somatic arousal, higher endorsement of dysfunctional beliefs about the consequences of insomnia on daytime functioning, and worry about insomnia and more frequent engagement in sleep-related safety behaviors. Patients with MS with ID did not differ from neurological disease-free individuals with insomnia on these measures. No link was found between MS clinical peculiarities and ID diagnosis. Conclusions: ID comorbid to MS is associated with the classical psychological factors perpetuating ID in neurological disease-free individuals with insomnia. Primary care providers and neurologists should consider target-oriented therapies like cognitive behavioral therapy for chronic insomnia as a treatment approach for ID comorbid to MS