[Therapeutic management of cognitive disorders in multiple sclerosis]

International audienceTaking in charge cognitive disorders is a new concept in the global care of MS patients. Cognitive disorders are observed in the all forms of the disease, sometimes early on in the evolution. These disorders can be evaluated in details even detected despite any complain in the patient. Because of the lack of clear demonstration that disease-modifying treatments could act on cognition, new specific therapeutic issues have emerged during last years. This article first discusses relationships between disease-modifying treatments and cognition for the different forms of the disease, then analyse the effects of symptomatic drug therapy especially the use of anticholinesterasics. In the last part of the article new issues about antagonists of excitatory amino-acids and individual or group cognitive training are discussed. Recent functional imaging data concerning cerebral adaptation and their modifications by drug or non-drug procedures in MS patients suggest interesting therapeutic development in a next future

[Therapeutic management of cognitive disorders in multiple sclerosis]

International audienceTaking in charge cognitive disorders is a new concept in the global care of MS patients. Cognitive disorders are observed in the all forms of the disease, sometimes early on in the evolution. These disorders can be evaluated in details even detected despite any complain in the patient. Because of the lack of clear demonstration that disease-modifying treatments could act on cognition, new specific therapeutic issues have emerged during last years. This article first discusses relationships between disease-modifying treatments and cognition for the different forms of the disease, then analyse the effects of symptomatic drug therapy especially the use of anticholinesterasics. In the last part of the article new issues about antagonists of excitatory amino-acids and individual or group cognitive training are discussed. Recent functional imaging data concerning cerebral adaptation and their modifications by drug or non-drug procedures in MS patients suggest interesting therapeutic development in a next future

Relapsing polychondritis revealed by basal ganglia lesions

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Secondary progressive multiple sclerosis: A national consensus paper on diagnostic criteria

International audienceBackground: In clinical practice, the diagnosis of secondary progressive multiple sclerosis (SPMS) is often delayed, retrospective and non-reproducible, as there are no consensus criteria that define the advent of SPMS. Early identification of SPMS is essential to improve patient care. Methods: Eight regional board meetings in France involving 56 multiple sclerosis (MS) experts (neurologists) were convened to discuss diagnostic criteria for SPMS. Subsequently, a national board meeting of 13 neurologists (with an expert representing each geographical region) was held to review points of convergence or divergence between regions and to develop a national consensus document. Results: Based on the discussions from the regional boards, the MS experts at the national board retained the worsening of the EDSS score, with compatible clinical features, as the only consensus criterion for the diagnosis of SPMS in clinical practice. The patient should have experienced during at least the previous 6 months and in the absence of any relapse, a worsening in the EDSS score of +1.0 point (if the previous EDSS was ≤ 5.0) or of +0.5 point (if the previous EDSS was ≥ 5.5), with a pyramidal or cerebellar functional system score ≥ 2 and without setting a minimum EDSS score; or, in case of a stable EDSS score ≥ 4.0, a worsening of a functional score. This worsening should be confirmed within 3 to 6 months. According to the MS experts, the patient's age, duration of illness and a minimal threshold EDSS score are only risk factors for transition to SPMS. Patient reports during consultation and cognitive impairment are important warning signs, which should trigger an objective assessment with specific tests or closer monitoring. Clinical relapse and/or MRI activities are non-discriminatory for making the diagnosis of SPMS. Conclusions: The experts defined precise diagnostic criteria adapted to clinical practice for earlier identification of SPMS, paving the way for better management of this stage of the disease

Secondary progressive multiple sclerosis: A national consensus paper on diagnostic criteria

International audienceBackground: In clinical practice, the diagnosis of secondary progressive multiple sclerosis (SPMS) is often delayed, retrospective and non-reproducible, as there are no consensus criteria that define the advent of SPMS. Early identification of SPMS is essential to improve patient care. Methods: Eight regional board meetings in France involving 56 multiple sclerosis (MS) experts (neurologists) were convened to discuss diagnostic criteria for SPMS. Subsequently, a national board meeting of 13 neurologists (with an expert representing each geographical region) was held to review points of convergence or divergence between regions and to develop a national consensus document. Results: Based on the discussions from the regional boards, the MS experts at the national board retained the worsening of the EDSS score, with compatible clinical features, as the only consensus criterion for the diagnosis of SPMS in clinical practice. The patient should have experienced during at least the previous 6 months and in the absence of any relapse, a worsening in the EDSS score of +1.0 point (if the previous EDSS was ≤ 5.0) or of +0.5 point (if the previous EDSS was ≥ 5.5), with a pyramidal or cerebellar functional system score ≥ 2 and without setting a minimum EDSS score; or, in case of a stable EDSS score ≥ 4.0, a worsening of a functional score. This worsening should be confirmed within 3 to 6 months. According to the MS experts, the patient's age, duration of illness and a minimal threshold EDSS score are only risk factors for transition to SPMS. Patient reports during consultation and cognitive impairment are important warning signs, which should trigger an objective assessment with specific tests or closer monitoring. Clinical relapse and/or MRI activities are non-discriminatory for making the diagnosis of SPMS. Conclusions: The experts defined precise diagnostic criteria adapted to clinical practice for earlier identification of SPMS, paving the way for better management of this stage of the disease

Adult-onset genetic leukoencephalopathies: a MRI pattern-based approach in a comprehensive study of 154 patients.

International audienceInherited white matter diseases are rare and heterogeneous disorders usually encountered in infancy. Adult-onset forms are increasingly recognized. Our objectives were to determine relative frequencies of genetic leukoencephalopathies in a cohort of adult-onset patients and to evaluate the effectiveness of a systematic diagnostic approach. Inclusion criteria of this retrospective study were: (i) symmetrical involvement of white matter on the first available brain MRI; (ii) age of onset above 16 years. Patients with acquired diseases were excluded. Magnetic resonance imaging analysis identified three groups (vascular, cavitary and non-vascular/non-cavitary) in which distinct genetic and/or biochemical testing were realized. One hundred and fifty-four patients (male/female = 60/94) with adult-onset leukoencephalopathies were identified. Mean age of onset was 38.6 years. In the vascular group, 41/55 patients (75%) finally had a diagnosis [including CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, n = 32) and COL4A1 mutation, n = 7]. In the cavitary group, 13/17 (76%) patients had a diagnosis of EIF2B-related disorder. In the third group (n = 82), a systematic biological screening allowed a diagnosis in 23 patients (28%) and oriented direct genetic screening identified 21 additional diseases (25.6%). Adult-onset genetic leukoencephalopathies are a rare but probably underestimated entity. Our study confirms the use of a magnetic resonance imaging-based classification with a final diagnosis rate of 64% (98/154) cases