Bailliere's clinical neurology最新文献

{"title":"Dementia with Lewy bodies.","authors":"Lennox Gg, Lowe Js","doi":"10.1016/b978-0-323-44781-2.50192-3","DOIUrl":"https://doi.org/10.1016/b978-0-323-44781-2.50192-3","url":null,"abstract":"","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 1","pages":"483-8"},"PeriodicalIF":0.0,"publicationDate":"2016-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"53977405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progressive supranuclear palsy and corticobasal degeneration.","authors":"D. Dickson, J. Hauw, Y. Agid, I. Litvan","doi":"10.1002/9781444341256.CH15","DOIUrl":"https://doi.org/10.1002/9781444341256.CH15","url":null,"abstract":"Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic neurodegenerative disorders of mid to late adult life that have shared clinical and pathologic features, but also notable differences that warrant their separation as clinicopathologic entities. Among shared clinical features are extrapyramidal signs similar to Parkinson's disease, but neither PSP nor CBD is responsive to levodopa therapy. Motor abnormalities in PSP are usually symmetrical, whereas asymmetry is the hallmark of CBD. Focal cortical signs, such as apraxia and aphasia, are common in CBD, but rare in PSP. Dementia is more common in CBD than PSP. Severe vertical gaze palsy early in the disease course is common in PSP, but is uncommon or a late manifestation of CBD. Pathologically, both PSP and CBD are associated with neuronal and glial filamentous inclusions that are composed of tau protein. The morphology of neuronal and glial lesions differs in PSP and CBD, but there are a number of lesions with transitional or overlapping features. Distribution of the lesions shows considerable overlap, but the overall distribution of the lesions differs in PSP and CBD. Cortical gray and white matter lesions are prominent in CBD, whereas deep gray matter lesions are more common in PSP. Biochemical studies of brain tissue from PSP and CBD show similar alterations in tau protein. Abnormal tau proteins in PSP and CBD are relatively insoluble and hyperphosphorylated. Furthermore, they appear to be composed of tau enriched in specific tau mRNA splice forms, specifically tau derived from alternative spicing of exon 10, which generates tau with four repeat regions in the microtubule-binding domain. It is unclear if this is due to disease-related differential expression of these tau isoforms, involvement of cell types that express these isoforms preferentially, or selective assembly of these specific tau isoforms in the lesions. Both PSP and CBD are considered to be nonfamilial or sporadic “tauopathies,” but genetic studies suggest that polymorphisms in the tau gene may confer some degree of genetic risk for these disorders. Given the relative rarity of these conditions, further clinical and pathologic studies are needed to define the diagnostic boundaries and to develop biologic markers for their clinical and pathologic differentiation.","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"78 7","pages":"135-155"},"PeriodicalIF":0.0,"publicationDate":"2011-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50791247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chronic inflammatory demyelinating polyradiculoneuropathy.","authors":"J. Pollard","doi":"10.1097/00019052-200206000-00009","DOIUrl":"https://doi.org/10.1097/00019052-200206000-00009","url":null,"abstract":"In chronic inflammatory demyelinating polyradiculopathy differing clinical subtypes are beginning to emerge as has already occurred with the Guillain-Barre syndrome. However, neither pathogenic correlates nor particular therapeutic approaches have yet been defined for these subgroups. The neurophysiological techniques of terminal latency index and of modified F ratio help differentiate chronic inflammatory demyelinating polyradiculopathy from IgM paraproteinaemic neuropathy. Diagnosis may be assisted by magnetic resonance imaging studies in which enlarged nerve roots and plexuses and gadolinium enhancement may be evident. Further insight into pathogenesis has come from studies showing pathogenic antibodies in a small percentage of patients. Immunohistological studies examining the presence of adhesion, co-stimulatory and antigen presenting molecules in nerve biopsies have shown that T cell activation can be initiated and perpetuated within nerve and that Schwann cells possess the necessary markers to function as antigen presenting cells. Recent clinical trials have confirmed the therapeutic short term efficacy of intravenous immunoglobulin and Prednisone.","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"113 1","pages":"107-27"},"PeriodicalIF":0.0,"publicationDate":"2002-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79857670","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The immunopathogenesis of multiple sclerosis.","authors":"G Giovannoni","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The aetiology of multiple sclerosis (MS) remains unknown. Evidence from clinical and animal studies strongly supports MS as an organ-specific autoimmune disease mediated by T helper 1 CD4+ autoreactive T cells. This chapter will review the evidence in favour of this theory, discuss central nervous system autoimmune hypotheses and outline the important inflammatory mechanisms involved in the immunopathogenesis of MS. The immunology applicable to the acute or isolated MS lesion is presented. Relevant clinical aspects of the disease are discussed to support and highlight potential inconsistencies in current thinking. Hypotheses are presented where parts of the immunopathogenic jigsaw puzzle remain incomplete, for example the mechanisms responsible for disease evolution. Where necessary, supportive evidence from the animal model experimental allergic encephalomyelitis is presented.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"387-407"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20973601","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Remyelination in demyelinating disease.","authors":"N J Scolding,&nbsp;R J Franklin","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>In multiple sclerosis, partial remyelination is conspicuous in many lesions, and is thought to contribute significantly to lasting recovery from acute relapse. However, myelin repair ultimately fails during progression of the disease, as disability and handicap accumulate. In this chapter we explore the biological background to myelin repair in CNS demyelinating disease, and the reasons underlying the failure of more widespread and lasting remyelination in multiple sclerosis. Experimental studies provide clear evidence that therapies promoting myelin repair can be highly successful in the CNS, and we discuss the clinical approaches which might allow the translation of these laboratory studies to neurological practice, together with some of the potential hazards and pitfalls likely to arise.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"525-48"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20974008","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Spasticity, ataxia and fatigue in multiple sclerosis.","authors":"J Kesselring,&nbsp;A J Thompson","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Multiple sclerosis frequently results in a wide range of symptoms which often coexist, creating a complex pattern of disability. Chief among these symptoms, both in relation to their frequency and their impact on the patient, are spasticity, ataxia and fatigue. This chapter discusses the pathological basis and current treatment of these symptoms and stresses the importance of a multidisciplinary approach to their management, producing a comprehensive care plan which incorporates these and any other coexisting problems.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"429-45"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20973437","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Copolymer-1.","authors":"G Comi,&nbsp;L Moiola","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Copolymer 1 (Cop-1) is a mixture of synthetic polypeptides composed of four amino acids. Cop-1 was very effective in suppression of experimental autoimmune encephalomyelitis (EAE), the animal model of multiple sclerosis (MS). Two principal mechanisms have been proposed to explain the suppressive activity of Cop-1 in EAE and in multiple sclerosis (MS): the induction of antigen-specific suppressor T cells and the interference with T-cell activation by competition with myelin antigens in binding to the major histocompatibility complex class II molecules. Clinical trials with Cop-1 have demonstrated that Cop-1 positively alters the course of relapsing-remitting multiple sclerosis (MS) by both reducing the relapse rate and slowing the progression of disability. In a 2-year multi-centre, randomized, double-blind, placebo-controlled trial of 251 patients, Cop-1 was shown to reduce relapses by an average of 29% when compared with placebo. A preliminary study on patients with relapsing-remitting MS treated with Cop-1 showed a reduced number of new enhancing lesions on MRI as well as a reduced accumulation of lesion load during Cop-1 treatment. Antibodies to copolymer-1, which are found during Cop-1 treatment, do not interfere with its clinical effects. The side effects of Cop-1 are minimal and acceptable. In vitro and in vivo animal studies have shown a very good safety profile of Cop-1 which is devoid of teratogenic or mutagenic effects. Cop-1 joins interferon beta as a good candidate for treatment of relapsing-remitting MS.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"495-509"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20973444","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcome measures in multiple sclerosis clinical trials.","authors":"P B Andersson,&nbsp;D E Goodkin","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Multiple sclerosis remains incurable. The urgency of this problem, together with the need to test an ever increasing number of promising therapeutic agents emerging from animal studies, has renewed interest in both the application and the development of efficient outcome measures for treatment trials. The selection and usage of instruments used to detect outcomes is probably the most important issue in treatment trial design. Thus, familiarity with them is essential not only for researchers, but also for clinicians who wish to interpret trial results critically. This chapter begins with a discussion of the properties of an ideal outcome instrument, then reviews the strengths and limitations of existing clinical, radiological and laboratory outcome measures. With this knowledge, the current consensus regarding appropriate outcome measure selection, as well as recommendations and future perspectives in trial design, are discussed.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"409-28"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20973602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Treatment of multiple sclerosis with interferon beta 1b.","authors":"S Dhib-Jalbut,&nbsp;H F McFarland","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Interferon beta 1b is now an established therapeutic option for relapsing remitting multiple sclerosis. More recently, it has also been shown to slow down disease progression in secondary progressive multiple sclerosis. Interferon beta 1b's clinical effect is reflected in MRI studies demonstrating a dramatic effect in reducing disease activity. The drug is generally well tolerated, but its efficacy can be compromised in some patients by the emergence of neutralizing antibodies. This chapter will focus on interferon beta 1b (Betaseron) treatment for multiple sclerosis, its clinical and MRI effects, and its putative mechanism of action.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"467-80"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20973441","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Other immunomodulatory therapies in multiple sclerosis.","authors":"C H Polman","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>This short review concentrates on immunomodulatory disease-modifying approaches that are under development for the treatment of multiple sclerosis based on present concepts of the immunopathogenesis of the disease that especially involve T-cells and macrophages as being prominently involved in inducing tissue destruction and on the advances that have been made in recent years by studying other treatment interventions that have been applied and that have been shown to be either successful (interferon beta, copolymer-1) or unsuccessful (many others).</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 3","pages":"511-24"},"PeriodicalIF":0.0,"publicationDate":"1997-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20974007","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}