Bailliere's clinical neurology最新文献

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Immune neuropathies in childhood. 儿童期的免疫神经病变。
Bailliere's clinical neurology Pub Date : 1996-03-01
J T Sladky
{"title":"Immune neuropathies in childhood.","authors":"J T Sladky","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>In contradistinction to older populations, immune-mediated disorders (principally demyelinating processes) account for nearly half of peripheral neuropathies in childhood. The largest single diagnostic entity is GBS, which makes up approximately 25% of sensorimotor neuropathies in patients under 18 years of age. The clinical features are similar to those encountered in adults, although the prognosis in youngsters appears to be better than in older populations. Despite the absence of prospective data, plasmapheresis seems to be an effective modality for hastening recovery during GBS in children. The use of human immunoglobulin has gained acceptance for the treatment of GBS in adults, but insufficient data exist to draw firm conclusions about it role in the management of paediatric GBS. CIDP is the second most common cause of chronic sensorimotor neuropathy in children. The clinical manifestations of this disorder are extremely variable, and it can mimic the phenotype of several genetically determined neuropathies. The prognosis in this disorder is also relatively good, although a small number of children have significant neurological disability or treatment side-effects. Other immune-mediated neuropathies are relatively infrequent in our experience. When they occur, they are often associated with collagen-vascular diseases or bone marrow transplantation. Peripheral neuropathy in association with HIV infection in children appears to be rare.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"233-44"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19705451","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}
引用次数: 0
Autoimmunity in the peripheral nervous system. 周围神经系统的自身免疫。
Bailliere's clinical neurology Pub Date : 1996-03-01
H P Hartung, R Kiefer, R Gold, K V Toyka
{"title":"Autoimmunity in the peripheral nervous system.","authors":"H P Hartung,&nbsp;R Kiefer,&nbsp;R Gold,&nbsp;K V Toyka","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The PNS is an immunocompetent organ. The participating cellular and humoral elements in the local immune circuitry have been identified. The model disorder EAN is extensively being used to examine the induction, amplification and effector phase of autoimmune responses to peripheral nerve antigens (Figure 9). Potential autoantigens contained in the myelin sheath and on the axolemma have been characterized. Recent years have seen a rapid growth of information concerning the pathogenesis of the Guillain-Barré syndrome, which has turned out to be a heterogeneous disorder, both in clinical characteristics, course and prognosis, and in the underlying pathology. It can be anticipated that using the better understanding of principal mechanisms of autoimmunity in the PNS will aid in the development of more specific and efficacious treatments.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"1-45"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19704410","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}
引用次数: 0
Neuropathies in HIV infection. HIV感染中的神经病变。
Bailliere's clinical neurology Pub Date : 1996-03-01
M C Dalakas, E J Cupler
{"title":"Neuropathies in HIV infection.","authors":"M C Dalakas,&nbsp;E J Cupler","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Peripheral neuropathies represent the most common neurological manifestation in patients infected with HIV infection occurring either early in the infection or during the course of the illness. They present as acute or chronic demyelinating neuropathies (Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy), mononeuritis multiplex, ganglioneuronitis, cytomegalovirus-related polyradiculoneuropathy, autonomic neuropathy or distal painful sensory neuropathy. They are multifactorial in aetiology. Their putative cause (viral, autoimmune, toxic, nutritional, co-infections) are often dictated by the stage of the underlying HIV disease. The virus, which is not found within ganglionic neurones or Schwann cells but only within the endoneurial macrophages, may generate a tissue-specific autoimmune attack by secretion of cytokines that promote trafficking of activated T cells and macrophages within the endoneurial parenchyma. The wide use of the neurotoxic antiretroviral nucleoside analogues ddC, ddI, d4T and 3TC, exacerbate or trigger subclinical neuropathy in many of these patients.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"199-218"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19706233","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}
引用次数: 0
Immunocytochemistry as a diagnostic tool. 免疫细胞化学作为诊断工具。
Bailliere's clinical neurology Pub Date : 1996-03-01
G Stoll
{"title":"Immunocytochemistry as a diagnostic tool.","authors":"G Stoll","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>ICC is a powerful technique to gain information about pathological processes in peripheral nerve beyond the limits of classical histopathology. ICC can aid in the identification of cellular infiltrates (T cells, B cells and macrophages), in the definition of their state of activation (MHC class I and II molecule expression), in the localization of cellular adhesion molecules (vascular and intercellular adhesion molecule 1) involved in trafficking of inflammatory cells through the endothelium and, finally, in the identification of locally produced cytokines (interferon gamma, interleukins and tumour necrosis factor). Moreover, ICC can identify pathological deposits, such as immunoglobulins and amyloid, within nerves and can be used to study the expression of myelin sheath proteins as they change in disease. In this chapter, basic immunocytochemical findings in experimental diseases of the peripheral nervous system, such as WD and immune-mediated demyelination, are summarized and discussed in the context of similar observations in sural nerve biopsies.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"129-42"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19706229","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}
引用次数: 0
Wallerian degeneration in peripheral nerve disease. 周围神经病变中的沃勒氏变性。
Bailliere's clinical neurology Pub Date : 1996-03-01
J W Griffin, E B George, V Chaudhry
{"title":"Wallerian degeneration in peripheral nerve disease.","authors":"J W Griffin,&nbsp;E B George,&nbsp;V Chaudhry","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Wallerian-like degeneration can be regarded as the final shared pathway of the most prevalent diseases of the peripheral nervous system, including many degenerative, metabolic, heritable, toxic, inflammatory and ischaemic disorders. The cellular responses that take place during Wallerian degeneration, including the elaboration of neurotrophins, are increasingly recognized to set the stage for the success or failure of subsequent regeneration, and manipulations of Wallerian degeneration are being investigated as a potential means of altering the outcome of nerve regeneration.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"65-75"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19704412","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}
引用次数: 0
Electron microscopy as a tool to diagnose neuropathies. 电子显微镜作为诊断神经病变的工具。
Bailliere's clinical neurology Pub Date : 1996-03-01
J M Vallat
{"title":"Electron microscopy as a tool to diagnose neuropathies.","authors":"J M Vallat","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Ultrastructural examination of a peripheral nerve biopsy may be particularly useful and sometimes indispensable for identification of the type of nerve lesion and of the aetiologies of peripheral neuropathies. The ultrastructural findings have anyway to be correlated with the clinical findings, the electrophysiological examination and the laboratory investigations. In this presentation, the various causes of peripheral neuropathies for which nerve biopsy study by electron microscope can provide diagnostic information are discussed. The principal aetiologies that will benefit from such an ultrastructural study are toxic, infectious, haemopathic, genetic and storage disorders. Sometimes the electron microscopic examination will help to determine not only the cause of the peripheral neuropathy, but also the mechanism of nerve lesions. Thus, in peripheral nerve pathology, ultrastructural study is an efficient tool among other available, complementary techniques.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"143-56"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19706230","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}
引用次数: 0
Immunology of the Schwann cell. 雪旺细胞免疫学。
Bailliere's clinical neurology Pub Date : 1996-03-01
P J Armati, J D Pollard
{"title":"Immunology of the Schwann cell.","authors":"P J Armati,&nbsp;J D Pollard","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The Schwann cell or its myelin membrane appears to be the focus of autoimmune attack in several peripheral neuropathies. Potential Schwann cell antigens have therefore been extensively studied. Of the known Schwann cell proteins, MBP, P2, MAG, PLP, PO, PMP-22 and Connexin 32, gene defects of the latter three have recently been shown to be responsible for some forms of hereditary sensory and motor neuropathies. The L2/HNK-1 epitope, common to MAG, Po and PMP-22, is the target of autoantibody damage in neuropathies associated with certain IgM paraproteinaemias. P2, the inciting antigen in rat EAN, has no demonstrated role in human neuropathies. The same appears true for the glycolipid galactocerebroside, a neuritogen in rabbits. Gangliosides are currently under intense study, but the antigen for the common inflammatory neuropathies remains undefined. Evidence for the potential role of Schwann cells in immune modulation is provided; Schwann cells produced MHC molecules, the adhesion molecules I-CAM1, L1, L2-HNK-1, Ng-CAM, N-cadherisn, the cytokines IL-1, IL-6 and TNF-alpha and the inflammatory prostanoids, PGE-2 AND TxA2.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"47-64"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19704411","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}
引用次数: 0
Animal models of neuropathies. 神经病动物模型。
Bailliere's clinical neurology Pub Date : 1996-03-01
H Schmalbruch, C Krarup
{"title":"Animal models of neuropathies.","authors":"H Schmalbruch,&nbsp;C Krarup","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Neuropathy in animals is either genetically determined or is provoked by chemical compounds or physical injury. Diabetes in mice and rats may be spontaneous or induced, but a true copy of diabetic neuropathy in man is not yet available. Painful neuropathy occurs after nerve constriction or neuroma formation. A mouse mutant with delayed Wallerian degeneration demonstrates the pivotal role of this process for the regeneration of injured axons. Surprisingly, the neurotoxic effect of cisplatin which is severe in cancer patients has not yet unambiguously been reproduced in animals. Genetically determined diseases in mutants or transgenic animals may affect the myelination of peripheral axons. 'Trembler mice' are deficient in myelin and possibly correspond to CMT IA in man. The relation of sensory neuronopathies in mice, rats and dogs to human diseases is not yet clear. Motor neuronopathies in experimental animals have attracted much interest, because the recent discovery of motoneuronotrophic factors has raised high hopes. Most of the mutants described have not been appropriately studied, and the mouse mutant 'motoneurone disease' (mnd) eventually was found to have Batten's disease. None of the few more thoroughly studied models is probably a copy of human disease, although they may none the less help to test new therapies.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"77-105"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19704413","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}
引用次数: 0
Leprous neuropathies. Leprous慢性疼痛。
Bailliere's clinical neurology Pub Date : 1996-03-01
M F Waters, J M Jacobs
{"title":"Leprous neuropathies.","authors":"M F Waters,&nbsp;J M Jacobs","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Once a terrifying disease, leprosy today has a very hopeful prognosis, provided that it is diagnosed early and treated with modern multidrug chemotherapy, any immunological reactions being recognized quickly and controlled well to prevent (further) peripheral nerve damage after commencing treatment. The diagnosis should be considered in all patients who present with peripheral neuropathy and/or anaesthetic skin lesions who come from or have lived in the tropics and subtropics. Although M. leprae cannot yet be grown in vitro, it is readily grown in experimental animals. A complete gene library has been developed, much of the genome mapped and a number species-specific and common mycobacterial antigens identified. The intricacies of the host-parasite relationship, especially of cell-mediated immunity, and of the important immunological reactions of ENL and reversal reaction have been widely investigated. Modern MDT has caused a dramatic fall in prevalence, although the world annual case detection rate remains at around 600,000 new patients, many being at an early stage of the disease. WHO has launched a campaign to eliminate leprosy as a significant public health risk by the 2000 (with a prevalence of less than 1:10 000 population), which may well be achieved in some endemic countries. Leprosy will, however, remain an important cause of peripheral neuropathy for at least several more decades.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"171-97"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19706232","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}
引用次数: 0
Paraproteinaemic neuropathies. Paraproteinaemic神经病变。
Bailliere's clinical neurology Pub Date : 1996-03-01
G C Miescher, A J Steck
{"title":"Paraproteinaemic neuropathies.","authors":"G C Miescher,&nbsp;A J Steck","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Paraproteinaemia and neuropathy are each relatively frequent and may be associated by chance. However, a number of significant relationships have to be ruled out in the differential diagnosis. Malignant gammopathy should be excluded: multiple myeloma can lead to compression of the spinal cord or cauda equina; primary amyloidosis is occasionally involved; the rare but intriguing POEMS syndrome, consisting of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes, usually accompanies osteosclerotic myeloma. It can be associated with angio-follicular lymph node hyperplasia and needs to be recognized because radioablative therapy is curative. The 'benign' monoclonal gammopathies of undetermined significance, known as MGUS, are much more frequent. There is an IgM MGUS group with predominantly distal sensorimotor demyelinating polyneuropathy and another rather heterogeneous group with IgG or IgA MGUS and a tendency to a favourable response to plasmapheresis. The role of the monoclonal IgG and IgA antibodies is unclear. This chapter has focused on the pathogenetic mechanisms of neuropathies associated with IgM MGUS. In the majority of cases, monoclonal autoantibodies specific for particular carbohydrate epitopes bind to myelin and are now recognized as the primary cause of the disease manifestations, including widening of the myelin lamellae. While the autoantibodies have been shown to bind complement, the presence of inhibitors is invoked to explain the absence of acute inflammatory changes. The epitopes recognized with the highest affinity by the auto-antibodies are present on the myelin-associated glycoprotein (MAG) and could interfere with cell adhesion and cellular signally processes. In addition, binding to antigenically similar glycoproteins, such as PO, PMP-22 and some acidic glycolipids, may be a contributory factor. It is generally accepted that the anti-MAG autoantibodies are inducing a progressive demyelinating polyneuropathy by modifying axon-Schwann cell interactions.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 1","pages":"219-32"},"PeriodicalIF":0.0,"publicationDate":"1996-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19706234","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}
引用次数: 0
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