1,721,122 research outputs found
Diagnosis and treatment of paraneoplastic neurological disorders
Full text linkIn about two thirds of cases, patients with paraneoplastic neurological disorders present to the neurologist without a known tumor. Due to the ongoing immune response, this tumor tends to stay biologically relatively benign, and therefore difficult to diagnose. In patients with a known tumor, the neurological symptoms often precede a tumor recurrence. In both scenarios, anti-neuronal antibodies are an invaluable diagnostic help to the clinician, and may be supplemented by other diagnostic tests such as MRI, CSF, and electrophysiology. Tumor therapy remains the mainstay of therapeutic options, although early immune therapy must be started in parallel. It is hoped that the recent fundamental advances in understanding the autoimmune pathology of these disorders, especially the role of cytotoxic T cells, will eventually lead to more effective treatment options
La crise monétaire du 14e siècle
No full textGraus F. La crise monétaire du 14e siècle. In: Revue belge de philologie et d'histoire, tome 29, fasc. 2-3, 1951. pp. 445-454
Origines de l'État et de la noblesse en Moravie et en Bohême
No full textGraus F. Origines de l'État et de la noblesse en Moravie et en Bohême. In: Revue des études slaves, tome 39, fascicule 1-4, 1961. pp. 43-58
Autoimmunity in paraneoplastic neurological syndromes
No full textIn patients with Paraneoplastic Neurological Disorders, the researcher detected several autoantibodies reacting with neuronal antigens and tumors; their characteristics supported the hypothesis that autoimmunity plays a part in these diseases and gave impetus to the study of these neurological disorders. The relationship between detection of anti-neuronal antibodies, clinical syndromes, and certain types of tumors suggested the utility of these antibodies as a new tool for clinical diagnosis, although their function in the pathogenesis of the various syndromes is still unclear. This paper intends to review the characteristics of the anti-neuronal antibodies so far identified, their correlation with clinical syndromes, and the function of antigens, In addition, the paper will offer some insights on the immunological mechanisms of neuronal damage and on treatment options
Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition
No full textThe concept of antibody mediated CNS disorders is relatively recent. The classical CNS paraneoplastic neurological syndromes are thought to be T cell mediated, and the onconeural antibodies merely biomarkers for the presence of the tumour. Thus it was thought that antibodies rarely, if ever, cause CNS disease. Over the past 10 years, identification of autoimmune forms of encephalitis with antibodies against neuronal surface antigens, particularly the voltage gated potassium channel complex proteins or the glutamate N-methyl-D-aspartate receptor, have shown that CNS disorders, often without associated tumours, can be antibody mediated and benefit from immunomodulatory therapies. The clinical spectrum of these diseases is not yet fully explored, there may be others yet to be discovered and some types of more common disorders (eg, epilepsy or psychosis) may prove to have an autoimmune basis. Here, the known conditions associated with neuronal surface antibodies are briefly reviewed, some general aspects of these syndromes are considered and guidelines that could help in the recognition of further disorders are suggested
Autoimmune paraneoplastic cerebellar degeneration: immunohistological localization of antibody-binding sites
No full textSera from 3 patients with breast or ovarian tumors and paraneoplastic cerebellar degeneration (PCD) contained anti-Purkinje cell antibodies (PCAbs) which also bind to other neurons on frozen sections of adult rodent brain. PCAbs tested on new-born rodent (rabbit, rat, mouse) brain tissue detected only oligodendrocyte-like cells (ODLC) in the white matter and allowed us to speculate on the nature of the antigenic structure in the neuronal cytoplasm. All these PCAbs appear histochemically identical and recognize antigens which belong to so-called "Yo" proteins. © 1994
Anti-GAD epileptic encephalopathy in a toddler with Parry-Romberg syndrome
No full textParry-Romberg syndrome (PRS) is a progressive facial hemiatrophy often associated with severe epilepsy. Although an immune-mediated vasculitic pathogenesis is widely assumed, no CNS-specific autoantibody has been described so far. A 2-year-old boy was admitted for a status epilepticus preceded by fever, restlessness, insomnia, and left facial rash. Cerebrospinal fluid was positive for glutamic acid decarboxylase (GAD)-antibodies. Brain MRI revealed FLAIR hyperintensities on left mediotemporal areas. He was successfully treated with intravenous methylprednisolone. One month later, seizures and facial rash reappeared and steroids were satisfactorily repeated. However, left hemifacial rash reappeared 5 months later, slowly followed by sclerotic skin lesions on frontal scalp and hemifacial sub-atrophy, leading to a diagnosis of PRS. Three years later, and despite chronic immunosuppression, new MRI lesions on left white matter are seen and left hemifacial atrophy has progressed. For the first time, we describe GAD autoantibodies in a PRS patient with epileptic encephalopathy. Epileptic syndromes with GAD autoantibodies are frequently described though with a questionable pathogenic significance. Given the clinical and MRI similarities of PRS with both Morphea and Rasmussen’s encephalitis, we suggest that, in our patient, the initial facial skin vasculitis spread into CNS vessels through perforating arteries, inducing neuronal MHC-class I presentation of GAD epitopes, ultimately causing CD8-mediated neuronal cytotoxicity and the epileptic encephalopathy. GAD autoantibodies might represent the missing pathophysiological link between PRS and neuropsychiatric manifestations
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