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Paraneoplastic Neurological Syndromes

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Cerebrospinal Fluid in Clinical Neurology

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Abstract

In this chapter, we summarise the current knowledge about a group of clinically diverse neurological syndromes that are unified by a common pathophysiology, mostly immune-mediated neuronal dysfunction or degeneration triggered by a distant tumour and thus are coined paraneoplastic neurological syndromes (PNS). In the majority of cases, antibodies against onconeural, intracellular antigens expressed by the tumour and the nervous system can be detected in serum and cerebrospinal fluid. Some clinical pictures are suggestive of an underlying PNS. These syndromes include progressive cerebellar degeneration, encephalomyelitis, limbic encephalitis, opsoclonus-myoclonus syndrome, subacute sensory neuronopathy, chronic gastrointestinal pseudo-obstruction and Lambert-Eaton myasthenic syndrome. These clinical manifestations are called classical paraneoplastic syndromes. Other neurological syndromes like brainstem encephalitides or neuropathies can have diverse aetiologies, amongst which a tumour-triggered immune response is one possibility amongst many others. The detection of onconeural antibodies in suspected PNS confirms the diagnosis and aids in diagnosis, tumour search and therapy. Here, we summarise the practical approach, typical findings and the most important syndromes including electrophysiological tests, imaging and cerebrospinal fluid analysis. Not obligatorily paraneoplastic autoimmune encephalitides associated with antibodies against neuronal surface proteins are covered by a separate chapter of this book.

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Authors and Affiliations

  1. Universitätsklinik für Neurologie, Universitäts- und Rehabilitationskliniken Ulm, Oberer Eselsberg 45, Ulm, 89081, Germany

    Jan Lewerenz

  2. Neuroimmunologie, Klinische Chemie und Neuroimmunologische Ambulanz, NeurologieUniversitätsklinik Schleswig-Holstein Campus, Kiel Arnold-Heller-Str. 3, Kiel, 24105, Germany

    Frank Leypoldt

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  1. Department of Neurology, Innsbruck Medical University, Innsbruck, Austria

    Florian Deisenhammer

  2. Department of Neurology, Danish Multiple Sclerosis Center Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

    Finn Sellebjerg

  3. Department of Clinical Chemistry, VU University Medical Center Amsterdam Neurochemistry Laboratory and Biobank, Amsterdam, The Netherlands

    Charlotte E Teunissen

  4. Department of Neurology, University of Ulm, Ulm, Germany

    Hayrettin Tumani

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Lewerenz, J., Leypoldt, F. (2015). Paraneoplastic Neurological Syndromes. In: Deisenhammer, F., Sellebjerg, F., Teunissen, C., Tumani, H. (eds) Cerebrospinal Fluid in Clinical Neurology. Springer, Cham. https://doi.org/10.1007/978-3-319-01225-4_21

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