Neurological Paraneoplastic Syndromes

7/03/02 (Ebert)

Question: what is currently known about the neurologic paraneoplastic syndromes?

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Unique Identifier:9511883

Authors: Dropcho EJ.

Institution: Department of Neurology, Indiana University Medical Center, Indianapolis 46202, USA. dropcho@iucc.iupui.edu

Title: Neurologic paraneoplastic syndromes. [Review] [96 refs]

Source: Journal of the Neurological Sciences. 153(2):264-78, 1998 Jan 8.

Abstract: Several neurologic paraneoplastic disorders are believed to be caused by an autoimmune reaction against antigen(s) co-expressed by tumour cells and neurons. Of the paraneoplastic syndromes, the evidence for an autoimmune etiology is strongest for the Lambert-Eaton myasthenic syndrome, in which autoantibodies downregulate voltage-gated calcium channels at the presynaptic nerve terminal. For other syndromes, including cerebellar degeneration, multifocal encephalomyelitis, sensory neuronopathy, limbic encephalitis, opsoclonus-myoclonus, stiff person syndrome, and retinal degeneration, the autoimmune theory is supported by the presence of specific antineuronal antibodies. These antibodies serve as a useful diagnostic tool, but their actual role in causing neuronal injury and clinical disease remains unclear. Further understanding of immunopathogenesis awaits successful experimental models. Among different syndromes, a varied proportion of patients shows neurologic improvement with immunosuppressive treatments; it is likely that many patients have already suffered irreversible neuronal injury at the time of diagnosis. [References: 96]

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Unique Identifier:8811308

Authors: Hinton RC.

Institution: Department of Neurology, University of Texas Southwestern Medical School, Dallas, USA.

Title: Paraneoplastic neurologic syndromes. [Review] [97 refs]

Source: Hematology - Oncology Clinics of North America. 10(4):909-25, 1996 Aug.

Abstract: Malignancies may produce indirect effects on the central and peripheral nervous systems, as well as on muscle. Certain autoantibodies have been detected in some of these paraneoplastic syndromes, and these antibodies most likely play a role in their pathogenesis. This article describes the currently accepted paraneoplastic neurologic syndromes along with current information regarding their pathogenesis. [References: 97] CAS Registry/EC Number 0 (Autoantibodies).