Title

Neurologic paraneoplastic syndromes

Author

Dropcho EJ

Address

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

Source

J Neurol Sci, 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.

Language

English

Unique Identifier

98170833

MESH Headings

Animal ; Autoimmune Diseases PA/PP/PX ; Human ; Nervous System Neoplasms PA/*PP/PX ; Paraneoplastic Syndromes PA/*PP/PX

Publication Type

JOURNAL ARTICLE; REVIEW; REVIEW, ACADEMIC

ISSN

0022-510X

Country of Publication

NETHERLANDS