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Paraneoplastic syndromes affecting the central nervous system

J B Posner¹, J O Dalmau

¹Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA. Posner@Neuro.mskcc.org

Annual Review of Medicine

|January 1, 1997

Summary

Paraneoplastic neurological syndromes are rare autoimmune disorders triggered by cancer. While some conditions respond to autoantibody removal, others like cerebellar degeneration remain challenging to treat.

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Area of Science:

Neurology
Oncology
Immunology

Background:

Paraneoplastic syndromes are rare neurologic disorders caused by cancer, distinct from metastases.
These syndromes involve an immune response targeting antigens common to both cancer and nervous system tissue.
Any part of the nervous system can be affected.

Purpose of the Study:

To summarize the nature and potential treatments of paraneoplastic neurological syndromes.
To highlight the heterogeneity in treatment responses among different paraneoplastic disorders.

Main Methods:

Review of existing literature on paraneoplastic neurological syndromes.
Analysis of pathogenesis, clinical presentation, and treatment outcomes.
Categorization of syndromes based on response to immunotherapy and cancer treatment.

Main Results:

Paraneoplastic syndromes involve immune reactions against shared antigens.
Lambert-Eaton myasthenic syndrome shows effective treatment via autoantibody removal.
Paraneoplastic cerebellar degeneration and sensory neuronopathy exhibit poor response to current treatments.

Conclusions:

Treatment efficacy for paraneoplastic neurological syndromes varies significantly.
Understanding the specific immune targets is crucial for developing effective therapies.
Further research is needed for refractory cases like paraneoplastic cerebellar degeneration.