Therapy of paraneoplastic disorders of the CNS

Paraneoplastic neurological syndromes affecting the CNS are rare, presenting in less than 1% of all those with cancer. However, they account for significant disability and may respond to treatment. The pathogenesis of paraneoplastic neurological syndromes is presumed to relate to loss of self-tolerance spilling over from the immune attack on the underlying neoplasm. Testing for anti-neuronal antibodies is now available in most tertiary laboratories, enabling targeted therapies. While the evidence base for treatment is limited, the response to treatment can be largely determined based on the location of the target antigen; antibodies against cell surface antigens responding well to treatments targeting the humoral response. Intracellular antigen-target syndromes respond less well, but may theoretically respond best to T-cell based therapies. In both cases, aggressive tumor therapy is indicated.