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2015 ; 21
(2 Neuro-oncology
): 452-75
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Paraneoplastic disorders
#MMPMID25837906
Lancaster E
Continuum (Minneap Minn)
2015[Apr]; 21
(2 Neuro-oncology
): 452-75
PMID25837906
show ga
PURPOSE OF REVIEW: Paraneoplastic disorders are autoimmune diseases associated
with risks for specific cancers and marked by specific autoantibodies. They cause
diverse clinical syndromes of the central and peripheral nervous systems. RECENT
FINDINGS: In the peripheral nervous system, autoimmunity to synaptic or axonal
proteins has long been recognized to associate with specific cancers. In these
disorders, typified by myasthenia gravis, the antibodies are directly toxic, and
recovery with immunotherapy is the rule. In contrast, the classic paraneoplastic
syndromes involve a higher risk of cancer, autoantibodies to intracellular
proteins (eg, Hu proteins), T-cell-dependent disease mechanisms targeting the CNS
or peripheral nervous system, and a poor response to treatment. Following the
discovery of N-methyl-D-aspartate (NMDA) receptor antibodies, a new and expanding
group of disorders involving autoantibodies to CNS synaptic and neuronal membrane
proteins and a favorable response to immunotherapy emerged. A final group of
disorders involves antibodies to intracellular synaptic proteins, such as
glutamic acid decarboxylase 65 (GAD65), and it is unclear whether these diseases
involve antibody or T-cell mechanisms. SUMMARY: Neurologists should recognize the
clinical syndromes associated with paraneoplastic disorders, utilize autoantibody
and other testing to confirm the diagnosis, understand the pathologic basis of
the diseases, and promptly give appropriate therapies.
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