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Acta Neuropathologica

, 122:381 | Cite as

Paraneoplastic encephalomyelopathies: pathology and mechanisms

  • Andrew McKeon
  • Sean J. Pittock
Review

Abstract

The last three decades have seen major advances in the understanding of paraneoplastic and idiopathic autoimmune disorders affecting the central nervous system (CNS). Neural-specific autoantibodies and their target antigens have been discovered, immunopathology and neuroimaging patterns recognized and pathogenic mechanisms elucidated. Disorders accompanied by autoantibody markers of neural peptide-specific cytotoxic effector T cells [such as anti-neuronal nuclear antibody type 1 (ANNA-1, aka anti-Hu), Purkinje cell antibody type 1 (PCA-1, aka anti-Yo) and CRMP-5 IgG] are generally poorly responsive to immunotherapy. Disorders accompanied by neural plasma membrane-reactive autoantibodies [the effectors of synaptic disorders, which include antibodies targeting voltage-gated potassium channel (VGKC) complex proteins, NMDA and GABA-B receptors] generally respond well to early immunotherapy. Here we describe in detail the neuropathological findings and pathophysiology of paraneoplastic CNS disorders with reference to antigen-specific serology and neurological and oncological contexts.

Keywords

Paraneoplastic Antibody Encephalopathy Encephalomyelopathy Cancer 

Notes

Acknowledgments

The authors wish to acknowledge Dr Vanda A. Lennon for critical revision of the manuscript and provision of Fig. 2.

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© Springer-Verlag 2011

Authors and Affiliations

  1. 1.Department of Laboratory Medicine and PathologyMayo Clinic, College of MedicineRochesterUSA
  2. 2.Department of NeurologyMayo Clinic, College of MedicineRochesterUSA
  3. 3.RochesterUSA

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