title: Paraneoplastic encephalomyelopathies: pathology and mechanisms.
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.
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, College of Medicine, Rochester, MN, 55905, USA. mckeon.andrew@mayo.edu
authors.forename: Andrew
authors.initials: A
authors.lastname: McKeon
authors.forename: Sean J
authors.initials: SJ
authors.lastname: Pittock
mesh_terms: D006801:Humans; D020361:Paraneoplastic Syndromes, Nervous System / Q000276:immunology / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology