Catalog

Paraneoplastic encephalomyelitis, neural autoantibody positive

No MMS code

Link to WHO Definition

Paraneoplastic encephalomyelitis, neural autoantibody positive – http://id.who.int/icd/entity/117504468

Paraneoplastic encephalomyelitis seropositive for neural autoantibodies. When the neural antibodies target plasma membrane antigens they are cause of damage (e.g. gamma-aminobutyric-acid type-B [GABA-B] associated with small cell lung cancer) through multiple effector mechanisms. However, when targeting nuclear or cytoplasmic antigens (e.g. antineuronal nuclear antibody type 1 [ANNA-1 or anti-Hu] associated with small cell lung cancer) they are markers of a T-cell effector mediated injury as they are inaccessible to immune attack in situ, but peptides from intracellular proteins are displayed on upregulated MHC class 1 molecules in a pro-inflammatory cytokine milieu and then accessible to peptide specific cytotoxic T-cells. Associated neural autoantibodies include: AMPA-R (amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antibodies); amphiphysin; ANNA-1(anti-Hu) (antineuronal nuclear autoantibody type 1); ANNA-2(anti-Ri) (antineuronal nuclear autoantibody type 2); ANNA-3 (antineuronal nuclear autoantibody type 3); CRMP5(anti-CV2) (collapsin response mediator protein 5); GABABR (Gamma-aminobutyric-acid antibodies); Ma1; Ma2; mGluR5 (metabotropic glutamate receptor antibody type 5); NMDA-R (N-methyl-D-aspartate receptor autoantibodies); PCA-2 (purkinje cell cytoplasmic autoantibody type 2);