Catalog

Paraneoplastic brainstem encephalitis, neural autoantibody positive

No MMS code

Link to WHO Definition

Paraneoplastic brainstem encephalitis, neural autoantibody positive – http://id.who.int/icd/entity/1483064286

Paraneoplastic brainstem encephalitis seropositive for neural autoantibodies. When these neural antibodies target plasma membrane antigens they are effectors of injury (e.g. voltage gated potassium channel complex autoantibodies) through multiple effector mechanisms. However, when targeting nuclear or cytoplasmic antigens (e.g. antineuronal nuclear antibody type 2 [ANNA-2 or anti-Ri] associated with lung and breast cancers) 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 type-A autoantibodies); Ma1; Ma2;