Catalog

Paraneoplastic encephalitis

No MMS code

Link to WHO Definition

Paraneoplastic encephalitis – http://id.who.int/icd/entity/704882202

Paraneoplastic encephalitis results from a targeted attack on the central nervous system as a consequence of a potentially effective tumour immune response initiated by onco-neural antigens derived from a systemic cancer. It has multifocal neurological accompaniments including: cortical dysfunction (seizures, aphasia, cognitive difficulties), basal ganglia dysfunction (see paraneoplastic movement disorder definition) or a brainstem syndrome (see brainstem encephalitis definition). Neurological symptoms typically precede cancer detection. The autoantibody profile in the serum or cerebrospinal fluid predicts the cancer type [see paraneoplastic encephalitis: neural autoantibody positive definition]. Investigations may reveal T2-signal abnormalities in involved areas on MRI and an inflammatory spinal fluid. Associated neural autoantibodies include amino- 3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antibodies (AMPA-R); amphiphysin; antineuronal nuclear autoantibody type 1 (ANNA-1[anti-Hu]); antineuronal nuclear autoantibody type 2 (ANNA-2[anti-Ri]); antineuronal nuclear autoantibody type 3 (ANNA-3); collapsin response mediator protein 5 (CRMP5 [anti-CV2]); Gamma-aminobutyric-acid type-B (GABABR); Ma1; Ma2; metabotropic glutamate receptor antibody type 5 (mGluR5); N-methyl-D-aspartate receptor autoantibodies (NMDA-R); purkinje cell cytoplasmic autoantibody type 2 (PCA-2); and purkinje cell autoantibody-Tr (PCA-Tr).