Autoantibodies against axonal neurofilaments in patients with Kuru and Creutzfeldt-Jakob disease.

The serums of some patients with subacute spongiform encephalopathies contain an autoantibody in higher titer against a normal fibrillar protein within the axon of mature central neurons in culture. The morphological features of this neurofilament, as demonstrated by immunofluorescence and immunoperoxidase staining, and the partial characterization of the antibody are described. The detection of this hetero-specific autoantibody is the first evidence of an immune reaction in the spongiform encephalopathies.