Definite Fatal Familial Insomnia (FFI) Disease with Voltage-Gated Potassium Channel (VGKC)-Complex Autoimmunity: A Rare Case of Molecular Mimicry or Incidental Finding? (P5.187)

Conclusions: To date, this is the first reported case of autopsy and genetically-confirmed FFI with a positive VGKC-complex autoantibody titer. Although low titers of VGKC-complex antibody may be an incidental finding, its coincidence in this case may have affected the phenotypic presentation of FFI. Global screening for paraneoplastic antibodies may provide important insight into understanding the complex pathophysiology, including the potential role of molecular mimicry, of prion diseases.Disclosure: Dr. Sanamandra has nothing to disclose. Dr. Youn has nothing to disclose. Dr. Maciel has nothing to disclose. Dr. Schrag has nothing to disclose. Dr. Machado has nothing to disclose. Dr. Gilmore has nothing to disclose.

Source: Neurology - April 3, 2016 Category: Neurology Authors: Sanamandra, S., Youn, T., Maciel, C., Schrag, M., Machado, D., Gilmore, E. Tags: Aging and Dementia: Atypical Dementia Source Type: research