Anti‐Neuronal Nuclear Antibody 3 Autoimmunity Targets Dachshund Homolog 1

The antigen specificity of Anti‐Neuronal Nuclear Antibody‐type 3 (ANNA3)‐IgG is unknown. We identified Dachshund‐homolog 1 (DACH1) as the ANNA3 autoantigen and confirmed it by antigen‐specific assays, immunohistochemical colocalization and immune absorption experiments. Patients' median age was 63.5 years (range, 49–88); 67% were female. Neurological manifestations (information available for 30 patients) included one or more of neuropathy, 12; cognitive difficulties, 11; ataxia, 8; dysautonomia, 7. Evidence of a neoplasm was present in 27 of 30 (90%), most of neuroendocrine lineage. DACH1‐IgG is rare and represents a novel proposed biomarker of neurological autoimmunity and cancer. ANN NEUROL 2022;91:670–675

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