Bilateral manual externalization of testis with self-castration in patient with prion disease

A 62-year-old man with Gerstmann-Straüssler-Scheinker syndrome, an inherited prion disease, presented after having manually externalized both his testes, including self-castration of his left testis. The left testis was avulsed, along with 12 in. of spermatic cord. Given the patient's hospice status and the wishes of his family, the patient was not taken to the operating room for an inguinal exploration. This represents an unusual case of genital self-mutilation.