We thank Schott and colleagues for their comments. We agree that Creutzfeldt-Jakob disease must be considered in the differential diagnosis of patients with encephalopathy, and brain biopsy is important because the 14-3-3 protein was found in the cerebrospinal fluid in 1 patient with Hashimoto encephalopathy.1 In addition, Doherty et al2 described a patient with Hashimoto encephalopathy in whom brain biopsy revealed vacuoles and gliosis suggestive of prion disease; a few inflammatory cells were seen in the parenchyma and perivascularly as well, which is atypical of Creutzfeldt-Jakob disease.
Chong JY, Rowland LP, Utiger RD. The Uncertain Nosology of Hashimoto Encephalopathy—Reply. Arch Neurol. 2003;60(12):1812. doi:10.1001/archneur.60.12.1812-a