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Prion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt–Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial insomnia, and Gerstmann–Straussler–Scheinker syndrome), and acquired (kuru, variant CJD, and iatrogenic CJD). This article will review the pathophysiology, genetics, clinical presentations, and diagnostic challenges in patients with prion disease. Case discussions, images, and tables will be used to highlight important characteristics of prion disease and prion mimics.

Antibody mediated limbic encephalitis is an increasingly recognized cause of seizures in cryptogenic epilepsy. Autoimmune encephalitis and epilepsy have been linked to both neuronal intracellular antibodies (GAD65, ANNA-1, and Ma) and neuronal cell surface antibodies (VGKC complex, NMDAR, AMPA, GABA-B, and GluR5). This article outlines the latest data on these various antibodies with a focus on their association with acute seizures in limbic encephalitis and likely increased risk for chronic epilepsy. There is mounting evidence that these antibodies may play a role in acute onset and chronic seizures in the general epilepsy population without manifesting typical limbic encephalitis symptoms. This review will discuss the data supporting early recognition and treatment options, beyond typical antiepileptic medications, necessary to improve outcomes in this epilepsy subgroup.

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