The clinical diagnosis of PNS requires the exclusion of other, more frequent causes, namely infectious and non-neoplastic-induced autoimmune disorders, cancers (including focal lesions as well as carcinomatous meningitis), rapidly progressive neurodegenerative disorders (e.g., prion disease, dementias, and motor neuron diseases which may present similarly to anti-Ma2-associated syndromes), and toxic/metabolic conditions [16]. Here, PNMA2 is linked to prion disease.