Mutations in the neuroserpin gene cause a rare autosomal-dominant dementia accompanied by epilepsy called Familial Encephalopathy with Neuroserpin Inclusion Bodies (FENIB), characterized by polymerization of neuroserpin, formation of inclusion bodies and subsequent neuronal degeneration (Davis et al., 1999, 2002; Takao et al., 2000; Gourfinkel-An et al., 2007; Coutelier et al., 2008; Hagen et al., 2011). Here, SERPINI1 is linked to epilepsy.