Specific mutations in the human neuroserpin gene are known to result in a form of autosomal dominant inherited dementia that is characterized by the presence of intraneuronal inclusion bodies, called Familial Encephalopathy with Neuroserpin Inclusion Bodies (FENIB; OMIM: 604218) [8-10]. Here, SERPINI1 is linked to familial encephalopathy with neuroserpin inclusion bodies.