Encephalopathy, Familial, with Neuroserpin Inclusion Bodies, also known as familial encephalopathy with neuroserpin inclusion bodies, is related to progressive dementia with neuroserpin inclusion bodies and progressive myoclonic epilepsy with neuroserpin inclusion bodies, and has symptoms including myoclonus, seizures and abnormality of extrapyramidal motor function. An important gene associated with Encephalopathy, Familial, with Neuroserpin Inclusion Bodies is SERPINI1 (Serpin Family I Member 1), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Signaling by Hedgehog. The drug Astragalus has been mentioned in the context of this disorder. Affiliated tissues include cortex and brain, and related phenotypes are seizure and nystagmus