Refractory epilepsy in PSEN 1 mutation (I83T)

Neurocase. 2020 Jun;26(3):167-170. doi: 10.1080/13554794.2020.1747632. Epub 2020 Apr 3.

Abstract

Mutations in the presenilin-1 gene (PSEN1) on chromosome 14 are the most common cause of autosomal dominant Alzheimer's disease (ADAD), which has a broad clinical phenotype, encompassing not only dementia but a variety of other neurological features. We report the case of a 32 years old man with a family history of early onset AD associated with a PSEN1 mutation in the exon 4 (I83T). The proband's, carrying the mutation, present a refractory epilepsy predating cognitive decline. We discuss the physiopathological mechanisms of epilepsy during AD associated with PSEN 1 mutation, the possibility of linking this epilepsy to the mutation?.

Keywords: Alzheimer disease; genetic; presenilin 1 mutation; refractory epilepsy.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Alzheimer Disease / genetics*
  • Drug Resistant Epilepsy / genetics*
  • Genetic Predisposition to Disease
  • Humans
  • Male
  • Mutation
  • Pedigree
  • Presenilin-1 / genetics*

Substances

  • PSEN1 protein, human
  • Presenilin-1