We have previously reported a new seipin-associated neurodegenerative syndrome (PELD, Progressive Encephalopathy with/without Lipodystrophy or Celia’s Encephalopathy, MIM:#615924) [17] due to a c.985C>T mutation in BSCL2 gene. This evidence concerns the gene BSCL2 and severe neurodegenerative syndrome with lipodystrophy.