Obstetric dilemma on the most appropriate management of Creutzfeldt-Jakob disease in pregnancy: Seventh case presentation, literature review and new insight

Stefania Di Gangi, Martina Bertin, Marco Noventa, Annachiara Cagnin, Erich Cosmi, Salvatore Gizzo

Research output: Contribution to journalArticlepeer-review

Abstract

Prion diseases (PDs) are fatal neurological disorders that are thought to be caused by the accumulation of an altered variant of a benign, widely expressed protein (PrPC) into a distinct pathological conformation(s) (PrPSc). The PDs are so rare but lethal pathologies that need an early diagnosis to adequately support the infected patient. A maternal-fetal transmission during pregnancy has been supposed to be on the basis of animal studies, but till now the effective vertical transmission in humans has not been proved. We present a case of infected pregnant woman with a peculiar pregnancy course and outcome. We also provided a systematic literature review to find the best obstetrical management of women affected by prionic disease during pregnancy. The available data underline the potential risk of prenatal and postnatal transmission of the disease but do not permit to define the exact molecular mechanism of transmission, the best follow-up and recommendations that are useful in both obstetrical and neonatal practice. At present awaiting for further clarifications about this topic, it is mandatory to personalize the management of this rare pregnancy complication according to the maternal-fetal well-being status.

Original languageEnglish
Pages (from-to)254-261
Number of pages8
JournalJournal of Maternal-Fetal and Neonatal Medicine
Volume28
Issue number3
DOIs
Publication statusPublished - Feb 1 2015

Keywords

  • Creutzfeldt-Jakob disease
  • Maternal-fetal transmission
  • Neurodegenerative disease
  • Pregnancy management
  • Pregnancy outcome
  • Prion disease
  • PrP protein

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Obstetrics and Gynaecology

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