Obstetric dilemma on the most appropriate management of Creutzfeldt–Jakob disease in pregnancy: seventh case presentation, literature review and new insight. (February 2015)

• Record Type: Journal Article
• Authors: Di Gangi, Stefania; Bertin, Martina; Noventa, Marco; Cagnin, Annachiara; Cosmi, Erich; Gizzo, Salvatore
• Is Part Of: Journal of maternal-fetal & neonatal medicine. Volume 28:Number 3(2015)
• Page Start: 254
• Page End: 261
• Publication Date: 2015-02
• Subjects: Obstetrics -- Periodicals; Perinatology -- Periodicals; Infants (Newborn) -- Diseases -- Periodicals; Neonatology -- Periodicals; 618.2
• DOI: 10.3109/14767058.2014.916678
• Languages: English
• ISSNs: 1476-7058

Abstract

<abstract> <title>Abstract</title> <p>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.</p> <p>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.</p> <p>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.</p> </abstract>