O Anesthetic challenge of rapidly progressing dementia- a case of fatal familial insomnia
DOI:
https://doi.org/10.25751/rspa.24932Keywords:
Anesthesiology, Gastrostomy, Insomnia, Fatal Familial Perioperative PeriodAbstract
Fatal familial insomnia (FFI) is a fatal and rare neurodegenerative prion disease. Our aim is to describe a case of a patient with FFI in need of a percutaneous endoscopic gastrostomy (PEG). This 59-year-old woman’s background included FFI diagnosed 10 months ago, and rapidly progressing – total dependency for daily activities and inability to communicate. She underwent a PEG with monitored anaesthetic care. The procedure was uneventful, with sedation using midazolam, fentanyl, propofol, local infiltration of lidocaine and oxygen therapy. The patient ended up dying one week later, due to an infectious complication of the FFI. Achieving the correct management of FFI in the perioperative period requires a preoperative evaluation, and a high degree of empathy to this terminal illness. The anaesthesiologist plays a major role in preserving what is left of this patient’s functionality. This case shows that although rare, FFI is an importante concern in the perioperative setting.
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Copyright (c) 2022 Mariana Vaz Gomes, Eunice Mendes , Ana Luisa Vieira, Conceição Martins
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