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Biot's breathing in a woman with fatal familial insomnia: is there a role for noninvasive ventilation?

Abstract
We present the case of a 48-year-old woman suffering from fatal familial insomnia (FFI)--a rare prion disease--who developed Biot's breathing and secondary respiratory failure during the early stages of the illness. Once hypercapnia was detected a trial of nocturnal noninvasive ventilation (NIV) was offered with important improvement of arterial blood gases (ABG), and subjective good quality of sleep. To our knowledge, this is the first report in the medical literature of the use of NIV in the management approach of this devastating disease. Its impact on the prognosis and survival of these patients, however, is yet to be elucidated.
AuthorsLuis Fernando Casas-Méndez, Manel Lujan, Laura Vigil, Gemma Sansa
JournalJournal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine (J Clin Sleep Med) Vol. 7 Issue 1 Pg. 89-91 (Feb 15 2011) ISSN: 1550-9397 [Electronic] United States
PMID21344052 (Publication Type: Case Reports, Journal Article)
Topics
  • Autopsy
  • Disease Progression
  • Fatal Outcome
  • Female
  • Humans
  • Hypercapnia (diagnosis, etiology, therapy)
  • Insomnia, Fatal Familial (diagnosis, genetics, therapy)
  • Middle Aged
  • Polysomnography (methods)
  • Pulmonary Gas Exchange
  • Rare Diseases
  • Respiration, Artificial
  • Respiratory Function Tests
  • Respiratory Insufficiency (complications, diagnosis)
  • Severity of Illness Index

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