Pulmonary Service, Corporació Parc Taulí, Hospital de Sabadell, Universitat Autònoma de Barcelona, Sabadell (Barcelona), Spain.
J Clin Sleep Med. 2011 Feb 15;7(1):89-91.
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.
我们报告了一例 48 岁女性患有致命性家族性失眠症（FFI）的病例，这是一种罕见的朊病毒病。在疾病早期，她出现了 Biot 呼吸和继发性呼吸衰竭。一旦发现高碳酸血症，就进行了夜间无创通气（NIV）试验，动脉血气（ABG）有了重要改善，睡眠质量也得到了主观改善。据我们所知，这是医学文献中首次报道使用 NIV 来治疗这种破坏性疾病。然而，它对这些患者预后和生存的影响仍有待阐明。
