Department of Palliative Medicine, St Oswald’s Hospice, Newcastle-upon-Tyne, UK.
J Neurol Neurosurg Psychiatry. 2012 Apr;83(4):371-6. doi: 10.1136/jnnp-2011-300480. Epub 2011 Aug 17.
In motor neurone disease (MND), respiratory muscle weakness causes substantial morbidity, and death is usually due to respiratory failure. Non-invasive ventilation (NIV) improves symptoms, quality of life and survival, but previous surveys showed that few patients with MND received NIV.
A postal survey was conducted of the clinical application of NIV in MND among consultant neurologists in the UK. The results were compared with those of a similar survey done in 2000.
Over 12 months, 612 patients were referred for NIV of whom 444 were successfully established on treatment (72.5% success rate). 38% of responding neurologists assessed respiratory function at presentation and 20% routinely monitored respiratory function; 32% relied on symptoms as the only criterion for NIV referral and 43% used a combination of symptoms and physiological impairment. 75% of responding neurologists accessed specialist palliative care services for their patients towards the end of life and 69% at an earlier stage.
Compared with 2000, there has been a marked increase in the number of patients referred for, and currently using, NIV (2.6 and 3.4-fold, respectively). The proportion successfully established on NIV has also increased, suggesting more appropriate selection and/or improvement in the methods of using NIV in this challenging group of patients. However, monitoring of respiratory function is suboptimal and uncontrolled oxygen is sometimes used inappropriately before the terminal phase.
在运动神经元病（MND）中，呼吸肌无力会导致严重的发病率，死亡通常是由于呼吸衰竭。无创通气（NIV）可改善症状、生活质量和存活率，但先前的调查显示，很少有 MND 患者接受 NIV。
对英国顾问神经科医生在 MND 中应用 NIV 的情况进行了邮寄调查。结果与 2000 年进行的类似调查进行了比较。
在 12 个月内，有 612 名患者被转介接受 NIV，其中 444 名成功建立治疗（成功率为 72.5%）。38%的应答神经科医生在就诊时评估呼吸功能，20%常规监测呼吸功能；32%仅将症状作为 NIV 转介的唯一标准，43%则将症状和生理损害结合使用。75%的应答神经科医生在患者生命末期为其患者寻求专门的姑息治疗服务，69%则在更早阶段寻求该服务。
与 2000 年相比，转介和目前正在使用 NIV 的患者数量明显增加（分别增加了 2.6 倍和 3.4 倍）。成功建立 NIV 的比例也有所增加，这表明在这组具有挑战性的患者中，对 NIV 的选择和/或使用方法进行了更恰当的改进。然而，呼吸功能的监测仍不理想，在终末期之前，有时会不恰当地使用未经控制的氧气。
