Similowski T, Attali V, Bensimon G, Salachas F, Mehiri S, Arnulf I, Lacomblez L, Zelter M, Meininger V, Derenne J P
Laboratoire de Physiopathologie Respiratoire, Groupe Hospitalier Pitié-Salpetrière, Paris, France.
Eur Respir J. 2000 Feb;15(2):332-7. doi: 10.1034/j.1399-3003.2000.15b19.x.
Amyotrophic lateral sclerosis (ALS) is a progressive disorder of unknown origin. Respiratory involvement is the principal cause of death, and dyspnoea is a major source of discomfort. In this study, diaphragm function is described and its relationship with dyspnoea examined in 48 ALS patients (32 male, age 26-80 yrs). The detailed neurological and respiratory evaluation (clinical examination, pulmonary function tests, static pressures, mouth twitch pressures (Pm,t), electromyographic responses to phrenic nerve stimulation and cortical magnetic stimulation were analysed after stratification according to dyspnoea. Dyspnoeic (group I) and nondyspnoeic (group II) patients were similar, bulbar signs being more frequent in group I. Vital capacity was lower in group I (mean+/-SD 67.9+/-22.7 versus 87.9+/-15.6% of the predicted value, p=0.0028), as were maximal static inspiratory pressure (41+/-24 versus 60+/-27% pred, p=0.0242) maximal static inspiratory pressure (18+/-11 versus 32+/-14% pred, p=0.0042), and Pm,t (3.71+/-2.5 versus 7.26+/-3.45 cmH2O, p=0.0011). Abdominal (Abd) paradox and respiratory pulse were frequent in group I (15 of 25 and 14 of 25) but absent or rare in group II (0 of 23 and four of 23) (p<0.05). The electromyographic responses to phrenic and cortical stimulation were generally abnormal in group I but subnormal in group II. Multivariate analysis selected only signs of diaphragm dysfunction (namely, Abd paradox and abnormal electromyographic responses) as significant predictors of dyspnoea. It is concluded that dyspnoea in amyotrophic lateral sclerosis patients should prompt diaphragm function tests.
肌萎缩侧索硬化症（ALS）是一种病因不明的进行性疾病。呼吸功能受累是主要死因，呼吸困难是不适的主要来源。在本研究中，描述了48例ALS患者（32例男性，年龄26 - 80岁）的膈肌功能，并研究了其与呼吸困难的关系。根据呼吸困难情况进行分层后，分析了详细的神经学和呼吸评估（临床检查、肺功能测试、静态压力、口部抽搐压力（Pm,t）、膈神经刺激和皮质磁刺激的肌电图反应）。呼吸困难组（I组）和无呼吸困难组（II组）患者相似，I组球部体征更常见。I组肺活量较低（均值±标准差为预测值的67.9±22.7%，而II组为87.9±15.6%，p = 0.0028），最大静态吸气压力（41±24%与60±27%预测值，p = 0.0242）、最大静态呼气压力（18±11%与32±14%预测值，p = 0.0042）以及Pm,t（3.71±2.5与7.26±3.45 cmH2O，p = 0.0011）也较低。I组腹部矛盾运动和呼吸脉搏常见（25例中有15例和14例），而II组不存在或罕见（23例中有0例和4例）（p<0.05）。I组对膈神经和皮质刺激的肌电图反应通常异常，而II组为轻度异常。多因素分析仅选择膈肌功能障碍体征（即腹部矛盾运动和异常肌电图反应）作为呼吸困难的重要预测指标。结论是，肌萎缩侧索硬化症患者出现呼吸困难时应进行膈肌功能测试。
