Vodoff M V, Cremer R, Martinot A, Cuvellier J C, Fourier C, Hue V, Nuyts J P, Leclerc F
Service de réanimation pédiatrique, hôpital Jeanne-de-Flandre, Lille, France.
Arch Pediatr. 1997 Sep;4(9):845-8. doi: 10.1016/s0929-693x(97)88149-3.
Myasthenia gravis is usually revealed by a ptosis or a diplopia. A respiratory muscle weakness often occurs during the course but an acute respiratory failure as initial feature is unusual.
Three girls, aged 8, 10 and 14 years, were hospitalised in an intensive care unit, along a 15 year-period, for an acute respiratory distress. The first two children suffered from skeletal and bulbar muscle weakness. The third, admitted with the diagnosis of unexplained pneumonia, was complaining of skeletal and bulbar muscle weakness for the last 18 months. Myasthenia gravis was confirmed with electromyography, and detection of the acetylcholine-receptors antibodies in all three cases.
Any unexplained acute respiratory distress must lead to search for skeletal and bulbar muscle weakness, specially after muscular exercise or at the end of day, manifestations which characterize myasthenia gravis.
重症肌无力通常表现为上睑下垂或复视。病程中常出现呼吸肌无力，但以急性呼吸衰竭为首发特征者并不常见。
在15年期间，有3名年龄分别为8岁、10岁和14岁的女孩因急性呼吸窘迫入住重症监护病房。前两名儿童患有骨骼肌和延髓肌无力。第三名儿童入院时诊断为不明原因肺炎，在过去18个月里一直诉说有骨骼肌和延髓肌无力。通过肌电图检查及检测所有3例患者的乙酰胆碱受体抗体确诊为重症肌无力。
任何不明原因的急性呼吸窘迫都必须排查骨骼肌和延髓肌无力，尤其是在肌肉运动后或一天结束时出现的此类症状，这些表现是重症肌无力的特征。
