LoMauro Antonella, Romei Marianna, Priori Rita, Laviola Marianna, D'Angelo Maria Grazia, Aliverti Andrea
TBM Lab, Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, Milano, Italy.
IRCCS E. Medea, Bosisio Parini, Lc, Italy.
Respir Physiol Neurobiol. 2014 Jun 15;197:1-8. doi: 10.1016/j.resp.2014.03.001. Epub 2014 Mar 12.
Spinal muscular atrophy (SMA) is characterized by degeneration of motor neurons resulting in muscle weakness. For the mild type III form, a sub-classification into type IIIA and IIIB, based on age of motor impairment, was recently proposed. To investigate if SMA IIIA (more severe) and IIIB differ also in terms of respiratory function, thoracoabdominal kinematics was measured during quiet breathing, inspiration preceding cough and inspiratory capacity on 5 type IIIA and 9 type IIIB patients. Four patients with SMA II (more severe than types III) and 19 healthy controls were also studied. Rib cage motion was similar in SMA IIIB and controls. Conversely, in SMA IIIA and SMA II it was significantly reduced and sometime paradoxical during quiet breathing in supine position. Our results suggest that in SMA IIIA intercostal muscles are weakened and the diaphragm is preserved similarly to SMA II, while in SMA IIIB the action of all inspiratory muscles is maintained. Sub-classification of type III seems feasible also for respiratory function.
脊髓性肌萎缩症（SMA）的特征是运动神经元退化导致肌肉无力。对于轻度III型，最近有人基于运动功能受损的年龄将其细分为IIIA型和IIIB型。为了研究IIIA型（更严重）和IIIB型在呼吸功能方面是否也存在差异，我们对5例IIIA型和9例IIIB型患者在安静呼吸、咳嗽前吸气以及吸气容量时的胸腹部运动学进行了测量。还研究了4例II型SMA患者（比III型更严重）和19名健康对照者。IIIB型SMA患者的胸廓运动与对照组相似。相反，在IIIA型SMA和II型SMA患者中，仰卧位安静呼吸时胸廓运动明显减少，有时甚至出现反常运动。我们的结果表明，在IIIA型SMA中，肋间肌与II型SMA一样被削弱而膈肌得以保留，而在IIIB型SMA中，所有吸气肌的功能得以维持。III型的细分在呼吸功能方面似乎也是可行的。
