Pulmonary complications of chronic neuromuscular diseases and their management

Abstract

Chronic neuromuscular diseases may affect all major respiratory muscles groups including inspiratory, expiratory, and bulbar, and respiratory complications are the major cause of morbidity and mortality. Untreated, many of these diseases lead inexorably to hypercapnic respiratory failure, precipitated in some cases by chronic aspiration and secretion retention or pneumonia, related to impairment of cough and swallowing mechanisms. Many measures are helpful including inhibition of salivation, cough-assist techniques, devices to enhance communication, and physical therapy. In addition, ventilatory assistance is an important part of disease management for patients with advanced neuromuscular disease. Because of its comfort, convenience, and portability advantages, noninvasive positive pressure ventilation (NPPV) has become the modality of first choice for most patients. Patients to receive NPPV should be selected using consensus guidelines, and initiation should be gradual to maximize the chances for success. Attention should be paid to individual preferences for interfaces and early identification of cough impairment that necessitates the use of cough-assist devices. For patients considered unsuitable for noninvasive ventilation, invasive mechanical ventilation should be considered, but only after a frank but compassionate discussion between the patient, family, physician, and other caregivers.

Concurrence of facioscapulohumeral muscular dystrophy and myasthenia gravis

“…6 In our Case 1, the diagnosis of MG may have been delayed because at least part of the initial symptoms of fatigability and shortness of breath attributed to FSHD were probably due to untreated MG. Although rare (Ͻ1%), respiratory dysfunction alone may be the presenting symptom of MG. 4 Although atypical phenotypes, such as progressive external ophthalmoplegia, have been described in one family with FSHD, 3 the diagnosis of FSHD was questioned when ptosis and severe bulbar dysfunction developed.…”

“…I read with interest the review by Perrin et al 6 and found it comprehensive except for one important omission. The authors failed to note "air stacking" or the importance of maximal lung insufflations prior to attempts at manually assisted coughing.…”

Lung Volumes

“…Bilateral diyafragma paralizisinde yatar pozisyonda VC'deki azalma %50'den fazla olabilir. Kas güçsüzlüğü olan hastalarda TLC ve IC azalırken FRC değişkenlik gösterir, azalabilir veya artabilir (29,36).…”

Exercise Training 3—exercise Training in Pulmonary Rehabilitation

“…Diseases within NMD can be divided into diseases of the central nervous system (cerebral cortex, brainstem, basal ganglia and spinal cord) and in disease of the peripheral nervous system (motor nerves, neuromuscular junction and myopathies). 30,31 For patients with NMD, one of the main causes of morbidity and mortality is due to complications of respiratory muscle dysfunction. 32 .…”