Kim EY, et al. Ann Rehabil Med. 2017 Apr;41(2):306-312. https://doi.org/10.5535/arm.2017.41.2.306

Maximum PtcCO2 was significantly greater than maximum PetCO2 for both groups and, therefore, tends to be higher than PetCO2 in this population. This should be taken into consideration when assessing patients for sleep hypoventilation.

Background: Duchenne muscular dystrophy (DMD) patients can have various issues that affect their quality of life, including eating and digestive conditions. Objective: We sought to identify the relationship between respiratory function and various eating and digestion related symptoms in patients with advanced Duchenne muscular dystrophy (DMD). Methods: Eating and digestive symptoms, including loss of appetite, nausea, vomiting, diarrhea, constipation, swallowing difficulty, mastication difficulty, early satiety, and aspiration, were evaluated among patients with advanced DMD who were nonambulatory and required noninvasive mechanical ventilatory support. In addition, various respiratory function parameters were measured, including forced vital capacity (FVC), maximal insufflation capacity (MIC), peak cough flow (PCF), assisted PCF (APCF), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). We then analyzed the relationship between gastrointestinal symptoms and respiratory function parameters. Results: A total of 180 patients (age, 22.3±5.0 years) were included in the analysis. Loss of appetite and early satiety showed no correlation with any of the respiratory function parameters. Constipation was correlated with MEP; swallowing difficulty was correlated with MIC, APCF, MIP and MEP; and mastication difficulty was correlated with FVC, PCF, APCF, MIP, and MEP. Notably, age did not correlate with any gastrointestinal symptoms. Conclusions: Eating and digestive symptoms are more closely correlated with respiratory function than with age in patients with DMD. We think this correlation is mainly caused by the skeletal muscle strength, which is major determinant of both digestive and respiratory function.

Lee JW, et al. Yonsei Med J. 2021 Apr;62(4):338-343. https://doi.org/10.3349/ymj.2021.62.4.338

Objective: To investigate the incidence, prevalence, and demographic factors of all amyotrophic lateral sclerosis (ALS) patients diagnosed in South Korea from 2011 to 2015, and to analyze cases misdiagnosed as myelopathy. Methods: The whole population registered under the Korean National Health Insurance Service (KNHIS) was applied. All 4551 patients who were registered as having ALS code from 2011 to 2015 were included. For all ALS patients, the incidence, prevalence, and demographic factors were assessed. Trends of diagnosis for myelopathy, and surgery prior to confirmation of ALS diagnosis were identified. Results: When the whole 48,135,715 KNHIS population enrolled in 2015, the incidence of ALS in 2015 was estimated to be 1.68 per 100,000 person-years, and the prevalence was 6.49 per 100,000 persons. Life expectancy of ALS can be calculated as 3.9 years after the diagnosis, and the mean age of diagnosis was 59.5 ± 13.1. A total of 1902 patients diagnosed with myelopathy before a diagnosis of ALS accounted for 0.13% of all myelopathy patients, and 41.8% of all ALS patients. It took an average of 471.7 d to confirm a diagnosis of ALS after the myelopathy diagnosis. Among the patients finally diagnosed with ALS, more patients underwent surgery for myelopathy (n = 263, 13.8%) than among patients who were diagnosed with myelopathy alone, and underwent surgery (n = 141,148, 9.8%). Conclusions: This whole-population nationwide demographic study confirmed the data from previous studies. Clinicians should consider the possibility of ALS when making a myelopathy diagnosis, especially if the symptoms are sufficiently severe to require surgery.