Epidemiology and diagnostic process of amyotrophic lateral sclerosis as distinct from myelopathy: 5-year cohort study of whole-population in South Korea.

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.