Abstract: Background: In the last years different studies have reported
an increase of amyotrophic lateral sclerosis (ALS) incidence,
highlighting the role of the environment in this disease. This
prompted us to review ALS cases diagnosed at our hospital
in the last decade and to compare them with a previous ALS
series reported in our region 30 years ago. Methods: We reviewed
those ALS cases diagnosed at our centre between
2004 and 2013. Subsequently, we compared them with the
previous series regarding clinical and epidemiological features.
Results: A total of 53 patients (30 males, 23 females)
were included. The annual incidence was 1.7 cases per
100,000 inhabitants (2.2 and 1.2 per 100,000 in males and
females, respectively), which was significantly higher than in
the previous series (1 case per 100,000 inhabitants). Otherwise,
the clinical and epidemiological features were similar
in both series. The median age at symptom onset was 67
years, with a median diagnosis delay of 6 months. About two
thirds of the patients presented with systemic ALS, whereas the remaining had a bulbar onset. Weakness, dysphagia, and
dysarthria were the most common clinical symptoms at diagnosis.
The median survival from symptom onset was 22
months. Conclusion: After 3 decades, the annual incidence
of ALS has almost doubled in our region. We did not find significant
differences regarding other clinical or epidemiological
features.