Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study

Abstract

Objective

This article presents an updated analysis of the LIGALS register, a prospective study conducted over a ten-year period (2009-2018) in Liguria, Italy, aimed at evaluating the incidence, prevalence, clinical presentation, and management of amyotrophic lateral sclerosis (ALS).

Methods

We calculated the mean annual crude incidence rate of ALS, assessed the point prevalence of ALS on January 1, 2018, and analyzed demographic factors, clinical characteristics, and clinical management strategies. Data analysis included Cox regression analysis to identify predictors of survival.

Results

The mean annual crude incidence rate of ALS was 3.16/100,000 per year (CI 95%) while the point prevalence of ALS on January 1, 2018, was 9.31/100,000 population (CI 95%). Among the patients, 6.5% were familial ALS, while 93.5% were sporadic cases. Clinical management strategies, including percutaneous endoscopic gastrostomy (PEG) and noninvasive ventilation (NIV), were employed. The study observed a stable frequency of NIV initiation and PEG placement over time, with a growing trend toward earlier PEG positioning. The mean survival from symptom onset was 39 months, whereas from diagnosis, it was 26 months. Cox regression analysis identified several predictors of survival, including gender, age at onset and diagnosis, site of onset, diagnostic category, phenotype, and diagnostic delay.

Conclusions

This comprehensive analysis provides valuable insights into the long-term trends in ALS epidemiology and clinical management in Liguria, Italy. It underscores the importance of continued research efforts in understanding and addressing the challenges posed by ALS, particularly in terms of early diagnosis and optimizing clinical interventions to improve patient outcomes.

Keywords:

• Amyotrophic lateral sclerosis
• LIGALS
• epidemiology
• motor neuron disease
• survival

Acknowledgement

We would like to express our sincere gratitude to all our Ligurian neurologist colleagues who have referred patients to us, supported us in data collection, and provided invaluable patient assistance. We are also grateful for the collaboration of AISLA, the Association of Family Members and Patients with ALS. The data that support the findings of this study are available from the corresponding author, upon reasonable request.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.