Objective:

Describe the clinical and sociodemographic characteristics of ALS cases in the unique multidisciplinary clinic of ALS in Puerto Rico (PR).

Background:

ALS is a neurodegenerative disease that affects the upper and lower motor neurons, causing progressive muscle weakness and ultimately leading to death within 2-5 years after symptoms onset with respiratory failure as the main cause. There have been several initiatives in the USA to study disease epidemiology but the literature about ALS in PR is limited. The mean age of diagnosis is 55 years, and the spinal onset is present in approximately more than 70% of cases. 

Design/Methods:

The “Cuestionario para Esclerosis Lateral Amiotrófica en Puerto Rico” (CELA-PR) was developed using REDCap to extract record information. With CELA-PR, we retrospectively reviewed all records from the multidisciplinary ALS clinic diagnosed exclusively by Dr. Brenda Deliz within the period of January 1, 2018, to December 31, 2022. Statistical analysis done using R V-4.2.1 and R studio V-2022.12.0.

Results:

A total of 76 cases were eligible for the study. The overall mean age (SD) of diagnosis is 59.57 (10.33) years; for bulbar onset is 63.42 (8.29) years, similar to the mean age of diagnosis for women 61.26 (10.31) years. Most of the cases, 71.23%, presented with spinal onset. On average there is a delay in diagnosis from symptom onset of 2.4 (2.55) years. 97.37% of cases were medically insured. Medications used for ALS were Riluzole (100%), Radicava (70.15%) and Relyvrio (11.76%).

Conclusions:

The CELA-PR allowed to develop the first database for ALS in Puerto Rico and get an overview of the descriptive epidemiology of ALS. Although some similarities with the reported data in USA were found this study provides a detailed description of the characteristics of our ALS population as well as valuable information of their pattern of care.