medRxiv
Prevalence of motor neuron disease and its clinical subtypes in the Valencian Community, Spain: a population-based cross-sectional study using linked electronic health records
By Ros-Arlanzon, P., Vazquez-Costa, J. F., Merchan-Cabanillas, A. and 5 others.
Abstract
Background
To determine the crude and age-adjusted point prevalence of motor neuron disease (MND) and its phenotypes (classical amyotrophic lateral sclerosis [ALS], primary lateral sclerosis [PLS] and progressive muscular atrophy [PMA]) using a comprehensive multi-source case ascertainment strategy.
Methods
Cross-sectional population-based study with a prevalence date of 1 January 2025. Cases were identified through five complementary regional data sources and confirmed by clinical review against the revised El Escorial and Gold Coast criteria. Cases were classified by phenotype as ALS, PLS or PMA. The reference population was the population aged 15 years or older in Valencian Community (n=4,433,999). Crude prevalence was calculated per 100,000 with 95% Poisson confidence intervals; age-adjusted rates were obtained by direct standardisation to the 2013 European Standard Population.