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
- 1Neurology Department, Dr. Balmis General University Hospital, Alicante Institute for Health and Biomedical Research (ISABIAL), Alicante, Spain
Abstract
BackgroundTo 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.
MethodsCross-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.
ResultsA total of 374 confirmed MND cases were identified. The overall crude prevalence of MND was 8.43 per 100,000 (95% CI: 7.60-9.29) and the age-adjusted prevalence was 7.85 per 100,000 (95% CI: 7.05-8.65). By phenotype, ALS accounted for 325 cases (86.9%; crude prevalence 7.33 per 100,000), PLS for 26 (7.0%; 0.59 per 100,000) and PMA for 23 (6.1%; 0.52 per 100,000). Prevalence was consistent across provinces. Mean age was 65.9 years, the male-to-female ratio was 1.37:1, and 83.4% were receiving riluzole.
ConclusionsThis is the first comprehensive, multi-source population-based estimate of MND prevalence and its subtypes in the Valencian Community. The estimate is higher than the previous single-unit figure and consistent with recent European data, underscoring the value of multi-source ascertainment.
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BibTeX
@misc{rosarlanzon2026prevalence,
title = {{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}},
author = {P. Ros-Arlanzon and J. F. Vazquez-Costa and A. Merchan-Cabanillas and R. Vilar-Ventura and A. Pareja and L. Lorente-Gomez and E. Ronda-Perez and C. Diaz-Marin},
howpublished = {medRxiv},
year = {2026},
month = oct,
doi = {10.64898/2026.10.07.26364981},
url = {https://www.medrxiv.org/content/10.64898/2026.10.07.26364981v1},
note = {CC BY-NC-ND},
}
RIS
TY - UNPB
TI - 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
AU - P. Ros-Arlanzon
AU - J. F. Vazquez-Costa
AU - A. Merchan-Cabanillas
AU - R. Vilar-Ventura
AU - A. Pareja
AU - L. Lorente-Gomez
AU - E. Ronda-Perez
AU - C. Diaz-Marin
PY - 2026
DA - 2026/10/07
PB - medRxiv
DO - 10.64898/2026.10.07.26364981
UR - https://www.medrxiv.org/content/10.64898/2026.10.07.26364981v1
AB - BackgroundTo 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. MethodsCross-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. ResultsA total of 374 confirmed MND cases were identified. The overall crude prevalence of MND was 8.43 per 100,000 (95% CI: 7.60-9.29) and the age-adjusted prevalence was 7.85 per 100,000 (95% CI: 7.05-8.65). By phenotype, ALS accounted for 325 cases (86.9%; crude prevalence 7.33 per 100,000), PLS for 26 (7.0%; 0.59 per 100,000) and PMA for 23 (6.1%; 0.52 per 100,000). Prevalence was consistent across provinces. Mean age was 65.9 years, the male-to-female ratio was 1.37:1, and 83.4% were receiving riluzole. ConclusionsThis is the first comprehensive, multi-source population-based estimate of MND prevalence and its subtypes in the Valencian Community. The estimate is higher than the previous single-unit figure and consistent with recent European data, underscoring the value of multi-source ascertainment.
ER -