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Open AccessArticle
Creatine Kinase and Respiratory Decline in Amyotrophic Lateral Sclerosis
by
João Pedro Correia
João Pedro Correia 1,†,
Marta Gromicho
Marta Gromicho 1,†
,
Ana Catarina Pronto-Laborinho
Ana Catarina Pronto-Laborinho 1
,
Miguel Oliveira Santos
Miguel Oliveira Santos 1,2,3
and
Mamede de Carvalho
Mamede de Carvalho 1,2,3,*
1
Faculdade de Medicina, Instituto de Medicina Molecular, Universidade de Lisboa, 1649-004 Lisboa, Portugal
2
Centro de Estudos Egas Moniz, Faculdade de Medicina, Universidade de Lisboa, 1649-004 Lisboa, Portugal
3
Departamento de Neurociências e Saúde Mental, Hospital (ULS) de Santa Maria, 1649-028 Lisboa, Portugal
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Brain Sci. 2024, 14(7), 661; https://doi.org/10.3390/brainsci14070661 (registering DOI)
Submission received: 25 May 2024
/
Revised: 24 June 2024
/
Accepted: 25 June 2024
/
Published: 28 June 2024
Abstract
Respiratory dysfunction is an important hallmark of amyotrophic lateral sclerosis (ALS). Elevation of creatine kinase (CK) has been reported in 23–75% of ALS patients, but the underlying mechanisms remain unknown. This work aims to enlighten the role of CK as a prognostic factor of respiratory dysfunction in ALS. A retrospective analysis of demographic and clinical variables, CK, functional decline per month (ΔFS), forced vital capacity (%FVC), and mean amplitude of the phrenic nerve compound motor action potential (pCMAP) in 319 ALS patients was conducted. These measurements were evaluated at study entry, and patients were followed from the moment of first observation until death or last follow-up visit. High CK values were defined as above the 90th percentile (CK ≥ P90) adjusted to sex. We analyzed survival and time to non-invasive ventilation (NIV) as proxies for respiratory impairment. Linear regression analysis revealed that high CK was associated with male sex (p < 0.001), spinal onset (p = 0.018), and FVC ≥ 80% (p = 0.038). CK was 23.4% higher in spinal-onset ALS patients (p < 0.001). High CK levels were not linked with an increased risk of death (p = 0.334) in Cox multivariate regression analysis. CK ≥ P90 (HR = 1.001, p = 0.038), shorter disease duration (HR = 0.937, p < 0.001), lower pCMAP (HR = 0.082, p < 0.001), and higher ΔFS (HR = 1.968, p < 0.001) were risk factors for respiratory failure. The association between high CK levels and poorer respiratory outcomes could derive from cellular metabolic stress or a specific phenotype associated with faster respiratory decline. Our study suggests that CK measurement at diagnosis should be more extensively investigated as a possible marker of poor respiratory outcome in future studies, including a larger population of patients.
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MDPI and ACS Style
Correia, J.P.; Gromicho, M.; Pronto-Laborinho, A.C.; Santos, M.O.; de Carvalho, M.
Creatine Kinase and Respiratory Decline in Amyotrophic Lateral Sclerosis. Brain Sci. 2024, 14, 661.
https://doi.org/10.3390/brainsci14070661
AMA Style
Correia JP, Gromicho M, Pronto-Laborinho AC, Santos MO, de Carvalho M.
Creatine Kinase and Respiratory Decline in Amyotrophic Lateral Sclerosis. Brain Sciences. 2024; 14(7):661.
https://doi.org/10.3390/brainsci14070661
Chicago/Turabian Style
Correia, João Pedro, Marta Gromicho, Ana Catarina Pronto-Laborinho, Miguel Oliveira Santos, and Mamede de Carvalho.
2024. "Creatine Kinase and Respiratory Decline in Amyotrophic Lateral Sclerosis" Brain Sciences 14, no. 7: 661.
https://doi.org/10.3390/brainsci14070661
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