Objective: Today the diagnosis of amyotrophic lateral sclerosis (ALS) is based on clinical assessment of upper and lower motor neuron signs in the limb and/or bulbar territories. Our aim was to establish the level of CSF chromogranin A (CgA) in patients with ALS to assess this protein as a candidate biomarker of this disease.

Background: A challenge for scientists is to search for biomarkers that would provide insight into pathogenesis of ALS and could also be clinically useful. Chromogranins may act as chaperone-like proteins to promote the secretion of mutant SOD1 into the extracellular milieu. Extracellular SOD1 protein may activate microglia, inducing motor neuron death in culture. Chromogranin – mediated secretion of mutant SOD1 may contribute to ALS pathogenesis.

Methods: We investigated 11 patients with ALS and 11 age matched controls. All patients fulfilled the El Escorial criteria for ALS. Clinical assessment, electromyography, brain and cervical spine MRI, blood test and lumbar puncture were performed in all patients.

Results: Levels of CgA were significantly increased in ALS CSF compared to controls. Plasma CSF levels could not have been statistically evaluated because the CgA level was below the lower limit of quantifiation in most controls.

Conclusions: Because CSF CgA level is elevated in patients with ALS, chromogranin A could become a candidate biomarker of ALS. Grant support: AZV (Czech Republic) No. 15-32715A.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/csf-level-of-chromogranin-a-in-amyotrophic-lateral-sclerosis/