ABSTRACT
Pathologic findings showed that neutrophils played an important role in the pathogenesis of NMO. This study aims to investigate the CSF levels of neutrophil-related chemokines in NMO. CXCL1, CXCL5, and CXCL7 were measured in 95 patients with NMO, 15 patients with MS, 18 patients with GFAP astrocytopathy, and 16 controls. The CSF level of CXCL1, CXCL5, and CXCL7 was significantly elevated in the NMO group but not correlated with the patient clinical severity. Besides, the CSF CXCL1, CXCL5, and CXCL7 could act as biomarkers to distinguish NMO from MS with good reliability, especially the CXCL7.
Subject(s)
Chemokine CXCL1/cerebrospinal fluid , Chemokine CXCL5/cerebrospinal fluid , Neuromyelitis Optica/cerebrospinal fluid , Neutrophil Activation , beta-Thromboglobulin/cerebrospinal fluid , Adult , Female , Humans , Male , Middle Aged , Neuromyelitis Optica/physiopathology , Severity of Illness IndexABSTRACT
BACKGROUND: The gangliosidoses (Tay-Sachs disease, Sandhoff disease, and GM1-gangliosidosis) are progressive neurodegenerative diseases caused by lysosomal enzyme activity deficiencies and consequent accumulation of gangliosides in the central nervous system (CNS). The infantile forms are distinguished from the juvenile forms by age of onset, rate of disease progression, and age of death. There are no approved treatments for the gangliosidoses. In search of potential biomarkers of disease, we quantified 188 analytes in CSF and serum from living human patients with longitudinal (serial) measurements. Notably, several associated with inflammation were elevated in the CSF of infantile gangliosidosis patients, and less so in more slowly progressing forms of juvenile gangliosidosis, but not in MPS disease. Thirteen CSF and two serum biomarker candidates were identified. Five candidate biomarkers were distinguished by persistent elevation in the CSF of patients with the severe infantile phenotype: ENA-78, MCP-1, MIP-1α, MIP-1ß, and TNFR2. Correspondence of abnormal elevation with other variables of disease-i.e., severity of clinical phenotype, differentiation from changes in serum, and lack of abnormality in other neurodegenerative lysosomal diseases-identifies these analytes as biomarkers of neuropathology specific to the gangliosidosis diseases.
