JNNP: The role of cerebrospinal fluid phosphate nerve wire heavy chains and shell triglycoside enzymes in primary lateral sclerosis.

Primary lateral sclerosis (PLS) is a rare degenerative disease of upper motor neurons (UMNs), clinically manifested as aggressive spasms.
disease progresses more slowly than amyotrophic lateral sclerosis (ALS).
PLS is complicated by a lack of specific neurochemical biomarkers.
Although the level of cerebrospinal fluid (CSF) nerve wire in ALS increased significantly, making it a recognized ALS biomarker that reflects axis mutations, the authors measured phosphatized neural strands (pNFH) and Chit1 in cerebrospinal fluid in patients with PLS, ALS and non-neurodegenerative neurological disorders.
each biomarker distinguishes between PLS and ALS.
: 10 patients (5 males, 5 females) with PLS had cerebrospinal fluid stored in a biolibrary.
patients meet the diagnostic criteria for PLS.
alS patients (n s 28; 16 men, 12 women) met the revised El Escorial diagnostic criteria and randomly selected ALS and NCs patients from the biolibrary in the study.
CSF samples at -80 degrees CC within 2 hours of waist-to-waist wear.
pNFH was detected in all patients; Chit1 was measured in 10 PLS patients, 20 out of 28 ALS patients, and 21 out of 30 NC patients.
use the ROC curve to evaluate the diagnostic performance of each individual biomarker and combined biomarker.
: The main findings of the study were that pNFH and Chit1 were different in all three diagnostic categories (PLS, ALC, NCs).
about the content of pNFH and Chit1, PLS showed moderate levels relative to ALS and NCs.
, this may reflect slower degeneration of the cortical spinal axons in PLS compared to ALS, and similarly, the decrease in Chit1 in PLS may reflect a small degree of small glial cell nerve inflammation, which may be associated with axon loss, which is reflected in the correlation between the two biomarkers.
From a clinical point of view, in addition to confirming the significant increase in cerebrospinal fluid levels of pNFH and Chit1 in known ALS and the prognossis value of pNFH in the disease, the authors' study also showed that both pNFH and Chit1 have potential usefulness in diagnosing PLS.
original link: Verde F, Zaina G, Bodio C, et al Cerebrospinal fluid phosphorylated neurofilament heavy chain and chitotriosidase in primary lateral sclerosis Journal of Neurology, Neurosurgery and Psimsy Published Online First: 27 August 2020. doi: 10.1136/jnnp-2020-324059 Source: MedSci Originals !-- End of Content Presentation -- !-- To Determine If Login Ends.