| Objectives: The first portion: To quantitate the concentrations of cystatin C (Cys-C)in the CSF employing an immuno-turbidimetric assay, and correlate the Cys-C levels toage of onset, gender, site of onset symptoms, rate of disease progression and revisedamyotrophic lateral sclerosis functional rating scale (ALSFRS-R). To investigate theeffect of body mass index (BMI) on Cys-C levels both in the CSF and serum, andanalyze the correlation between BMI and clinical indicators in sporadic amyotrophiclateral sclerosis (sALS) patients. The second portion: To examine the serum creatinine(SCr) levels of sALS patients and evaluate the relationship between SCr levels andclinical indicators.Methods: The first portion: The study was conducted on121definite and probablesALS patients which consecutively admitted to the Department of Neurology. We alsoanalyzed data from22multiple system atrophy (MSA) and18age-matched, unrelatedhealthy controls who were enrolled in this study. We evaluated Cys-C in CSF and thecorrelation between Cys-C levels and age of onset, site of onset symptoms, BMI,disease duration, ALSFRS-R, forced vital capacity (FVC) and rate of diseaseprogression were analysed. The second portion:80sALS,80MSA and80tension typeheadache (TTH) patients were recruited for the study, and the differences of SCr levelsbetween experimental and control groups were compared, the correlation between SCrlevels and ALSFRS-R, FVC, site of onset symptoms, disease duration and rate ofdisease progression in sALS patients were analysed.Results： The first portion:(1) The estimated means of CSF Cys-C were notsignificantly different from patients with sALS (4.17±1.32) mg/L, HC (4.58±1.97)mg/L and MSA (4.05±1.18) mg/L. No meaningful correlation was observed between CSF Cys-C and each indicator.(2) The serum Cys-C levels were not significantlydifferent from patients with sALS (1.03±0.18) mg/L, HC (1.10±0.54) mg/L and MSA(1.10±0.21) mg/L. We observed a negative correlation between serum Cys-C andALSFRS-R (r=-0.355, P=0.007) and a positive correlation between serum Cys-C andsite of onset symptoms (r=0.274, P=0.041).(3) We compared the mean Cys-C levels inCSF and serum of sALS patients and found significant differences between bothbiofluid (P<0.001), the correlation analysis indicated that there is no correlationbetween CSF and serum Cys-C levels (r=0.071, P=0.605).(4) We found that the BMIvalues correlated with FVC (Pearson r=0.321, P=0.003), neither measure of Cys-C inCSF nor serum differed significantly by BMI, as well as the other clinical indicators.The second portion:(1) SCr levels in sALS patients was significantly decreased thanthose of the controls [(60.86±16.80) μmol/l vs.(70.05±12.79) μmol/l vs.(66.97±14.14) μmol/l, P<0.01].(2) There was no statistical difference between bulbar onset andspinal onset sALS patients in SCr levels [（62.82±10.62）μmol/L vs.（60.64±13.26）μmol/L, P=0.544]；The levels of SCr were not significantly different from sALSpatients with short duration（62.38±13.57）μmol/L and long duration（58.24±11.09）μmol/L.(3) In sALS patients, the SCr levels was positively correlated with theALSFRS-R (r=0.315, P=0.005), but not with other indicators.Conclusions: The first portion: The data demonstrate that the levels of Cys-C in CSFshould not be considered as a biomarker of ALS. The interference factors in assay andwell-directed statistical method for data analysis that clearly deserve more attention infuture studies. The second portion: The SCr is an important biochemical index insALS patients and might play an important role in monitoring disease progression. |
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