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Acta Neurol Scand. 2013 Dec;128(6):386-90. doi: 10.1111/ane.12134. Epub 2013 Jun 27.

Utility of cystatin C for renal function in amyotrophic lateral sclerosis.

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  • 1Division of Neurology, Department of Internal Medicine, Jichi Medical University, Shimotsuke, Japan.



Creatinine (Cr) as a marker of renal function has limited value in amyotrophic lateral sclerosis (ALS) because patients with ALS have reduced muscle mass. Thus, there is a need for alternative methods of assessing renal function. Cystatin C (CysC), which is unaffected by muscle mass, is potentially an ideal biomarker of nephrotoxicity in ALS; however, its utility requires validation.


One hundred and six subjects were recruited for the study: 76 ALS patients and 30 healthy controls. We compared the Cr-based estimated glomerular filtration rate (eGFR) with the CysC-based eGFR in the ALS patients and healthy controls. The results were further analysed according to the severity of ALS in the patients.


The mean Cr-based eGFRs were 257.2 ± 383.1 ml/min/1.73 m(2) in the ALS group and 98.1 ± 34.9 in the control group; however, the mean CysC-based eGFRs were not significantly different between both groups. Thus, the Cr-based eGFR in the ALS group was markedly higher than any of the other values. Although serum CysC levels did not correlate with the severity of ALS according to the ALS Functional Rating Scale-Revised, strong simple correlations were observed between serum Cr levels and the severity of ALS (correlation coefficient = 0.734, P < 0.001).


This study demonstrates the potential usefulness of CysC as a biomarker of renal function in ALS patients. Furthermore, its applicability could be extended to other neuromuscular diseases.

© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.


amyotrophic lateral sclerosis; creatinine; cystatin C; estimated glomerular filtration rates; renal function

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