• Jie Zhou, Lifang Lei, Yuting Shi, Junling Wang, Hong Jiang, Lu Shen, and Beisha Tang.
• Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, China.
• Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2011 Jun 1;36(6):504-10.

ObjectiveTo determine the neuronal damage or loss and gliosis at the cellular level in spinocerebellar ataxia type 3/Machado-Joseph disease(SCA3/MJD), and evaluate the potential use of neuron-specific enolase (NSE) and protein S 100 B(S100B) serum concentrations as biochemical markers.MethodsSerum concentrations of NSE and S100B were measured in 102 SCA3/MJD patients and 100 healthy subjects matched by sex and age. The correlations between both markers and age, age of onset, disease duration, CAG repeat size, scores of international cooperative ataxia rating scale(ICARS), and scale for the assessment and rating of ataxia(SARA) were analyzed.ResultsCompared with the healthy controls, patients with SCA3/MJD had higher NSE serum concentrations [(6.95 ± 2.83)ng/mL vs (4.83 ± 1.70) ng/mL, P<0.05] and higher S100B serum concentrations [(0.07 ± 0.06) ng/mL vs (0.05 ± 0.02) ng/mL, P<0.05]. In the SCA3/MJD patients group, NSE levels presented a positive correlation with age, disease duration, ICARS scores and SARA scores, whereas S100B levels did not correlate with age, age of onset, disease duration, ICARS scores and SARA scores. CAG repeat size did not correlate with the NSE levels and S100B levels in different age groups of SCA3/MJD patients.ConclusionSerum NSE might be a useful marker to monitor disease progression and represent the degree of severity of a certain disease. Elevated S100B serum concentrations in patients compared to healthy controls may suggest an application of this protein as a peripheral marker of brain impairment in SCA3/MJD.

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