Reliability and validity of the Chinese version of the Scale for Assessment and Rating of Ataxia

Background The Scale for the Assessment and Rating of Ataxia （SARA） was shown to be a reliable and valid measurement for patients with spinocerebellar ataxia （SCA）. The Brazilian version and the Japanese version of SARA were favorable for good reliability and validity. This study aimed to translate SARA into Chinese and test its reliability and validity in measurement of cerebellar ataxia. Methods SARA was translated into Chinese. A total 39 patients with degeneration cerebellar ataxia were evaluated independently by two neurologists with the Chinese version of SARA. Then the patients were evaluated by one of above neurologists with International Cooperative Ataxia Rating Scale （ICARS）. The statistical analyses were performed using SPSS 17.0 for Windows. Results The Cronbach＇s alpha coefficient of the Chinese version of SARA was 0.78, which represents a good internal consistence. The correlation coefficient of the Chinese version of SARA scores between the two evaluators was 0.86, illustrating that the inter-rater reliability of Chinese version of SARA was good. The correlation coefficient between the Chinese version of SARA and ICARS was 0.91, illustrating that the criterion validity of Chinese version of SARA was not bad. Conclusions The Chinese version of SARA is reliable and effective for the assessment of degeneration cerebellar ataxia. Compared with ICARS, the evaluation of Chinese version of SARA is more objective, the assessment time is shortened, and the maneuverability is better.

Nerve Growth Factor for the Treatment of Spinocerebellar Ataxia Type 3： An Open-label Study

Background： Spinocerebellar ataxia type 3 （SCA3） is the most common subtype of SCA worldwide, and runs a slowly progressive and unremitting disease course. There is currently no curable treatment available. Growing evidence has suggested that nerve growth factor （NGF） may have therapeutic effects in neurodegenerative diseases, and possibly also in SCA3. The objective of this study was to test the efficacy of NGF in SCA3 patients. Methods： We performed an open-label prospective study in genetically confirmed adult （〉18 years old） SCA3 patients. NGF was administered by intramuscular injection （18 μg once daily） fbr 28 days consecutively. All the patients were evaluated at baseline and 2 and 4 weeks after treatment using the Chinese version of the scale for assessment and rating of ataxia （SARA）. Results： Twenty-one SCA3 patients （ 10 men and 11 women, mean age 39.14 ± 7.81 years, mean disease duration 4.14 ± 1.90 years, mean CAG repeats number 77,57 ± 2.27） were enrolled. After 28 days of NGF treatment, the mean total SARA score decreased significantly from a baseline of 8.48± 2.40 to 6.30 ± 1.87 （P 〈 0.001 ）. Subsections SARA scores also showed significant improvements in stance （P = 0.003）, speech （P = 0.023）, finger chase （P = 0.015）, fast alternating hand movements （P = 0.009）, and heel-shin slide （P = 0.001）. Conclusions： Our preliminary data suggest that NGF may be effective in treating patients with SCA3.