Acetyl-DL-leucine improves gait variability in patients with cerebellar ataxia-a case series.

Q3 Medicine Cerebellum and Ataxias Pub Date : 2016-04-12 eCollection Date: 2016-01-01 DOI:10.1186/s40673-016-0046-2
Roman Schniepp, Michael Strupp, Max Wuehr, Klaus Jahn, Marianne Dieterich, Thomas Brandt, Katharina Feil
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引用次数: 34

Abstract

Acetyl-DL-leucine is a modified amino acid that was observed to improve ataxic symptoms in patients with sporadic and hereditary forms of ataxia. Here, we investigated the effect of the treatment with Acetyl-DL-leucine on the walking stability of patients with cerebellar ataxia (10x SAOA, 2x MSA-C, 2x ADA, 1x CACNA-1A mutation, 2x SCA 2, 1x SCA 1). Treatment with Acetyl-DL-leucine (500 mg; 3-3-4) significantly improved the coefficient of variation of stride time in 14 out of 18 patients. Moreover, subjective ambulatory scores (FES-I and ABC) and the SARA scores were also improved under treatment. Further prospective studies are necessary to support these class III observational findings.

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乙酰- dl -亮氨酸改善小脑性共济失调患者的步态变异性-一个病例系列。
乙酰- dl -亮氨酸是一种被观察到改善散发性和遗传性共济失调患者的共济失调症状的修饰氨基酸。在这里,我们研究了乙酰- dl -亮氨酸治疗对小脑性共济失调患者行走稳定性的影响(10倍SAOA, 2倍MSA-C, 2倍ADA, 1倍CACNA-1A突变,2倍SCA 2, 1倍SCA 1)。3-3-4)显著改善了18例患者中14例的步幅时间变异系数。此外,主观动态评分(FES-I和ABC)和SARA评分也在治疗后得到改善。需要进一步的前瞻性研究来支持这些III级观察性发现。
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Cerebellum and Ataxias
Cerebellum and Ataxias Medicine-Neurology (clinical)
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