Da Yuwei, Lei Lin, Jurkat-Rott Karin, Lehmann-Horn Frank
Department of Neurology, Xuan Wu Hospital, Capital Medical University, Beijing, China.
Department of Neurosurgery, Ulm University, Germany.
Acta Myol. 2016 Oct;35(2):107-108.
Primary periodic paralyses (PPs) are autosomal dominant ion channel disorders characterized by episodic flaccid weakness associated with variations in serum potassium level. The main prophylactic therapy of choice for PPsis carbonic anhydrase inhibitors that are not always effective. In this report, we described two PP patients who were successfully treated with coenzyme Q10. They remained asymptomatic since initiation of treatment, which may be associated with promotion of energy synthesis, anti-oxidant activity, influence of the fiber type composition and regulation of the expression of gene. To our knowledge, this is the first report of primary periodic paralyses which have been successfully treated with CoQ10. More observations need to substantiate this clinical finding in PPs.
原发性周期性麻痹（PPs）是常染色体显性离子通道疾病，其特征为发作性弛缓性肌无力，并伴有血清钾水平的变化。PPs的主要预防性治疗药物是碳酸酐酶抑制剂，但并非总是有效。在本报告中，我们描述了两名成功接受辅酶Q10治疗的PP患者。自开始治疗以来，他们一直无症状，这可能与促进能量合成、抗氧化活性、纤维类型组成的影响以及基因表达的调节有关。据我们所知，这是首例成功使用辅酶Q10治疗原发性周期性麻痹的报告。需要更多观察来证实PPs中的这一临床发现。
