The exercise test in Andersen syndrome
The exercise test in Andersen syndrome
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DOI：
10.1001/archneur.56.3.352
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发表时间：
1999-03-01
影响因子：
--
通讯作者：
Barohn, RJ
中科院分区：
文献类型：
--
作者：
Katz, JS;Wolfe, GI;Barohn, RJ
背景：Andersen综合征是一种罕见的周期性麻痹（PP），伴有畸形特征和潜在致命的心律失常。到目前为止，还没有任何超声诊断异常的报道可以用来确认这种情况下PP的存在。目的：探讨运动试验在Andersen综合征PP诊断中的应用价值。评价运动试验作为评估治疗期间神经肌肉状态的一种手段。方法：对2例安德森综合征患者进行运动试验。在1例患者中，我们使用了一种修改版本的最小值来记录对托卡因胺治疗的反应性。结果：两例患者的研究均显示，运动后复合肌肉动作电位幅度逐渐下降，这是其他形式PP的特征。1例患者发作间强度的改善和无力发作次数的减少与试验结果的改善相关。结论：我们的病例表明，运动试验可以确诊安德森综合征的PP。一种改良版的运动试验也可以被认为是记录PP治疗反应的客观方法。
Background: Andersen syndrome is a rare form of periodic paralysis (PP) associated with dysmorphic features and potentially fatal cardiac dysrhythmias. To date, no flcctrodiagnostic abnormalities have been reported that can be used to confirm the presence of PP in this condition.Objectives: To determine if the exercise test could be used to confirm the diagnosis of PP in Andersen syndrome. To evaluate the exercise test as a means to assess neuromuscular status during treatment.Methods: We performed the exercise test on 2 patients with Andersen syndrome. In 1 patient, we used a modified version of the lest to document responsiveness to treatment with tocainide.Results: Studies in both patients demonstrated a progressive decline in the compound muscle action potential amplitude after exercise that was characteristic of the phenomenon seen in other forms of PP. In 1 patient, im provement in interattack strength and a reduction in the number of attacks of weakness correlated with improvement ill the test results.Conclusions: Our cases demonstrate that the exercise test can confirm the diagnosis of PP in Andersen syndrome. A modified version of exercise testing may also be considered as an objective method for documenting treatment responses in PP.