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The Association of Methylprednisolone Dosing to Cessation of Myotonia in a Patient with Myotonic Dystrophy Type 1
Neuromuscular Disorders ( IF 2.7 ) Pub Date : 2020-03-01 , DOI: 10.1016/j.nmd.2020.03.004
Magda Horáková 1 , Tomáš Horák 1 , Josef Bednařík 1 , Stanislav Voháňka 1
Affiliation  

We report the case of a patient suffering from duplicity of myotonic dystrophy type 1 and ulcerative colitis whose treatment for ulcerative colitis included repeated administrations of descending doses of methylprednisolone and in whom we found an association between methylprednisolone dosing and cessation of myotonia. Myotonia severity was expressed as relaxation time after voluntary contraction and as a patient-reported outcome using the Czech version of the Myotonia Behavior Scale. The patient was being treated for a flare of ulcerative colitis, starting with 32 mg of methylprednisolone and reducing the dose by 4 mg a week. The symptoms of myotonia began to wear off three weeks after starting methylprednisolone and had totally disappeared by four weeks after starting methylprednisolone. The first symptoms of myotonia returned about a month after the last dose of methylprednisolone and reached a peak of severity more than two months after the final dose.

中文翻译:

甲泼尼龙剂量与 1 型肌强直性营养不良患者肌强直停止的关联

我们报告了一例患有双重性肌强直性营养不良 1 型和溃疡性结肠炎的患者,其溃疡性结肠炎的治疗包括重复给药递减剂量的甲基强的松龙,我们发现甲基强的松龙剂量与肌强直停止之间存在关联。肌强直严重程度表示为自主收缩后的松弛时间,并表示为使用捷克版肌强直行为量表的患者报告结果。该患者正在接受溃疡性结肠炎发作的治疗,从 32 毫克甲基强的松龙开始,每周减少 4 毫克剂量。开始服用甲泼尼龙三周后,肌强直症状开始消失,并在开始服用甲泼尼龙 4 周后完全消失。
更新日期:2020-03-01
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