Corbett A, Kingston W, Griggs R C, Moxley R T
Arch Neurol. 1984 Jul;41(7):740-3. doi: 10.1001/archneur.1984.04050180062019.
Acetazolamide is effective treatment for myotonia in certain patients with myotonia congenita. Since potassium metabolism may be abnormal in myotonia congenita, we studied the effect of acetazolamide administration on potassium regulation and glucose disposal, using the euglycemic insulin clamp technique in patients with myotonic disorders and in normal subjects. Glucose disposal was normal in patients with myotonia congenita; administration of acetazolamide increased glucose disposal in normal subjects and in patients with myotonia congenita. By contrast, patients with myotonic dystrophy showed insulin resistance and decreased glucose disposal that was not improved by acetazolamide administration. Patients with myotonia congenita had elevated potassium levels in the basal state and a greater fall in potassium level during the insulin clamp procedure than controls. Patients with myotonic dystrophy had normal or low basal potassium levels and a subnormal decrease in potassium level during the insulin clamp procedure. Administration of acetazolamide did not alter these abnormalities in potassium metabolism in patients with either myotonia congenita or myotonic dystrophy.
乙酰唑胺对某些先天性肌强直患者的肌强直是有效的治疗方法。由于先天性肌强直患者的钾代谢可能异常，我们采用正常血糖胰岛素钳夹技术，研究了乙酰唑胺给药对肌强直疾病患者和正常受试者钾调节及葡萄糖处置的影响。先天性肌强直患者的葡萄糖处置正常；乙酰唑胺给药可增加正常受试者和先天性肌强直患者的葡萄糖处置。相比之下，强直性肌营养不良患者表现出胰岛素抵抗且葡萄糖处置减少，给予乙酰唑胺后无改善。先天性肌强直患者基础状态下钾水平升高，胰岛素钳夹过程中钾水平下降幅度大于对照组。强直性肌营养不良患者基础钾水平正常或偏低，胰岛素钳夹过程中钾水平下降低于正常。给予乙酰唑胺并未改变先天性肌强直或强直性肌营养不良患者钾代谢的这些异常情况。
