Bradley W G, Taylor R, Rice D R, Hausmanowa-Petruzewicz I, Adelman L S, Jenkison M, Jedrzejowska H, Drac H, Pendlebury W W
Department of Neurology, University of Vermont School of Medicine, Medical Center Hospital, Burlington 05405.
Arch Neurol. 1990 Sep;47(9):1013-7. doi: 10.1001/archneur.1990.00530090091018.
A progressive degenerative myopathy has been well described in hypokalemic periodic paralysis but is not as widely recognized in hyperkalemic periodic paralysis. We studied four families with the latter disease in which some members developed a progressive myopathy. Episodes of paralysis were prolonged, lasting for months in some cases, and in one case paralysis was sufficiently severe to require ventilatory support. The progressive myopathy tended to develop at a time when attacks of paralysis were decreasing in frequency. Muscle biopsy specimens showed variability in fiber size, internal nuclei, and fibers with vacuoles. Electron microscopy showed myofibrillary degeneration and tubular aggregates. An abnormal biopsy specimen was more common in older patients. Our experience suggests that a progressive myopathy is as common in hyperkalemic periodic paralysis as it is in the hypokalemic disorder.
进行性退行性肌病在低钾性周期性麻痹中已有详细描述，但在高钾性周期性麻痹中尚未得到广泛认识。我们研究了四个患有后一种疾病的家族，其中一些成员出现了进行性肌病。麻痹发作持续时间延长，在某些情况下持续数月，并且在一个病例中，麻痹严重到需要通气支持。进行性肌病往往在麻痹发作频率降低时出现。肌肉活检标本显示纤维大小、内部细胞核以及有空泡的纤维存在变异性。电子显微镜显示肌原纤维变性和管状聚集物。异常活检标本在老年患者中更常见。我们的经验表明，进行性肌病在高钾性周期性麻痹中与在低钾性疾病中一样常见。
