Cruz-Martínez A, Arpa J
Hospital La Luz, Madrid, Spain.
Acta Neurol Scand. 1997 Oct;96(4):229-35. doi: 10.1111/j.1600-0404.1997.tb00274.x.
To clarify the diagnostic yield of conduction velocity along muscle fibers in situ (MFCV) in hypokalemic periodic paralyses (HOPPs).
MFCV of the short head of biceps brachii was performed in 3 subjects with primary HOPP and in another 3 with secondary HOPP.
MFCV was reduced during the recovery period in 2 of the patients with hereditary HOPP and in 3 with secondary hypokalemia due to hyperaldosteronism, diuretics, and renal tubular acidosis. One case with familiar HOPP showed fluctuating muscle weakness, but never had paralytic attacks. MFCV slowing was similar in both hereditary and secondary HOPP. Direct stimulation during a major attack with quadriplegia showed inexcitability of most muscle fibers.
Inexcitability during major attacks and MFCV slowing in interictal or recovery periods evidenced sarcolemmal altered function. MFCV is a sensitive method for the detection of membrane muscle fiber defects in HOPPs and is also an additional diagnostic criterion of these diseases.
明确原位肌纤维传导速度（MFCV）在低钾性周期性麻痹（HOPPs）中的诊断价值。
对3例原发性HOPP患者和另外3例继发性HOPP患者的肱二头肌短头进行MFCV检测。
2例遗传性HOPP患者和3例因醛固酮增多症、利尿剂及肾小管酸中毒导致继发性低钾血症的患者在恢复期MFCV降低。1例家族性HOPP患者表现为波动性肌无力，但从未发生麻痹发作。遗传性和继发性HOPP中MFCV减慢情况相似。四肢瘫大发作期间直接刺激显示大多数肌纤维无兴奋性。
大发作期间的无兴奋性以及发作间期或恢复期的MFCV减慢证明肌膜功能改变。MFCV是检测HOPPs中肌膜肌纤维缺陷的敏感方法，也是这些疾病的一项额外诊断标准。
