Midwestern University, Chicago College of Osteopathic Medicine, Downers Grove, IL, 60515, USA.
Midwestern University, MABS, Downers Grove, IL, USA.
J Med Case Rep. 2022 Feb 11;16(1):65. doi: 10.1186/s13256-022-03283-0.
Hypokalemic periodic paralysis is a chronic condition characterized by sporadic attacks of weakness associated with acute hypokalemia. Attacks are typically associated with specific triggers, such as prolonged rest following exercise or consumption of a high-carbohydrate meal. Most commonly, this condition is caused by an autosomal dominant calcium channel mutation, and patients typically have an established family medical history of hypokalemic periodic paralysis. Long-term complications include the development of progressive proximal myopathy. Oral potassium chloride may be considered for the treatment of an acute attack, with administration of acetazolamide or dichlorphenamide as long-term prophylaxis. Nephrologists can play an important role in the recognition and treatment of previously undiagnosed hypokalemic periodic paralysis.
We summarize the case of a 19-year-old white man who presented to the emergency department with undiagnosed attacks of hypokalemic periodic paralysis, and who reported, at follow-up, improvement in the severity and frequency of attacks with dichlorphenamide.
This case demonstrates the crucial role nephrologists can play, not only in the diagnosis of hypokalemic periodic paralysis, but also in the ongoing management of this condition. Patients should be advised to regularly follow up with their nephrology team for evaluation due to the risk of developing myopathy.
低钾周期性麻痹是一种慢性疾病,其特征为伴有急性低钾血症的间歇性无力发作。发作通常与特定的诱因有关,如运动后长时间休息或摄入高碳水化合物餐。大多数情况下,这种情况是由常染色体显性钙通道突变引起的,患者通常有明确的低钾周期性麻痹家族病史。长期并发症包括进行性近端肌病的发展。口服氯化钾可考虑用于急性发作的治疗,长期预防可使用乙酰唑胺或二氯苯氧胺。肾脏病医生在识别和治疗以前未诊断的低钾周期性麻痹方面可以发挥重要作用。
我们总结了一位 19 岁白人男性的病例,他因未确诊的低钾周期性麻痹发作而到急诊科就诊,并在随访中报告二氯苯氧胺可改善发作的严重程度和频率。
该病例表明肾脏病医生不仅可以在低钾周期性麻痹的诊断中发挥关键作用,而且在该疾病的持续管理中也可以发挥关键作用。由于发生肌病的风险,应建议患者定期到肾脏病团队进行评估。