中国现代神经疾病杂志 ›› 2012, Vol. 12 ›› Issue (2): 171-174. doi: 10.3969/j.issn.1672-6731.2012.02.016

• 神经免疫学临床与基础研究 • 上一篇    下一篇

2 干燥综合征伴低钾性周期性麻痹:二例报告并文献复习

程晓娟,王枫,赵玉武   

  1. 200233 上海交通大学附属第六人民医院神经内科
  • 出版日期:2012-04-16 发布日期:2012-04-21
  • 通讯作者: 赵玉武(Email:zhaoyuwu2005@126.com)

Sjogren's syndrome combined with hypokalemic periodic paralysis (report of 2 cases with review of literature)

CHENG Xiao-juan, WANG Feng, ZHAO Yu-wu   

  1. Department of Neurology, the Sixth People's Hospital, Shanghai Jiaotong University, Shanghai 200233, China
  • Online:2012-04-16 Published:2012-04-21
  • Contact: ZHAO Yu-wu (Email: zhaoyuwu2005@126.com)

摘要: 目的 探讨干燥综合征伴低钾性周期性麻痹的早期诊断和治疗。方法 回顾分析2 例以低钾性周期性麻痹为首发症状的干燥综合征患者的诊断与治疗经过。结果 均以突发性或发作性四肢进行性无力发病,血清钾和氯离子水平显著降低并伴碱性尿;临床免疫学检测抗SS-A 抗体、抗SS-B 抗体阳性,同时可伴甲状腺功能亢进或减退(血清游离T3、T4 水平降低),或肾功能衰竭;唇腺活检提示小唾液腺组织多灶性淋巴细胞浸润或自然唾液流率测定阳性。经枸橼酸钾、氯化钾,以及碳酸氢钠和左甲状腺素(优甲乐)等药物治疗后症状改善。结论 对干燥综合征并发低钾血症患者的诊断,依赖于详细的病史询问、体格检查和实验室检查,早期确诊、及时治疗有利于预后。治疗原则以补钾、纠正酸中毒、改善甲状腺功能及对症营养支持治疗为主。

关键词: 干燥综合征, 低钾性周期性麻痹, 酸中毒, 肾小管性, 病例报告

Abstract: Objective To explore the early diagnosis and the therapy of Sjogren's syndrome combined with hypokalemic periodic paralysis. Methods Clinical data of 2 cases with Sjogren's syndrome and hypokalemic periodic paralysis were analyzed. Results The first symptom of both two cases was suddenly or paroxysmal progressive four limbs weakness. The levels of serum potassium and chloride ion were decreased significantly, combined with alkaline urine, anti SS-A (+), anti SS-B (+), and sometimes with hyperthyroidism or hypothyroidism (the level of serum FT3 and FT4 being lower), or renal failure. In pathological examination of labial gland, mulifocality lymphocytes were seen in glandulae saliviae minores tissue in lower lip, or nature saliva flow rate measurement positive. All the patients' symptom improved after they were given potassium citrate, potassium chloride, sodium bicarbonate and levothyroxine (euthyrox). Conclusion The diagnosis of Sjogren's syndrome and hypokalemic periodic paralysis depends on comprehensive analysis of patient history, physical and laboratory examination. Early diagnosis and treatment can improve the prognosis. The treatment principle includes potassium supplement, correction of acidosis, improvement of thyroid function, and expectant alimentary support.

Key words: Sjogren's syndrome, Hypokalemic periodic paralysis, Acidosis, renal tubular, Case reports