El Otmani H, Moutaouakil F, Aghai R, Rafai M A, Bourezgui M, Benkirane A, Slassi I
Service de Neurologie et Explorations Fonctionnelles, CHU ibn Rochd, Casablanca, Maroc.
Rev Neurol (Paris). 2006 May;162(5):640-2. doi: 10.1016/s0035-3787(06)75059-7.
Hypokalaemic periodic paralysis can be primitive or secondary to potassium deficiency which can arise from several causes. Primary Sjogren's syndrome is a rare cause related to kidney involvement.
A 50-year-old woman has been admitted for hypotonic tetraparesis which had appeared a few days earlier. History taking revealed three previous similar episodes with a notion of oral and lacrimal dryness. Laboratory tests revealed severe hypokalaemia, hyperchloremia, alkaline urinary pH and a minima 24h proteinuria. Additional investigations led to the diagnosis of a primary Sjogren's syndrome defined on the basis of international criteria. Kidney biopsy revealed tubular-interstitial nephritis. Oral corticosteroïd therapy and potassium supplementation led to symptom improvement. A recurrent episode also responded to treatment. Additional urinary alkalinisation has prevented further relapse.
Primary Sjogren's syndrome is an exocrine disease causing systemic disorders. Tubular-interstitial nephropathy may occur in 25 percent of patients leading to distal tubular acidosis defined by the association of hypokalaemia, hyperchloremia and alkaline urinary pH. When hypokalaemia is severe, periodic paralysis may occur.
Primary Sjogren's syndrome can lead to nephropathy and subsequent hypokalaemic periodic paralysis. Urinary alkalinisation is essential to prevent this catastrophic presentation from recurring.
低钾性周期性麻痹可为原发性或继发于钾缺乏，钾缺乏可由多种原因引起。原发性干燥综合征是一种与肾脏受累相关的罕见病因。
一名50岁女性因数天前出现的低渗性四肢轻瘫入院。病史询问发现此前有三次类似发作，并伴有口眼干燥症状。实验室检查显示严重低钾血症、高氯血症、尿pH值呈碱性以及微量24小时蛋白尿。进一步检查依据国际标准诊断为原发性干燥综合征。肾脏活检显示肾小管间质性肾炎。口服皮质类固醇治疗及补钾使症状改善。一次复发发作也对治疗有反应。额外的尿液碱化预防了进一步复发。
原发性干燥综合征是一种导致全身紊乱的外分泌腺疾病。25%的患者可能发生肾小管间质性肾病，导致以低钾血症、高氯血症和尿pH值呈碱性为特征的远端肾小管酸中毒。当低钾血症严重时，可能发生周期性麻痹。
原发性干燥综合征可导致肾病及随后的低钾性周期性麻痹。尿液碱化对于预防这种灾难性表现的复发至关重要。
