Ramachandiran Nandhagopal
Department of Neurology, SVIMS, India.
South Med J. 2008 Sep;101(9):940-2. doi: 10.1097/SMJ.0b013e3181809186.
A 19-year-old woman presented with recurrent hypokalemic paralysis, followed by apparently persistent symptoms due to coexisting osteomalacia. Distal renal tubular acidosis type 1 (dRTA1) linked the metabolic abnormalities and occurred as an extraglandular feature of Sjögren syndrome (SS). This case highlights the fact that in the setting of recurrent hypokalemia, apparently progressive weakness should be distinguished from primary hypokalemic paralysis and evaluated for dRTA1, as the metabolic alterations are potentially treatable. Further dRTA1 may precede the occurrence of sicca syndrome in SS.
一名19岁女性出现反复低钾性麻痹,随后因并存骨软化症而出现明显持续症状。1型远端肾小管性酸中毒(dRTA1)将这些代谢异常联系起来,它作为干燥综合征(SS)的腺外表现出现。该病例凸显了这样一个事实,即在反复低钾血症的情况下,应将明显进行性肌无力与原发性低钾性麻痹相区分,并评估是否存在dRTA1,因为这些代谢改变可能是可治疗的。此外,dRTA1可能在SS的干燥综合征出现之前发生。