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Apparently Persistent Weakness After Recurrent Hypokalemic Paralysis: A Tale of Two Disorders

Nandhagopal Ramachandiran, DM
Volume: 101 Issue: 9 September, 2008

Abstract:

Abstract: 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.


Key Points


* Evaluation of the underlying cause(s) of hypokalemic paralysis has therapeutic and prognostic implications.


* Progressive weakness and pain after recurrent hypokalemic paralysis may point towards coexisting osteomalacia complicating distal renal tubular acidosis type 1.


* Sjögren syndrome may rarely present with distal renal tubular acidosis type 1 preceding sicca syndrome.

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References:

1. Lin SH, Lin YF, Chen DT, et al. Laboratory tests to determine the cause of hypokalemia and paralysis. Arch Intern Med 2004;164:1561–1566.
 
2. Fulop M, Mackay M. Renal tubular acidosis, Sjögren’s syndrome, and bone disease. Arch Intern Med 2004;164:905–909.
 
3. Links TP, Zwarts MJ, Wilmink JT, et al. Permanent muscle weakness in familial hypokalaemic periodic paralysis. Clinical, radiological and pathological aspects. Brain 1990;113:1873–1889.
 
4. Eriksson P, Denneberg T, Eneström S, et al. Urolithiasis and distal renal tubular acidosis preceding primary Sjögren’s syndrome: a retrospective study 5–53 years after the presentation of urolithiasis. J Intern Med 1996;239:483–488.
 
5. Takemoto F, Hoshino J, Sawa N, et al. Autoantibodies against carbonic anhydrase II are increased in renal tubular acidosis associated with Sjögren syndrome. Am J Med 2005;118:181–184.
 
6. Takemoto F, Katori H, Sawa N, et al. Induction of anti-carbonic-anhydrase-II antibody causes renal tubular acidosis in a mouse model of Sjögren’s syndrome. Nephron Physiol 2007;106:63–68.

CME:

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