ESPN 51th Annual Meeting

ESPN 2018


 
CENTRAL PONTINE AND EXTRAPONTINE MYELINOLYSIS IN GIRL WITH SJÖGREN’S SYNDROME
MARINA AKSENOVA TATJANA LEPAEVA 1 VLADIMIR DLIN 1

1- Y.VELTISCHEV RESEARCH AND CLINICAL INSTITUTE FOR PEDIATRICS AT N.PIROGOV RUSSIAN NATIONAL RESEARCH MEDICAL UNIVERSITY
 
Introduction:

Central pontine and extrapontine myelinolysis (CPEPM) usually develops due to acute hyponatremia. 

Material and methods:

We describe the case of CPEPM in 12-year-old girl with Sjögren’s syndrome(SS).

Results:

A girl was admitted in the regional hospital with a history of fatigue, paresthesia, weight loss, polyuria, anemia. She had severe hypokalemic metabolic acidosis (pH 7,14, K 1,31mmol/l), elevated ESR(45mm/h) and serum IgG(30g/l), eGFR 52ml/min, proteinuria 1,5g/l, enlarged kidneys on US; CT of chest, abdomen were normal. A few days later the girl had painless progressive flaccid quadriplegia. Autoimmune, oncological, infectious screening, spinal puncture didnt reveal pathology; MRT showed CPEPM. The girl was treated with IVIG, antibiotics, potassium supplements.She was admitted in our hospital because of the persistenсe of symptoms. The positive blood anti-SSA/anti-SSB in the absence of another autoimmune markers were revealed. A renal biopsy showed tubulo-interstitial nephritis.The diagnosis of primary SS was confirmed. Treatment with steroids led to marked clinical improvement. 

Conclusions:

SS should be suspect in patient with inexplained hypokalemic paralysis.