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Central pontine myelinolysis a rare manifestation of CNS Sjogren's syndrome

Rheumatology Section, Department of Medicine, National University Hospital, National University of Singapore, 5 Lower Kent Ridge Road, Singapore 119074. Fax: (+ 65) 7794112; mdcykhinus.edu.sg

K Y Fong

D R Koh

R Suri

Rheumatology Section, Department of Medicine, National University Hospital, National University of Singapore, Singapore

Central nervous involvement in Sjogren's syndrome (CNS-SS) is not uncommon and has a variety of manifestations. We describe a 47-year-old woman with Sjogren's syndrome who presented with distal renal tubular acidosis with severe hypokalemia and hypokalemic myopathy. She developed progressive obtundation after years of stable disease. ANA, anti-Ro antibodies were positive. Brain MRI showed a cleft in the mid pons which was hypointense on T1 and hyperintense on T2 which was considered to be classical of central pontine myelinolysis. Serial MRI showed initial enlargement of the lesion which persisted despite successful immunosuppressive therapy with pulse methyprednisolone, pulse cyclophosphamide, plasmapharesis and IVIG.

Key Words: central pontine myelinolysis • Sjogren's syndrome

Lupus, Vol. 9, No. 6, 471-473 (2000)
DOI: 10.1191/096120300678828550


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