Total external ophthalmoplegia: First clinical manifestation of Sjögren’s syndrome

Abstract

Sjögren’s syndrome (SS) is an autoimmune disease causing destruction of the exocrine glands secondary to lymphocytic infiltration. Common clinical symptoms of SS are xerostomia, xerophthalmia, myalgia, arthritis, and vasculitis. Neurological symptoms may precede the diagnosis of SS by up to 2 years in about 80% of patients. A 28-year-old female presented to us with complaints of horizontal and vertical diplopia along with inability to move the right eye and sudden drooping of the right upper eyelid. She was a recently diagnosed case of type 2 diabetes mellitus and had a history of foreign body sensation in both eyes for 4 months. Schirmers I test revealed less than 5 mm of wetting in both eyes after 5 min, suggesting dry eyes. On immunological blood investigations, serum c-peptide was normal, rheumatoid factor was negative, antinuclear antibodies were positive, and proliferating cell nuclear antigens were positive. Extractable Nuclear Ag profile RO 52 was strongly positive suggestive of primary SS. Lip biopsy revealed mild-to-moderate chronic inflammation showing irregular acanthotic epidermis, and dermis had perivascular lymphocytic infiltrate. Thus, the possible diagnosis of primary SS with type 2 diabetes mellitus and right-sided total external ophthalmoplegia was made. Patient was prescribed subcutaneous insulin, multivitamins, tear substitutes and alternate patching. Later on insulin was replaced by oral hypoglycemics. The external ophthalmoplegia completely resolved within 6 weeks. So, total external ophthalmoplegia can be added to the spectrum of central nervous system involvement in SS.