Sjogrens disease and Diplopia- extremely rare case discussion
A 45-year-old woman presented with complain of double vision on horizontal and vertical gaze, which started 2 weeks prior to admission. She had some blurring of vision in her left eye and retro-orbital pain which subsided later. Her symptoms progressed to involve her right eye. A week before admission, she had noticed grittiness in her eyes, and thought her eyelids were droopy.On examination she had no deviation on primary gaze but had limited adduction as well as abduction in both eyes. Vertical gaze was limited in the right eye, and downgaze was only slightly impaired on the left. She had limited convergence of both eyes. Ptosis was more prominent on the right side. Pupils were equal and reactive to light on both sides.
Several other serum labs including, ANA, ENA, ANCA, Serum protein electrophoresis, TSH, vitamin B12, angiotensin converting en- zyme level, VDRL, anti RNP, anti SSA, anti SSB, anti Jo1, myeloperoxidase antibody, HIV, beta 2 glycoprotein, along with paraneoplastic panel were performed and were within normal limits. CSF analysis showed normal cell count, protein level, IgG index and no oligoclonal bands. Anti GM1, anti GD1a, anti GD1b in serum were negative. Anti SSA anti- body were detected in her serum at concentration of 65.2 AAU/mL (normal 0–24.9 AAU/mL). MRI of the brain showed thickening bilateral oculomotor and abducens nerves, with enhancement after gadolinium administration. There was progression and worsening of the enhancement and thickening on repeat imaging performed within 4 days of the initial scan.
Initial differential included Lymes disease, CNS vasculitis, sarcoidosis, lupus, and other connective tissue disorders such as Behcets disease and rheumatoid arthritis. However, the serum work up and lack of supporting history and clinical exam excluded these diseases from our differential.
She was treated with intravenous methylprednisolone 1 g daily dose for 5 days and responded with full recovery. In a week her symptoms of diplopia had improved and within 2 weeks of start of therapy she had complete range of extraocular movements with no ptosis on either side. During her hospitalization she did not complain of any further episodes of dry eyes or mouth. Lip biopsy performed 4 weeks after steroid treatment showed presence of plasma cell in?ltrates without destruction of salivary gland acini or any granulomas. She has not had a relapse of her symptoms since 2 years