A Difficult Day with Double Trouble (.pdf)

A 59 year old woman with a history of hypothyroidism, an inner ear implant and lupus (complicated by autoimmune hemolytic anemia and raynaud's disease) presented with 1 week of intermittent horizontal binocular diplopia. She denied headache and giant cell arteritis symptomatology. Examination demonstrated normal afferent function, full ductions and an incomitant esotropia worse in right gaze. She was diagnosed with a subtle microvascular right sixth nerve palsy. Two weeks later, she developed intractable bifrontal headaches and photosensitivity. On neuro-ophthalmic evaluation she had bilateral limitation of abduction, left greater than right, and slight limitation of elevation of both eyes. A head CT with enhancement demonstrated slightly enlarged extraocular muscles and crowding of the orbital apex bilaterally. Bloodwork was significant for a slightly elevated WBC count with a longstanding microcytic anemia (Hb 10.4) and thrombocytosis. Thyroid function studies and myasthenia gravis work-up was normal. However, antithyroglobulin antibody was elevated at 59 (N<2). Thyroid orbitopathy was considered a probable diagnosis at this time. However, the patient's complaint of relentless headaches prompted persuing an MRI brain and orbits with contrast that demonstrated diffuse pachymeningeal enhancement, abnormal enhancing soft tissues in the orbital apices bilaterally particularly surrounding the intercanalicular segments of the optic nerves and enlarged enhancing extraocular muscles. Lumbar puncture demonstrated an opening pressure of 24 cm H20 with normal constituents. Blood ACE, lysozyme, RPR, VDRL, ANCAs, complement level and inflammatory markers were unremarkable. The patient improved significantly upon solumedrol although diagnosis was unclear. An immunoglobulin and IgG4 level was ordered and the serum IgG4 level was significantly elevated.