The authors reported a rare case with immunoglobulin (Ig) G4-related ophthalmic disease presented with chronic progressive bilateral complete ophthalmoplegia and blindness from orbital apex syndrome. MRI brain and orbit demonstrated ill-defined infiltrative lesions at bilateral orbital apexes, bilateral optic canals, and bilateral Meckel’s caves, causing optic nerve compression and possibly optic neuropathy with generalized leptomeningeal enhancement at dura, cavernous sinus, and parotid gland. Lumbar puncture revealed few small lymphocytes, rare monocytes, very rare neutrophils with degenerative cells in background, and negative for malignancy. Serology titers for IgG subclass 4 (IgG4) had resulted in 5.959 grams per deciliter (g/dL). A dural biopsy revealed aggregate histiocytes with chronic inflammation and focal foreign body type giant cells. Motility improvement was achieved in the patient after systemic corticosteroids treatment. IgG4 serology should be considered for workup when patients present with chronic idiopathic orbital inflammation.

Keywords: IgG4-related ophthalmic disease, Ophthalmoplegia, Orbital apex syndrome

DOI: doi.org/10.35755/jmedassocthai.2020.07.11128

Received 4 Feb 2020 | Revised 13 May 2020 | Accepted 15 May 2020