IgG-4 Disease; Panuveitis; 3rd Nerve Palsy; 6th Nerve Palsy; Skull Base Mass
History
33 year-old man presented with 3 days of binocular diplopia. Visual acuity was 20/30 OU and pupillary exam was normal. Motility testing demonstrated right partial 3rd nerve palsy (no adduction and limited supraduction with complete ptosis). There were +1/2 anterior chamber cells and few vitreous cells bilaterally. Fundoscopy was normal with mild bilateral cystoid macular edema on OCT. Of note, the patient was diagnosed with bilateral panuveitis six years ago for which no identifiable cause was found. He was treated with several topical and systemic immunosuppressants, but was receiving only adalimumab injections regularly for past two years; he continued to demonstrate low-grade panuveitis while on treatment. He recalled having three previous episodes of intermittent diplopia with right ptosis during last three years which spontaneously resolved. Urgent MRI/MRA revealed densely enhancing osteodestructive lesion around right sphenoid sinus, extending into adjacent meninges, right cavernous sinus and right superior orbital fissure; associated soft tissue thickening had low signal on T2. Radiological differential was between inflammatory or neoplastic entities. After short course of oral prednisone, motility deficits and ptosis resolved. One month later, diplopia recurred; right 6th nerve palsy was diagnosed. At the same time, central vision deteriorated to counting fingers with right RAPD. CT chest, abdomen and pelvis were normal. Extensive serological testing was unrevealing. Transphenoidal biopsy of skull base mass demonstrated inflammatory infiltrate with lymphocytes and slight increase in B cells, but was otherwise unrevealing. Bone marrow biopsy was normal. Adalimumab was discontinued and, two weeks after biopsy, acuity had improved to 20/40 OD; motility deficits had resolved. There were still +1/2 cells in AC bilaterally. CT brain and orbits demonstrated destructive changes in left sphenoid sinus and thickening of left cavernous sinus and orbital apex. Radiological differential was unchanged. A diagnostic procedure was performed.
Date
2016-02-28
Language
eng
Format
video/mp4
Type
Image/MovingImage
Relation is Part of
NANOS Annual Meeting Frank B. Walsh Sessions; 2016
