Objective:

To report a unique and rare case of Ig4 disease presenting as chronic headaches. 

Background:

NA 

Design/Methods:

Retrospective chart review of a case

Results:

A 35-year-old woman presented with a worsening bitemporal, throbbing headache for two
months, associated with nausea and vomiting. The headache partially improved with topiramate,
however, she developed amenorrhea, galactorrhea, and diplopia after two months of treatment.
Her serum prolactin level was found to be elevated and MRI showed heterogeneously enhancing pituitary mass. A diagnosis of benign pituitary macroadenoma was made and galactorrhea improved with initiation of cabergoline therapy. However, her headache and amenorrhea
persisted despite topiramate and cabergoline therapy. She also developed blurry vision and
ophthalmoplegia with evidence of sixth cranial nerve palsy. MRI showed an enlarged suprasellar lesion with mass effect compressing right pre-chiasmatic optic nerve. A CSF study revealed elevated CSF protein with oligoclonal bands and normal opening pressure. She underwent transsphenoidal pituitary macroadenoma resection and biopsy showed an inflammatory process and lymphocytic hypophysitis, consistent with IgG4-related disease. Following surgery, she also
developed right-sided weakness and sensory deficits, due to cavernous sinus involvement causing ICA stenosis and bilateral watershed infarcts. She was initiated on treatment with prednisone and rituximab which improved her symptoms and is currently receiving cyclophosphamide infusions for her IgG4-related disease.

Conclusions:

IgG4-related disease can present with pituitary hypophysitis with a slow indolent course which may mimic pituitary adenoma in presentation. Dural enhancement, involvement of other cranial nerves and persistent headache as a presenting symptom should raise suspicion for this entity in these patients.