Objective:

We report a case of IgG4 related disease with isolated meningeal involvement resulting in persistent intracranial hypertension.

Background:

IgG4 related disease is a chronic, immune mediated fibroinflammatory disorder with a wide range of manifestations.

Design/Methods:

N/A

Results:

32-year-old obese female presented to outside hospital with progressive headaches, blurred vision and new onset seizures. Initial examination revealed visual acuity of 20/40 with bilateral grade 4 disc edema. MRI brain demonstrated dural enhancement, hydrocephalus, and Chiari 1 malformation. A ventriculoperitoneal shunt was placed and meningeal biopsy demonstrated nonspecific acute and chronic inflammation with adjacent cerebritis. The patient improved following shunt placement but required shunt removal later due to intracranial infection. Nasal biopsy done showed acute on chronic inflammation. Following shunt removal, the patient noted worsening vision prompting presentation to the ED. She was found to have 20/400 vision in both eyes with pale edematous disc. MRI again showed diffuse dural and leptomeningeal enhancement. Lumbar puncture showed opening pressure of 29 with positive oligoclonal bands and IgG index of 1.3 -- consistent with CSF inflammation. Repeat brain biopsy and nasal biopsy showed similar findings to prior. Disc edema improved with oral acetazolamide, however, patient had persistent seizures prompting re-admission. Broad infectious and autoimmune lab workup, including serum IgG4 levels were within normal limits. The third repeat meningeal biopsy met criteria for probable IgG4 related disease. The patient had stabilization of symptoms on oral steroids. She has been maintained on Rituximab without recurrent symptoms. 

Conclusions:

IgG4 related disease has a wide range of manifestations that can include intracranial hypertension due to meningeal inflammation. Serum IgG4 levels in IgG4 related disease may not be elevated if disease process is isolated to a single organ system. Repeat biopsy may be warranted in cases of persistent inflammation to achieve the diagnosis, as in this case which required multiple biopsies to confirm diagnosis.