Abstract Details

Title IgG4 Related Disease Presenting as Persistent Intracranial Hypertension

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) P9 - Poster Session 9 (11:45 AM-12:45 PM)

Poster/Presentation
Number 11-011

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.

Authors/Disclosures
Momina Qureshi (The Cleveland Clinic Foundation) PRESENTER	Miss Qureshi has nothing to disclose.
Benjamin Katz, MD	Dr. Katz has nothing to disclose.
Poonam Bansal, MD (Henry Ford Health System)	Dr. Bansal has nothing to disclose.
Mirela Cerghet, MD, PhD, FAAN (Henry Ford Hospital)	Dr. Cerghet has nothing to disclose.

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