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Washington University Experience | MISCELLANEOUS | Idiopathic Hypertrophic Pachymeningitis (IgG4 Disease) | 8A0 Case 8 History
Case 8 History ---- The patient is a 59yo female with a PMHx of congestive heart failure (CHF), type 2 diabetes, atrial fibrillation on Eliquis, hypertension, OSA, GERD and depression. She reported a 1 month history of tinnitus and aural fullness in her right ear and was diagnosed with an ear infection and given 1 week of antibiotics. She did not improve and was given an additional week of antibiotics. On 8/29 the patient was admitted to an OSH for CHF exacerbation. During her admission, she complained of headache, right ear pain and tinnitus. A CT showed right sided acute mastoiditis. An MRI confirmed right sided mastoiditis, pachymeningitis, cerebritis and possible subdural empyema. She was admitted on 9/10 to BJH for right sided mastoiditis and possible subdural empyema. Vancomycin, Cefepime, and Flagyl were begun. The patient underwent a right tympanoplasty with mastoidectomy and encephalocele repair with bone graft on 9/12. Blood and OR cultures did not identify an infectious agent. She was discharged on a 10 day course of Augmentin. Her headache had significantly worsened over the last day resulting in readmission to BJH with suspicion of meningitis and cerebritis in the setting of recent mastoiditis and possible dural venous sinus thrombosis. An MRI showed worsening right temporal pachymeningeal and leptomeningeal enhancement, vasogenic edema with mass effect, partial effacement of the right lateral ventricle, and possible subocclusive filling defect at the junction of the right sigmoid/ transverse sinuses. She was given Cefepime, Vancomycin, and Flagyl along with Reglan, Benadryl, and Hydrocodone. The patient was then transferred to BJH NNICU for further neurological evaluation. On arrival to the NNICU, she reported that her headache became severe on 10/3 and she also noticed sensitivity to light and decreased hearing. Tinnitus in her right ear was unchanged from prior to surgery. Since her first surgery she was living with one of her daughters who had not noticed any confusion, emotional lability or significant weakness. The patient underwent craniotomy on 10/4 for lesion resection. She was transferred subsequently to the medicine service on 10/8 where she was continued on empiric antibiotics. The specimen from her Initial mastoid surgery received a diagnosis of “mixed inflammatory cell infiltrate with polyclonal plasmacytosis consistent with chronic inflammation and no evidence of malignancy or infectious etiology”. Pathologic findings on her second surgery showed dura, leptomeninges and brain parenchyma with granulomatous inflammation, small vessel vasculitis/vasculopathy, and increased IgG4+ plasma cells. She received rituximab beginning on 10/21 with plan for repeat dosing within a month and close outpatient follow-up.
