You're Too Young for That! (Slides)

An 11-year old boy presented with right orbital pain for two months with recent double vision. He had no recent viral illnesses or sick contacts. He had no past medical history. His brother had Kawasaki disease; his paternal grandmother had rheumatoid arthritis; and his father died of a myocardial infarction at age 48. Our patient's exam showed only inability to abduct OD. Brain MRI showed dural thickening and enhancement along the right lateral cavernous sinus, right orbital apex, and tentorium. His lumbar puncture showed 6 white blood cells, 1 red blood cell, normal protein, glucose, and CSF ACE, and negative oligoclonal bands, gram stain, and cultures. The working diagnosis was Tolosa Hunt. He was started on high dose oral steroids with taper. Five months after initial presentation, he developed new right V2 numbness, complete right ophthalmoplegia, and weakness and numbness of his right hand and leg. These symptoms were again responsive to steroids. He had further workup that showed elevated ESR (44mm/hr), CRP (4.7mg/dL), and C4 (37mg/dL) while on oral steroids. He was hepatitis immune. Serum ACE, IgG4, B12, RF, ANA, quantiferon gold, VZV IgM, and cytometry were normal or negative. Repeat MRI brain showed decreased dural thickening. His symptoms resolved, and his disease was stable for 15 months. He returned with two weeks of left-sided headaches and acute diplopia, and on exam had; a left sixth nerve palsy. He also reported pain in his knees and ankles with mild swelling on exam. MRI Brain showed new hypertrophic pachymeningitis isolated to the right middle cranial fossa with slight extension superiorly overlying the lateral sulcus and frontal parietal junction. Extensive work up was negative: CSF Lyme, coccidioides, and aspergillus antibodies, meningitis/encephalitis PCR panel, CSF gram stain, culture, cytology, and cytometry, serum electrophoresis, dsDNA, RF, and lysozyme. A diagnostic procedure was performed