Case 1: A 68-year-old female presents with a 2-year history of progressive bilateral ptosis and blurry vision as well as 6 months of diplopia found to have bilateral ptosis, upward gaze fatigability, and right CN VI palsy. MRI brain revealed diffuse pachymeningeal inflammation probable sarcoidosis, CSF ACE elevated at 3.1 U/L.
Case 2: A 48-year-old male with left episcleritis presents with a 2-year history of leg weakness and acute onset of headache and vertigo. MRI brain revealed pachymeningeal enhancement at the cerebral tentorium with repeat scan in January 2020 revealing diffuse pachymeningeal enhancement. Right temporal dural biopsy revealed necrotizing pachymeninges secondary to ANCA + vasculitis.
Case 3: A 64-year-old-male develops new onset chronic daily headache, predominantly bifrontal and significant fatigue. MRI brain showed diffuse pachymeningeal thickening and enhancement. LP was normal with normal ACE level. We are currently awaiting biopsy for this patient with possibility of sarcoidosis.
Case 4: A 37-year-old male who at age 25 developed new onset intractable holocephalic, throbbing headache and seizures. He had extensive work up done prior to establishing with our practice which included left temporal lobe biopsy of a mass-like lesion which revealed Idiopathic Hypertrophic Pachymeningitis. Initially treated with Imuran and later switched to Rituximab.