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Abstract Details

Granulomatosis with Polyangiitis-Associated Hypertrophic Pachymeningitis as a Neurosarcoidosis Mimic
Autoimmune Neurology
P15 - Poster Session 15 (12:00 PM-1:00 PM)
15-010
To bring awareness to an important neurologic manifestation of granulomatosis with polyangiitis (GPA).
GPA is an autoimmune vasculitis affecting small-to-medium vessels that typically involves the upper and lower respiratory tract and the kidneys. CNS involvement occurs in 7-11% of patients with GPA. Hypertrophic pachymeningitis (HP) accounts for a third of the neurologic complications in GPA; however, patients with HP have a lower incidence of systemic manifestations which leads to significant delay in diagnosis.
A 47-year-old Hispanic male with a history of episcleritis of the left eye (taking adalimumab and methotrexate for the past 2 years) presented with vertigo, severe headache, and diplopia. He developed nystagmus, ophthalmoplegia, and later right peripheral cranial nerve 7 palsy, as well as nosebleeds. Methylprednisolone led to resolution of his headache.
Brain MRI revealed dural thickening and enhancement of the right tentorium as well as meningeal enhancement along the right internal auditory canal and cerebellopontine angle. Lumbar puncture demonstrated 6 nucleated cells (78% lymphocytes), 16 RBCs, protein 114, and glucose 91. CSF ACE and IL-2 receptor antibody were negative. Serum C-ANCA was positive. CT chest was negative for granulomas. Biopsy of the dura is currently pending. Although his clinical presentation with multiple cranial nerve palsies and dural thickening was concerning for neurosarcoidosis, workup was more consistent with GPA-associated HP and he was started on rituximab.
Dural thickening is common to both neurosarcoidosis and hypertorphic pachymeningitis, and these may be confused because both are granulomatous processes. Some studies suggest that C-ANCA positive HP should be recognized as a distinct clinical entity known as CNS-limited GPA, with lesions limited to the dura mater and upper airways. It is important to recognize severe headache and HP as presenting symptoms of CNS-limited GPA since early treatment with rituximab or cyclophosphamide leads to improved clinical outcome and lower relapse rate.
Authors/Disclosures
Joshua Kramer, MD
PRESENTER 		Dr. Kramer has nothing to disclose.
Jessica Frey, MD (West Virginia University) The institution of Dr. Frey has received research support from Tourette Association of America.
Ann Murray, MD (West Virginia Department of Neurology) Dr. Murray has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Insightec. Dr. Murray has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medtronic .