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Abstract Details

Intracranial Pachymeningitis: A Case-Series Describing Clinical Features, Imaging, Serological and Cerebrospinal Fluid Markers, Biopsy Findings and Management
Autoimmune Neurology
P9 - Poster Session 9 (5:30 PM-6:30 PM)
6-018
Hypertrophic pachymeningitis is characterized by focal/diffuse thickening of intracranial and/or intraspinal dura.
We present a case-series of patients with intracranial pachymeningitis and outline their management for successful outcomes.

Case-1: 48-year-old-woman with Crohn's disease, relapsing polychondritis, presented with persistent left-sided headaches. MRI brain revealed left-sided contrast-enhancing pachymeningeal thickening; CSF showed lymphocytic pleocytosis. Glucocorticoids, adalimumab, and methotrexate resulted in clinical and neuroimaging improvement.

Case-2: 78-year-old-woman presented with intractable headache, bilateral sensorineural-hearing-loss and vision-impairment. MRI brain showed nodular, multifocal dural-thickening at clivus, parasellar region, cavernous-sinuses extension encasing major vessels at skull-base. CSF showed lymphocytic pleocytosis, protein and serology demonstrated IgG4-subclasses, attributing etiology to IgG4-related-disease. Steroids, methotrexate, and later IV-rituximab resulted in equivocal response. Intrathecal rituximab, resulted in clinical and imaging stabilization, from better CNS penetration.

Case-3: 60-year-old woman, with sarcoidosis presented with severe headaches. MRI demonstrated contrast-enhancing dural thickening in left posterior-temporo-occipital regions. Brain-biopsy showed non-necrotizing granulomas involving dura, parenchyma consistent with sarcoidosis. Steroids and infliximab, lead to clinical and radiological improvement.

Case-4: 76-year-old man with prior metastatic melanoma, post-chemotherapy (nivolumab+ipilimumab), presented with headaches. MRI showed diffuse intracranial contrast-enhancing pachymeningeal thickening. Chest CT-scan showed circumferential thickening, contrast-enhancing thoracic aorta indicating systemic vasculitis. Serologically, ANA 1:320, +SSA/SSB, ESR/CRP both >100 and ANCA was negative. Meningeal biopsy demonstrated granulomatosis with polyangiitis; etiology considered paraneoplastic. Steroids and methotrexate demonstrated clinical and radiological response.

Case-5: 56-year-old woman presented with headaches and CN-VII palsy. MRI brain revealed hypertrophic pachymeningitis causing mass-effect on right lateral ventricle. ANCA serological positivity and biopsy demonstrated AFB-stain-negative caseating granulomas, consistent with ANCA-vasculitis. Rituximab led to complete resolution of both pachymeningeal and CN-VII enhancement.

Clinical resolution and radiological stability was achieved for all 5 patients.

Intracranial pachymeningitis presents in setting of systemic autoimmune disorders and sarcoidosis. Brain imaging, CSF-analysis, serological immune-markers, and biopsy aid in diagnosis. Immunosuppressive therapies provide for clinical and radiologic stabilization.

Authors/Disclosures
Aditi Vian Varma-Doyle, MD (Brigham and Women's Hospital, Mass General Brigham)
PRESENTER
Dr. Varma-Doyle has nothing to disclose.
Giovanna Manzano, MD (MGH Department of Neurology) Dr. Manzano has nothing to disclose.
Nagagopal Venna, MBBS, FAAN (Massachusetts General Hospital) Dr. Venna has nothing to disclose.