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A 61-year-old man with rheumatoid arthritis presented with acute confusion in the setting of chronic bifrontal headaches. His headaches began 7 months previously after bilateral sinus uncinectomies and ethmoidectomies for sinusitis, and responded significantly to prednisone therapy. Further history revealed he suffered from fatigue, weight loss, blurred vision and episodes of nausea and vomiting since 3 months prior.
Upon presentation, he was febrile, tachycardic and hypertensive. Neurologic examination demonstrated disorientation, poor attention, agitation, anisocoria, left-sided hemiparesis and hyperreflexia throughout with bilateral Babinski signs. Initial testing revealed an elevated white blood cell count. Blood cultures were negative. A serum autoimmune workup including C3 and C4 complement, antinuclear antibodies, anti-neutrophil cytoplasmic antibody and rheumatoid factor was unremarkable. Serum erythrocyte sedimentation rate and C-reactive protein were elevated. Head CT scan was negative while brain MRI revealed diffuse pachymeningeal enhancement (Fig. 1).
Fig. 1Axial T1-weighted brain MRI with gadolinium showing diffuse pachymeningeal thickening and enhancement.
Lumbar puncture revealed an elevated opening pressure (>55 cm H2O) and cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis (22 white blood cells/μL with 72% lymphocytes), elevated protein concentration (124 mg/dL; normal: 15–60 mg/dL) and hypoglycorrachia. CSF cytology analysis was negative. An extensive CSF infectious workup was negative for Streptococcus, Mycobacterium, Neisseria, Listeria, Bartonella, human immunodeficiency virus, Herpes, Varicella, Lyme, syphilis, Nocardia, Cryptococcus, Blastomyces, Histoplasma, Coccidioides, and Aspergillus. Angiotensin-converting enzyme levels in the serum and CSF were normal.
The patient underwent a brain biopsy, which showed significant inflammatory cell infiltration in the dura as well as foci of necrosis (Fig. 2). A Gram stain, Grocott’s methenamine silver stain, and an acid-fast stain were negative.
Fig. 2Histopathology slides of the dura demonstrating nodular chronic inflammatory infiltrates, scattered giant cells, and foci of necrosis. (A) Hematoxylin and eosin (H&E) stain at low power showing infiltration of inflammatory cells in the dura mater. (B) H&E stain at medium power showing a nodule of inflammation in the dura. (C) H&E stain at medium power showing a focus of necrosis in the dura. CD3, CD20 and CD68 immunohistochemistry showed prominent T-cell , B-cell and histiocytic infiltration in the dura (Supp. Fig. 1). (This figure is available in colour at http://www.sciencedirect.com.)
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