Brain paracoccidioidomycosis in an immunosuppressed patient with systemic lupus erythematosus

Brain paracoccidioidomycosis (PCM) or neuroparacoccidioidomycosis (NPCM) is a fungal infection of the central nervous system (CNS) caused by Paracoccidioides brasiliensis, a dimorphic fungus. The CNS involvement is through bloodstream dissemination. The association between NPCM and systemic lupus erythematous (SLE) is rare. However, SLE patients are under risk of opportunistic infections given their immunosuppression status.

The aim of this case report is to present a 37-year-old female with diagnosis of SLE who presented with progressive and persistent headache in the past 4 months accompanied by the right arm weakness with general and neurologic examination unremarkable. The computerized tomography of the head showed left extra-axial parietooccipital focal hypoattenuation with adjacent bone erosion. The brain magnetic resonance imaging reported left parietooccipital subdural collection associated with focal leptomeningeal thickening with restriction to diffusion and peripheral contrast enhancement. The patient underwent a left craniotomy and dura mater biopsy showed noncaseous granulomatosis with multinucleated giant cells with rounded birefringent structures positive for silver stain, consistent with PCM. Management with itraconazole 200 mg daily was started with a total of 12 months of treatment, with patient presenting resolution of headache and right arm weakness.

The diagnosis of NPCM is challenging and a high degree of suspicious should be considered in patients with persistent headache and immunosuppression.