Objective:

To describe a case of Coccidioides immitis leptomeningitis mimicking neurosarcoidosis.

Background:

Neurosarcoidosis involves granulomatous inflammation of the central and/or peripheral nervous system. Leptomeningitis complicates 10-20% of Neurosarcoid cases. Treatment involves immunosuppression. Coccidioides immitis (C. Immitis) is endemic to south and southwest United States and commonly causes “Valley Fever,” a self-limiting respiratory illness. Rarely, C. Immitis can invade the leptomeninges with CNS dissemination and treatment involves fluconazole.

Design/Methods:

Retrospective Chart Review.

Results:

A 29-year-old man with no medical history, who immigrated from Colombia to Arizona in 2022 and then to New York in 2023, presented with progressive headaches with nausea and vomiting. Neurological exam was nonfocal and CTH revealed new communicating hydrocephalus when compared to a CTH ordered for a mechanical fall 4 months prior. Contrasted MRI brain revealed diffuse and thick leptomeningeal enhancement. Lumbar puncture revealed lymphocytic pleocytosis, hypoglycorrhachia, elevated protein, and negative CSF bacterial and fungal culture, acid-fast staining, meningitis/encephalitis PCR panel, and cytology, but elevated CSF ACE. Body CT imaging was unremarkable. Patient required CSF diversion with an EVD due to depressed mental status. Biopsy of the cervical leptomeninges revealed non-necrotizing granulomas with multinucleated giant cells surrounded by lymphoplasmacytic infiltrate. Neurosarcoidosis was initially diagnosed given clinical presentation, negative infectious work up, extensive leptomeningitis, elevated CSF ACE, and biopsy result consistent with sarcoid. Corticosteroid therapy was initiated with mild clinical improvement. Routine CSF culture from EVD was sent prior to VPS planning and grew C. Immitis concerning for C. Immitis leptomeningitis. Steroid therapy was discontinued, and patient was treated with Fluconazole with clinical improvement. 

Conclusions:

This case report highlights that C. Immitis leptomeningitis may mimic noninfectious neurological conditions, such as neurosarcoidosis. C. Immitis should be suspected in patients with leptomeningitis who are from or travel from endemic areas despite initial negative CSF culture and with non-necrotizing granulomas on biopsy.