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Coccidioidomycosis has been associated with various cerebrovascular complications, including infarctions, aneurysms, intracranial hemorrhage, vasospasm, and transient ischemic attacks. Mortality is typically high, with limited targeted therapeutic options.

A 36-year-old immunocompromised female with SLE, Lupus Nephritis, antiphospholipid antibody syndrome on warfarin, and a history of coccidioidomycosis meningitis two years prior (on lifelong antifungal therapy), presented with confusion and speech difficulties. One month prior, she was hospitalized in Tucson, Arizona, for hydrocephalus and possible meningitis. Cerebrospinal fluid (CSF) analysis showed low glucose, mildly elevated white cells, and protein. Infectious work-up, including fungal and bacterial cultures, GMS stain, next-generation sequencing, and coccidioidomycosis serology, revealed no evidence of bacterial, fungal, or mycobacterial infection in CSF, blood, or peritoneal fluid.

On presentation, MRI revealed scattered infarcts across multiple vascular territories, and arterial imaging showed occlusion of right middle cerebral artery (MCA) branches and the left anterior cerebral artery (ACA), with multifocal stenosis in bilateral M1 segments. She was treated with intravenous methylprednisolone, continued on anticoagulation with warfarin and discharged to rehabilitation. 48 hours after discharge, she developed worsening confusion, fever, right-sided weakness, left gaze deviation, and global aphasia. Repeat MRI showed increased stroke burden, with new occlusion of the left M1 and distal branches, and extensive irregular stenosis of the supraclinoid right internal carotid artery, bilateral M1 and M2 segments of MCA, proximal A2 segment of ACA, V4 segments of bilateral vertebral arteries and the basilar artery. Fungal vasculitis was suspected as the underlying etiology based on cerebrovascular imaging, hypoglycorrhachia and prior history of coccidioidomycosis.

This case highlights the risk of recurrent multifocal strokes and cerebrovascular complications in patients with a history of coccidioidomycosis, even while on long-term antifungal treatment.