Coccidioides species are endemic to the southwestern United States and typically cause a mild or asymptomatic primary infection. In some instances, infection can disseminate and involve the central nervous system with meningitis being the most common manifestation. Non-osseous spinal cord involvement is exceedingly rare. We report a case of disseminated coccidioidomycosis in an otherwise healthy 20 year old man with diffuse leptomeningeal enhancement, cerebrospinal fluid findings suggestive of meningitis, and intramedullary spinal cord abscesses. Response to treatment occurred with prolonged systemic liposomal amphotericin B and voriconazole. An extended course of steroids was needed to blunt inflammation.

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Orbitofacial neurofibromatosis (OFNF) is considered a variant of neurofibromatosis type 1 (NF1). OFNF most often affects the eye, orbit and one side of the face. It is characterized by the development of relatively aggressive and disfiguring lesions, including plexiform and diffuse neurofibromas. Ciliochoroidal ganglioneuromas have not been previously reported in patients with this syndrome. We report the case of a 50-year-old man with OFNF, ciliochoroidal ganglioneuroma and a large ipsilateral frontoethmoidal encephalocele.