A 56-year-old Caucasian man presented to clinic with a 6-week history of progressive dysesthesia in bilateral arms and legs up to the buttocks and gait difficulties.
He was found to have a multi-focal enhancing and longitudinally extensive lesion with dorsal subpial enhancement on MRI of the cervical and thoracic spine extending from C2 to mid T4 level (Figure 1). Brain MRI was unremarkable. Biopsy of a pulmonary mass showed a granuloma that was not definitive for sarcoidosis. CSF analysis twice revealed persistent pleocytosis (18-61 cells/mm3) and elevated protein but no hypoglycorrhachia. CSF lymphoma markers and ACE levels were unremarkable. Neuromyelitis optica and myelin oligodendrocyte glycoprotein antibodies were also negative. Given the lack of tissue diagnosis and an inconclusive prior lung biopsy, a spinal cord biopsy was done and showed noncaseating granulomas consistent with definite neurosarcoidosis. He was discharged on an oral taper of dexamethasone starting at 15 mg daily along with combination of methotrexate 15 mg weekly and infliximab 5 mg/kg.
Two months later while on this combination regimen of dexamethasone taper, methotrexate 15 mg weekly and infliximab (received 2 infusions), he developed dyspnea and was found to have new pulmonary nodules and cavitary lesions. Simultaneously, he developed right eye blindness. Repeat brain and orbital MRI showed innumerable ring-enhancing lesions in the brain, right endophthalmitis, episcleritis and orbital cellulitis extending from adjacent frontal lesions with surrounding edema concerning for infection (Figure 2). Previous sarcoidosis-related spinal cord abnormalities had resolved completely and immunosuppressants were discontinued. Fungitell test came back positive with a value greater than 500 pg/mL (reference range: <80 pg/mL) and liposomal amphotericin B was initiated. A biopsy of his largest cavitary lung nodule and the vitreous culture of the right eye confirmed Aspergillus fumigatus. Specifically, tuberculosis and malignancy were absent. With a diagnosis of disseminated aspergillosis, amphotericin was transitioned to voriconazole for continued maintenance therapy of disseminated aspergillosis. Unfortunately, he lost all vision in the right eye and underwent enucleation, which substantiated the diagnosis of aspergillosis on histological analysis (Figure 3).
Further surveillance MRIs demonstrated regression of the newly developed lesions, yet persistent contrast enhancement in the brain, despite two years of voriconazole treatment with clinical improvement. Voriconazole was ultimately discontinued due to exfoliative dermatitis, and given his clinical status, no other antifungals were initiated. The patient remains in clinical and radiological remission without immunosuppressive therapy as of 30 months later.
Written informed consent was acquired from the patient for clinical information and medical images to be published.