A 28-year-old man with a history of childhood epilepsy was admitted to the hospital with recurrent headache and ataxia thought to be secondary to meningitis. Magnetic resonance imaging (MRI) of the brain showed diffuse leptomeningeal enhancement. Cerebrospinal fluid analysis revealed mild pleocytosis, hypoglycorrhachia and elevated protein. Cultures, viral panel, cytology, and flow cytometry were unremarkable. Extensive infectious and inflammatory work up was unrevealing. Follow-up computed tomography (CT) head showed hydrocephalus requiring ventriculoperitoneal shunt placement. Brain biopsy reported mild gliosis.
Three months later, patient was readmitted with left sided weakness and dysarthria. Initial head CT revealed acute right frontal hemorrhage. CT angiogram and conventional angiography were unrevealing. Hospital course was complicated by multiple subsequent spontaneous intracranial hematomas in different vascular territories. MRI of brain and cervical spine showed a longitudinally extensive spinal cord lesion and flow voids concerning for dural AV fistula. Spinal angiogram was unremarkable, and findings were thought to be secondary to venous congestion in the setting of over-shunting (Miyazaki Syndrome). Another brain biopsy with extensive pathology staining revealed diffuse leptomeningeal melanomatosis (MART-1 and HMB-45 positive). No ocular or cutaneous melanoma was found. Due to devastating neurological deterioration, patient was not a candidate for any further treatment. Patient was transitioned to comfort measures and ultimately died.