Abstract
### Case report. A 12-year-old boy with multiple nevi presented with 6 months of polydipsia (>4 L/day), polyuria, and a 40-pound weight loss that progressed over weeks to intractable headache and vomiting. The patient subsequently developed ataxia, complex partial seizures, and encephalopathy. Medical history was significant for numerous (40 to 50) benign congenital nevi ranging in diameter from 1 to 10 cm. He did not have a previous diagnosis of neurocutaneous melanosis (NCM) or a history of CNS disease. The initial workup revealed hyperglycemia (random blood glucose of 400) and hemoglobin (Hgb) A1c 11.9%. Anti–glutamic acid decarboxylase (GAD) antibody was 2.1 U/mL (normal <0.1). There was diffuse leptomeningeal enhancement on MRI. Initial lumbar puncture revealed an opening pressure of 40 cm H2O, white blood cell count of 23, glucose 40, and protein 193. Cytology from the CSF demonstrated atypical melanocytes consistent …
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