1. Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology A 27-year-old man with bright basal ganglia and thalami on MRI

2. Vignette A 27-year-old man with cholestasis presented with 4 weeks of progressive spastic quadriparesis and hypophonia. Lakhan et al.

3. Brain MRI 4 weeks after symptoms Lakhan et al.

4. Vignette (cont.) He was treated with IV methylprednisolone for presumed acute disseminated encephalomyelitis. He clinically worsened. Lakhan et al.

5. Brain MRI 8 weeks after symptoms Lakhan et al.

6. Vignette (cont.) Blood manganese was elevated (25.4 ug/L, reference range 4.4 - 15.2). There was no occupational, environmental, or medical exposure. He was treated with chelation therapy and levodopa with clinical response. Lakhan et al.

7. Manganese neurotoxicity of the basal ganglia and thalamus This case represents manganese toxicity without known exposure. Cholestasis was the sole risk factor as manganese undergoes biliary excretion. Other workup were negative: – toxic (carbon monoxide, methanol, cyanide), – metabolic (hyperammonemeia, nonketotic hyperglycemia, hypoglycemia, hypoxic ischemic encephalopathy, Leigh's disease, Wilson's disease, osmotic myelinolysis, Wernicke's encephalopathy), – vascular (deep cerebral vein thrombosis), – degenerative (pantothenate kinase-associated neurodegeneration, Creutzfeldt-Jakob disease), and – infectious (flavivirus, toxoplasmosis) diseases. Lakhan et al.