For 3 months, a 37-year-old man developed memory loss and personality changes, followed by fever, seizures, bilateral upper motor neuron signs, and increased CSF protein with skin induration that was compatible with scleromyxedema. An IgG-type paraproteinemia was identified. He improved with plasmapheresis, but died in acute respiratory and renal failure. Autopsy revealed right temporal demyelination and meningeal inflammation.
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