Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP-5 neuronal antibody

Abstract

Paraneoplastic limbic encephalitis (PLE) is characterized by progressive dementia, psychiatric disturbance, and seizures.1 Small cell lung carcinoma is the main associated tumor (50 to 60%),1 and various antineuronal autoantibodies have been described. Recently, a novel autoantibody to collapsin response-mediating protein-5 (CRMP-5) has been described as a marker of lung cancer and thymoma-related autoimmunity.2 Although stereotypic clinical features have not been described with the CRMP-5 antibody, chorea is a prominent associated clinical feature.2,3⇓ We report an autopsy proven case of PLE with multiple coexisting paraneoplastic syndromes including chorea and unusual MRI and autopsy findings in association with CRMP-5 neuronal antibody. A 62-year-old woman presented with vague abdominal symptoms and weight loss. She had a 40-pack-year smoking history. Extensive gastrointestinal tests, chest x-rays, and routine laboratory investigations had normal results. Anxiety and depression were diagnosed and the patient was put on mirtazapine and discharged. The patient subsequently became intermittently agitated and developed neurologic signs necessitating readmission. General examination was significant …