Ophelia syndrome: Hodgkin lymphoma with limbic encephalitis.

Abstract

BACKGROUND: Limbic encephalitis, an immune-mediated encephalitis, results from inflammation in the medial temporal lobes. The paraneoplastic form is rare in pediatric population, and frequently precedes tumor diagnosis. CASE CHARACTERISTICS: A 9-year-old boy receiving chemotherapy for Hodgkin lymphoma, developed headache, temporal lobe seizures, anxiety, hallucinations, short-term memory loss and autonomic disturbances. Magnetic resonance imaging of brain showed features suggestive of limbic encephalitis. Electro-encephalography showed diffuse slowing with no epileptiform discharges. OUTCOME: We diagnosed paraneoplastic form of limbic encephalitis. Treatment with steroids and intravenous immunoglobulin failed, and the child died 4 weeks after onset of symptoms. MESSAGE: Limbic encephalitis should be kept as differential diagnosis in a child with sub-acutely evolving neuropsychiatric symptoms.