Limbic Encephalitis as Presentation of Hodgkin Lymphoma in a Pediatric Patient: The Ophelia Syndrome (P4.6-041)

Objective: NA Background: Ophelia syndrome is an exceedingly rare paraneoplastic neurological syndrome characterized by Hodgkin Lymphoma and limbic encephalitis. A previously healthy 15-year-old female presented with a 10-day history of low-grade fever and headaches that progressed to altered mental status, slurred speech and mouth numbness. She quickly decompensated, resulting in intubation. Lumbar puncture showed findings consistent with aseptic meningitis. A MRI brain was normal. She was treated empirically with antivirals and had seemingly recovered within three days. She re-presented five days after discharge due to abnormal hand movements in sleep, minor confusion, and excessive sleepiness. Repeat MRI brain revealed findings consistent with limbic encephalitis in the left temporal lobe. EEG displayed focal slowing and epileptiform discharges in left central-temporal regions. IVIG was initiated, but the patient exhibited continued decline in mental status with increasing confusion, hallucinations and extreme agitation. The decision to initiate plasma exchange was made. A mediastinal mass was fortuitously discovered via a chest x-ray to assess placement of pheresis catheter, and the patient was ultimately diagnosed with classical nodular sclerosing Hodgkin Lymphoma. A paraneoplastic panel was mildly positive for neuronal voltage gated potassium channel antibodies (with negative LGI1 and Caspr2 antibodies) and striational antibody. Antibodies to metabotropic glutamate receptor 5 (mGluR5) were negative. After two cycles of chemotherapy, the patient is in remission of her Hodgkin Lymphoma with robust neurological recovery. Notably, a sequela of this patient’s disease is temporal lobe epilepsy. There are only 14 cases of Ophelia syndrome in adult and pediatric patients combined. Recognition of this syndrome is of crucial importance, as the mainstay of therapy is identification and treatment of the underlying neoplasm, and neurological symptoms can precede oncologic symptoms. It is classically associated with antibodies to mGluR5. Prognosis is excellent in patients who are diagnosed and treated for Hodgkin’s disease promptly. Design/Methods: NA Results: NA Conclusions: NA Disclosure: Dr. Tuttrup has nothing to disclose. Dr. Miller has nothing to disclose. Dr. Cruz Flores has nothing to disclose. Dr. Prasad has nothing to disclose. Dr. Yu has nothing to disclose.