Abstract Details

Title A Case Report: Ophelia Syndrome Associated with a Novel Subtype of Hodgkin’s Lymphoma

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (11:45 AM-12:45 PM)

Poster/Presentation
Number 8-007

Objective

To describe a case of Ophelia Syndrome in the setting of a novel subtype of Hodgkin’s Lymphoma.

Background Ophelia syndrome is a rare combination of a paraneoplastic neurologic syndrome in the setting of classical Hodgkin’s lymphoma (HL) usually accompanied by onconeural metabotropic glutamate receptor 5 (mGluR5) antibodies with known targets in the hippocampus and extralimbic areas. It is often characterized by neuropsychiatric symptoms such as hallucinations and behavioral/cognitive changes. In 1982, Ian Carr coined this syndrome by reporting a case of his own daughter’s personality and mental status change in the setting of Hodgkin’s lymphoma, rightly pointing to a paraneoplastic syndrome prior to the discovery of the associated onconeural antibody.

Design/Methods NA

Results 49-year-old male presents with subacute, progressive short-term memory (STM) deficits, inattention, and mood changes, which led to poor work performance. MRI and EEGs were unremarkable, however CSF studies showed mild pleocytosis and oligoclonal banding. Otherwise, he had negative infectious/metabolic encephalopathy workups. PET scan however revealed multi-focal hypermetabolic lymphadenopathy with biopsy-confirmed Nodular Lymphocyte Predominant HL. He was diagnosed with possible paraneoplastic autoimmune encephalitis and started IVIG. Due to low disease burden and preferred treatments of nonclassical HL being more akin to that of B cell lymphoma, he was additionally started on rituximab leading to STM improvement. Over the next year, he regained near complete neurologic recovery.

Conclusions This patient was diagnosed with Ophelia syndrome based on coincident neuropsychiatric changes and HL, which can be classified under the criteria: intermediate-risk phenotypes for paraneoplastic neurologic syndromes if anti-mGluR5 is found, which we are currently unable to test for. To our knowledge, less than 20 patients have been reported, all in the setting of classical HL. This case would be a novel presentation of Ophelia syndrome in the context of nonclassical HL, which should be considered in the differential of paraneoplastic neurologic syndrome.

Authors/Disclosures
Alessandra LaJeunesse, MD PRESENTER	Miss LaJeunesse has nothing to disclose.
Amanda F. Abuaf, MD (University of Wisconsin)	Dr. Abuaf has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech.

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