Abstract
Background/Objectives When encephalitis presents with nonspecific inflammatory cerebrospinal fluid (CSF) changes and an unremarkable computed tomography (CT) and magnetic resonance imaging (MRI), ascertaining a cause may present a challenge. In terms of aetiologies, Hodgkin Lymphoma (HL) associated with paraneoplastic limbic encephalitis (PLE) should be considered.
Methods In this case report, a patient is described who presented with a PLE associated with HL, also known as Ophelia Syndrome (OS).
Results A 53-year-old male presented via ambulance to the emergency department with 2 weeks of vomiting, headache, dizziness, fever, fatigue, anorexia, night sweats and weight loss. Initial assessment demonstrated new confusion.
Investigations showed neutrophilia with lymphopenia and microcytic hypochromic anaemia. A comprehensive septic screen detected nil infections. CSF showed an inflammatory picture (monocytes 47, polymorphonuclear cells 4, protein 0.87g/L and glucose 3.2mmol/L), although CSF viral testing and culture were negative. CT and MRI brain with contrast demonstrated no abnormalities. However, a positron emission tomography (PET) scan revealed extensive FDG-avid mediastinal and cervical lymphadenopathy. Biopsy of an involved lymph node confirmed Classic HL.
Subsequent neurological assessment confirmed a severe amnestic and dysexecutive syndrome with deficits in semantic knowledge and scarce, circumlocutory speech. A likely diagnosis of PLE associated with HL was made, with neuronal immunoblot and anti-mGluR5 antibodies awaiting. This OS was managed with chemotherapy.
Conclusion This case serves as a reminder that HL can present with a preceding PLE. Features include an amnestic and dysexecutive syndrome with language deficits. Consideration of a paraneoplastic syndrome is vital when investigating undifferentiated subacute encephalitis.
