Ophelia-like Paraneoplastic Limbic Encephalitis with Haemorrhagic Temporal Lobe Involvement and Cauda Equina...

Ophelia syndrome is a rare paraneoplastic limbic encephalitis associated with classical Hodgkin lymphoma (cHL), most often with antibodies against metabotropic glutamate receptor 5 (mGluR5). We describe a 19-year-old man with newly diagnosed cHL who presented with generalised seizures, cognitive dysfunction, spastic paraparesis, cauda equina-related autonomic dysfunction, and a 35 × 31 mm haemorrhagic inflammatory lesion in the right temporal lobe. Brain biopsy showed dense intravascular and perivascular inflammatory infiltrates without neoplastic cells. Cerebrospinal fluid demonstrated pleocytosis and intrathecal IgG synthesis with type III oligoclonal bands. Serum and cerebrospinal fluid neuronal autoantibody panels were negative, but mGluR5 antibodies were not assessed. Cervical lymph node biopsy confirmed nodular sclerosis cHL, stage IIA. After exclusion of infectious encephalitis and central nervous system lymphoma, the presentation was considered most consistent with Ophelia-like paraneoplastic limbic encephalitis. ABVD chemotherapy was initiated, with rapid neurological improvement after the first cycle. Complete metabolic response was achieved after two cycles and sustained after six cycles. At 15-month follow-up, major neurological symptoms had not recurred, although bladder and bowel dysfunction persisted. This case highlights the importance of considering paraneoplastic limbic encephalitis in cHL despite negative standard neuronal antibody testing and of documenting whether mGluR5 antibodies were assessed.